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Who is at risk for Bronchiectasis? ?
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People who have conditions that damage the lungs or increase the risk of lung infections are at risk for bronchiectasis. Such conditions include:
Cystic fibrosis. This disease leads to almost half of the cases of bronchiectasis in the United States.
Immunodeficiency disorders, such as common variable immunodeficiency and, less often, HIV and AIDS.
Allergic bronchopulmonary aspergillosis. This is an allergic reaction to a fungus called aspergillus. The reaction causes swelling in the airways.
Disorders that affect cilia function, such as primary ciliary dyskinesia. Cilia are small, hair-like structures that line your airways. They help clear mucus (a slimy substance) out of your airways.
Bronchiectasis can develop at any age. Overall, two-thirds of people who have the condition are women. However, in children, the condition is more common in boys than in girls.
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Bronchiectasis
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What are the symptoms of Bronchiectasis ?
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The initial airway damage that leads to bronchiectasis often begins in childhood. However, signs and symptoms may not appear until months or even years after you start having repeated lung infections.
The most common signs and symptoms of bronchiectasis are:
A daily cough that occurs over months or years
Daily production of large amounts of sputum (spit). Sputum, which you cough up and spit out, may contain mucus (a slimy substance), trapped particles, and pus.
Shortness of breath and wheezing (a whistling sound when you breathe)
Chest pain
Clubbing (the flesh under your fingernails and toenails gets thicker)
If your doctor listens to your lungs with a stethoscope, he or she may hear abnormal lung sounds.
Over time, you may have more serious symptoms. You may cough up blood or bloody mucus and feel very tired. Children may lose weight or not grow at a normal rate.
Complications of Bronchiectasis
Severe bronchiectasis can lead to other serious health conditions, such as respiratory failure and atelectasis.
Respiratory failure is a condition in which not enough oxygen passes from your lungs into your blood. The condition also can occur if your lungs can't properly remove carbon dioxide (a waste gas) from your blood.
Respiratory failure can cause shortness of breath, rapid breathing, and air hunger (feeling like you can't breathe in enough air). In severe cases, signs and symptoms may include a bluish color on your skin, lips, and fingernails; confusion; and sleepiness.
Atelectasis is a condition in which one or more areas of your lungs collapse or don't inflate properly. As a result, you may feel short of breath. Your heart rate and breathing rate may increase, and your skin and lips may turn blue.
If bronchiectasis is so advanced that it affects all parts of your airways, it may cause heart failure. Heart failure is a condition in which the heart can't pump enough blood to meet the body's needs.
The most common signs and symptoms of heart failure are shortness of breath or trouble breathing, tiredness, and swelling in the ankles, feet, legs, abdomen, and veins in the neck.
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Bronchiectasis
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How to diagnose Bronchiectasis ?
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Your doctor may suspect bronchiectasis if you have a daily cough that produces large amounts of sputum (spit).
To find out whether you have bronchiectasis, your doctor may recommend tests to:
Identify any underlying causes that require treatment
Rule out other causes of your symptoms
Find out how much your airways are damaged
Diagnostic Tests and Procedures
Chest CT Scan
A chest computed tomography (to-MOG-ra-fee) scan, or chest CT scan, is the most common test for diagnosing bronchiectasis.
This painless test creates precise pictures of your airways and other structures in your chest. A chest CT scan can show the extent and location of lung damage. This test gives more detailed pictures than a standard chest x ray.
Chest X Ray
This painless test creates pictures of the structures in your chest, such as your heart and lungs. A chest x ray can show areas of abnormal lung and thickened, irregular airway walls.
Other Tests
Your doctor may recommend other tests, such as:
Blood tests. These tests can show whether you have an underlying condition that can lead to bronchiectasis. Blood tests also can show whether you have an infection or low levels of certain infection-fighting blood cells.
A sputum culture. Lab tests can show whether a sample of your sputum contains bacteria (such as the bacteria that cause tuberculosis) or fungi.
Lung function tests. These tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. Lung function tests help show how much lung damage you have.
A sweat test or other tests for cystic fibrosis.
Bronchoscopy
If your bronchiectasis doesn't respond to treatment, your doctor may recommend bronchoscopy (bron-KOS-ko-pee). Doctors use this procedure to look inside the airways.
During bronchoscopy, a flexible tube with a light on the end is inserted through your nose or mouth into your airways. The tube is called a bronchoscope. It provides a video image of your airways. You'll be given medicine to numb your upper airway and help you relax during the procedure.
Bronchoscopy can show whether you have a blockage in your airways. The procedure also can show the source of any bleeding in your airways.
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Bronchiectasis
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What are the treatments for Bronchiectasis ?
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Bronchiectasis often is treated with medicines, hydration, and chest physical therapy (CPT). Your doctor may recommend surgery if the bronchiectasis is isolated to a section of lung or you have a lot of bleeding.
If the bronchiectasis is widespread and causing respiratory failure, your doctor may recommend oxygen therapy.
The goals of treatment are to:
Treat any underlying conditions and lung infections.
Remove mucus (a slimy substance) from your lungs. Maintaining good hydration helps with mucus removal.
Prevent complications.
Early diagnosis and treatment of the underlying cause of bronchiectasis may help prevent further lung damage.
In addition, any disease associated with the bronchiectasis, such as cystic fibrosisor immunodeficiency, also should be treated.
Medicines
Your doctor may prescribe antibiotics, bronchodilators, expectorants, or mucus-thinning medicines to treat bronchiectasis.
Antibiotics
Antibiotics are the main treatment for the repeated lung infections that bronchiectasis causes. Oral antibiotics often are used to treat these infections.
For hard-to-treat infections, your doctor may prescribe intravenous (IV) antibiotics. These medicines are given through an IV line inserted into your arm. Your doctor may help you arrange for a home care provider to give you IV antibiotics at home.
Expectorants and Mucus-Thinning Medicines
Your doctor may prescribe expectorants and mucus thinners to help you cough up mucus.
Expectorants help loosen the mucus in your lungs. They often are combined with decongestants, which may provide extra relief. Mucus thinners, such as acetylcysteine, loosen the mucus to make it easier to cough up.
For some of these treatments, little information is available to show how well they work.
Hydration
Drinking plenty of fluid, especially water, helps prevent airway mucus from becoming thick and sticky. Good hydration helps keep airway mucus moist and slippery, which makes it easier to cough up.
Chest Physical Therapy
CPT also is called physiotherapy (FIZ-e-o-THER-ah-pe) or chest clapping or percussion. This technique is generally performed by a respiratory therapist but can be done by a trained member of the family. It involves the therapist pounding your chest and back over and over with his or her hands or a device. Doing this helps loosen the mucus from your lungs so you can cough it up.
You can sit with your head tilted down or lie on your stomach with your head down while you do CPT. Gravity and force help drain the mucus from your lungs.
Some people find CPT hard or uncomfortable to do. Several devices can help with CPT, such as:
An electric chest clapper, known as a mechanical percussor.
An inflatable therapy vest that uses high-frequency air waves to force mucus toward your upper airways so you can cough it up.
A small handheld device that you breathe out through. It causes vibrations that dislodge the mucus.
A mask that creates vibrations to help break loose mucus from your airway walls.
Some of these methods and devices are popular with patients and doctors, but little information is available on how well they actually work. Choice usually is based on convenience and cost.
Several breathing techniques also are used to help move mucus to the upper airway so it can be coughed up. These techniques include forced expiration technique (FET) and active cycle breathing (ACB).
FET involves forcing out a couple of breaths and then doing relaxed breathing. ACB is FET that involves deep breathing exercises.
Other Treatments
Depending on your condition, your doctor also may recommend bronchodilators, inhaled corticosteroids, oxygen therapy, or surgery.
Bronchodilators
Bronchodilators relax the muscles around your airways. This helps open your airways and makes breathing easier. Most bronchodilators are inhaled medicines. You will use an inhaler or a nebulizer to breathe in a fine mist of medicine.
Inhaled bronchodilators work quickly because the medicine goes straight to your lungs. Your doctor may recommend that you use a bronchodilator right before you do CPT.
Inhaled Corticosteroids
If you also have wheezing or asthma with your bronchiectasis, your doctor may prescribe inhaled corticosteroids (used to treat inflammation in the airways).
Oxygen Therapy
Oxygen therapy can help raise low blood oxygen levels. For this treatment, you'll receive oxygen through nasal prongs or a mask. Oxygen therapy can be done at home, in a hospital, or in another health facility. (For more information, go to the Health Topics Oxygen Therapy article.)
Surgery
Your doctor may recommend surgery if no other treatments have helped and only one part of your airway is affected. If you have major bleeding in your airway, your doctor may recommend surgery to remove part of your airway or a procedure to control the bleeding.
In very rare instances of severe bronchiectasis, your doctor may recommend that you receive a lung transplant replacing your diseased lungs with a healthy set of lungs.
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Bronchiectasis
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How to prevent Bronchiectasis ?
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To prevent bronchiectasis, it's important to prevent the lung infections and lung damage that can cause it.
Childhood vaccines for measles and whooping cough prevent infections related to these illnesses. These vaccines also reduce complications from these infections, such as bronchiectasis.
Avoiding toxic fumes, gases, smoke, and other harmful substances also can help protect your lungs.
Proper treatment of lung infections in children also may help preserve lung function and prevent lung damage that can lead to bronchiectasis.
Stay alert to keep children (and adults) from inhaling small objects (such as pieces of toys and food that might stick in a small airway). If you think you, your child, or someone else has inhaled a small object, seek prompt medical care.
In some cases, treating the underlying cause of bronchiectasis can slow or prevent its progression.
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Bronchiectasis
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What is (are) Oxygen Therapy ?
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Oxygen therapy is a treatment that provides you with extra oxygen, a gas that your body needs to work well. Normally, your lungs absorb oxygen from the air. However, some diseases and conditions can prevent you from getting enough oxygen.
Oxygen therapy may help you function better and be more active. Oxygen is supplied in a metal cylinder or other container. It flows through a tube and is delivered to your lungs in one of the following ways:
Through a nasal cannula, which consists of two small plastic tubes, or prongs, that are placed in both nostrils.
Through a face mask, which fits over your nose and mouth.
Through a small tube inserted into your windpipe through the front of your neck. Your doctor will use a needle or small incision (cut) to place the tube. Oxygen delivered this way is called transtracheal oxygen therapy.
Oxygen therapy can be done in a hospital, another medical setting, or at home. If you need oxygen therapy for a chronic (ongoing) disease or condition, you might receive home oxygen therapy.
Overview
To learn how oxygen therapy works, it helps to understand how your respiratory system works. This system is a group of organs and tissues that help you breathe. The respiratory system includes the airways and lungs.
The airways carry oxygen-rich air to your lungs. They also carry carbon dioxide (a waste gas) out of your lungs.
Air enters your body through your nose or mouth, which moistens and warms the air. The air then travels through your voice box and down your windpipe. The windpipe divides into two tubes called bronchi that enter your lungs.
Within your lungs, your bronchi branch into thousands of smaller, thinner tubes called bronchioles (BRONG-ke-ols). These tubes end in bunches of tiny round air sacs called alveoli (al-VEE-uhl-eye).
Each of the air sacs is covered in a mesh of tiny blood vessels called capillaries (KAP-ih-lare-ees). The capillaries connect to a network of arteries and veins that move blood throughout your body.
When air reaches the air sacs, the oxygen in the air passes through the air sac walls into the blood in the capillaries.
The oxygen-rich blood then travels to the heart through the pulmonary vein and its branches. The heart pumps the oxygen-rich blood to your organs. (For more information, go to the Health Topics How the Lungs Work article.)
Certain acute (short-term) and chronic (ongoing) diseases and conditions can affect the transfer of oxygen from the alveoli into the blood. Examples include pneumonia (nu-MO-ne-ah) and COPD (chronic obstructive pulmonary disease).
Your doctor will decide whether you need oxygen therapy based on the results of tests, such as an arterial blood gas test and a pulse oximetry test. These tests measure how much oxygen is in your blood. A low oxygen level is a sign that you need oxygen therapy.
Oxygen is considered a medicine, so your doctor must prescribe it.
Outlook
Oxygen therapy helps many people function better and be more active. It also may help:
Decrease shortness of breath and fatigue (tiredness)
Improve sleep in some people who have sleep-related breathing disorders
Increase the lifespan of some people who have COPD
Although you may need oxygen therapy long term, it doesn't have to limit your daily routine. Portable oxygen units can make it easier for you to move around and do many daily activities. Talk with your doctor if you have questions about whether certain activities are safe for you.
A home equipment provider will work with you to make sure you have the supplies and equipment you need. Trained staff also will show you how to use the equipment correctly and safely.
Oxygen therapy generally is safe, but it can pose a fire hazard. To use your oxygen safely, follow the instructions you receive from your home equipment provider.
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Oxygen Therapy
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What is the outlook for Oxygen Therapy ?
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During an emergencysuch as a serious accident, possible heart attack, or other life-threatening eventyou might be started on oxygen therapy right away.
Otherwise, your doctor will decide whether you need oxygen therapy based on test results. An arterial blood gas test and a pulse oximetry test can measure the amount of oxygen in your blood.
For an arterial blood gas test, a small needle is inserted into an artery, usually in your wrist. A sample of blood is taken from the artery. The sample is then sent to a laboratory, where its oxygen level is measured.
For a pulse oximetry test, a small sensor is attached to your fingertip or toe. The sensor uses light to estimate how much oxygen is in your blood.
If the tests show that your blood oxygen level is low, your doctor may prescribe oxygen therapy. In the prescription, your doctor will include the number of liters of oxygen per minute that you need (oxygen flow rate). He or she also will include how often you need to use the oxygen (frequency of use).
Frequency of use includes when and for how long you should use the oxygen. Depending on your condition and blood oxygen level, you may need oxygen only at certain times, such as during sleep or while exercising.
If your doctor prescribes home oxygen therapy, he or she can help you find a home equipment provider. The provider will give you the equipment and other supplies you need.
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Oxygen Therapy
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What is the outlook for Oxygen Therapy ?
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During an emergencysuch as a serious accident, possible heart attack, or other life-threatening eventyou might be started on oxygen therapy right away.
While you're in the hospital, your doctor will check on you to make sure you're getting the right amount of oxygen. Nurses or respiratory therapists also may assist with the oxygen therapy.
If you're having oxygen therapy at home, a home equipment provider will help you set up the oxygen therapy equipment at your house.
Trained staff will show you how to use and take care of the equipment. They'll supply the oxygen and teach you how to safely handle it.
Because oxygen poses a fire risk, you'll need to take certain safety steps. Oxygen isn't explosive, but it can worsen a fire. In the presence of oxygen, a small fire can quickly get out of control. Also, the cylinder that compressed oxygen gas comes in can explode if it's exposed to heat.
Your home equipment provider will give you a complete list of safety steps that you'll need to follow at home and in public. For example, while on oxygen, you should:
Never smoke or be around people who are smoking
Never use paint thinners, cleaning fluids, gasoline, aerosol sprays, and other flammable materials
Stay at least 5 feet away from gas stoves, candles, and other heat sources
When you're not using the oxygen, keep it in a large, airy room. Never store compressed oxygen gas cylinders and liquid oxygen containers in small, enclosed places, such as in closets, behind curtains, or under clothes.
Oxygen containers let off small amounts of oxygen. These small amounts can build up to harmful levels if they're allowed to escape into small spaces.
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Oxygen Therapy
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Who is at risk for Oxygen Therapy? ?
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Oxygen therapy can cause complications and side effects. These problems might include a dry or bloody nose, skin irritation from the nasal cannula or face mask, fatigue (tiredness), and morning headaches.
If these problems persist, tell your doctor and home equipment provider. Depending on the problem, your doctor may need to change your oxygen flow rate or the length of time you're using the oxygen.
If nose dryness is a problem, your doctor may recommend a nasal spray or have a humidifier added to your oxygen equipment.
If you have an uncomfortable nasal cannula or face mask, your home equipment provider can help you find a device that fits better. Your provider also can recommend over-the-counter gels and devices that are designed to lessen skin irritation.
Complications from transtracheal oxygen therapy can be more serious. With this type of oxygen therapy, oxygen is delivered through a tube inserted into your windpipe through the front of your neck.
With transtracheal oxygen therapy:
Mucus balls might develop on the tube inside the windpipe. Mucus balls tend to form as a result of the oxygen drying out the airways. Mucus balls can cause coughing and clog the windpipe or tube.
Problems with the tube slipping or breaking.
Infection.
Injury to the lining of the windpipe.
Proper medical care and correct handling of the tube and other supplies may reduce the risk of complications.
Other Risks
In certain people, oxygen therapy may suppress the drive to breathe, affecting how well the respiratory system works. This is managed by adjusting the oxygen flow rate.
Oxygen poses a fire risk, so you'll need to take certain safety steps. Oxygen itself isn't explosive, but it can worsen a fire. In the presence of oxygen, a small fire can quickly get out of control. Also, the cylinder that compressed oxygen gas comes in might explode if exposed to heat.
Your home equipment provider will give you a complete list of safety steps you'll need to take at home and when out in public.
For example, when you're not using the oxygen, keep it in an airy room. Never store compressed oxygen gas cylinders and liquid oxygen containers in small, enclosed places, such as in closets, behind curtains, or under clothes.
Oxygen containers let off small amounts of oxygen. These small amounts can build up to harmful levels if they're allowed to escape into small spaces.
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Oxygen Therapy
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What is (are) Polycythemia Vera ?
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Polycythemia vera (POL-e-si-THEE-me-ah VAY-rah or VE-rah), or PV, is a rare blood disease in which your body makes too many red blood cells.
The extra red blood cells make your blood thicker than normal. As a result, blood clots can form more easily. These clots can block blood flow through your arteries and veins, which can cause a heart attack or stroke.
Thicker blood also doesn't flow as quickly to your body as normal blood. Slowed blood flow prevents your organs from getting enough oxygen, which can cause serious problems, such as angina (an-JI-nuh or AN-juh-nuh) and heart failure. (Angina is chest pain or discomfort.)
Overview
Red blood cells carry oxygen to all parts of your body. They also remove carbon dioxide (a waste product) from your body's cells and carry it to the lungs to be exhaled.
Red blood cells are made in your bone marrowa sponge-like tissue inside the bones. White blood cells and platelets (PLATE-lets) also are made in your bone marrow. White blood cells help fight infection. Platelets stick together to seal small cuts or breaks on blood vessel walls and stop bleeding.
If you have PV, your bone marrow makes too many red blood cells. It also can make too many white blood cells and platelets.
A mutation, or change, in the body's JAK2 gene is the major cause of PV. This gene makes a protein that helps the body produce blood cells. What causes the change in the JAK2 gene isn't known. PV generally isn't inheritedthat is, passed from parents to children through genes.
PV develops slowly and may not cause symptoms for years. The disease often is found during routine blood tests done for other reasons.
When signs and symptoms are present, they're the result of the thick blood that occurs with PV. This thickness slows the flow of oxygen-rich blood to all parts of your body. Without enough oxygen, many parts of your body won't work normally.
For example, slower blood flow deprives your arms, legs, lungs, and eyes of the oxygen they need. This can cause headaches, dizziness, itching, and vision problems, such as blurred or double vision.
Outlook
PV is a serious, chronic (ongoing) disease that can be fatal if not diagnosed and treated. PV has no cure, but treatments can help control the disease and its complications.
PV is treated with procedures, medicines, and other methods. You may need one or more treatments to manage the disease.
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Polycythemia Vera
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What causes Polycythemia Vera ?
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Primary Polycythemia
Polycythemia vera (PV) also is known as primary polycythemia. A mutation, or change, in the body's JAK2 gene is the main cause of PV. The JAK2 gene makes a protein that helps the body produce blood cells.
What causes the change in the JAK2 gene isn't known. PV generally isn't inheritedthat is, passed from parents to children through genes. However, in some families, the JAK2 gene may have a tendency to mutate. Other, unknown genetic factors also may play a role in causing PV.
Secondary Polycythemia
Another type of polycythemia, called secondary polycythemia, isn't related to the JAK2 gene. Long-term exposure to low oxygen levels causes secondary polycythemia.
A lack of oxygen over a long period can cause your body to make more of the hormone erythropoietin (EPO). High levels of EPO can prompt your body to make more red blood cells than normal. This leads to thicker blood, as seen in PV.
People who have severe heart or lung disease may develop secondary polycythemia. People who smoke, spend long hours at high altitudes, or are exposed to high levels of carbon monoxide where they work or live also are at risk.
For example, working in an underground parking garage or living in a home with a poorly vented fireplace or furnace can raise your risk for secondary polycythemia.
Rarely, tumors can make and release EPO, or certain blood problems can cause the body to make more EPO.
Sometimes doctors can cure secondary polycythemiait depends on whether the underlying cause can be stopped, controlled, or cured.
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Polycythemia Vera
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Who is at risk for Polycythemia Vera? ?
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Polycythemia vera (PV) is a rare blood disease. The disease affects people of all ages, but it's most common in adults who are older than 60. PV is rare in children and young adults. Men are at slightly higher risk for PV than women.
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Polycythemia Vera
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What are the symptoms of Polycythemia Vera ?
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Polycythemia vera (PV) develops slowly. The disease may not cause signs or symptoms for years.
When signs and symptoms are present, they're the result of the thick blood that occurs with PV. This thickness slows the flow of oxygen-rich blood to all parts of your body. Without enough oxygen, many parts of your body won't work normally.
The signs and symptoms of PV include:
Headaches, dizziness, and weakness
Shortness of breath and problems breathing while lying down
Feelings of pressure or fullness on the left side of the abdomen due to an enlarged spleen (an organ in the abdomen)
Double or blurred vision and blind spots
Itching all over (especially after a warm bath), reddened face, and a burning feeling on your skin (especially your hands and feet)
Bleeding from your gums and heavy bleeding from small cuts
Unexplained weight loss
Fatigue (tiredness)
Excessive sweating
Very painful swelling in a single joint, usually the big toe (called gouty arthritis)
In rare cases, people who have PV may have pain in their bones.
Polycythemia Vera Complications
If you have PV, the thickness of your blood and the slowed blood flow can cause serious health problems.
Blood clots are the most serious complication of PV. Blood clots can cause a heart attack or stroke. They also can cause your liver and spleen to enlarge. Blood clots in the liver and spleen can cause sudden, intense pain.
Slowed blood flow also prevents enough oxygen-rich blood from reaching your organs. This can lead to angina (chest pain or discomfort) and heart failure. The high levels of red blood cells that PV causes can lead to stomach ulcers, gout, or kidney stones.
Some people who have PV may develop myelofibrosis (MY-e-lo-fi-BRO-sis). This is a condition in which your bone marrow is replaced with scar tissue. Abnormal bone marrow cells may begin to grow out of control.
This abnormal growth can lead to acute myelogenous (my-eh-LOJ-eh-nus) leukemia (AML), a cancer of the blood and bone marrow. This disease can worsen very quickly.
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Polycythemia Vera
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How to diagnose Polycythemia Vera ?
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Polycythemia vera (PV) may not cause signs or symptoms for years. The disease often is found during routine blood tests done for other reasons. If the results of your blood tests aren't normal, your doctor may want to do more tests.
Your doctor will diagnose PV based on your signs and symptoms, your age and overall health, your medical history, a physical exam, and test results.
During the physical exam, your doctor will look for signs of PV. He or she will check for an enlarged spleen, red skin on your face, and bleeding from your gums.
If your doctor confirms that you have polycythemia, the next step is to find out whether you have primary polycythemia (polycythemia vera) or secondary polycythemia.
Your medical history and physical exam may confirm which type of polycythemia you have. If not, you may have tests that check the level of the hormone erythropoietin (EPO) in your blood.
People who have PV have very low levels of EPO. People who have secondary polycythemia usually have normal or high levels of EPO.
Specialists Involved
If your primary care doctor thinks you have PV, he or she may refer you to a hematologist. A hematologist is a doctor who specializes in diagnosing and treating blood diseases and conditions.
Diagnostic Tests
You may have blood tests to diagnose PV. These tests include a complete blood count (CBC) and other tests, if necessary.
Complete Blood Count
Often, the first test used to diagnose PV is a CBC. The CBC measures many parts of your blood.
This test checks your hemoglobin (HEE-muh-glow-bin) and hematocrit (hee-MAT-oh-crit) levels. Hemoglobin is an iron-rich protein that helps red blood cells carry oxygen from the lungs to the rest of the body. Hematocrit is a measure of how much space red blood cells take up in your blood. A high level of hemoglobin or hematocrit may be a sign of PV.
The CBC also checks the number of red blood cells, white blood cells, and platelets in your blood. Abnormal results may be a sign of PV, a blood disorder, an infection, or another condition.
In addition to high red blood cell counts, people who have PV also may have high white blood cell and/or platelet counts.
Other Blood Tests
Blood smear. For this test, a small sample of blood is drawn from a vein, usually in your arm. The blood sample is examined under a microscope.
A blood smear can show whether you have a higher than normal number of red blood cells. The test also can show abnormal blood cells that are linked to myelofibrosis and other conditions related to PV.
Erythropoietin level. This blood test measures the level of EPO in your blood. EPO is a hormone that prompts your bone marrow to make new blood cells. People who have PV have very low levels of EPO. People who have secondary polycythemia usually have normal or high levels of EPO.
Bone Marrow Tests
Bone marrow tests can show whether your bone marrow is healthy. These tests also show whether your bone marrow is making normal amounts of blood cells.
The two bone marrow tests are aspiration (as-pi-RA-shun) and biopsy. For aspiration, your doctor removes a small amount of fluid bone marrow through a needle. For a biopsy, your doctor removes a small amount of bone marrow tissue through a larger needle. The samples are then examined under a microscope.
If the tests show that your bone marrow is making too many blood cells, it may be a sign that you have PV.
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Polycythemia Vera
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What are the treatments for Polycythemia Vera ?
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Polycythemia vera (PV) doesn't have a cure. However, treatments can help control the disease and its complications. PV is treated with procedures, medicines, and other methods. You may need one or more treatments to manage the disease.
Goals of Treatment
The goals of treating PV are to control symptoms and reduce the risk of complications, especially heart attack and stroke. To do this, PV treatments reduce the number of red blood cells and the level of hemoglobin (an iron-rich protein) in the blood. This brings the thickness of your blood closer to normal.
Blood with normal thickness flows better through the blood vessels. This reduces the chance that blood clots will form and cause a heart attack or stroke.
Blood with normal thickness also ensures that your body gets enough oxygen. This can help reduce some of the signs and symptoms of PV, such as headaches, vision problems, and itching.
Studies show that treating PV greatly improves your chances of living longer.
The goal of treating secondary polycythemia is to control its underlying cause, if possible. For example, if the cause is carbon monoxide exposure, the goal is to find the source of the carbon monoxide and fix or remove it.
Treatments To Lower Red Blood Cell Levels
Phlebotomy
Phlebotomy (fle-BOT-o-me) is a procedure that removes some blood from your body. For this procedure, a needle is inserted into one of your veins. Blood from the vein flows through an airtight tube into a sterile container or bag. The process is similar to the process of donating blood.
Phlebotomy reduces your red blood cell count and starts to bring your blood thickness closer to normal.
Typically, a pint (1 unit) of blood is removed each week until your hematocrit level approaches normal. (Hematocrit is the measure of how much space red blood cells take up in your blood.)
You may need to have phlebotomy done every few months.
Medicines
Your doctor may prescribe medicines to keep your bone marrow from making too many red blood cells. Examples of these medicines include hydroxyurea and interferon-alpha.
Hydroxyurea is a medicine generally used to treat cancer. This medicine can reduce the number of red blood cells and platelets in your blood. As a result, this medicine helps improve your blood flow and bring the thickness of your blood closer to normal.
Interferon-alpha is a substance that your body normally makes. It also can be used to treat PV. Interferon-alpha can prompt your immune system to fight overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal.
Radiation Treatment
Radiation treatment can help suppress overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal.
However, radiation treatment can raise your risk of leukemia (blood cancer) and other blood diseases.
Treatments for Symptoms
Aspirin can relieve bone pain and burning feelings in your hands or feet that you may have as a result of PV. Aspirin also thins your blood, so it reduces the risk of blood clots.
Aspirin can have side effects, including bleeding in the stomach and intestines. For this reason, take aspirin only as your doctor recommends.
If your PV causes itching, your doctor may prescribe medicines to ease the discomfort. Your doctor also may prescribe ultraviolet light treatment to help relieve your itching.
Other ways to reduce itching include:
Avoiding hot baths. Cooler water can limit irritation to your skin.
Gently patting yourself dry after bathing. Vigorous rubbing with a towel can irritate your skin.
Taking starch baths. Add half a box of starch to a tub of lukewarm water. This can help soothe your skin.
Experimental Treatments
Researchers are studying other treatments for PV. An experimental treatment for itching involves taking low doses of selective serotonin reuptake inhibitors (SSRIs). This type of medicine is used to treat depression. In clinical trials, SSRIs reduced itching in people who had PV.
Imatinib mesylate is a medicine that's approved for treating leukemia. In clinical trials, this medicine helped reduce the need for phlebotomy in people who had PV. This medicine also helped reduce the size of enlarged spleens.
Researchers also are trying to find a treatment that can block or limit the effects of an abnormal JAK2 gene. (A mutation, or change, in the JAK2 gene is the major cause of PV.)
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Polycythemia Vera
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How to prevent Polycythemia Vera ?
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Primary polycythemia (polycythemia vera) can't be prevented. However, with proper treatment, you can prevent or delay symptoms and complications.
Sometimes you can prevent secondary polycythemia by avoiding things that deprive your body of oxygen for long periods. For example, you can avoid mountain climbing, living at a high altitude, or smoking.
People who have serious heart or lung diseases may develop secondary polycythemia. Treatment for the underlying disease may improve the secondary polycythemia. Following a healthy lifestyle to lower your risk of heart and lung diseases also will help you prevent secondary polycythemia.
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Polycythemia Vera
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What is (are) Childhood Interstitial Lung Disease ?
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Childhood interstitial (in-ter-STISH-al) lung disease, or chILD, is a broad term for a group of rare lung diseases that can affect babies, children, and teens. These diseases have some similar symptoms, such as chronic cough, rapid breathing, and shortness of breath.
These diseases also harm the lungs in similar ways. For example, they damage the tissues that surround the lungs' alveoli (al-VEE-uhl-eye; air sacs) and bronchial tubes (airways). Sometimes these diseases directly damage the air sacs and airways.
The various types of chILD can decrease lung function, reduce blood oxygen levels, and disturb the breathing process.
Overview
Researchers have only begun to study, define, and understand chILD in the last decade. Currently, they don't know how many children have chILD. They also don't know how many children have each type of chILD.
Diagnosing chILD and its specific diseases is hard because chILD is rare and complex. Also, chILD is a broad term for a group of diseases with similar symptomsit's not a precise diagnosis.
Interstitial lung disease (ILD) also occurs in adults. However, the cause of ILD in adults may be different than the cause in children. Some types of chILD are similar to the adult forms of the disease. They may even have the same names as the adult forms, such as hypersensitivity pneumonitis (noo-mo-NI-tis), immunodeficiency-associated lung disease, and bronchiolitis (brong-ke-o-LI-tis) obliterans.
However, research shows that the course and outcomes of these diseases often are very different for children than for adults.
Some ILDs only occur in children. They include:
Lung growth abnormalities
Neuroendocrine (noor-o-EN-do-krin) cell hyperplasia (hi-per-PLA-ze-ah) of infancy (NEHI)
Pulmonary interstitial glycogenosis (gli-ko-JEN-eh-sis)
Developmental disorders, such as alveolar (al-VE-o-lar) capillary dysplasia
Outlook
Each form of chILD may differ in its severity and how it's treated. Thus, getting a correct diagnosis is vital for understanding and treating your child's illness.
You may want to consult a pediatric pulmonologist. This is a doctor who specializes in diagnosing and treating children who have lung diseases and conditions. This doctor's training and experience can help him or her diagnose chILD.
The outlook for children who have chILD also depends on the specific type of disease they have. Some diseases are very severe and lead to early death. Others are chronic (long-term) diseases that parents and the child's medical team must work together to manage.
At this time, chILD has no cure. However, some children who have certain diseases, such as NEHI, may slowly improve over time.
Researchers are now starting to learn more about the causes of chILD. They're also trying to find distinct patterns and traits for the various forms of chILD. This information may help doctors better understand these diseases.
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Childhood Interstitial Lung Disease
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What causes Childhood Interstitial Lung Disease ?
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Researchers don't yet know all of the causes of childhood interstitial lung disease (chILD). Many times, these diseases have no clear cause.
Some conditions and factors that may cause or lead to chILD include:
Inherited conditions, such as surfactant disorders. Surfactant is a liquid that coats the inside of the lungs. It helps with breathing and may help protect the lungs from bacterial and viral infections.
Birth defects that cause problems with the structure or function of the lungs.
Aspiration (as-pih-RA-shun). This term refers to inhaling substancessuch as food, liquid, or vomitinto the lungs. Inhaling these substances can injure the lungs. Aspiration may occur in children who have swallowing problems or gastroesophageal (GAS-tro-eh-so-fa-JE-al) reflux disease (GERD). GERD occurs if acid from the stomach backs up into the throat.
Immune system disorders. The immune system protects the body against bacteria, viruses, and toxins. Children who have immune system disorders aren't able to fight illness and disease as well as children who have healthy immune systems.
Exposure to substances in the environment that can irritate the lungs, such as molds and chemicals.
Some cancer treatments, such as radiation and chemotherapy.
Systemic or autoimmune diseases, such as collagen vascular disease or inflammatory bowel disease. Systemic diseases are diseases that involve many of the body's organs. Autoimmune diseases occur if the body's immune system mistakenly attacks the body's tissues and cells.
A bone marrow transplant or a lung transplant.
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Childhood Interstitial Lung Disease
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Who is at risk for Childhood Interstitial Lung Disease? ?
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Childhood interstitial lung disease (chILD) is rare. Most children are not at risk for chILD. However, some factors increase the risk of developing chILD. These risk factors include:
Having a family history of interstitial lung disease or chILD.
Having an inherited surfactant disorder or a family history of this type of disorder. Surfactant is a liquid that coats the inside of the lungs. It helps with breathing and may help protect the lungs from bacterial and viral infections.
Having problems with aspiration. This term "aspiration" refers to inhaling substancessuch as food, liquid, or vomitinto the lungs.
Having an immune system disorder. The immune system protects the body against bacteria, viruses, and toxins. Children who have immune system disorders aren't able to fight illness and disease as well as children who have healthy immune systems.
Being exposed to substances in the environment that can irritate the lungs, such as molds and chemicals.
Having a systemic or autoimmune disease, such as collagen vascular disease or inflammatory bowel disease. Systemic diseases are diseases that involve many of the body's organs. Autoimmune diseases occur if the body's immune system mistakenly attacks the body's tissues and cells.
Undergoing some cancer treatments, such as radiation and chemotherapy.
Having a bone marrow transplant or a lung transplant.
Certain types of chILD are more common in infants and young children, while others can occur in children of any age. For more information, go to "Types of Childhood Interstitial Lung Disease."
The risk of death seems to be higher for children who have chILD and pulmonary hypertension, developmental or growth disorders, bone marrow transplants, or certain surfactant problems.
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Childhood Interstitial Lung Disease
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What are the symptoms of Childhood Interstitial Lung Disease ?
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Childhood interstitial lung disease (chILD) has many signs and symptoms because the disease has many forms. Signs and symptoms may include:
Fast breathing, which also is called tachypnea (tak-ip-NE-ah)
Labored breathing, which also is called respiratory distress
Low oxygen levels in the blood, which also is called hypoxemia (hi-POK-se-ah)
Recurrent coughing, wheezing, or crackling sounds in the chest
Shortness of breath during exercise (in older children) or while eating (in infants), which also is called dyspnea (disp-NE-ah)
Poor growth or failure to gain weight
Recurrent pneumonia or bronchiolitis
If your child has any of these signs and symptoms, contact his or her doctor. The doctor may refer you to a pediatric pulmonologist. This is a doctor who specializes in diagnosing and treating children who have lung diseases and conditions.
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Childhood Interstitial Lung Disease
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How to diagnose Childhood Interstitial Lung Disease ?
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Doctors diagnose childhood interstitial lung disease (chILD) based on a child's medical and family histories and the results from tests and procedures. To diagnose chILD, doctors may first need to rule out other diseases as the cause of a child's symptoms.
Early diagnosis of chILD may help doctors stop or even reverse lung function problems. Often though, doctors find chILD hard to diagnose because:
There are many types of the disease and a range of underlying causes
The disease's signs and symptoms are the same as those for many other diseases
The disease may coexist with other diseases
Going to a pediatric pulmonologist who has experience with chILD is helpful. A pediatric pulmonologist is a doctor who specializes in diagnosing and treating children who have lung diseases and conditions.
Medical and Family Histories
Your child's medical history can help his or her doctor diagnose chILD. The doctor may ask whether your child:
Has severe breathing problems that occur often.
Has had severe lung infections.
Had serious lung problems as a newborn.
Has been exposed to possible lung irritants in the environment, such as birds, molds, dusts, or chemicals.
Has ever had radiation or chemotherapy treatment.
Has an autoimmune disease, certain birth defects, or other medical conditions. (Autoimmune diseases occur if the body's immune system mistakenly attacks the bodys tissues and cells.)
The doctor also may ask how old your child was when symptoms began, and whether other family members have or have had severe lung diseases. If they have, your child may have an inherited form of chILD.
Diagnostic Tests and Procedures
No single test can diagnose the many types of chILD. Thus, your child's doctor may recommend one or more of the following tests. For some of these tests, infants and young children may be given medicine to help them relax or sleep.
A chest x ray. This painless test creates pictures of the structures inside your child's chest, such as the heart, lungs, and blood vessels. A chest x ray can help rule out other lung diseases as the cause of your child's symptoms.
A high-resolution CT scan (HRCT). An HRCT scan uses x rays to create detailed pictures of your child's lungs. This test can show the location, extent, and severity of lung disease.
Lung function tests. These tests measure how much air your child can breathe in and out, how fast he or she can breathe air out, and how well your child's lungs deliver oxygen to the blood. Lung function tests can assess the severity of lung disease. Infants and young children may need to have these tests at a center that has special equipment for children.
Bronchoalveolar lavage (BRONG-ko-al-VE-o-lar lah-VAHZH). For this procedure, the doctor injects a small amount of saline (salt water) through a tube inserted in the child's lungs. The fluid helps bring up cells from the tissues around the air sacs. The doctor can then look at these cells under a microscope. This procedure can help detect an infection, lung injury, bleeding, aspiration, or an airway problem.
Various tests to rule out conditions such as asthma, cystic fibrosis, acid reflux, heart disease, neuromuscular disease, and immune deficiency.
Various tests for systemic diseases linked to chILD. Systemic diseases are diseases that involve many of the body's organs.
Blood tests to check for inherited (genetic) diseases and disorders.
If these tests don't provide enough information, your child's doctor may recommend a lung biopsy. A lung biopsy is the most reliable way to diagnose chILD and the specific disease involved.
A lung biopsy is a surgical procedure that's done in a hospital. Before the biopsy, your child will receive medicine to make him or her sleep.
During the biopsy, the doctor will take small samples of lung tissue from several places in your child's lungs. This often is done using video-assisted thoracoscopy (thor-ah-KOS-ko-pe).
For this procedure, the doctor inserts a small tube with a light and camera (endoscope) into your child's chest through small cuts between the ribs. The endoscope provides a video image of the lungs and allows the doctor to collect tissue samples.
After the biopsy, the doctor will look at these samples under a microscope.
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Childhood Interstitial Lung Disease
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What are the treatments for Childhood Interstitial Lung Disease ?
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Childhood interstitial lung disease (chILD) is rare, and little research has been done on how to treat it. At this time, chILD has no cure. However, some children who have certain diseases, such as neuroendocrine cell hyperplasia of infancy, may slowly improve over time.
Current treatment approaches include supportive therapy, medicines, and, in the most serious cases, lung transplants.
Supportive Therapy
Supportive therapy refers to treatments that help relieve symptoms or improve quality of life. Supportive approaches used to relieve common chILD symptoms include:
Oxygen therapy. If your child's blood oxygen level is low, he or she may need oxygen therapy. This treatment can improve breathing, support growth, and reduce strain on the heart.
Bronchodilators. These medications relax the muscles around your childs airways, which helps open the airways and makes breathing easier.
Breathing devices. Children who have severe disease may need ventilators or other devices to help them breathe easier.
Extra nutrition. This treatment can help improve your child's growth and help him or her gain weight. Close monitoring of growth is especially important.
Techniques and devices to help relieve lung congestion. These may include chest physical therapy (CPT) or wearing a vest that helps move mucus (a sticky substance) to the upper airways so it can be coughed up. CPT may involve pounding the chest and back over and over with your hands or a device to loosen mucus in the lungs so that your child can cough it up.
Supervised pulmonary rehabilitation (PR). PR is a broad program that helps improve the well-being of people who have chronic (ongoing) breathing problems.
Medicines
Corticosteroids are a common treatment for many children who have chILD. These medicines help reduce lung inflammation.
Other medicines can help treat specific types or causes of chILD. For example, antimicrobial medicines can treat a lung infection. Acid-blocking medicines can prevent acid reflux, which can lead to aspiration.
Lung Transplant
A lung transplant may be an option for children who have severe chILD if other treatments haven't worked.
Currently, lung transplants are the only effective treatment for some types of chILD that have a high risk of death, such as alveolar capillary dysplasia and certain surfactant dysfunction mutations.
Early diagnosis of these diseases gives children the chance to receive lung transplants. So far, chILD doesn't appear to come back in patients' transplanted lungs.
For more information about this treatment, go to the Health Topics Lung Transplant article.
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Childhood Interstitial Lung Disease
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How to prevent Childhood Interstitial Lung Disease ?
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At this time, most types of childhood interstitial lung disease (chILD) can't be prevented. People who have a family history of inherited (genetic) interstitial lung disease may want to consider genetic counseling. A counselor can explain the risk of children inheriting chILD.
You and your child can take steps to help prevent infections and other illnesses that worsen chILD and its symptoms. For example:
Make hand washing a family habit to avoid germs and prevent illnesses.
Try to keep your child away from people who are sick. Even a common cold can cause problems for someone who has chILD.
Talk with your child's doctor about vaccines that your child needs, such as an annual flu shot. Make sure everyone in your household gets all of the vaccines that their doctors recommend.
Talk with your child's doctor about how to prevent your child from getting respiratory syncytial (sin-SIT-e-al) virus. This common virus leads to cold and flu symptoms for most people. However, it can make children who have lung diseases very sick.
Avoid exposing your child to air pollution, tobacco smoke, and other substances that can irritate his or her lungs. Strongly advise your child not to smoke now or in the future.
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Childhood Interstitial Lung Disease
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What is (are) Pleurisy and Other Pleural Disorders ?
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Pleurisy (PLUR-ih-se) is a condition in which the pleura is inflamed. The pleura is a membrane that consists of two large, thin layers of tissue. One layer wraps around the outside of your lungs. The other layer lines the inside of your chest cavity.
Between the layers of tissue is a very thin space called the pleural space. Normally this space is filled with a small amount of fluidabout 4 teaspoons full. The fluid helps the two layers of the pleura glide smoothly past each other as you breathe in and out.
Pleurisy occurs if the two layers of the pleura become irritated and inflamed. Instead of gliding smoothly past each other, they rub together every time you breathe in. The rubbing can cause sharp pain.
Many conditions can cause pleurisy, including viral infections.
Other Pleural Disorders
Pneumothorax
Air or gas can build up in the pleural space. When this happens, it's called a pneumothorax (noo-mo-THOR-aks). A lung disease or acute lung injury can cause a pneumothorax.
Some lung procedures also can cause a pneumothorax. Examples include lung surgery, drainage of fluid with a needle,bronchoscopy (bron-KOS-ko-pee), andmechanical ventilation.
Sometimes the cause of a pneumothorax isn't known.
The most common symptoms of a pneumothorax are sudden pain in one side of the lung and shortness of breath. The air or gas in the pleural space also can put pressure on the lung and cause it to collapse.
Pleurisy and Pneumothorax
A small pneumothorax may go away without treatment. A large pneumothorax may require a procedure to remove air or gas from the pleural space.
A very large pneumothorax can interfere with blood flow through your chest and cause your blood pressure to drop. This is called a tension pneumothorax.
Pleural Effusion
In some cases of pleurisy, excess fluid builds up in the pleural space. This is called a pleural effusion. A lot of extra fluid can push the pleura against your lung until the lung, or part of it, collapses. This can make it hard for you to breathe.
Sometimes the extra fluid gets infected and turns into an abscess. When this happens, it's called an empyema (em-pi-E-ma).
You can develop a pleural effusion even if you don't have pleurisy. For example,pneumonia, (nu-MO-ne-ah),heart failure, cancer, or pulmonary embolism (PULL-mun-ary EM-bo-lizm) can lead to a pleural effusion.
Hemothorax
Blood also can build up in the pleural space. This condition is called a hemothorax (he-mo-THOR-aks). An injury to your chest, chest or heart surgery, or lung or pleural cancer can cause a hemothorax.
A hemothorax can put pressure on the lung and cause it to collapse. A hemothorax also can cause shock. In shock, not enough blood and oxygen reach your body's vital organs.
Outlook
Pleurisy and other pleural disorders can be serious, depending on their causes. If the condition that caused the pleurisy or other pleural disorder isn't too serious and is diagnosed and treated early, you usually can expect a full recovery.
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Pleurisy and Other Pleural Disorders
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What causes Pleurisy and Other Pleural Disorders ?
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Pleurisy
Many conditions can cause pleurisy. Viral infections are likely the most common cause. Other causes of pleurisy include:
Bacterial infections, such as pneumonia (nu-MO-ne-ah) and tuberculosis, and infections from fungi or parasites
Pulmonary embolism, a blood clot that travels through the blood vessels to the lungs
Autoimmune disorders, such as lupus and rheumatoid arthritis
Cancer, such as lung cancer, lymphoma, and mesothelioma (MEZ-o-thee-lee-O-ma)
Chest and heart surgery, especially coronary artery bypass grafting
Lung diseases, such as LAM (lymphangioleiomyomatosis) or asbestosis (as-bes-TO-sis)
Inflammatory bowel disease
Familial Mediterranean fever, an inherited condition that often causes fever and swelling in the abdomen or lungs
Other causes of pleurisy include chest injuries, pancreatitis (an inflamed pancreas), and reactions to certain medicines. Reactions to certain medicines can cause a condition similar to lupus. These medicines include procainamide, hydralazine, and isoniazid.
Sometimes doctors can't find the cause of pleurisy.
Pneumothorax
Lung diseases or acute lung injury can make it more likely that you will develop a pneumothorax (a buildup of air or gas in the pleural space). Such lung diseases may include COPD (chronic obstructive pulmonary disease), tuberculosis, and LAM.
Surgery or a chest injury also may cause a pneumothorax.
You can develop a pneumothorax without having a recognized lung disease or chest injury. This is called a spontaneous pneumothorax. Smoking increases your risk of spontaneous pneumothorax. Having a family history of the condition also increases your risk.
Pleural Effusion
The most common cause of a pleural effusion (a buildup of fluid in the pleural space) is heart failure. Lung cancer, LAM, pneumonia, tuberculosis, and other lung infections also can lead to a pleural effusion.
Sometimes kidney or liver disease can cause fluid to build up in the pleural space. Asbestosis, sarcoidosis (sar-koy-DO-sis), and reactions to some medicines also can lead to a pleural effusion.
Hemothorax
An injury to the chest, chest or heart surgery, or lung or pleural cancer can cause a hemothorax (buildup of blood in the pleural space).
A hemothorax also can be a complication of an infection (for example, pneumonia), tuberculosis, or a spontaneous pneumothorax.
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Pleurisy and Other Pleural Disorders
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What are the symptoms of Pleurisy and Other Pleural Disorders ?
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Pleurisy
The main symptom of pleurisy is a sharp or stabbing pain in your chest that gets worse when you breathe in deeply or cough or sneeze.
The pain may stay in one place or it may spread to your shoulders or back. Sometimes the pain becomes a fairly constant dull ache.
Depending on what's causing the pleurisy, you may have other symptoms, such as:
Shortness of breath or rapid, shallow breathing
Coughing
Fever and chills
Unexplained weight loss
Pneumothorax
The symptoms of pneumothorax include:
Sudden, sharp chest pain that gets worse when you breathe in deeply or cough
Shortness of breath
Chest tightness
Easy fatigue (tiredness)
A rapid heart rate
A bluish tint to the skin caused by lack of oxygen
Other symptoms of pneumothorax include flaring of the nostrils; anxiety, stress, and tension; and hypotension (low blood pressure).
Pleural Effusion
Pleural effusion often has no symptoms.
Hemothorax
The symptoms of hemothorax often are similar to those of pneumothorax. They include:
Chest pain
Shortness of breath
Respiratory failure
A rapid heart rate
Anxiety
Restlessness
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Pleurisy and Other Pleural Disorders
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How to diagnose Pleurisy and Other Pleural Disorders ?
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Your doctor will diagnose pleurisy or another pleural disorder based on your medical history, a physical exam, and test results.
Your doctor will want to rule out other causes of your symptoms. He or she also will want to find the underlying cause of the pleurisy or other pleural disorder so it can be treated.
Medical History
Your doctor may ask detailed questions about your medical history. He or she likely will ask you to describe any pain, especially:
What it feels like
Where it's located and whether you can feel it in your arms, jaw, or shoulders
When it started and whether it goes away and then comes back
What makes it better or worse
Your doctor also may ask whether you have other symptoms, such as shortness of breath, coughing, or palpitations. Palpitations are feelings that your heart is skipping a beat, fluttering, or beating too hard or fast.
Your doctor also may ask whether you've ever:
Had heart disease.
Smoked.
Traveled to places where you may have been exposed to tuberculosis.
Had a job that exposed you to asbestos. Asbestos is a mineral that, at one time, was widely used in many industries.
Your doctor also may ask about medicines you take or have taken. Reactions to some medicines can cause pleurisy or other pleural disorders.
Physical Exam
Your doctor will listen to your breathing with a stethoscope to find out whether your lungs are making any abnormal sounds.
If you have pleurisy, the inflamed layers of the pleura make a rough, scratchy sound as they rub against each other when you breathe. Doctors call this a pleural friction rub. If your doctor hears the friction rub, he or she will know that you have pleurisy.
If you have a pleural effusion, fluid buildup in the pleural space will prevent a friction rub. But if you have a lot of fluid, your doctor may hear a dull sound when he or she taps on your chest. Or, he or she may have trouble hearing any breathing sounds.
Muffled or dull breathing sounds also can be a sign of a pneumothorax (a buildup of air or gas in the pleural space).
Diagnostic Tests
Depending on the results of your physical exam, your doctor may recommend tests.
Chest X Ray
Achest x rayis a painless test that creates a picture of the structures in your chest, such as your heart, lungs, and blood vessels. This test may show air or fluid in the pleural space.
A chest x ray also may show what's causing a pleural disorderfor example, pneumonia, a fractured rib, or a lung tumor.
Sometimes a chest x ray is taken while you lie on your side. This position can show fluid that didn't appear on an x ray taken while you were standing.
Chest CT Scan
A chest computed tomography (to-MOG-rah-fee) scan, orchest CT scan,is a painless test that creates precise pictures of the structures in your chest.
This test provides a computer-generated picture of your lungs that can show pockets of fluid. A chest CT scan also may show signs of pneumonia, a lung abscess, a tumor, or other possible causes of pleural disorders.
Ultrasound
This test uses sound waves to create pictures of your lungs. An ultrasound may show where fluid is located in your chest. The test also can show some tumors.
Chest MRI
A chest magnetic resonance imaging scan, orchest MRI,uses radio waves, magnets, and a computer to created detailed pictures of the structures in your chest. This test can show pleural effusions and tumors.
This test also is called a magnetic resonance (MR) scan or a nuclear magnetic resonance (NMR) scan.
Blood Tests
Blood testscan show whether you have an illness that increases your risk of pleurisy or another pleural disorder. Such illnesses include bacterial or viral infections, pneumonia, pancreatitis (an inflamed pancreas), kidney disease, or lupus.
Arterial Blood Gas Test
For this test, a blood sample is taken from an artery, usually in your wrist. The blood's oxygen and carbon dioxide levels are checked. This test shows how well your lungs are taking in oxygen.
Thoracentesis
Once your doctor knows whether fluid has built up in the pleural space and where it is, he or she can remove a sample for testing. This is done using a procedure calledthoracentesis(THOR-ah-sen-TE-sis).
During the procedure, your doctor inserts a thin needle or plastic tube into the pleural space and draws out the excess fluid. After the fluid is removed from your chest, it's sent for testing.
The risks of thoracentesissuch as pain, bleeding, and infectionusually are minor. They get better on their own, or they're easily treated. Your doctor may do a chest x ray after the procedure to check for complications.
Fluid Analysis
The fluid removed during thoracentesis is examined under a microscope. It's checked for signs of infection, cancer, or other conditions that can cause fluid or blood to build up in the pleural space.
Biopsy
Your doctor may suspect that tuberculosis or cancer has caused fluid to build up in your pleural space. If so, he or she may want to look at a small piece of the pleura under a microscope.
To take a tissue sample, your doctor may do one of the following procedures:
Insert a needle into your chest to remove a small sample of the pleura's outer layer.
Insert a tube with a light on the end (endoscope) into tiny cuts in your chest wall so that he or she can see the pleura. Your doctor can then snip out small pieces of tissue. This procedure must be done in a hospital. You'll be given medicine to make you sleep during the procedure.
Snip out a sample of the pleura through a small cut in your chest wall. This is called an open pleural biopsy. It's usually done if the sample from the needle biopsy is too small for an accurate diagnosis. This procedure must be done in a hospital. You'll be given medicine to make you sleep during the procedure.
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Pleurisy and Other Pleural Disorders
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What are the treatments for Pleurisy and Other Pleural Disorders ?
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Pleurisy and other pleural disorders are treated with procedures, medicines, and other methods. The goals of treatment include:
Relieving symptoms
Removing the fluid, air, or blood from the pleural space (if a large amount is present)
Treating the underlying condition
Relieving Symptoms
To relieve pleurisy symptoms, your doctor may recommend:
Acetaminophen or anti-inflammatory medicines (such as ibuprofen) to control pain.
Codeine-based cough syrups to controlcoughing.
Lying on your painful side. This might make you more comfortable.
Breathing deeply and coughing to clear mucus as the pain eases. Otherwise, you may developpneumonia.
Getting plenty of rest.
Removing Fluid, Air, or Blood From the Pleural Space
Your doctor may recommend removing fluid, air, or blood from your pleural space to prevent a lung collapse.
The procedures used to drain fluid, air, or blood from the pleural space are similar.
Duringthoracentesis, your doctor will insert a thin needle or plastic tube into the pleural space. An attached syringe will draw fluid out of your chest. This procedure can remove more than 6 cups of fluid at a time.
If your doctor needs to remove a lot of fluid, he or she may use a chest tube. Your doctor will inject a painkiller into the area of your chest wall where the fluid is. He or she will then insert a plastic tube into your chest between two ribs. The tube will be connected to a box that suctions out the fluid. Your doctor will use achest x ray to check the tube's position.
Your doctor also can use a chest tube to drain blood and air from the pleural space. This process can take several days. The tube will be left in place, and you'll likely stay in the hospital during this time.
Sometimes the fluid in the pleural space contains thick pus or blood clots. It may form a hard skin or peel, which makes the fluid harder to drain. To help break up the pus or blood clots, your doctor may use a chest tube to deliver medicines called fibrinolytics to the pleural space. If the fluid still won't drain, you may need surgery.
If you have a small, persistent air leak into the pleural space, your doctor may attach a one-way valve to the chest tube. The valve allows air to exit the pleural space, but not reenter. Using this type of valve may allow you to continue your treatment from home.
Treat the Underlying Condition
The fluid sample that was removed during thoracentesis will be checked under a microscope. This can tell your doctor what's causing the fluid buildup, and he or she can decide the best way to treat it.
If the fluid is infected, treatment will involve antibiotics and drainage. If you have tuberculosis or a fungal infection, treatment will involve long-term use of antibiotics or antifungal medicines.
If tumors in the pleura are causing fluid buildup, the fluid may quickly build up again after it's drained. Sometimes antitumor medicines will prevent further fluid buildup. If they don't, your doctor may seal the pleural space. Sealing the pleural space is called pleurodesis (plur-OD-eh-sis).
For this procedure, your doctor will drain all of the fluid out of your chest through a chest tube. Then he or she will push a substance through the chest tube into the pleural space. The substance will irritate the surface of the pleura. This will cause the two layers of the pleura to stick together, preventing more fluid from building up.
Chemotherapy or radiation treatment also may be used to reduce the size of the tumors.
Ifheart failureis causing fluid buildup, treatment usually includes diuretics (medicines that help reduce fluid buildup) and other medicines.
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Pleurisy and Other Pleural Disorders
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What is (are) Pulmonary Hypertension ?
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Pulmonary hypertension (PULL-mun-ary HI-per-TEN-shun), or PH, is increased pressure in the pulmonary arteries. These arteries carry blood from your heart to your lungs to pick up oxygen.
PH causes symptoms such as shortness of breath during routine activity (for example, climbing two flights of stairs), tiredness, chest pain, and a racing heartbeat. As the condition worsens, its symptoms may limit all physical activity.
Overview
To understand PH, it helps to understand how your heart and lungs work. Your heart has two sides, separated by an inner wall called the septum.
Each side of your heart has an upper and lower chamber. The lower right chamber of your heart, the right ventricle (VEN-trih-kul), pumps blood to your pulmonary arteries. The blood then travels to your lungs, where it picks up oxygen.
The upper left chamber of your heart, the left atrium (AY-tree-um), receives the oxygen-rich blood from your lungs. The blood is then pumped into the lower left chamber of your heart, the left ventricle. From the left ventricle, the blood is pumped to the rest of your body through an artery called the aorta.
For more information about the heart and lungs, go to the Diseases and Conditions Index How the Heart Works and How the Lungs Work articles.
PH begins with inflammation and changes in the cells that line your pulmonary arteries. Other factors also can affect the pulmonary arteries and cause PH. For example, the condition may develop if:
The walls of the arteries tighten.
The walls of the arteries are stiff at birth or become stiff from an overgrowth of cells.
Blood clots form in the arteries.
These changes make it hard for your heart to push blood through your pulmonary arteries and into your lungs. As a result, the pressure in your arteries rises. Also, because your heart is working harder than normal, your right ventricle becomes strained and weak.
Your heart may become so weak that it can't pump enough blood to your lungs. This causes heart failure. Heart failure is the most common cause of death in people who have PH.
PH is divided into five groups based on its causes. In all groups, the average pressure in the pulmonary arteries is higher than 25 mmHg at rest or 30 mmHg during physical activity. The pressure in normal pulmonary arteries is 820 mmHg at rest. (The mmHg is millimeters of mercurythe units used to measure blood pressure.)
Other diseases or conditions, such as heart and lung diseases or blood clots, usually cause PH. Some people inherit the condition (that is, their parents pass the genes for PH on to them). In some cases, the cause isn't known.
Outlook
PH has no cure. However, research for new treatments is ongoing. The earlier PH is treated, the easier it is to control.
Treatments include medicines, procedures, and other therapies. These treatments can relieve PH symptoms and slow the progress of the disease. Lifestyle changes also can help control symptoms.
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Pulmonary Hypertension
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What causes Pulmonary Hypertension ?
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Pulmonary hypertension (PH) begins with inflammation and changes in the cells that line your pulmonary arteries. Other factors also can affect the pulmonary arteries and cause PH. For example, the condition may develop if:
The walls of the arteries tighten.
The walls of the arteries are stiff at birth or become stiff from an overgrowth of cells.
Blood clots form in the arteries.
These changes make it hard for your heart to push blood through your pulmonary arteries and into your lungs. Thus, the pressure in the arteries rises, causing PH.
Many factors can contribute to the process that leads to the different types of PH.
Group 1 pulmonary arterial hypertension (PAH) may have no known cause, or the condition may be inherited. ("Inherited" means the condition is passed from parents to children through genes.)
Some diseases and conditions also can cause group 1 PAH. Examples include HIV infection, congenital heart disease, and sickle cell disease. Also, the use of street drugs (such as cocaine) and certain diet medicines can lead to PAH.
Many diseases and conditions can cause groups 2 through 5 PH (often called secondary PH), including:
Mitral valve disease
Lung diseases, such as COPD (chronic obstructive pulmonary disease)
Sleep apnea
Sarcoidosis
For more information about the types of PH and the diseases, conditions, and factors that can cause them, go to "Types of Pulmonary Hypertension."
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Pulmonary Hypertension
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Who is at risk for Pulmonary Hypertension? ?
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The exact number of people who have pulmonary hypertension (PH) isn't known.
Group 1 pulmonary arterial hypertension (PAH) without a known cause is rare. It affects women more often than men. People who have group 1 PAH tend to be overweight.
PH that occurs with another disease or condition is more common.
PH usually develops between the ages of 20 and 60, but it can occur at any age. People who are at increased risk for PH include:
Those who have a family history of the condition.
Those who have certain diseases or conditions, such as heart and lung diseases, liver disease, HIV infection, or blood clots in the pulmonary arteries. (For more information about the diseases, conditions, and factors that cause PH, go to "Types of Pulmonary Hypertension.")
Those who use street drugs (such as cocaine) or certain diet medicines.
Those who live at high altitudes.
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Pulmonary Hypertension
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What are the symptoms of Pulmonary Hypertension ?
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Signs and symptoms of pulmonary hypertension (PH) may include:
Shortness of breath during routine activity, such as climbing two flights of stairs
Tiredness
Chest pain
A racing heartbeat
Pain on the upper right side of the abdomen
Decreased appetite
As PH worsens, you may find it hard to do any physical activities. At this point, other signs and symptoms may include:
Feeling light-headed, especially during physical activity
Fainting at times
Swelling in your legs and ankles
A bluish color on your lips and skin
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Pulmonary Hypertension
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How to diagnose Pulmonary Hypertension ?
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Your doctor will diagnose pulmonary hypertension (PH) based on your medical and family histories, a physical exam, and the results from tests and procedures.
PH can develop slowly. In fact, you may have it for years and not know it. This is because the condition has no early signs or symptoms.
When symptoms do occur, they're often like those of other heart and lung conditions, such as asthma. This makes PH hard to diagnose.
Medical and Family Histories
Your doctor may ask about your signs and symptoms and how and when they began. He or she also may ask whether you have other medical conditions that can cause PH.
Your doctor will want to know whether you have any family members who have or have had PH. People who have a family history of PH are at higher risk for the condition.
Physical Exam
During the physical exam, your doctor will listen to your heart and lungs with a stethoscope. He or she also will check your ankles and legs for swelling and your lips and skin for a bluish color. These are signs of PH.
Diagnostic Tests and Procedures
Your doctor may recommend tests and procedures to confirm a diagnosis of PH and to look for its underlying cause. Your doctor also will use test results to find out the severity of your PH.
Tests and Procedures To Confirm a Diagnosis
Echocardiography. Echocardiography (EK-o-kar-de-OG-ra-fee), or echo, uses sound waves to create a moving picture of your heart. This test can estimate the pressure in your pulmonary arteries. Echo also can show the size and thickness of your right ventricle and how well it's working.
Chest x ray. A chest x ray takes pictures of the structures in your chest, such as your heart, lungs, and blood vessels. This test can show whether your pulmonary arteries and right ventricle are enlarged.
The pulmonary arteries and right ventricle may get larger if the right ventricle has to work hard to pump blood through the pulmonary arteries.
A chest x ray also may show signs of an underlying lung disease that's causing or contributing to PH.
EKG (electrocardiogram). An EKG is a simple, painless test that records the heart's electrical activity. This test also shows whether your heart's rhythm is steady or irregular. An EKG may show whether your right ventricle is enlarged or strained.
Right heart catheterization. This procedure measures the pressure in your pulmonary arteries. It also shows how well your heart is pumping blood to the rest of your body. Right heart catheterization (KATH-e-ter-ih-ZA-shun) can find any leaks between the left and right side of the heart.
During this procedure, a thin, flexible tube called a catheter is put into a blood vessel in your groin (upper thigh) or neck. The tube is threaded into the right side of your heart and into the pulmonary arteries. Through the tube, your doctor can do tests and treatments on your heart.
Tests To Look for the Underlying Cause of Pulmonary Hypertension
PH has many causes, so many tests may need to be done to find its underlying cause.
Chest CT scan. A chest computed tomography (to-MOG-ra-fee) scan, or chest CT scan, creates pictures of the structures inside your chest, such as your heart, lungs, and blood vessels. These pictures can show signs of PH or a condition that may be causing PH.
Chest MRI. Chest magnetic resonance imaging, or chest MRI, shows how your right ventricle is working. The test also shows blood flow in your lungs. Chest MRI also can help detect signs of PH or an underlying condition causing PH.
Lung function tests. Lung function tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. These tests can help detect a lung disease that may be causing PH.
Polysomnogram (PSG). This test records brain activity, eye movements, heart rate, and blood pressure while you sleep. A PSG also measures the level of oxygen in your blood. A low oxygen level during sleep is common in PH, and it can make the condition worse.
A PSG usually is done while you stay overnight at a sleep center. For more information about this test, go to the Diseases and Conditions Index Sleep Studies article.
Lung ventilation/perfusion (VQ) scan. A lung VQ scan measures air and blood flow in your lungs. This test can help detect blood clots in your lung's blood vessels.
Blood tests. Blood tests are used to rule out other diseases, such as HIV, liver disease, and autoimmune diseases (such as rheumatoid arthritis).
Finding Out the Severity of Pulmonary Hypertension
Exercise testing is used to find out the severity of PH. This testing consists of either a 6-minute walk test or a cardiopulmonary exercise test.
A 6-minute walk test measures the distance you can quickly walk in 6 minutes. A cardiopulmonary exercise test measures how well your lungs and heart work while you exercise on a treadmill or bicycle.
During exercise testing, your doctor will rate your activity level. Your level is linked to the severity of your PH. The rating system ranges from class 1 to class 4.
Class 1 has no limits. You can do regular physical activities, such as walking or climbing stairs. These activities don't cause PH symptoms, such as tiredness, shortness of breath, or chest pain.
Class 2 has slight or mild limits. You're comfortable while resting, but regular physical activity causes PH symptoms.
Class 3 has marked or noticeable limits. You're comfortable while resting. However, walking even one or two blocks or climbing one flight of stairs can cause PH symptoms.
Class 4 has severe limits. You're not able to do any physical activity without discomfort. You also may have PH symptoms while at rest.
Over time, you may need more exercise tests to find out how well your treatments are working. Each time testing is done, your doctor will compare your activity level with the previous one.
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Pulmonary Hypertension
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What are the treatments for Pulmonary Hypertension ?
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Pulmonary hypertension (PH) has no cure. However, treatment may help relieve symptoms and slow the progress of the disease.
PH is treated with medicines, procedures, and other therapies. Treatment will depend on what type of PH you have and its severity. (For more information, go to "Types of Pulmonary Hypertension.")
Group 1 Pulmonary Arterial Hypertension
Group 1 pulmonary arterial hypertension (PAH) includes PH that's inherited, that has no known cause, or that's caused by certain drugs or conditions. Treatments for group 1 PAH include medicines and medical procedures.
Medicines
Your doctor may prescribe medicines to relax the blood vessels in your lungs and reduce excess cell growth in the blood vessels. As the blood vessels relax, more blood can flow through them.
Your doctor may prescribe medicines that are taken by mouth, inhaled, or injected.
Examples of medicines for group 1 PAH include:
Phosphodiesterase-5 inhibitors, such as sildenafil
Prostanoids, such as epoprostenol
Endothelin receptor antagonists, such as bosentan and ambrisentan
Calcium channel blockers, such as diltiazem
Your doctor may prescribe one or more of these medicines. To find out which of these medicines works best, you'll likely have an acute vasoreactivity test. This test shows how the pressure in your pulmonary arteries reacts to certain medicines. The test is done during right heart catheterization.
Medical and Surgical Procedures
If you have group 1 PAH, your doctor may recommend one or more of the following procedures.
Atrial septostomy (sep-TOS-toe-me). For this procedure, a thin, flexible tube called a catheter is put into a blood vessel in your leg and threaded to your heart. The tube is then put through the wall that separates your right and left atria (the upper chambers of your heart). This wall is called the septum.
A tiny balloon on the tip of the tube is inflated. This creates an opening between the atria. This procedure relieves the pressure in the right atria and increases blood flow. Atrial septostomy is rarely done in the United States.
Lung transplant. A lung transplant is surgery to replace a person's diseased lung with a healthy lung from a deceased donor. This procedure may be used for people who have severe lung disease that's causing PAH.
Heartlung transplant. A heartlung transplant is surgery in which both the heart and lung are replaced with healthy organs from a deceased donor.
Group 2 Pulmonary Hypertension
Conditions that affect the left side of the heart, such as mitral valve disease, can cause group 2 PH. Treating the underlying condition will help treat PH. Treatments may include lifestyle changes, medicines, and surgery.
Group 3 Pulmonary Hypertension
Lung diseases, such as COPD (chronic obstructive pulmonary disease) and interstitial lung disease, can cause group 3 PH. Certain sleep disorders, such as sleep apnea, also can cause group 3 PH.
If you have this type of PH, you may need oxygen therapy. This treatment raises the level of oxygen in your blood. You'll likely get the oxygen through soft, plastic prongs that fit into your nose. Oxygen therapy can be done at home or in a hospital.
Your doctor also may recommend other treatments if you have an underlying lung disease.
Group 4 Pulmonary Hypertension
Blood clots in the lungs or blood clotting disorders can cause group 4 PH. If you have this type of PH, your doctor will likely prescribe blood-thinning medicines. These medicines prevent clots from forming or getting larger.
Sometimes doctors use surgery to remove scarring in the pulmonary arteries due to old blood clots.
Group 5 Pulmonary Hypertension
Various diseases and conditions, such as thyroid disease and sarcoidosis, can cause group 5 PH. An object, such as a tumor, pressing on the pulmonary arteries also can cause group 5 PH.
Group 5 PH is treated by treating its cause.
All Types of Pulmonary Hypertension
Several treatments may be used for all types of PH. These treatments include:
Diuretics, also called water pills. These medicines help reduce fluid buildup in your body, including swelling in your ankles and feet.
Blood-thinning medicines. These medicines help prevent blood clots from forming or getting larger.
Digoxin. This medicine helps the heart beat stronger and pump more blood. Digoxin sometimes is used to control the heart rate if abnormal heart rhythms, such as atrial fibrillation or atrial flutter, occur.
Oxygen therapy. This treatment raises the level of oxygen in your blood.
Physical activity. Regular activity may help improve your ability to be active. Talk with your doctor about a physical activity plan that's safe for you.
Research is ongoing for better PH treatments. These treatments offer hope for the future.
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Pulmonary Hypertension
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What is (are) Thrombocytopenia ?
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Thrombocytopenia (THROM-bo-si-to-PE-ne-ah) is a condition in which your blood has a lower than normal number of blood cell fragments called platelets (PLATE-lets).
Platelets are made in your bone marrow along with other kinds of blood cells. They travel through your blood vessels and stick together (clot) to stop any bleeding that may happen if a blood vessel is damaged. Platelets also are called thrombocytes (THROM-bo-sites) because a clot also is called a thrombus.
Overview
When your blood has too few platelets, mild to serious bleeding can occur. Bleeding can occur inside your body (internal bleeding) or underneath your skin or from the surface of your skin (external bleeding).
A normal platelet count in adults ranges from 150,000 to 450,000 platelets per microliter of blood. A platelet count of less than 150,000 platelets per microliter is lower than normal. If your blood platelet count falls below normal, you have thrombocytopenia.
However, the risk for serious bleeding doesn't occur until the count becomes very lowless than 10,000 or 20,000 platelets per microliter. Mild bleeding sometimes occurs when the count is less than 50,000 platelets per microliter.
Many factors can cause a low platelet count, such as:
The body's bone marrow doesn't make enough platelets.
The bone marrow makes enough platelets, but the body destroys them or uses them up.
The spleen holds on to too many platelets. The spleen is an organ that normally stores about one-third of the body's platelets. It also helps your body fight infection and remove unwanted cell material.
A combination of the above factors.
How long thrombocytopenia lasts depends on its cause. It can last from days to years.
The treatment for this condition also depends on its cause and severity. Mild thrombocytopenia often doesn't require treatment. If the condition causes or puts you at risk for serious bleeding, you may need medicines or blood or platelet transfusions. Rarely, the spleen may need to be removed.
Outlook
Thrombocytopenia can be fatal, especially if the bleeding is severe or occurs in the brain. However, the overall outlook for people who have the condition is good, especially if the cause of the low platelet count is found and treated.
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Thrombocytopenia
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What causes Thrombocytopenia ?
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Many factors can cause thrombocytopenia (a low platelet count). The condition can be inherited or acquired. "Inherited" means your parents pass the gene for the condition to you. "Acquired" means you aren't born with the condition, but you develop it. Sometimes the cause of thrombocytopenia isn't known.
In general, a low platelet count occurs because:
The body's bone marrow doesn't make enough platelets.
The bone marrow makes enough platelets, but the body destroys them or uses them up.
The spleen holds on to too many platelets.
A combination of the above factors also may cause a low platelet count.
The Bone Marrow Doesn't Make Enough Platelets
Bone marrow is the sponge-like tissue inside the bones. It contains stem cells that develop into red blood cells, white blood cells, and platelets. When stem cells are damaged, they don't grow into healthy blood cells.
Many conditions and factors can damage stem cells.
Cancer
Cancer, such as leukemia (lu-KE-me-ah) or lymphoma (lim-FO-ma), can damage the bone marrow and destroy blood stem cells. Cancer treatments, such as radiation and chemotherapy, also destroy the stem cells.
Aplastic Anemia
Aplastic anemia is a rare, serious blood disorder in which the bone marrow stops making enough new blood cells. This lowers the number of platelets in your blood.
Toxic Chemicals
Exposure to toxic chemicalssuch as pesticides, arsenic, and benzenecan slow the production of platelets.
Medicines
Some medicines, such as diuretics and chloramphenicol, can slow the production of platelets. Chloramphenicol (an antibiotic) rarely is used in the United States.
Common over-the-counter medicines, such as aspirin or ibuprofen, also can affect platelets.
Alcohol
Alcohol also slows the production of platelets. A temporary drop in the platelet count is common among heavy drinkers, especially if they're eating foods that are low in iron, vitamin B12, or folate.
Viruses
Chickenpox, mumps, rubella, Epstein-Barr virus, or parvovirus can decrease your platelet count for a while. People who have AIDS often develop thrombocytopenia.
Genetic Conditions
Some genetic conditions can cause low numbers of platelets in the blood. Examples include Wiskott-Aldrich and May-Hegglin syndromes.
The Body Destroys Its Own Platelets
A low platelet count can occur even if the bone marrow makes enough platelets. The body may destroy its own platelets due to autoimmune diseases, certain medicines, infections, surgery, pregnancy, and some conditions that cause too much blood clotting.
Autoimmune Diseases
Autoimmune diseases occur if the body's immune system mistakenly attacks healthy cells in the body. If an autoimmune disease destroys the body's platelets, thrombocytopenia can occur.
One example of this type of autoimmune disease is immune thrombocytopenia (ITP). ITP is a bleeding disorder in which the blood doesn't clot as it should. An autoimmune response is thought to cause most cases of ITP.
Normally, your immune system helps your body fight off infections and diseases. But if you have ITP, your immune system attacks and destroys its own platelets. Why this happens isn't known. (ITP also may occur if the immune system attacks your bone marrow, which makes platelets.)
Other autoimmune diseases that destroy platelets include lupus and rheumatoid arthritis.
Medicines
A reaction to medicine can confuse your body and cause it to destroy its platelets. Examples of medicines that may cause this to happen include quinine; antibiotics that contain sulfa; and some medicines for seizures, such as Dilantin, vancomycin, and rifampin. (Quinine is a substance often found in tonic water and nutritional health products.)
Heparin is a medicine commonly used to prevent blood clots. But an immune reaction may trigger the medicine to cause blood clots and thrombocytopenia. This condition is called heparin-induced thrombocytopenia (HIT). HIT rarely occurs outside of a hospital.
In HIT, the body's immune system attacks a substance formed by heparin and a protein on the surface of the platelets. This attack activates the platelets and they start to form blood clots.
Blood clots can form deep in the legs (deep vein thrombosis), or they can break loose and travel to the lungs (pulmonary embolism).
Infection
A low platelet count can occur after blood poisoning from a widespread bacterial infection. A virus, such as mononucleosis or cytomegalovirus, also can cause a low platelet count.
Surgery
Platelets can be destroyed when they pass through man-made heart valves, blood vessel grafts, or machines and tubing used for blood transfusions or bypass surgery.
Pregnancy
About 5 percent of pregnant women develop mild thrombocytopenia when they're close to delivery. The exact cause isn't known for sure.
Rare and Serious Conditions That Cause Blood Clots
Some rare and serious conditions can cause a low platelet count. Two examples are thrombotic thrombocytopenic purpura (TTP) and disseminated intravascular coagulation (DIC).
TTP is a rare blood condition. It causes blood clots to form in the body's small blood vessels, including vessels in the brains, kidneys, and heart.
DIC is a rare complication of pregnancy, severe infections, or severe trauma. Tiny blood clots form suddenly throughout the body.
In both conditions, the blood clots use up many of the blood's platelets.
The Spleen Holds On to Too Many Platelets
Usually, one-third of the body's platelets are held in the spleen. If the spleen is enlarged, it will hold on to too many platelets. This means that not enough platelets will circulate in the blood.
An enlarged spleen often is due to cancer or severe liver disease, such as cirrhosis (sir-RO-sis). Cirrhosis is a disease in which the liver is scarred. This prevents it from working well.
An enlarged spleen also might be due to a bone marrow condition, such as myelofibrosis (MI-eh-lo-fi-BRO-sis). With this condition, the bone marrow is scarred and isn't able to make blood cells.
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Thrombocytopenia
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Who is at risk for Thrombocytopenia? ?
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People who are at highest risk for thrombocytopenia are those affected by one of the conditions or factors discussed in "What Causes Thrombocytopenia?" This includes people who:
Have certain types of cancer, aplastic anemia, or autoimmune diseases
Are exposed to certain toxic chemicals
Have a reaction to certain medicines
Have certain viruses
Have certain genetic conditions
People at highest risk also include heavy alcohol drinkers and pregnant women.
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Thrombocytopenia
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What are the symptoms of Thrombocytopenia ?
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Mild to serious bleeding causes the main signs and symptoms of thrombocytopenia. Bleeding can occur inside your body (internal bleeding) or underneath your skin or from the surface of your skin (external bleeding).
Signs and symptoms can appear suddenly or over time. Mild thrombocytopenia often has no signs or symptoms. Many times, it's found during a routine blood test.
Check with your doctor if you have any signs of bleeding. Severe thrombocytopenia can cause bleeding in almost any part of the body. Bleeding can lead to a medical emergency and should be treated right away.
External bleeding usually is the first sign of a low platelet count. External bleeding may cause purpura (PURR-purr-ah) or petechiae (peh-TEE-key-ay). Purpura are purple, brown, and red bruises. This bruising may happen easily and often. Petechiae are small red or purple dots on your skin.
Purpura and Petechiae
Other signs of external bleeding include:
Prolonged bleeding, even from minor cuts
Bleeding or oozing from the mouth or nose, especially nosebleeds or bleeding from brushing your teeth
Abnormal vaginal bleeding (especially heavy menstrual flow)
A lot of bleeding after surgery or dental work also might suggest a bleeding problem.
Heavy bleeding into the intestines or the brain (internal bleeding) is serious and can be fatal. Signs and symptoms include:
Blood in the urine or stool or bleeding from the rectum. Blood in the stool can appear as red blood or as a dark, tarry color. (Taking iron supplements also can cause dark, tarry stools.)
Headaches and other neurological symptoms. These problems are very rare, but you should discuss them with your doctor.
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Thrombocytopenia
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How to diagnose Thrombocytopenia ?
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Your doctor will diagnose thrombocytopenia based on your medical history, a physical exam, and test results. A hematologist also may be involved in your care. This is a doctor who specializes in diagnosing and treating blood diseases and conditions.
Once thrombocytopenia is diagnosed, your doctor will begin looking for its cause.
Medical History
Your doctor may ask about factors that can affect your platelets, such as:
The medicines you take, including over-the-counter medicines and herbal remedies, and whether you drink beverages that contain quinine. Quinine is a substance often found in tonic water and nutritional health products.
Your general eating habits, including the amount of alcohol you normally drink.
Your risk for AIDS, including questions about blood transfusions, sexual partners, intravenous (IV) drugs, and exposure to infectious blood or bodily fluids at work.
Any family history of low platelet counts.
Physical Exam
Your doctor will do a physical exam to look for signs and symptoms of bleeding, such as bruises or spots on the skin. He or she will check your abdomen for signs of an enlarged spleen or liver. You also will be checked for signs of infection, such as a fever.
Diagnostic Tests
Your doctor may recommend one or more of the following tests to help diagnose a low platelet count. For more information about blood tests, go to the Health Topics Blood Tests article.
Complete Blood Count
A complete blood count (CBC) measures the levels of red blood cells, white blood cells, and platelets in your blood. For this test, a small amount of blood is drawn from a blood vessel, usually in your arm.
If you have thrombocytopenia, the results of this test will show that your platelet count is low.
Blood Smear
A blood smear is used to check the appearance of your platelets under a microscope. For this test, a small amount of blood is drawn from a blood vessel, usually in your arm.
Bone Marrow Tests
Bone marrow tests check whether your bone marrow is healthy. Blood cells, including platelets, are made in your bone marrow. The two bone marrow tests are aspiration (as-pih-RA-shun) and biopsy.
Bone marrow aspiration might be done to find out why your bone marrow isn't making enough blood cells. For this test, your doctor removes a sample of fluid bone marrow through a needle. He or she examines the sample under a microscope to check for faulty cells.
A bone marrow biopsy often is done right after an aspiration. For this test, your doctor removes a sample of bone marrow tissue through a needle. He or she examines the tissue to check the number and types of cells in the bone marrow.
Other Tests
If a bleeding problem is suspected, you may need other blood tests as well. For example, your doctor may recommend PT and PTT tests to see whether your blood is clotting properly.
Your doctor also may suggest an ultrasound to check your spleen. An ultrasound uses sound waves to create pictures of your spleen. This will allow your doctor to see whether your spleen is enlarged.
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Thrombocytopenia
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What are the treatments for Thrombocytopenia ?
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Treatment for thrombocytopenia depends on its cause and severity. The main goal of treatment is to prevent death and disability caused by bleeding.
If your condition is mild, you may not need treatment. A fully normal platelet count isn't necessary to prevent bleeding, even with severe cuts or accidents.
Thrombocytopenia often improves when its underlying cause is treated. People who inherit the condition usually don't need treatment.
If a reaction to a medicine is causing a low platelet count, your doctor may prescribe another medicine. Most people recover after the initial medicine has been stopped. For heparin-induced thrombocytopenia (HIT), stopping the heparin isn't enough. Often, you'll need another medicine to prevent blood clotting.
If your immune system is causing a low platelet count, your doctor may prescribe medicines to suppress the immune system.
Severe Thrombocytopenia
If your thrombocytopenia is severe, your doctor may prescribe treatments such as medicines, blood or platelet transfusions, or splenectomy.
Medicines
Your doctor may prescribe corticosteroids, also called steroids for short. Steroids may slow platelet destruction. These medicines can be given through a vein or by mouth. One example of this type of medicine is prednisone.
The steroids used to treat thrombocytopenia are different from illegal steroids taken by some athletes to enhance performance.
Your doctor may prescribe immunoglobulins or medicines like rituximab to block your immune system. These medicines are given through a vein. He or she also may prescribe other medicines, such as eltrombopag or romiplostim, to help your body make more platelets. The former comes as a tablet to take by mouth and the latter is given as an injection under the skin.
Blood or Platelet Transfusions
Blood or platelet transfusions are used to treat people who have active bleeding or are at a high risk of bleeding. During this procedure, a needle is used to insert an intravenous (IV) line into one of your blood vessels. Through this line, you receive healthy blood or platelets.
For more information about this procedure, go to the Health Topics Blood Transfusion article.
Splenectomy
A splenectomy is surgery to remove the spleen. This surgery may be used if treatment with medicines doesn't work. This surgery mostly is used for adults who have immune thrombocytopenia (ITP). However, medicines often are the first course of treatment.
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Thrombocytopenia
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How to prevent Thrombocytopenia ?
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Whether you can prevent thrombocytopenia depends on its specific cause. Usually the condition can't be prevented. However, you can take steps to prevent health problems associated with thrombocytopenia. For example:
Avoid heavy drinking. Alcohol slows the production of platelets.
Try to avoid contact with toxic chemicals. Chemicals such as pesticides, arsenic, and benzene can slow the production of platelets.
Avoid medicines that you know have decreased your platelet count in the past.
Be aware of medicines that may affect your platelets and raise your risk of bleeding. Two examples of such medicines are aspirin and ibuprofen. These medicines may thin your blood too much.
Talk with your doctor about getting vaccinated for viruses that can affect your platelets. You may need vaccines for mumps, measles, rubella, and chickenpox. You may want to have your child vaccinated for these viruses as well. Talk with your child's doctor about these vaccines.
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Thrombocytopenia
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What is (are) Peripheral Artery Disease ?
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Peripheral artery disease (P.A.D.) is a disease in which plaque builds up in the arteries that carry blood to your head, organs, and limbs. Plaque is made up of fat, cholesterol, calcium, fibrous tissue, and other substances in the blood.
When plaque builds up in the body's arteries, the condition is called atherosclerosis. Over time, plaque can harden and narrow the arteries. This limits the flow of oxygen-rich blood to your organs and other parts of your body.
P.A.D. usually affects the arteries in the legs, but it also can affect the arteries that carry blood from your heart to your head, arms, kidneys, and stomach. This article focuses on P.A.D. that affects blood flow to the legs.
Normal Artery and Artery With Plaque Buildup
Overview
Blocked blood flow to your legs can cause pain and numbness. It also can raise your risk of getting an infection in the affected limbs. Your body may have a hard time fighting the infection.
If severe enough, blocked blood flow can cause gangrene (tissue death). In very serious cases, this can lead to leg amputation.
If you have leg pain when you walk or climb stairs, talk with your doctor. Sometimes older people think that leg pain is just a symptom of aging. However, the cause of the pain could be P.A.D. Tell your doctor if you're feeling pain in your legs and discuss whether you should be tested for P.A.D.
Smoking is the main risk factor for P.A.D. If you smoke or have a history of smoking, your risk of P.A.D. increases. Other factors, such as age and having certain diseases or conditions, also increase your risk of P.A.D.
Outlook
P.A.D. increases your risk of coronary heart disease, heart attack, stroke, and transient ischemic attack("mini-stroke").Although P.A.D. is serious, it's treatable. If you have the disease, see your doctor regularly and treat the underlying atherosclerosis.P.A.D. treatment may slow or stop disease progress and reduce the risk of complications. Treatments include lifestyle changes, medicines, and surgery or procedures. Researchers continue to explore new therapies for P.A.D.
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Peripheral Artery Disease
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What causes Peripheral Artery Disease ?
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The most common cause of peripheral arterydisease (P.A.D.) is atherosclerosis. Atherosclerosis is a disease in which plaque builds up in your arteries. The exact cause of atherosclerosis isn't known.
The disease may start if certain factors damage the inner layers of the arteries. These factors include:
Smoking
High amounts of certain fats and cholesterol in the blood
High blood pressure
High amounts of sugar in the blood due to insulin resistance or diabetes
When damage occurs, your body starts a healing process. The healing may cause plaque to build up where the arteries are damaged.
Eventually, a section of plaque can rupture (break open), causing a blood clot to form at the site. The buildup of plaque or blood clots can severely narrow or block the arteries and limit the flow of oxygen-rich blood to your body.
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Peripheral Artery Disease
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Who is at risk for Peripheral Artery Disease? ?
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Peripheral artery disease (P.A.D.) affects millions of people in the United States. The disease is more common in blacks than any other racial or ethnic group.The major risk factors for P.A.D. are smoking, older age, and having certain diseases or conditions.
Smoking
Smoking is the main risk factor for P.A.D. and your risk increases if you smoke or have a history of smoking. Quitting smoking slows the progress of P.A.D. People who smoke and people who have diabetes are at highest risk for P.A.D. complications, such as gangrene (tissue death) in the leg from decreased blood flow.
Older Age
Older age also is a risk factor for P.A.D. Plaque builds up in your arteries as you age.Older age combined with other risk factors, such as smoking or diabetes, also puts you at higher risk for P.A.D.
Diseases and Conditions
Many diseases and conditions can raise your risk of P.A.D., including:
Diabetes
High blood pressure
High blood cholesterol
Coronary heart disease
Stroke
Metabolic syndrome
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Peripheral Artery Disease
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What are the symptoms of Peripheral Artery Disease ?
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Many people who have peripheral artery disease (P.A.D.) dont have any signs or symptoms.
Even if you don't have signs or symptoms, ask your doctor whether you should get checked for P.A.D. if you're:
Aged 70 or older
Aged 50 or older and have a history of smoking or diabetes
Younger than 50 and have diabetes and one or more risk factors for atherosclerosis
Intermittent Claudication
People who have P.A.D. may have symptoms when walking or climbing stairs, which may include pain, numbness, aching, or heaviness in the leg muscles.Symptoms also may include cramping in the affected leg(s) and in the buttocks, thighs, calves, and feet. Symptoms may ease after resting.These symptoms are called intermittent claudication.
During physical activity, your muscles need increased blood flow. If your blood vessels are narrowed or blocked, your muscles won't get enough blood, which will lead to symptoms. When resting, the muscles need less blood flow, so the symptoms will go away.
Other Signs and Symptoms
Other signs and symptoms of P.A.D. include:
Weak or absent pulses in the legs or feet
Sores or wounds on the toes, feet, or legs that heal slowly, poorly, or not at all
A pale or bluish color to the skin
A lower temperature in one leg compared to the other leg
Poor nail growth on the toes and decreased hair growth on the legs
Erectile dysfunction, especially among men who have diabetes
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Peripheral Artery Disease
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How to diagnose Peripheral Artery Disease ?
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Peripheral artery disease (P.A.D.) is diagnosed based on your medical and family histories, a physical exam, and test results.
P.A.D. often is diagnosed after symptoms are reported. A correct diagnosis is important because people who have P.A.D. are at higher risk for coronary heart disease (CHD), heart attack, stroke, and transient ischemic attack ("mini-stroke"). If you have P.A.D., your doctor also may want to check for signs of these diseases and conditions.
Specialists Involved
Primary care doctors, such as internists and family doctors, may treat people who have mild P.A.D. For more advanced P.A.D., a vascular specialist may be involved. This is a doctor who specializes in treating blood vessel diseases and conditions.
A cardiologist also may be involved in treating people who have P.A.D. Cardiologists treat heart problems, such as CHD and heart attack, which often affect people who have P.A.D.
Medical and Family Histories
Your doctor may ask:
Whether you have any risk factors for P.A.D. For example, he or she may ask whether you smoke or have diabetes.
About your symptoms, including any symptoms that occur when walking, exercising, sitting, standing, or climbing.
About your diet.
About any medicines you take, including prescription and over-the-counter medicines.
Whether anyone in your family has a history of heart or blood vessel diseases.
Physical Exam
During the physical exam, your doctor will look for signs of P.A.D. He or she may check the blood flow in your legs or feet to see whether you have weak or absent pulses.
Your doctor also may check the pulses in your leg arteries for an abnormal whooshing sound called a bruit. He or she can hear this sound with a stethoscope. A bruit may be a warning sign of a narrowed or blocked artery.
Your doctor may compare blood pressure between your limbs to see whether the pressure is lower in the affected limb. He or she also may check for poor wound healing or any changes in your hair, skin, or nails that may be signs of P.A.D.
Diagnostic Tests
Ankle-Brachial Index
A simple test called an ankle-brachial index (ABI) often is used to diagnose P.A.D. The ABI compares blood pressure in your ankle to blood pressure in your arm. This test shows how well blood is flowing in your limbs.
ABI can show whether P.A.D. is affecting your limbs, but it won't show which blood vessels are narrowed or blocked.
A normal ABI result is 1.0 or greater (with a range of 0.90 to 1.30). The test takes about 10 to 15 minutes to measure both arms and both ankles. This test may be done yearly to see whether P.A.D. is getting worse.
Ankle-Brachial Index
Doppler Ultrasound
A Doppler ultrasound looks at blood flow in the major arteries and veins in the limbs. During this test, a handheld device is placed on your body and passed back and forth over the affected area. A computer converts sound waves into a picture of blood flow in the arteries and veins.
The results of this test can show whether a blood vessel is blocked. The results also can help show the severity of P.A.D.
Treadmill Test
A treadmill test can show the severity of symptoms and the level of exercise that brings them on. You'll walk on a treadmill for this test. This shows whether you have any problems during normal walking.
You may have an ABI test before and after the treadmill test. This will help compare blood flow in your arms and legs before and after exercise.
Magnetic Resonance Angiogram
A magnetic resonance angiogram (MRA) uses magnetic and radio wave energy to take pictures of your blood vessels. This test is a type of magnetic resonance imaging (MRI).
An MRA can show the location and severity of a blocked blood vessel. If you have a pacemaker, man-made joint, stent, surgical clips, mechanical heart valve, or other metallic devices in your body, you might not be able to have an MRA. Ask your doctor whether an MRA is an option for you.
Arteriogram
An arteriogram provides a "road map" of the arteries. Doctors use this test to find the exact location of a blocked artery.
For this test, dye is injected through a needle or catheter (tube) into one of your arteries. This may make you feel mildly flushed. After the dye is injected, an x ray is taken. The xray can show the location, type, and extent of the blockage in the artery.
Some doctors use a newer method of arteriogram that uses tiny ultrasound cameras. These cameras take pictures of the insides of the blood vessels. This method is called intravascular ultrasound.
Blood Tests
Your doctor may recommend blood tests to check for P.A.D. risk factors. For example, blood tests can help diagnose conditions such as diabetes and high blood cholesterol.
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Peripheral Artery Disease
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What are the treatments for Peripheral Artery Disease ?
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Treatments for peripheral artery disease (P.A.D.) include lifestyle changes, medicines, and surgery or procedures.
The overall goals of treating P.A.D. include reducing risk of heart attack and stroke; reducing symptoms of claudication; improving mobility and overall quality of life; and preventing complications. Treatment is based on your signs and symptoms, risk factors, and the results of physical exams and tests.
Treatment may slow or stop the progression of the disease and reduce the risk of complications. Without treatment, P.A.D. may progress, resulting in serious tissue damage in the form of sores or gangrene (tissue death) due to inadequate blood flow. In extreme cases of P.A.D., also referred to as critical limb ischemia (CLI), removal (amputation) of part of the leg or foot may be necessary.
Lifestyle Changes
Treatment often includes making long-lasting lifestyle changes, such as:
Physical activity
Quitting smoking
Heart-healthy eating
Physical Activity
Routine physical activity can improve P.A.D. symptoms and lower many risk factors for atherosclerosis, including LDL (bad) cholesterol, high blood pressure, and excess weight. Exercise can improve the distances you can comfortably walk.
Talk with your doctor about taking part in a supervised exercise program. If a supervised program is not an option, ask your doctor to help you develop an exercise plan. Most exercise programs begin slowly, which includes simple walking alternating with rest. Over time, most people build up the amount of time they can walk before developing pain. The more active you are, the more you will benefit.
Quitting Smoking
If you smoke, quit. Smoking raises your risk for P.A.D. Smoking also raises your risk for other diseases, such as coronary heart disease and heart attack, and worsens other coronary heart disease risk factors. Talk with your doctor about programs and products that can help you quit smoking. Also, try to avoid secondhandsmoke.
If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking.
Read more about quitting smoking at Smoking and Your Heart.
Heart-Healthy Eating
Your doctor may recommend heart-healthy eating to treat atherosclerosis, the most common cause of P.A.D. Following heart-healthy eating can help control blood pressure and cholesterol levels, which can lead to atherosclerosis.
Medicines
Your doctor may prescribe medicines to:
Prevent blood clots from forming due to low blood flow with anticlotting medicines, such as aspirin.
Treat unhealthy cholesterol levels with statins. Statins control or lower blood cholesterol. By lowering your blood cholesterol level, you can decrease your chance of developing complications from P.A.D.
Treat high blood pressure with one of many high blood pressure medicines.
Help ease leg pain that occurs when you walk or climb stairs.
Reduce the symptoms of intermittent claudication, measured by increased walking distance with certain platelet-aggregation inhibitors.
Surgery or Procedures
Bypass Grafting
Your doctor may recommend bypass grafting surgery if blood flow in your limb is blocked or nearly blocked. For this surgery, your doctor uses a blood vessel from another part of your body or a synthetic tube to make a graft.
This graft bypasses (that is, goes around) the blocked part of the artery. The bypass allows blood to flow around the blockage. This surgery doesnt cure P.A.D., but it may increase blood flow to the affected limb.
Angioplasty and Stent Placement
Your doctor may recommend angioplasty to restore blood flow through a narrowed or blockedartery.
During this procedure, a catheter (thin tube) with a balloon at the tip is inserted into a blocked artery. The balloon is then inflated, which pushes plaque outward against the artery wall. This widens the artery and restores blood flow.
A stent (a small mesh tube) may be placed in the artery during angioplasty. A stent helps keep the artery open after angioplasty is done. Some stents are coated with medicine to help prevent blockages in the artery.
Atherectomy
Atherectomy is a procedure that removes plaque buildup from an artery. During the procedure, a catheter is used to insert a small cutting device into the blocked artery. The device is used to shave or cut off plaque.
The bits of plaque are removed from the body through the catheter or washed away in the bloodstream (if theyre small enough).
Doctors also can perform atherectomy using a special laser that dissolves the blockage.
Other Types of Treatment
Researchers are studying cell and gene therapies to treat P.A.D. However, these treatments arent yet available outside of clinical trials. Read more about clinicaltrials.
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How to prevent Peripheral Artery Disease ?
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Taking action to control your risk factors can help prevent or delay peripheral artery disease (P.A.D.) and its complications. Know your family history of health problems related to P.A.D. If you or someone in your family has the disease, be sure to tell your doctor. Controlling risk factors includes the following.
Be physically active.
Be screened for P.A.D. A simple office test, called an ankle-brachial index or ABI, can help determine whether you have P.A.D.
Follow heart-healthy eating.
If you smoke, quit. Talk with your doctor about programs and products that can help you quitsmoking.
If youre overweight or obese, work with your doctor to create a reasonable weight-loss plan.
The lifestyle changes described above can reduce your risk of developing P.A.D. These changes also can help prevent and control conditions that can be associated with P.A.D., such as coronary heart disease, diabetes, high blood pressure, high blood cholesterol, andstroke.
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Peripheral Artery Disease
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Who is at risk for Smoking and Your Heart? ?
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The chemicals in tobacco smoke harm your heart and blood vessels in many ways. For example, they:
Contribute to inflammation, which may trigger plaque buildup in your arteries.
Damage blood vessel walls, making them stiff and less elastic (stretchy). This damage narrows the blood vessels and contributes to the damage caused by unhealthy cholesterol levels.
Disturb normal heart rhythms.
Increase your blood pressure and heart rate, making your heart work harder thannormal.
Lower your HDL (good) cholesterol and raise your LDL (bad) cholesterol. Smoking also increases your triglyceride level. Triglycerides are a type of fat found in theblood.
Thicken your blood and make it harder for your blood to carry oxygen.
Smoking and Heart Disease Risk
Smoking is a major risk factor for coronary heart disease, a condition in which plaque builds up inside the coronary arteries. These arteries supply your heart muscle with oxygen-rich blood.
When plaque builds up in the arteries, the condition is called atherosclerosis.
Plaque narrows the arteries and reduces blood flow to your heart muscle. The buildup of plaque also makes it more likely that blood clots will form in your arteries. Blood clots can partially or completely block blood flow.
Over time, smoking contributes to atherosclerosis and increases your risk of having and dying from heart disease, heart failure, or a heartattack.
Compared with nonsmokers, people who smoke are more likely to have heart disease and suffer from a heart attack. The risk of having or dying from a heart attack is even higher among people who smoke and already have heart disease.
For some people, such as women who use birth control pills and people who have diabetes, smoking poses an even greater risk to the heart and blood vessels.
Smoking is a major risk factor for heart disease. When combined with other risk factorssuch as unhealthy blood cholesterol levels, high blood pressure, and overweight or obesitysmoking further raises the risk of heart disease.
Smoking and the Risk of Peripheral Artery Disease
Peripheral artery disease (P.A.D.) is a disease in which plaque builds up in the arteries that carry blood to your head, organs, and limbs. Smoking is a major risk factor for P.A.D.
P.A.D. usually affects the arteries that carry blood to your legs. Blocked blood flow in the leg arteries can cause cramping, pain, weakness, and numbness in your hips, thighs, and calf muscles.
Blocked blood flow also can raise your risk of getting an infection in the affected limb. Your body might have a hard time fighting the infection.
If severe enough, blocked blood flow can cause gangrene (tissue death). In very serious cases, this can lead to leg amputation.
If you have P.A.D., your risk of heart disease and heart attack is higher than the risk for people who dont have P.A.D.
Smoking even one or two cigarettes a day can interfere with P.A.D. treatments. People who smoke and people who have diabetes are at highest risk for P.A.D. complications, including gangrene in the leg from decreased blood flow.
Secondhand Smoke Risks
Secondhand smoke is the smoke that comes from the burning end of a cigarette, cigar, or pipe. Secondhand smoke also refers to smoke thats breathed out by a person who is smoking.
Secondhand smoke contains many of the same harmful chemicals that people inhale when they smoke. It can damage the heart and blood vessels of people who dont smoke in the same way that active smoking harms people who do smoke. Secondhand smoke greatly increases adults risk of heart attack and death.
Secondhand smoke also raises the risk of future coronary heart disease in children and teens because it:
Damages heart tissues
Lowers HDL cholesterol
Raises blood pressure
The risks of secondhand smoke are especially high for premature babies who have respiratory distress syndrome and children who have conditions such asasthma.
Cigar and Pipe Smoke Risks
Researchers know less about how cigar and pipe smoke affects the heart and blood vessels than they do about cigarette smoke.
However, the smoke from cigars and pipes contains the same harmful chemicals as the smoke from cigarettes. Also, studies have shown that people who smoke cigars are at increased risk of heart disease.
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Smoking and Your Heart
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What is (are) Angina ?
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Espaol
Angina (an-JI-nuh or AN-juh-nuh) is chest pain or discomfort that occurs if an area of your heart muscle doesn't get enough oxygen-rich blood.
Angina may feel like pressure or squeezing in your chest. The pain also can occur in your shoulders, arms, neck, jaw, or back. Angina pain may even feel like indigestion.
Angina isn't a disease; it's a symptom of an underlying heart problem. Angina usually is a symptom of coronary heart disease (CHD).
CHD is the most common type of heart disease in adults. It occurs if a waxy substance called plaque (plak) builds up on the inner walls of your coronary arteries. These arteries carry oxygen-rich blood to your heart.
Plaque Buildup in an Artery
Plaque narrows and stiffens the coronary arteries. This reduces the flow of oxygen-rich blood to the heart muscle, causing chest pain. Plaque buildup also makes it more likely that blood clots will form in your arteries. Blood clots can partially or completely block blood flow, which can cause a heart attack.
Angina also can be a symptom of coronary microvascular disease (MVD). This is heart disease that affects the hearts smallest coronary arteries. In coronary MVD, plaque doesn't create blockages in the arteries like it does in CHD.
Studies have shown that coronary MVD is more likely to affect women than men. Coronary MVD also is called cardiac syndrome X and nonobstructive CHD.
Types of Angina
The major types of angina are stable, unstable, variant (Prinzmetal's), and microvascular. Knowing how the types differ is important. This is because they have different symptoms and require different treatments.
Stable Angina
Stable angina is the most common type of angina. It occurs when the heart is working harder than usual. Stable angina has a regular pattern. (Pattern refers to how often the angina occurs, how severe it is, and what factors trigger it.)
If you have stable angina, you can learn its pattern and predict when the pain will occur. The pain usually goes away a few minutes after you rest or take your angina medicine.
Stable angina isn't a heart attack, but it suggests that a heart attack is more likely to happen in the future.
Unstable Angina
Unstable angina doesn't follow a pattern. It may occur more often and be more severe than stable angina. Unstable angina also can occur with or without physical exertion, and rest or medicine may not relieve the pain.
Unstable angina is very dangerous and requires emergency treatment. This type of angina is a sign that a heart attack may happen soon.
Variant (Prinzmetal's) Angina
Variant angina is rare. A spasm in a coronary artery causes this type of angina. Variant angina usually occurs while you're at rest, and the pain can be severe. It usually happens between midnight and early morning. Medicine can relieve this type of angina.
Microvascular Angina
Microvascular angina can be more severe and last longer than other types of angina. Medicine may not relieve this type of angina.
Overview
Experts believe that nearly 7 million people in the United States suffer from angina. The condition occurs equally among men and women.
Angina can be a sign of CHD, even if initial tests don't point to the disease. However, not all chest pain or discomfort is a sign of CHD.
Other conditions also can cause chest pain, such as:
Pulmonary embolism (a blockage in a lung artery)
A lung infection
Aortic dissection (tearing of a major artery)
Aortic stenosis (narrowing of the hearts aortic valve)
Hypertrophic cardiomyopathy (KAR-de-o-mi-OP-ah-thee; heart muscle disease)
Pericarditis (inflammation in the tissues that surround the heart)
A panic attack
All chest pain should be checked by a doctor.
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Angina
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What causes Angina ?
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Underlying Causes
Angina usually is a symptom of coronary heart disease (CHD). This means that the underlying causes of angina generally are the same as the underlying causes of CHD.
Research suggests that CHD starts when certain factors damage the inner layers of the coronary arteries. These factors include:
Smoking
High amounts of certain fats and cholesterol in the blood
High blood pressure
High amounts of sugar in the blood due to insulin resistance or diabetes
Plaque may begin to build up where the arteries are damaged. When plaque builds up in the arteries, the condition is called atherosclerosis (ath-er-o-skler-O-sis).
Plaque narrows or blocks the arteries, reducing blood flow to the heart muscle. Some plaque is hard and stable and causes the arteries to become narrow and stiff. This can greatly reduce blood flow to the heart and cause angina.
Other plaque is soft and more likely to rupture (break open) and cause blood clots. Blood clots can partially or totally block the coronary arteries and cause angina or a heart attack.
Immediate Causes
Many factors can trigger angina pain, depending on the type of angina you have.
Stable Angina
Physical exertion is the most common trigger of stable angina. Severely narrowed arteries may allow enough blood to reach the heart when the demand for oxygen is low, such as when you're sitting.
However, with physical exertionlike walking up a hill or climbing stairsthe heart works harder and needs more oxygen.
Other triggers of stable angina include:
Emotional stress
Exposure to very hot or cold temperatures
Heavy meals
Smoking
Unstable Angina
Blood clots that partially or totally block an artery cause unstable angina.
If plaque in an artery ruptures, blood clots may form. This creates a blockage. A clot may grow large enough to completely block the artery and cause a heart attack. For more information, go to the animation in "What Causes a Heart Attack?"
Blood clots may form, partially dissolve, and later form again. Angina can occur each time a clot blocks an artery.
Variant Angina
A spasm in a coronary artery causes variant angina. The spasm causes the walls of the artery to tighten and narrow. Blood flow to the heart slows or stops. Variant angina can occur in people who have CHD and in those who dont.
The coronary arteries can spasm as a result of:
Exposure to cold
Emotional stress
Medicines that tighten or narrow blood vessels
Smoking
Cocaine use
Microvascular Angina
This type of angina may be a symptom of coronary microvascular disease (MVD). Coronary MVD is heart disease that affects the hearts smallest coronary arteries.
Reduced blood flow in the small coronary arteries may cause microvascular angina. Plaque in the arteries, artery spasms, or damaged or diseased artery walls can reduce blood flow through the small coronary arteries.
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Angina
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Who is at risk for Angina? ?
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Angina is a symptom of an underlying heart problem. Its usually a symptom of coronary heart disease (CHD), but it also can be a symptom of coronary microvascular disease (MVD). So, if youre at risk for CHD or coronary MVD, youre also at risk for angina.
The major risk factors for CHD and coronary MVD include:
Unhealthy cholesterol levels.
High blood pressure.
Smoking.
Insulin resistance or diabetes.
Overweight or obesity.
Metabolic syndrome.
Lack of physical activity.
Unhealthy diet.
Older age. (The risk increases for men after 45 years of age and for women after 55 years of age.)
Family history of early heart disease.
For more detailed information about CHD and coronary MVD risk factors, visit the Diseases and Conditions Index Coronary Heart Disease, Coronary Heart Disease Risk Factors, and Coronary Microvascular Disease articles.
People sometimes think that because men have more heart attacks than women, men also suffer from angina more often. In fact, overall, angina occurs equally among men and women.
Microvascular angina, however, occurs more often in women. About 70 percent of the cases of microvascular angina occur in women around the time of menopause.
Unstable angina occurs more often in older adults. Variant angina is rare; it accounts for only about 2 out of 100 cases of angina. People who have variant angina often are younger than those who have other forms of angina.
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Angina
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What are the symptoms of Angina ?
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Pain and discomfort are the main symptoms of angina. Angina often is described as pressure, squeezing, burning, or tightness in the chest. The pain or discomfort usually starts behind the breastbone.
Pain from angina also can occur in the arms, shoulders, neck, jaw, throat, or back. The pain may feel like indigestion. Some people say that angina pain is hard to describe or that they can't tell exactly where the pain is coming from.
Signs and symptoms such as nausea (feeling sick to your stomach), fatigue (tiredness), shortness of breath, sweating, light-headedness, and weakness also may occur.
Women are more likely to feel discomfort in the neck, jaw, throat, abdomen, or back. Shortness of breath is more common in older people and those who have diabetes. Weakness, dizziness, and confusion can mask the signs and symptoms of angina in elderly people.
Symptoms also vary based on the type of angina you have.
Because angina has so many possible symptoms and causes, all chest pain should be checked by a doctor. Chest pain that lasts longer than a few minutes and isn't relieved by rest or angina medicine may be a sign of a heart attack. Call 911 right away.
Stable Angina
The pain or discomfort:
Occurs when the heart must work harder, usually during physical exertion
Doesn't come as a surprise, and episodes of pain tend to be alike
Usually lasts a short time (5 minutes or less)
Is relieved by rest or medicine
May feel like gas or indigestion
May feel like chest pain that spreads to the arms, back, or other areas
Unstable Angina
The pain or discomfort:
Often occurs at rest, while sleeping at night, or with little physical exertion
Comes as a surprise
Is more severe and lasts longer than stable angina (as long as 30 minutes)
Usually isnt relieved by rest or medicine
May get worse over time
May mean that a heart attack will happen soon
Variant Angina
The pain or discomfort:
Usually occurs at rest and during the night or early morning hours
Tends to be severe
Is relieved by medicine
Microvascular Angina
The pain or discomfort:
May be more severe and last longer than other types of angina pain
May occur with shortness of breath, sleep problems, fatigue, and lack of energy
Often is first noticed during routine daily activities and times of mental stress
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Angina
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How to diagnose Angina ?
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The most important issues to address when you go to the doctor with chest pain are:
What's causing the chest pain
Whether you're having or are about to have a heart attack
Angina is a symptom of an underlying heart problem, usually coronary heart disease (CHD). The type of angina pain you have can be a sign of how severe the CHD is and whether it's likely to cause a heart attack.
If you have chest pain, your doctor will want to find out whether it's angina. He or she also will want to know whether the angina is stable or unstable. If it's unstable, you may need emergency medical treatment to try to prevent a heart attack.
To diagnose chest pain as stable or unstable angina, your doctor will do a physical exam, ask about your symptoms, and ask about your risk factors for and your family history of CHD or other heart diseases.
Your doctor also may ask questions about your symptoms, such as:
What brings on the pain or discomfort and what relieves it?
What does the pain or discomfort feel like (for example, heaviness or tightness)?
How often does the pain occur?
Where do you feel the pain or discomfort?
How severe is the pain or discomfort?
How long does the pain or discomfort last?
Diagnostic Tests and Procedures
If your doctor thinks that you have unstable angina or that your angina is related to a serious heart condition, he or she may recommend one or more tests.
EKG (Electrocardiogram)
An EKG is a simple, painless test that detects and records the hearts electrical activity. The test shows how fast the heart is beating and its rhythm (steady or irregular). An EKG also records the strength and timing of electrical signals as they pass through the heart.
An EKG can show signs of heart damage due to CHD and signs of a previous or current heart attack. However, some people who have angina have normal EKGs.
Stress Testing
During stress testing, you exercise to make your heart work hard and beat fast while heart tests are done. If you cant exercise, you may be given medicine to make your heart work hard and beat fast.
When your heart is working hard and beating fast, it needs more blood and oxygen. Plaque-narrowed arteries can't supply enough oxygen-rich blood to meet your heart's needs.
A stress test can show possible signs and symptoms of CHD, such as:
Abnormal changes in your heart rate or blood pressure
Shortness of breath or chest pain
Abnormal changes in your heart rhythm or your heart's electrical activity
As part of some stress tests, pictures are taken of your heart while you exercise and while you rest. These imaging stress tests can show how well blood is flowing in various parts of your heart. They also can show how well your heart pumps blood when it beats.
Chest X Ray
A chest x ray takes pictures of the organs and structures inside your chest, such as your heart, lungs, and blood vessels.
A chest x ray can reveal signs of heart failure. It also can show signs of lung disorders and other causes of symptoms not related to CHD. However, a chest x ray alone is not enough to diagnose angina or CHD.
Coronary Angiography and Cardiac Catheterization
Your doctor may recommend coronary angiography (an-jee-OG-ra-fee) if he or she suspects you have CHD. This test uses dye and special x rays to show the inside of your coronary arteries.
To get the dye into your coronary arteries, your doctor will use a procedure called cardiac catheterization (KATH-e-ter-ih-ZA-shun).
A thin, flexible tube called a catheter is put into a blood vessel in your arm, groin (upper thigh), or neck. The tube is threaded into your coronary arteries, and the dye is released into your bloodstream.
Special x rays are taken while the dye is flowing through your coronary arteries. The dye lets your doctor study the flow of blood through your heart and blood vessels.
Cardiac catheterization usually is done in a hospital. You're awake during the procedure. It usually causes little or no pain, although you may feel some soreness in the blood vessel where your doctor inserts the catheter.
Computed Tomography Angiography
Computed tomography (to-MOG-rah-fee) angiography (CTA) uses dye and special x rays to show blood flow through the coronary arteries. This test is less invasive than coronary angiography with cardiac catheterization.
For CTA, a needle connected to an intravenous (IV) line is put into a vein in your hand or arm. Dye is injected through the IV line during the scan. You may have a warm feeling when this happens. The dye highlights your blood vessels on the CT scan pictures.
Sticky patches called electrodes are put on your chest. The patches are attached to an EKG machine to record your heart's electrical activity during the scan.
The CT scanner is a large machine that has a hollow, circular tube in the middle. You lie on your back on a sliding table. The table slowly slides into the opening of the machine.
Inside the scanner, an x-ray tube moves around your body to take pictures of different parts of your heart. A computer puts the pictures together to make a three-dimensional (3D) picture of the whole heart.
Blood Tests
Blood tests check the levels of certain fats, cholesterol, sugar, and proteins in your blood. Abnormal levels may show that you have risk factors for CHD.
Your doctor may recommend a blood test to check the level of a protein called C-reactive protein (CRP) in your blood. Some studies suggest that high levels of CRP in the blood may increase the risk for CHD and heart attack.
Your doctor also may recommend a blood test to check for low levels of hemoglobin (HEE-muh-glow-bin) in your blood. Hemoglobin is an iron-rich protein in red blood cells. It helps the blood cells carry oxygen from the lungs to all parts of your body. If your hemoglobin level is low, you may have a condition called anemia (uh-NEE-me-uh).
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Angina
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What are the treatments for Angina ?
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Treatments for angina include lifestyle changes, medicines, medical procedures, cardiac rehabilitation (rehab), and other therapies. The main goals of treatment are to:
Reduce pain and discomfort and how often it occurs
Prevent or lower your risk for heart attack and death by treating your underlying heart condition
Lifestyle changes and medicines may be the only treatments needed if your symptoms are mild and aren't getting worse. If lifestyle changes and medicines don't control angina, you may need medical procedures or cardiac rehab.
Unstable angina is an emergency condition that requires treatment in a hospital.
Lifestyle Changes
Making lifestyle changes can help prevent episodes of angina. You can:
Slow down or take rest breaks if physical exertion triggers angina.
Avoid large meals and rich foods that leave you feeling stuffed if heavy meals trigger angina.
Try to avoid situations that make you upset or stressed if emotional stress triggers angina. Learn ways to handle stress that can't be avoided.
You also can make lifestyle changes that help lower your risk for coronary heart disease. One of the most important changes is to quit smoking. Smoking can damage and tighten blood vessels and raise your risk for CHD. Talk with your doctor about programs and products that can help you quit. Also, try to avoid secondhand smoke.
If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking.
For more information about how to quit smoking, go to the Diseases and Conditions Index (DCI) Smoking and Your Heart article and the National Heart, Lung, and Blood Institutes (NHLBIs) "Your Guide to a Healthy Heart."
Following a healthy diet is another important lifestyle change. A healthy diet can prevent or reduce high blood pressure and high blood cholesterol and help you maintain a healthy weight.
A healthy diet includes a variety of fruits and vegetables (including beans and peas). It also includes whole grains, lean meats, poultry without skin, seafood, and fat-free or low-fat milk and dairy products. A healthy diet also is low in sodium (salt), added sugars, solid fats, and refined grains.
For more information about following a healthy diet, go to the NHLBIs Your Guide to Lowering Your Blood Pressure With DASH and the U.S. Department of Agricultures ChooseMyPlate.gov Web site. Both resources provide general information about healthy eating.
Other important lifestyle changes include:
Being physically active. Check with your doctor to find out how much and what kinds of activity are safe for you. For more information, go to the DCI Physical Activity and Your Heart article.
Maintaining a healthy weight. If youre overweight or obese, work with your doctor to create a reasonable weight-loss plan. Controlling your weight helps you control CHD risk factors.
Taking all medicines as your doctor prescribes, especially if you have diabetes.
Medicines
Nitrates are the medicines most commonly used to treat angina. They relax and widen blood vessels. This allows more blood to flow to the heart, while reducing the hearts workload.
Nitroglycerin (NI-tro-GLIS-er-in) is the most commonly used nitrate for angina. Nitroglycerin that dissolves under your tongue or between your cheek and gum is used to relieve angina episodes.
Nitroglycerin pills and skin patches are used to prevent angina episodes. However, pills and skin patches act too slowly to relieve pain during an angina attack.
Other medicines also are used to treat angina, such as beta blockers, calcium channel blockers, ACE inhibitors, oral antiplatelet medicines, or anticoagulants (blood thinners). These medicines can help:
Lower blood pressure and cholesterol levels
Slow the heart rate
Relax blood vessels
Reduce strain on the heart
Prevent blood clots from forming
People who have stable angina may be advised to get annual flu shots.
Medical Procedures
If lifestyle changes and medicines don't control angina, you may need a medical procedure to treat the underlying heart disease. Both angioplasty (AN-jee-oh-plas-tee) and coronary artery bypass grafting (CABG) are commonly used to treat heart disease.
Angioplasty opens blocked or narrowed coronary arteries. During angioplasty, a thin tube with a balloon or other device on the end is threaded through a blood vessel to the narrowed or blocked coronary artery.
Once in place, the balloon is inflated to push the plaque outward against the wall of the artery. This widens the artery and restores blood flow.
Angioplasty can improve blood flow to your heart and relieve chest pain. A small mesh tube called a stent usually is placed in the artery to help keep it open after the procedure.
During CABG, healthy arteries or veins taken from other areas in your body are used to bypass (that is, go around) your narrowed coronary arteries. Bypass surgery can improve blood flow to your heart, relieve chest pain, and possibly prevent a heart attack.
You will work with your doctor to decide which treatment is better for you.
Cardiac Rehabilitation
Your doctor may recommend cardiac rehab for angina or after angioplasty, CABG, or a heart attack. Cardiac rehab is a medically supervised program that can help improve the health and well-being of people who have heart problems.
The cardiac rehab team may include doctors, nurses, exercise specialists, physical and occupational therapists, dietitians or nutritionists, and psychologists or other mental health specialists.
Rehab has two parts:
Exercise training. This part helps you learn how to exercise safely, strengthen your muscles, and improve your stamina. Your exercise plan will be based on your personal abilities, needs, and interests.
Education, counseling, and training. This part of rehab helps you understand your heart condition and find ways to reduce your risk for future heart problems. The rehab team will help you learn how to adjust to a new lifestyle and deal with your fears about the future.
For more information about cardiac rehab, go to the DCI Cardiac Rehabilitation article.
Enhanced External Counterpulsation Therapy
Enhanced external counterpulsation (EECP) therapy is helpful for some people who have angina. Large cuffs, similar to blood pressure cuffs, are put on your legs. The cuffs are inflated and deflated in sync with your heartbeat.
EECP therapy improves the flow of oxygen-rich blood to your heart muscle and helps relieve angina. You typically get 35 1-hour treatments over 7 weeks.
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Angina
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How to prevent Angina ?
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You can prevent or lower your risk for angina and heart disease by making lifestyle changes and treating related conditions.
Making Lifestyle Changes
Healthy lifestyle choices can help prevent or delay angina and heart disease. To adopt a healthy lifestyle, you can:
Quit smoking and avoid secondhand smoke
Avoid angina triggers
Follow a healthy diet
Be physically active
Maintain a healthy weight
Learn ways to handle stress and relax
Take your medicines as your doctor prescribes
For more information about these lifestyle changes, go to How Is Angina Treated? For more information about preventing and controlling heart disease risk factors, visit the Diseases and Conditions Index Coronary Heart Disease, Coronary Heart Disease Risk Factors, and Coronary Microvascular Disease articles.
Treating Related Conditions
You also can help prevent or delay angina and heart disease by treating related conditions, such as high blood cholesterol, high blood pressure, diabetes, and overweight or obesity.
If you have one or more of these conditions, talk with your doctor about how to control them. Follow your treatment plan and take all of your medicines as your doctor prescribes.
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Angina
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What is (are) Heart Failure ?
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Heart failure is a condition in which the heart can't pump enough blood to meet the body's needs. In some cases, the heart can't fill with enough blood. In other cases, the heart can't pump blood to the rest of the body with enough force. Some people have both problems.
The term "heart failure" doesn't mean that your heart has stopped or is about to stop working. However, heart failure is a serious condition that requires medical care.
Overview
Heart failure develops over time as the heart's pumping action grows weaker. The condition can affect the right side of the heart only, or it can affect both sides of the heart. Most cases involve both sides of the heart.
Right-side heart failure occurs if the heart can't pump enough blood to the lungs to pick up oxygen. Left-side heart failure occurs if the heart can't pump enough oxygen-rich blood to the rest of the body.
Right-side heart failure may cause fluid to build up in the feet, ankles, legs, liver, abdomen, and the veins in the neck. Right-side and left-side heart failure also may cause shortness of breath and fatigue (tiredness).
The leading causes of heart failure are diseases that damage the heart. Examples include coronary heart disease (CHD), high blood pressure, and diabetes.
Outlook
Heart failure is a very common condition. About 5.7 million people in the United States have heart failure.Both children and adults can have the condition, although the symptoms and treatments differ. The Health Topicfocuses on heart failure in adults.
Currently, heart failure has no cure. However, treatmentssuch as medicines and lifestyle changescan help people who have the condition live longer and more active lives. Researchers continue to study new ways to treat heart failure and its complications.
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Heart Failure
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What causes Heart Failure ?
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Conditions that damage or overwork the heart muscle can cause heart failure. Over time, the heart weakens. It isnt able to fill with and/or pump blood as well as it should. As the heart weakens, certain proteins and substances might be released into the blood. These substances have a toxic effect on the heart and blood flow, and they worsen heart failure.
Causes of heart failure include:
Coronary heart disease
Diabetes
High blood pressure
Other heart conditions or diseases
Other factors
Coronary Heart Disease
Coronary heart disease is a condition in which a waxy substance called plaque builds up inside the coronary arteries. These arteries supply oxygen-rich blood to your heart muscle.
Plaque narrows the arteries and reduces blood flow to your heart muscle. The buildup of plaque also makes it more likely that blood clots will form in your arteries. Blood clots can partially or completely block blood flow. Coronary heart disease can lead to chest pain or discomfort calledangina, aheart attack, and heart damage.
Diabetes
Diabetes is a disease in which the bodys blood glucose (sugar) level is too high. The body normally breaks down food into glucose and then carries it to cells throughout the body. The cells use a hormone called insulin to turn the glucose into energy.
In diabetes, the body doesnt make enough insulin or doesnt use its insulin properly. Over time, high blood sugar levels can damage and weaken the heart muscle and the blood vessels around the heart, leading to heart failure.
High Blood Pressure
Blood pressure is the force of blood pushing against the walls of the arteries. If this pressure rises and stays high over time, it can weaken your heart and lead to plaque buildup.
Blood pressure is considered high if it stays at or above 140/90 mmHg over time. (The mmHg is millimeters of mercurythe units used to measure blood pressure.) If you have diabetes or chronic kidney disease, high blood pressure is defined as 130/80 mmHg or higher.
Other Heart Conditions or Diseases
Other conditions and diseases also can lead to heart failure, such as:
Arrhythmia. Happens when a problem occurs with the rate or rhythm of the heartbeat.
Cardiomyopathy.Happens when the heart muscle becomes enlarged, thick, or rigid.
Congenital heart defects. Problems with the hearts structure are present at birth.
Heart valve disease. Occurs if one or more of your heart valves doesnt work properly, which can be present at birth or caused by infection, other heart conditions, and age.
Other Factors
Other factors also can injure the heart muscle and lead to heart failure. Examples include:
Alcohol abuse or cocaine and other illegal drug use
HIV/AIDS
Thyroid disorders (having either too much or too little thyroid hormone in the body)
Too much vitamin E
Treatments for cancer, such as radiation and chemotherapy
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Heart Failure
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Who is at risk for Heart Failure? ?
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About 5.7 million people in the United States have heart failure. The number of people who have this condition is growing.
Heart failure is more common in:
People who are age 65 or older. Aging can weaken the heart muscle. Older people also may have had diseases for many years that led to heart failure. Heart failure is a leading cause of hospital stays among people on Medicare.
Blacks are more likely to have heart failure than people of other races. Theyre also more likely to have symptoms at a younger age, have more hospital visits due to heart failure, and die from heart failure.
People who areoverweight. Excess weight puts strain on the heart. Being overweight also increases your risk of heart disease and type 2diabetes. These diseases can lead to heart failure.
People who have had a heart attack. Damage to the heart muscle from a heart attack and can weaken the heart muscle.
Children who havecongenital heart defectsalso can develop heart failure. These defects occur if the heart, heart valves, or blood vessels near the heart dont form correctly while a baby is in the womb. Congenital heart defects can make the heart work harder. This weakens the heart muscle, which can lead to heart failure. Children dont have the same symptoms of heart failure or get the same treatments as adults. This Health Topic focuses on heart failure in adults.
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Heart Failure
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What are the symptoms of Heart Failure ?
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The most common signs and symptoms of heart failure are:
Shortness of breath or trouble breathing
Fatigue (tiredness)
Swelling in the ankles, feet, legs, abdomen, and veins in the neck
All of these symptoms are the result of fluid buildup in your body. When symptoms start, you may feel tired and short of breath after routine physical effort, like climbing stairs.
As your heart grows weaker, symptoms get worse. You may begin to feel tired and short of breath after getting dressed or walking across the room. Some people have shortness of breath while lying flat.
Fluid buildup from heart failure also causes weight gain, frequent urination, and a cough that's worse at night and when you're lying down. This cough may be a sign of acute pulmonary edema (e-DE-ma). This is a condition in which too much fluid builds up in your lungs. The condition requires emergency treatment.
Heart Failure Signs and Symptoms
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Heart Failure
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How to diagnose Heart Failure ?
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Your doctor will diagnose heart failure based on your medical and family histories, a physical exam, and test results. The signs and symptoms of heart failure also are common in other conditions. Thus, your doctor will:
Find out whether you have a disease or condition that can cause heart failure, such as coronary heart disease (CHD), high blood pressure, or diabetes
Rule out other causes of your symptoms
Find any damage to your heart and check how well your heart pumps blood
Early diagnosis and treatment can help people who have heart failure live longer, more active lives.
Medical and Family Histories
Your doctor will ask whether you or others in your family have or have had a disease or condition that can cause heart failure.
Your doctor also will ask about your symptoms. He or she will want to know which symptoms you have, when they occur, how long you've had them, and how severe they are. Your answers will help show whether and how much your symptoms limit your daily routine.
Physical Exam
During the physical exam, your doctor will:
Listen to your heart for sounds that aren't normal
Listen to your lungs for the sounds of extra fluid buildup
Look for swelling in your ankles, feet, legs, abdomen, and the veins in your neck
Diagnostic Tests
No single test can diagnose heart failure. If you have signs and symptoms of heart failure, your doctor may recommend one or more tests.
Your doctor also may refer you to a cardiologist. A cardiologist is a doctor who specializes in diagnosing and treating heart diseases and conditions.
EKG (Electrocardiogram)
An EKG is a simple, painless test that detects and records the heart's electrical activity. The test shows how fast your heart is beating and its rhythm (steady or irregular). An EKG also records the strength and timing of electrical signals as they pass through your heart.
An EKG may show whether the walls in your heart's pumping chambers are thicker than normal. Thicker walls can make it harder for your heart to pump blood. An EKG also can show signs of a previous or current heart attack.
Chest X Ray
A chest x raytakes pictures of the structures inside your chest, such as your heart, lungs, and blood vessels. This test can show whether your heart is enlarged, you have fluid in your lungs, or you have lung disease.
BNP Blood Test
This test checks the level of a hormone in your blood called BNP. The level of this hormone rises during heart failure.
Echocardiography
Echocardiography (echo) uses sound waves to create a moving picture of your heart. The test shows the size and shape of your heart and how well your heart chambers and valves work.
Echo also can identify areas of poor blood flow to the heart, areas of heart muscle that aren't contracting normally, and heart muscle damage caused by lack of blood flow.
Echo might be done before and after a stress test (see below). A stress echo can show how well blood is flowing through your heart. The test also can show how well your heart pumps blood when it beats.
Doppler Ultrasound
A Doppler ultrasound uses sound waves to measure the speed and direction of blood flow. This test often is done with echo to give a more complete picture of blood flow to the heart and lungs.
Doctors often use Doppler ultrasound to help diagnose right-side heart failure.
Holter Monitor
A Holter monitor records your heart's electrical activity for a full 24- or 48-hour period, while you go about your normal daily routine.
You wear small patches called electrodes on your chest. Wires connect the patches to a small, portable recorder. The recorder can be clipped to a belt, kept in a pocket, or hung around your neck.
Nuclear Heart Scan
A nuclear heart scan shows how well blood is flowing through your heart and how much blood is reaching your heart muscle.
During a nuclear heart scan, a safe, radioactive substance called a tracer is injected into your bloodstream through a vein. The tracer travels to your heart and releases energy. Special cameras outside of your body detect the energy and use it to create pictures of your heart.
A nuclear heart scan can show where the heart muscle is healthy and where it's damaged.
A positron emission tomography (PET) scan is a type of nuclear heart scan. It shows the level of chemical activity in areas of your heart. This test can help your doctor see whether enough blood is flowing to these areas. A PET scan can show blood flow problems that other tests might not detect.
Cardiac Catheterization
During cardiac catheterization (KATH-eh-ter-ih-ZA-shun), a long, thin, flexible tube called a catheter is put into a blood vessel in your arm, groin (upper thigh), or neck and threaded to your heart. This allows your doctor to look inside your coronary (heart) arteries.
During this procedure, your doctor can check the pressure and blood flow in your heart chambers, collect blood samples, and use x rays to look at your coronary arteries.
Coronary Angiography
Coronary angiography (an-jee-OG-rah-fee) usually is done with cardiac catheterization. A dye that can be seen on x ray is injected into your bloodstream through the tip of the catheter.
The dye allows your doctor to see the flow of blood to your heart muscle. Angiography also shows how well your heart is pumping.
Stress Test
Some heart problems are easier to diagnose when your heart is working hard and beating fast. During stress testing, you exercise to make your heart work hard and beat fast.
You may walk or run on a treadmill or pedal a bicycle. If you can't exercise, you may be given medicine to raise your heart rate.
Heart tests, such as nuclear heart scanning and echo, often are done during stress testing.
Cardiac MRI
Cardiac MRI (magnetic resonance imaging) uses radio waves, magnets, and a computer to create pictures of your heart as it's beating. The test produces both still and moving pictures of your heart and major blood vessels.
A cardiac MRI can show whether parts of your heart are damaged. Doctors also have used MRI in research studies to find early signs of heart failure, even before symptoms appear.
Thyroid Function Tests
Thyroid function tests show how well your thyroid gland is working. These tests include blood tests, imaging tests, and tests to stimulate the thyroid. Having too much or too little thyroid hormone in the blood can lead to heart failure.
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Heart Failure
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What are the treatments for Heart Failure ?
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Early diagnosis and treatment can help people who have heart failure live longer, more active lives. Treatment for heart failure depends on the type and severity of the heart failure.
The goals of treatment for all stages of heart failure include:
Treating the conditions underlying cause, such ascoronary heart disease,high blood pressure, ordiabetes
Reducing symptoms
Stopping the heart failure from getting worse
Increasing your lifespan and improving your quality of life
Treatments usually include lifestyle changes, medicines, and ongoing care. If you have severe heart failure, you also may need medical procedures or surgery.
Heart-Healthy Lifestyle Changes
Your doctor may recommend heart-healthy lifestyle changes if you have heart failure. Heart-healthy lifestyle changes include:
Heart-healthy eating
Maintaining a healthy weight
Physical activity
Quitting smoking
Heart-Healthy Eating
Your doctor may recommend a heart-healthy eating plan, which should include:
Fat-free or low-fat dairy products, such as skim milk
Fish high in omega-3 fatty acids, such as salmon, tuna, and trout, about twice a week
Fruits, such as apples, bananas, oranges, pears, and prunes
Legumes, such as kidney beans, lentils, chickpeas, black-eyed peas, and lima beans
Vegetables, such as broccoli, cabbage, and carrots
Whole grains, such as oatmeal, brown rice, and corn tortillas
When following a heart-healthy diet, you should avoid eating:
A lot of red meat
Palm and coconut oils
Sugary foods and beverages
Two nutrients in your diet make blood cholesterol levels rise:
Saturated fatfound mostly in foods that come from animals
Trans fat (trans fatty acids)found in foods made with hydrogenated oils and fats, such as stick margarine; baked goods, such as cookies, cakes, and pies; crackers; frostings; and coffee creamers. Some trans fats also occur naturally in animal fats andmeats.
Saturated fat raises your blood cholesterol more than anything else in your diet. When you follow a heart-healthy eating plan, only 5 percent to 6 percent of your daily calories should come from saturated fat. Food labels list the amounts of saturated fat. To help you stay on track, here are some examples:
If you eat:
Try to eat no more than:
1,200 calories a day
8 grams of saturated fat a day
1,500 calories a day
10 grams of saturated fat a day
1,800 calories a day
12 grams of saturated fat a day
2,000 calories a day
13 grams of saturated fat a day
2,500 calories a day
17 grams of saturated fat a day
Not all fats are bad. Monounsaturated and polyunsaturated fats actually help lower blood cholesterollevels.
Some sources of monounsaturated and polyunsaturated fats are:
Avocados
Corn, sunflower, and soybean oils
Nuts and seeds, such as walnuts
Olive, canola, peanut, safflower, and sesame oils
Peanut butter
Salmon and trout
Tofu
Sodium
You should try to limit the amount of sodium that you eat. This means choosing and preparing foods that are lower in salt and sodium. Try to use low-sodium and no added salt foods and seasonings at the table or while cooking. Food labels tell you what you need to know about choosing foods that are lower in sodium. Try to eat no more than 2,300 milligrams of sodium a day. If you have high blood pressure, you may need to restrict your sodium intake even more.
Dietary Approaches to Stop Hypertension
Your doctor may recommend the Dietary Approaches to Stop Hypertension (DASH) eating plan if you have high blood pressure. The DASH eating plan focuses on fruits, vegetables, whole grains, and other foods that are heart healthy and low in fat, cholesterol, and sodium and salt.
The DASH eating plan is a good heart-healthy eating plan, even for those who dont have high blood pressure. Read more about DASH.
Liquid Intake
Its important for people who have heart failure to take in the correct amounts and types of liquids. Consuming too much liquid can worsen heart failure. Also, if you have heart failure, you shouldnt drink alcohol. Talk with your doctor about what amounts and types of liquids you should have each day.
Maintaining a Healthy Weight
Maintaining a healthy weight is important for overall health and can lower your risk for heart failure and coronary heart disease. Aim for a Healthy Weight by following a heart-healthy eating plan and keeping physically active.
Knowing your body mass index (BMI) helps you find out if youre a healthy weight in relation to your height and gives an estimate of your total body fat. To figure out your BMI, check out the National Heart, Lung, and Blood Institutes (NHLBI) online BMI calculator or talk to your doctor. A BMI:
Below 18.5 is a sign that you are underweight.
Between 18.5 and 24.9 is in the normal range.
Between 25 and 29.9 is considered overweight.
Of 30 or more is considered obese.
A general goal to aim for is a BMI below 25. Your doctor or health care provider can help you set an appropriate BMI goal.
Measuring waist circumference helps screen for possible health risks. If most of your fat is around your waist rather than at your hips, youre at a higher risk for heart disease and type 2 diabetes. This risk may be higher with a waist size that is greater than 35 inches for women or greater than 40 inches for men. To learn how to measure your waist, visit Assessing Your Weight and Health Risk.
If youre overweight or obese, try to lose weight. A loss of just 3 percent to 5 percent of your current weight can lower your triglycerides, blood glucose, and the risk of developing type 2 diabetes. Greater amounts of weight loss can improve blood pressure readings, lower LDL cholesterol, and increase HDL cholesterol.
Physical Activity
Routine physical activity can lower many coronary heart disease risk factors, including LDL (bad) cholesterol, high blood pressure, and excess weight. Physical activity also can lower your risk for diabetes and raise your HDL cholesterol level. HDL is the good cholesterol that helps prevent coronary heart disease.
Everyone should try to participate in moderate-intensity aerobic exercise at least 2hours and 30minutes per week, or vigorous aerobic exercise for 1hour and 15minutes per week. Aerobic exercise, such as brisk walking, is any exercise in which your heart beats faster and you use more oxygen than usual. The more active you are, the more you will benefit. Participate in aerobic exercise for at least 10minutes at a time spread throughout the week.
Read more about physical activity at:
Physical Activity and Your Heart
U.S. Department of Health and Human Services 2008 Physical Activity Guidelines forAmericans
Talk with your doctor before you start a new exercise plan. Ask your doctor how much and what kinds of physical activity are safe for you.
Quitting Smoking
If you smoke, quit. Smoking can raise your risk for coronary heart disease and heart attack and worsen heart failure. Talk with your doctor about programs and products that can help you quit smoking. Also, try to avoid secondhand smoke.
If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking.
Read more about quitting smoking at Smoking and Your Heart.
Medicines
Your doctor will prescribe medicines based on the type of heart failure you have, how severe it is, and your response to certain medicines. The following medicines are commonly used to treat heart failure:
ACE inhibitors lower blood pressure and reduce strain on your heart. They also may reduce the risk of a futureheart attack.
Aldosterone antagonists trigger the body to remove excess sodium through urine. This lowers the volume of blood that the heart must pump.
Angiotensin receptor blockers relax your blood vessels and lower blood pressure to decrease your hearts workload.
Beta blockers slow your heart rate and lower your blood pressure to decrease your hearts workload.
Digoxin makes the heart beat stronger and pump more blood.
Diuretics (fluid pills) help reduce fluid buildup in your lungs and swelling in your feet and ankles.
Isosorbide dinitrate/hydralazine hydrochloride helps relax your blood vessels so your heart doesnt work as hard to pump blood. Studies have shown that this medicine can reduce the risk of death in blacks. More studies are needed to find out whether this medicine will benefit other racial groups.
Take all medicines regularly, as your doctor prescribes. Dont change the amount of your medicine or skip a dose unless your doctor tells you to. You should still follow a heart healthy lifestyle, even if you take medicines to treat your heart failure.
Ongoing Care
You should watch for signs that heart failure is getting worse. For example, weight gain may mean that fluids are building up in your body. Ask your doctor how often you should check your weight and when to report weight changes.
Getting medical care for other related conditions is important. If you have diabetes or high blood pressure, work with your health care team to control these conditions. Have your blood sugar level and blood pressure checked. Talk with your doctor about when you should have tests and how often to take measurements at home.
Try to avoid respiratory infections like the flu andpneumonia. Talk with your doctor or nurse about getting flu and pneumonia vaccines.
Many people who have severe heart failure may need treatment in a hospital from time to time. Your doctor may recommend oxygen therapy, which can be given in a hospital or at home.
Medical Procedures and Surgery
As heart failure worsens, lifestyle changes and medicines may no longer control your symptoms. You may need a medical procedure or surgery.
In heart failure, the right and left sides of the heart may no longer contract at the same time. This disrupts the hearts pumping. To correct this problem, your doctor might implant a cardiac resynchronization therapy device (a type ofpacemaker) near your heart. This device helps both sides of your heart contract at the same time, which can decrease heart failure symptoms.
Some people who have heart failure have very rapid, irregular heartbeats. Without treatment, these heartbeats can causesudden cardiac arrest. Your doctor might implant an implantable cardioverter defibrillator (ICD) near your heart to solve this problem. An ICD checks your heart rate and uses electrical pulses to correct irregular heart rhythms.
People who have severe heart failure symptoms at rest, despite other treatments, may need:
A mechanical heart pump, such as aleft ventricular assist device. This device helps pump blood from the heart to the rest of the body. You may use a heart pump until you have surgery or as a long-term treatment.
Heart transplant. A heart transplant is an operation in which a persons diseased heart is replaced with a healthy heart from a deceased donor. Heart transplants are done as a life-saving measure for end-stage heart failure when medical treatment and less drastic surgery have failed.
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Heart Failure
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How to prevent Heart Failure ?
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You can take steps to prevent heart failure. The sooner you start, the better your chances of preventing or delaying the condition.
For People Who Have Healthy Hearts
If you have a healthy heart, you can take action to prevent heart disease and heart failure. To reduce your risk of heart disease:
Avoid using illegal drugs.
Be physically active. The more active you are, the more you will benefit.
Follow a heart-healthy eating plan.
If yousmoke, make an effort to quit. Talk with your doctor about programs and products that can help you quit smoking. Also, try to avoid secondhand smoke.
Maintain a healthy weight. Work with your health care team to create a reasonable weight-loss plan.
For People Who Are at High Risk for Heart Failure
Even if youre at high risk for heart failure, you can take steps to reduce your risk. People at high risk include those who have coronary heart disease,high blood pressure, ordiabetes.
Follow all of the steps listed above. Talk with your doctor about what types and amounts of physical activity are safe for you.
Treat and control any conditions that can cause heart failure. Take medicines as your doctor prescribes.
Avoid drinking alcohol.
See your doctor for ongoing care.
For People Who Have Heart Damage but No Signs of Heart Failure
If you have heart damage but no signs of heart failure, you can still reduce your risk of developing the condition. In addition to the steps above, take your medicines as prescribed to reduce your hearts workload.
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Heart Failure
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What is (are) Thrombocythemia and Thrombocytosis ?
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Thrombocythemia (THROM-bo-si-THE-me-ah) and thrombocytosis (THROM-bo-si-TO-sis) are conditions in which your blood has a higher than normal number of platelets (PLATE-lets).
Platelets are blood cell fragments. They're made in your bone marrow along with other kinds of blood cells.
Platelets travel through your blood vessels and stick together (clot). Clotting helps stop any bleeding that may occur if a blood vessel is damaged. Platelets also are called thrombocytes (THROM-bo-sites) because a blood clot also is called a thrombus.
A normal platelet count ranges from 150,000 to 450,000 platelets per microliter of blood.
Overview
The term "thrombocythemia" is preferred when the cause of a high platelet count isn't known. The condition sometimes is called primary or essential thrombocythemia.
This condition occurs if faulty cells in the bone marrow make too many platelets. Bone marrow is the sponge-like tissue inside the bones. It contains stem cells that develop into red blood cells, white blood cells, and platelets. What causes the bone marrow to make too many platelets often isn't known.
With primary thrombocythemia, a high platelet count may occur alone or with other blood cell disorders. This condition isn't common.
When another disease or condition causes a high platelet count, the term "thrombocytosis" is preferred. This condition often is called secondary or reactive thrombocytosis. Secondary thrombocytosis is more common than primary thrombocythemia.
Often, a high platelet count doesn't cause signs or symptoms. Rarely, serious or life-threatening symptoms can develop, such as blood clots and bleeding. These symptoms are more likely to occur in people who have primary thrombocythemia.
Outlook
People who have primary thrombocythemia with no signs or symptoms don't need treatment, as long as the condition remains stable.
Other people who have this condition may need medicines or procedures to treat it. Most people who have primary thrombocythemia will live a normal lifespan.
Treatment and outlook for secondary thrombocytosis depend on its underlying cause.
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Thrombocythemia and Thrombocytosis
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What causes Thrombocythemia and Thrombocytosis ?
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Primary Thrombocythemia
In this condition, faulty stem cells in the bone marrow make too many platelets. What causes this to happen usually isn't known. When this process occurs without other blood cell disorders, it's called essential thrombocythemia.
A rare form of thrombocythemia is inherited. ("Inherited" means the condition is passed from parents to children through the genes.) In some cases, a genetic mutation may cause the condition.
In addition to the bone marrow making too many platelets, the platelets also are abnormal in primary thrombocythemia. They may form blood clots or, surprisingly, cause bleeding when they don't work well.
Bleeding also can occur because of a condition that develops called von Willebrand disease. This condition affects the blood clotting process.
After many years, scarring of the bone marrow can occur.
Secondary Thrombocytosis
This condition occurs if another disease, condition, or outside factor causes the platelet count to rise. For example, 35 percent of people who have high platelet counts also have cancermostly lung, gastrointestinal, breast, ovarian, and lymphoma. Sometimes a high platelet count is the first sign of cancer.
Other conditions or factors that can cause a high platelet count are:
Iron-deficiency anemia (uh-NEE-me-uh)
Hemolytic (HEE-moh-lit-ick) anemia
Absence of a spleen (after surgery to remove the organ)
Inflammatory or infectious diseases, such as connective tissue disorders, inflammatory bowel disease, and tuberculosis
Reactions to medicine
Some conditions can lead to a high platelet count that lasts for only a short time. Examples of such conditions include:
Recovery from serious blood loss
Recovery from a very low platelet count caused by excessive alcohol use and lack of vitamin B12 or folate
Acute (short-term) infection or inflammation
Response to physical activity
Although the platelet count is high in secondary thrombocytosis, the platelets are normal (unlike in primary thrombocythemia). Thus, people who have secondary thrombocytosis have a lower risk of blood clots and bleeding.
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Thrombocythemia and Thrombocytosis
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Who is at risk for Thrombocythemia and Thrombocytosis? ?
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Primary Thrombocythemia
Thrombocythemia isn't common. The exact number of people who have the condition isn't known. Some estimates suggest that 24 out of every 100,000 people have primary thrombocythemia.
Primary thrombocythemia is more common in people aged 50 to 70, but it can occur at any age. For unknown reasons, more women around the age of 30 have primary thrombocythemia than men of the same age.
Secondary Thrombocytosis
You might be at risk for secondary thrombocytosis if you have a disease, condition, or factor that can cause it. (For more information, go to "What Causes Thrombocythemia and Thrombocytosis?")
Secondary thrombocytosis is more common than primary thrombocythemia. Studies have shown that most people who have platelet counts over 500,000 have secondary thrombocytosis.
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Thrombocythemia and Thrombocytosis
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What are the symptoms of Thrombocythemia and Thrombocytosis ?
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People who have thrombocythemia or thrombocytosis may not have signs or symptoms. These conditions might be discovered only after routine blood tests.
However, people who have primary thrombocythemia are more likely than those who have secondary thrombocytosis to have serious signs and symptoms.
The signs and symptoms of a high platelet count are linked to blood clots and bleeding. They include weakness, bleeding, headache, dizziness, chest pain, and tingling in the hands and feet.
Blood Clots
In primary thrombocythemia, blood clots most often develop in the brain, hands, and feet. But they can develop anywhere in the body, including in the heart and intestines.
Blood clots in the brain may cause symptoms such as chronic (ongoing) headache and dizziness. In extreme cases, stroke may occur.
Blood clots in the tiny blood vessels of the hands and feet leave them numb and red. This may lead to an intense burning and throbbing pain felt mainly on the palms of the hands and the soles of the feet.
Other signs and symptoms of blood clots may include:
Changes in speech or awareness, ranging from confusion to passing out
Seizures
Upper body discomfort in one or both arms, the back, neck, jaw, or abdomen
Shortness of breath and nausea (feeling sick to your stomach)
In pregnant women, blood clots in the placenta can cause miscarriage or problems with fetal growth and development.
Women who have primary thrombocythemia or secondary thrombocytosis and take birth control pills are at increased risk for blood clots.
Blood clots are related to other conditions and factors as well. Older age, prior blood clots, diabetes, high blood pressure, and smoking also increase your risk for blood clots.
Bleeding
If bleeding occurs, it most often affects people who have platelet counts higher than 1million platelets per microliter of blood. Signs of bleeding include nosebleeds, bruising, bleeding from the mouth or gums, or blood in the stools.
Although bleeding usually is associated with a low platelet count, it also can occur in people who have high platelet counts. Blood clots that develop in thrombocythemia or thrombocytosis may use up your body's platelets. This means that not enough platelets are left in your bloodstream to seal off cuts or breaks on the blood vessel walls.
Another cause of bleeding in people who have very high platelets counts is a condition called von Willebrand Disease. This condition affects the blood clotting process.
In rare cases of primary thrombocythemia, the faulty bone marrow cells will cause a form of leukemia (lu-KE-me-ah). Leukemia is a cancer of the blood cells.
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Thrombocythemia and Thrombocytosis
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How to diagnose Thrombocythemia and Thrombocytosis ?
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Your doctor will diagnose thrombocythemia or thrombocytosis based on your medical history, a physical exam, and test results. A hematologist also may be involved in your care. This is a doctor who specializes in blood diseases and conditions.
Medical History
Your doctor may ask you about factors that can affect your platelets, such as:
Any medical procedures or blood transfusions you've had
Any recent infections or vaccines you've had
The medicines you take, including over-the-counter medicines
Your general eating habits, including the amount of alcohol you normally drink
Any family history of high platelet counts
Physical Exam
Your doctor will do a physical exam to look for signs and symptoms of blood clots and bleeding. He or she also will check for signs of conditions that can cause secondary thrombocytosis, such as an infection.
Primary thrombocythemia is diagnosed only after all possible causes of a high platelet count are ruled out. For example, your doctor may recommend tests to check for early, undiagnosed cancer. If another disease, condition, or factor is causing a high platelet count, the diagnosis is secondary thrombocytosis.
Diagnostic Tests
Your doctor may recommend one or more of the following tests to help diagnose a high platelet count.
Complete Blood Count
A complete blood count (CBC) measures the levels of red blood cells, white blood cells, and platelets in your blood. For this test, a small amount of blood is drawn from a blood vessel, usually in your arm.
If you have thrombocythemia or thrombocytosis, the CBC results will show that your platelet count is high.
Blood Smear
A blood smear is used to check the condition of your platelets. For this test, a small amount of blood is drawn from a blood vessel, usually in your arm. Some of your blood is put on a glass slide. A microscope is then used to look at your platelets.
Bone Marrow Tests
Bone marrow tests check whether your bone marrow is healthy. Blood cells, including platelets, are made in the bone marrow. The two bone marrow tests are aspiration (as-pih-RA-shun) and biopsy.
Bone marrow aspiration might be done to find out whether your bone marrow is making too many platelets. For this test, your doctor removes a sample of fluid bone marrow through a needle. He or she examines the sample under a microscope to check for faulty cells.
A bone marrow biopsy often is done right after an aspiration. For this test, your doctor removes a small amount of bone marrow tissue through a needle. He or she examines the tissue to check the number and types of cells in the bone marrow.
With thrombocythemia and thrombocytosis, the bone marrow has a higher than normal number of the very large cells that make platelets.
Other Tests
Your doctor may recommend other blood tests to look for genetic factors that can cause a high platelet count.
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Thrombocythemia and Thrombocytosis
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What are the treatments for Thrombocythemia and Thrombocytosis ?
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Primary Thrombocythemia
This condition is considered less harmful today than in the past, and its outlook often is good. People who have no signs or symptoms don't need treatment, as long as the condition remains stable.
Taking aspirin may help people who are at risk for blood clots (aspirin thins the blood). However, talk with your doctor about using aspirin because it can cause bleeding.
Doctors prescribe aspirin to most pregnant women who have primary thrombocythemia. This is because it doesn't have a high risk of side effects for the fetus.
Some people who have primary thrombocythemia may need medicines or medical procedures to lower their platelet counts.
Medicines To Lower Platelet Counts
You may need medicines to lower your platelet count if you:
Have a history of blood clots or bleeding
Have risk factors for heart disease, such as high blood cholesterol, high blood pressure, or diabetes
Are older than 60
Have a platelet count over 1 million
You'll need to take these medicines throughout your life.
Hydroxyurea. This platelet-lowering medicine is used to treat cancers and other life-threatening diseases. Hydroxyurea most often is given under the care of doctors who specialize in cancer or blood diseases. Patients on hydroxyurea are closely monitored.
Currently, hydroxyurea plus aspirin is the standard treatment for people who have primary thrombocythemia and are at high risk for blood clots.
Anagrelide. This medicine also has been used to lower platelet counts in people who have thrombocythemia. However, research shows that when compared with hydroxyurea, anagrelide has worse outcomes. Anagrelide also has side effects, such as fluid retention, palpitations (pal-pih-TA-shuns), arrhythmias (ah-RITH-me-ahs), heart failure, and headaches.
Interferon alfa. This medicine lowers platelet counts, but 20 percent of patients can't handle its side effects. Side effects include a flu-like feeling, decreased appetite, nausea (feeling sick to the stomach), diarrhea, seizures, irritability, and sleepiness.
Doctors may prescribe this medicine to pregnant women who have primary thrombocythemia because it's safer for a fetus than hydroxyurea and anagrelide.
Plateletpheresis
Plateletpheresis (PLATE-let-fe-REH-sis) is a procedure used to rapidly lower your platelet count. This procedure is used only for emergencies. For example, if you're having a stroke due to primary thrombocythemia, you may need plateletpheresis.
An intravenous (IV) needle that's connected to a tube is placed in one of your blood vessels to remove blood. The blood goes through a machine that removes platelets from the blood. The remaining blood is then put back into you through an IV line in one of your blood vessels.
One or two procedures might be enough to reduce your platelet count to a safe level.
Secondary Thrombocytosis
Secondary thrombocytosis is treated by addressing the condition that's causing it.
People who have secondary thrombocytosis usually don't need platelet-lowering medicines or procedures. This is because their platelets usually are normal (unlike in primary thrombocythemia).
Also, secondary thrombocytosis is less likely than primary thrombocythemia to cause serious problems related to blood clots and bleeding.
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Thrombocythemia and Thrombocytosis
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How to prevent Thrombocythemia and Thrombocytosis ?
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You can't prevent primary thrombocythemia. However, you can take steps to reduce your risk for complications. For example, you can control many of the risk factors for blood clots, such as high blood cholesterol, high blood pressure, diabetes, and smoking.
To reduce your risk, quit smoking, adopt healthy lifestyle habits, and work with your doctor to manage your risk factors.
It's not always possible to prevent conditions that lead to secondary thrombocytosis. But, if you have routine medical care, your doctor may detect these conditions before you develop a high platelet count.
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Thrombocythemia and Thrombocytosis
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What is (are) Thrombotic Thrombocytopenic Purpura ?
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Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder. In TTP, blood clots form in small blood vessels throughout the body.
The clots can limit or block the flow of oxygen-rich blood to the body's organs, such as the brain, kidneys, and heart. As a result, serious health problems can develop.
The increased clotting that occurs in TTP also uses up platelets (PLATE-lets) in the blood. Platelets are blood cell fragments that help form blood clots. These cell fragments stick together to seal small cuts and breaks on blood vessel walls and stop bleeding.
With fewer platelets available in the blood, bleeding problems can occur. People who have TTP may bleed inside their bodies, underneath the skin, or from the surface of the skin. When cut or injured, they also may bleed longer than normal.
"Thrombotic" (throm-BOT-ik) refers to the blood clots that form. "Thrombocytopenic" (throm-bo-cy-toe-PEE-nick) means the blood has a lower than normal number of platelets. "Purpura" (PURR-purr-ah) refers to purple bruises caused by bleeding under the skin.
Bleeding under the skin also can cause tiny red or purple dots on the skin. These pinpoint-sized dots are called petechiae (peh-TEE-kee-ay). Petechiae may look like a rash.
Purpura and Petechiae
TTP also can cause red blood cells to break apart faster than the body can replace them. This leads to hemolytic anemia (HEE-moh-lit-ick uh-NEE-me-uh)a rare form of anemia. Anemia is a condition in which the body has a lower than normal number of red blood cells.
A lack of activity in the ADAMTS13 enzyme (a type of protein in the blood) causes TTP. The ADAMTS13 gene controls the enzyme, which is involved in blood clotting. The enzyme breaks up a large protein called von Willebrand factor that clumps together with platelets to form blood clots.
Types of Thrombotic Thrombocytopenic Purpura
The two main types of TTP are inherited and acquired. "Inherited" means the condition is passed from parents to children through genes. This type of TTP mainly affects newborns and children.
In inherited TTP, the ADAMTS13 gene is faulty and doesn't prompt the body to make a normal ADAMTS13 enzyme. As a result, enzyme activity is lacking or changed.
Acquired TTP is the more common type of the disorder. "Acquired" means you aren't born with the disorder, but you develop it. This type of TTP mostly occurs in adults, but it can affect children.
In acquired TTP, the ADAMTS13 gene isn't faulty. Instead, the body makes antibodies (proteins) that block the activity of the ADAMTS13 enzyme.
It's not clear what triggers inherited and acquired TTP, but some factors may play a role. These factors may include:
Some diseases and conditions, such as pregnancy, cancer, HIV, lupus, and infections
Some medical procedures, such as surgery and blood and marrow stem cell transplant
Some medicines, such as chemotherapy, ticlopidine, clopidogrel, cyclosporine A, and hormone therapy and estrogens
Quinine, which is a substance often found in tonic water and nutritional health products
If you have TTP, you may sometimes hear it referred to as TTPHUS. HUS, or hemolytic-uremic syndrome, is a disorder that resembles TTP, but is more common in children. Kidney problems also tend to be worse in HUS. Although some researchers think TTP and HUS are two forms of a single syndrome, recent evidence suggests that each has different causes.
Outlook
TTP is a rare disorder. It can be fatal or cause lasting damage, such as brain damage or a stroke, if it's not treated right away.
TTP usually occurs suddenly and lasts for days or weeks, but it can continue for months. Relapses (or flareups) can occur in up to 60 percent of people who have the acquired type of TTP. Many people who have inherited TTP have frequent flareups that need to be treated.
Treatments for TTP include infusions of fresh frozen plasma and plasma exchange, also called plasmapheresis (PLAZ-ma-feh-RE-sis). These treatments have greatly improved the outlook of the disorder.
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Thrombotic Thrombocytopenic Purpura
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What causes Thrombotic Thrombocytopenic Purpura ?
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A lack of activity in the ADAMTS13 enzyme (a type of protein in the blood) causes thrombotic thrombocytopenic purpura (TTP). The ADAMTS13 gene controls the enzyme, which is involved in blood clotting.
Not having enough enzyme activity causes overactive blood clotting. In TTP, blood clots form in small blood vessels throughout the body. These clots can limit or block the flow of oxygen-rich blood to the body's organs, such as the brain, kidneys, and heart. As a result, serious health problems can develop.
The increased clotting that occurs in TTP also uses up many of the blood's platelets. With fewer platelets available in the blood, bleeding problems can occur.
People who have TTP may bleed inside their bodies, underneath the skin, or from the surface of the skin. When cut or injured, they also may bleed longer than normal.
TTP also can cause red blood cells to break apart faster than the body can replace them. This leads to hemolytic anemia.
Inherited Thrombotic Thrombocytopenic Purpura
In inherited TTP, the ADAMTS13 gene is faulty. It doesn't prompt the body to make a normal ADAMTS13 enzyme. As a result, enzyme activity is lacking or changed.
"Inherited" means that the condition is passed from parents to children through genes. A person who inherits TTP is born with two copies of the faulty geneone from each parent.
Most often, the parents each have one copy of the faulty gene, but have no signs or symptoms TTP.
Acquired Thrombotic Thrombocytopenic Purpura
In acquired TTP, the ADAMTS13 gene isn't faulty. Instead, the body makes antibodies (proteins) that block the activity of the ADAMTS13 enzyme.
"Acquired" means you aren't born with the condition, but you develop it sometime after birth.
Triggers for Thrombotic Thrombocytopenic Purpura
It's unclear what triggers inherited and acquired TTP, but some factors may play a role. These factors may include:
Some diseases and conditions, such as pregnancy, cancer, HIV, lupus, and infections
Some medical procedures, such as surgery and blood and marrow stem cell transplant
Some medicines, such as chemotherapy, ticlopidine, clopidogrel, cyclosporine A, and hormone therapy and estrogens
Quinine, which is a substance often found in tonic water and nutritional health products
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Thrombotic Thrombocytopenic Purpura
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Who is at risk for Thrombotic Thrombocytopenic Purpura? ?
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Thrombotic thrombocytopenic purpura (TTP) is a rare disorder. Most cases of TTP are acquired. Acquired TTP mostly occurs in adults, but it can affect children. The condition occurs more often in women and in Black people than in other groups.
Inherited TTP mainly affects newborns and children. Most people who have inherited TTP begin to have symptoms soon after birth. Some, however, don't have symptoms until they're adults.
It isn't clear what triggers inherited and acquired TTP, but some factors may play a role. These factors may include:
Some diseases and conditions, such as pregnancy, cancer, HIV, lupus, and infections
Some medical procedures, such as surgery and blood and marrow stem cell transplant
Some medicines, such as chemotherapy, ticlopidine, clopidogrel, cyclosporine A, and hormone therapy and estrogens
Quinine, which is a substance often found in tonic water and nutritional health products
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Thrombotic Thrombocytopenic Purpura
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What are the symptoms of Thrombotic Thrombocytopenic Purpura ?
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Blood clots, a low platelet count, and damaged red blood cells cause the signs and symptoms of thrombotic thrombocytopenic purpura (TTP).
The signs and symptoms include:
Purplish bruises on the skin or mucous membranes (such as in the mouth). These bruises, called purpura, are caused by bleeding under the skin.
Pinpoint-sized red or purple dots on the skin. These dots, called petechiae, often are found in groups and may look like a rash. Bleeding under the skin causes petechiae.
Paleness or jaundice (a yellowish color of the skin or whites of the eyes).
Fatigue (feeling very tired and weak).
Fever.
A fast heart rate or shortness of breath.
Headache, speech changes, confusion, coma, stroke, or seizure.
A low amount of urine, or protein or blood in the urine.
If you've had TTP and have any of these signs or symptoms, you may be having a relapse (flareup). Ask your doctor when to call him or her or seek emergency care.
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Thrombotic Thrombocytopenic Purpura
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How to diagnose Thrombotic Thrombocytopenic Purpura ?
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Your doctor will diagnosis thrombotic thrombocytopenic purpura (TTP) based on your medical history, a physical exam, and test results.
If TTP is suspected or diagnosed, a hematologist will be involved in your care. A hematologist is a doctor who specializes in diagnosing and treating blood disorders.
Medical History
Your doctor will ask about factors that may affect TTP. For example, he or she may ask whether you:
Have certain diseases or conditions, such as cancer, HIV, lupus, or infections (or whether you're pregnant).
Have had previous medical procedures, such as a blood and marrow stem cell transplant.
Take certain medicines, such as ticlopidine, clopidogrel, cyclosporine A, or hormone therapy and estrogens, or whether you've had chemotherapy.
Have used any products that contain quinine. Quinine is a substance often found in tonic water and nutritional health products.
Physical Exam
As part of the medical history and physical exam, your doctor will ask about any signs or symptoms you've had. He or she will look for signs such as:
Bruising and bleeding under your skin
Fever
Paleness or jaundice (a yellowish color of the skin or whites of the eyes)
A fast heart rate
Speech changes or changes in awareness that can range from confusion to passing out
Changes in urine
Diagnostic Tests
Your doctor also may recommend tests to help find out whether you have TTP.
Complete Blood Count
This test measures the number of red blood cells, white blood cells, and platelets in your blood. For this test, a sample of blood is drawn from a vein, usually in your arm.
If you have TTP, you'll have a lower than normal number of platelets and red blood cells (anemia).
Blood Smear
For this test, a sample of blood is drawn from a vein, usually in your arm. Some of your blood is put on a glass slide. A microscope is then used to look at your red blood cells. In TTP, the red blood cells are torn and broken.
Platelet Count
This test counts the number of platelets in a blood smear. People who have TTP have a lower than normal number of platelets in their blood. This test is used with the blood smear to help diagnose TTP.
Bilirubin Test
When red blood cells die, they release a protein called hemoglobin (HEE-muh-glow-bin) into the bloodstream. The body breaks down hemoglobin into a compound called bilirubin. High levels of bilirubin in the bloodstream cause jaundice.
For this blood test, a sample of blood is drawn from a vein, usually in your arm. The level of bilirubin in the sample is checked. If you have TTP, your bilirubin level may be high because your body is breaking down red blood cells faster than normal.
Kidney Function Tests and Urine Tests
These tests show whether your kidneys are working well. If you have TTP, your urine may contain protein or blood cells. Also, your blood creatinine (kre-AT-ih-neen) level may be high. Creatinine is a blood product that's normally removed by the kidneys.
Coombs Test
This blood test is used to find out whether TTP is the cause of hemolytic anemia. For this test, a sample of blood is drawn from a vein, usually in your arm.
In TTP, hemolytic anemia occurs because red blood cells are broken into pieces as they try to squeeze around blood clots.
When TTP is the cause of hemolytic anemia, the Coombs test is negative. The test is positive if antibodies (proteins) are destroying your red blood cells.
Lactate Dehydrogenase Test
This blood test measures a protein called lactate dehydrogenase (LDH). For this test, a sample of blood is drawn from a vein, usually in your arm.
Hemolytic anemia causes red blood cells to break down and release LDH into the blood. LDH also is released from tissues that are injured by blood clots as a result of TTP.
ADAMTS13 Assay
A lack of activity in the ADAMTS13 enzyme causes TTP. For this test, a sample of blood is drawn from a vein, usually in your arm. The blood is sent to a special lab to test for the enzyme's activity.
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Thrombotic Thrombocytopenic Purpura
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What are the treatments for Thrombotic Thrombocytopenic Purpura ?
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Thrombotic thrombocytopenic purpura (TTP) can be fatal or cause lasting damage, such as brain damage or a stroke, if it's not treated right away.
In most cases, TTP occurs suddenly and lasts for days or weeks, but it can go on for months. Relapses (flareups) can occur in up to 60 percent of people who have acquired TTP. Flareups also occur in most people who have inherited TTP.
Plasma treatments are the most common way to treat TTP. Other treatments include medicines and surgery. Treatments are done in a hospital.
Plasma Therapy
Plasma is the liquid part of your blood. It carries blood cells, hormones, enzymes, and nutrients to your body.
TTP is treated with plasma therapy. This includes:
Fresh frozen plasma for people who have inherited TTP
Plasma exchange for people who have acquired TTP
Plasma therapy is started in the hospital as soon as TTP is diagnosed or suspected.
For inherited TTP, fresh frozen plasma is given through an intravenous (IV) line inserted into a vein. This is done to replace the missing or changed ADAMTS13 enzyme.
Plasma exchange (also called plasmapheresis) is used to treat acquired TTP. This is a lifesaving procedure. It removes antibodies (proteins) from the blood that damage your ADAMTS13 enzyme. Plasma exchange also replaces the ADAMTS13 enzyme.
If plasma exchange isn't available, you may be given fresh frozen plasma until it is available.
During plasma exchange, an IV needle or tube is placed in a vein in your arm to remove blood. The blood goes through a cell separator, which removes plasma from the blood. The nonplasma part of the blood is saved, and donated plasma is added to it.
Then, the blood is put back into you through an IV line inserted into one of your blood vessels. The time required to complete the procedure varies, but it often takes about 2hours.
Treatments of fresh frozen plasma or plasma exchange usually continue until your blood tests results and signs and symptoms improve. This can take days or weeks, depending on your condition. You'll stay in the hospital while you recover.
Some people who recover from TTP have flareups. This can happen in the hospital or after you go home. If you have a flareup, your doctor will restart plasma therapy.
Other Treatments
Other treatments are used if plasma therapy doesn't work well or if flareups occur often.
For acquired TTP, medicines can slow or stop antibodies to the ADAMTS13 enzyme from forming. Medicines used to treat TTP include glucocorticoids, vincristine, rituximab, and cyclosporine A.
Sometimes surgery to remove the spleen (an organ in the abdomen) is needed. This is because cells in the spleen make the antibodies that block ADAMTS13 enzyme activity.
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Thrombotic Thrombocytopenic Purpura
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How to prevent Thrombotic Thrombocytopenic Purpura ?
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Both inherited and acquired thrombotic thrombocytopenic purpura (TTP) occur suddenly with no clear cause. You can't prevent either type.
If you've had TTP, watch for signs and symptoms of a relapse (flareup). (For more information, go to "Living With Thrombotic Thrombocytopenic Purpura.")
Ask your doctor about factors that may trigger TTP or a flareup, including:
Some diseases or conditions, such as pregnancy, cancer, HIV, lupus, or infections.
Some medical procedures, such as surgery and blood and marrow stem cell transplant.
Some medicines, such as ticlopidine, clopidogrel, cyclosporine A, chemotherapy, and hormone therapy and estrogens. If you take any of these medicines, your doctor may prescribe a different medicine.
Quinine, which is a substance often found in tonic water and nutritional health products.
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Thrombotic Thrombocytopenic Purpura
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What is (are) Atherosclerosis ?
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Espaol
Atherosclerosis is a disease in which plaque builds up inside your arteries. Arteries are blood vessels that carry oxygen-rich blood to your heart and other parts of your body.
Plaque is made up of fat, cholesterol, calcium, and other substances found in the blood. Over time, plaque hardens and narrows your arteries. This limits the flow of oxygen-rich blood to your organs and other parts of your body.
Atherosclerosis can lead to serious problems, including heart attack, stroke, or even death.
Atherosclerosis
Atherosclerosis-Related Diseases
Atherosclerosis can affect any artery in the body, including arteries in the heart, brain, arms, legs, pelvis, and kidneys. As a result, different diseases may develop based on which arteries are affected.
Coronary Heart Disease
Coronary heart disease (CHD), also called coronary artery disease, occurs when plaque builds up in the coronary arteries. These arteries supply oxygen-rich blood to your heart.
Plaque narrows the coronary arteries and reduces blood flow to your heart muscle. Plaque buildup also makes it more likely that blood clots will form in your arteries. Blood clots can partially or completely block blood flow.
If blood flow to your heart muscle is reduced or blocked, you may have angina (chest pain or discomfort) or a heart attack.
Plaque also can form in the heart's smallest arteries. This disease is called coronary microvascular disease (MVD). In coronary MVD, plaque doesn't cause blockages in the arteries as it does in CHD.
Carotid Artery Disease
Carotid (ka-ROT-id) artery disease occurs if plaque builds up in the arteries on each side of your neck (the carotid arteries). These arteries supply oxygen-rich blood to your brain. If blood flow to your brain is reduced or blocked, you may have a stroke.
Peripheral Artery Disease
Peripheral artery disease (P.A.D.) occurs if plaque builds up in the major arteries that supply oxygen-rich blood to your legs, arms, and pelvis.
If blood flow to these parts of your body is reduced or blocked, you may have numbness, pain, and, sometimes, dangerous infections.
Chronic Kidney Disease
Chronic kidney disease can occur if plaque builds up in the renal arteries. These arteries supply oxygen-rich blood to your kidneys.
Over time, chronic kidney disease causes a slow loss of kidney function. The main function of the kidneys is to remove waste and extra water from the body.
Overview
The cause of atherosclerosis isn't known. However, certain traits, conditions, or habits may raise your risk for the disease. These conditions are known as risk factors.
You can control some risk factors, such as lack of physical activity, smoking, and an unhealthy diet. Others you can't control, such as age and a family history of heart disease.
Some people who have atherosclerosis have no signs or symptoms. They may not be diagnosed until after a heart attack or stroke.
The main treatment for atherosclerosis is lifestyle changes. You also may need medicines and medical procedures. These treatments, along with ongoing medical care, can help you live a healthier life.
Outlook
Improved treatments have reduced the number of deaths from atherosclerosis-related diseases. These treatments also have improved the quality of life for people who have these diseases. However, atherosclerosis remains a common health problem.
You may be able to prevent or delay atherosclerosis and the diseases it can cause. Making lifestyle changes and getting ongoing care can help you avoid the problems of atherosclerosis and live a long, healthy life.
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Atherosclerosis
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What causes Atherosclerosis ?
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The exact cause of atherosclerosis isn't known. However, studies show that atherosclerosis is a slow, complex disease that may start in childhood. It develops faster as you age.
Atherosclerosis may start when certain factors damage the inner layers of the arteries. These factors include:
Smoking
High amounts of certain fats and cholesterol in the blood
High blood pressure
High amounts of sugar in the blood due to insulin resistance or diabetes
Plaque may begin to build up where the arteries are damaged. Over time, plaque hardens and narrows the arteries. Eventually, an area of plaque can rupture (break open).
When this happens, blood cell fragments called platelets (PLATE-lets) stick to the site of the injury. They may clump together to form blood clots. Clots narrow the arteries even more, limiting the flow of oxygen-rich blood to your body.
Depending on which arteries are affected, blood clots can worsen angina (chest pain) or cause a heart attack or stroke.
Researchers continue to look for the causes of atherosclerosis. They hope to find answers to questions such as:
Why and how do the arteries become damaged?
How does plaque develop and change over time?
Why does plaque rupture and lead to blood clots?
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Atherosclerosis
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Who is at risk for Atherosclerosis? ?
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The exact cause of atherosclerosis isn't known. However, certain traits, conditions, or habits may raise your risk for the disease. These conditions are known as risk factors. The more risk factors you have, the more likely it is that you'll develop atherosclerosis.
You can control most risk factors and help prevent or delay atherosclerosis. Other risk factors can't be controlled.
Major Risk Factors
Unhealthy blood cholesterol levels. This includes high LDL cholesterol (sometimes called "bad" cholesterol) and low HDL cholesterol (sometimes called "good" cholesterol).
High blood pressure. Blood pressure is considered high if it stays at or above 140/90 mmHg over time. If you have diabetes or chronic kidney disease, high blood pressure is defined as 130/80 mmHg or higher. (The mmHg is millimeters of mercurythe units used to measure blood pressure.)
Smoking. Smoking can damage and tighten blood vessels, raise cholesterol levels, and raise blood pressure. Smoking also doesn't allow enough oxygen to reach the body's tissues.
Insulin resistance. This condition occurs if the body can't use its insulin properly. Insulin is a hormone that helps move blood sugar into cells where it's used as an energy source. Insulin resistance may lead to diabetes.
Diabetes. With this disease, the body's blood sugar level is too high because the body doesn't make enough insulin or doesn't use its insulin properly.
Overweight or obesity. The terms "overweight" and "obesity" refer to body weight that's greater than what is considered healthy for a certain height.
Lack of physical activity. A lack of physical activity can worsen other risk factors for atherosclerosis, such as unhealthy blood cholesterol levels, high blood pressure, diabetes, and overweight and obesity.
Unhealthy diet. An unhealthy diet can raise your risk for atherosclerosis. Foods that are high in saturated and trans fats, cholesterol, sodium (salt), and sugar can worsen other atherosclerosis risk factors.
Older age. As you get older, your risk for atherosclerosis increases. Genetic or lifestyle factors cause plaque to build up in your arteries as you age. By the time you're middle-aged or older, enough plaque has built up to cause signs or symptoms. In men, the risk increases after age 45. In women, the risk increases after age 55.
Family history of early heart disease. Your risk for atherosclerosis increases if your father or a brother was diagnosed with heart disease before 55 years of age, or if your mother or a sister was diagnosed with heart disease before 65 years of age.
Although age and a family history of early heart disease are risk factors, it doesn't mean that you'll develop atherosclerosis if you have one or both. Controlling other risk factors often can lessen genetic influences and prevent atherosclerosis, even in older adults.
Studies show that an increasing number of children and youth are at risk for atherosclerosis. This is due to a number of causes, including rising childhood obesity rates.
Emerging Risk Factors
Scientists continue to study other possible risk factors for atherosclerosis.
High levels of a protein called C-reactive protein (CRP) in the blood may raise the risk for atherosclerosis and heart attack. High levels of CRP are a sign of inflammation in the body.
Inflammation is the body's response to injury or infection. Damage to the arteries' inner walls seems to trigger inflammation and help plaque grow.
People who have low CRP levels may develop atherosclerosis at a slower rate than people who have high CRP levels. Research is under way to find out whether reducing inflammation and lowering CRP levels also can reduce the risk for atherosclerosis.
High levels of triglycerides (tri-GLIH-seh-rides) in the blood also may raise the risk for atherosclerosis, especially in women. Triglycerides are a type of fat.
Studies are under way to find out whether genetics may play a role in atherosclerosis risk.
Other Factors That Affect Atherosclerosis
Other factors also may raise your risk for atherosclerosis, such as:
Sleep apnea. Sleep apnea is a disorder that causes one or more pauses in breathing or shallow breaths while you sleep. Untreated sleep apnea can raise your risk for high blood pressure, diabetes, and even a heart attack or stroke.
Stress. Research shows that the most commonly reported "trigger" for a heart attack is an emotionally upsetting event, especially one involving anger.
Alcohol. Heavy drinking can damage the heart muscle and worsen other risk factors for atherosclerosis. Men should have no more than two drinks containing alcohol a day. Women should have no more than one drink containing alcohol a day.
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Atherosclerosis
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What are the symptoms of Atherosclerosis ?
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Atherosclerosis usually doesn't cause signs and symptoms until it severely narrows or totally blocks an artery. Many people don't know they have the disease until they have a medical emergency, such as a heart attack or stroke.
Some people may have signs and symptoms of the disease. Signs and symptoms will depend on which arteries are affected.
Coronary Arteries
The coronary arteries supply oxygen-rich blood to your heart. If plaque narrows or blocks these arteries (a disease called coronary heart disease, or CHD), a common symptom is angina. Angina is chest pain or discomfort that occurs when your heart muscle doesn't get enough oxygen-rich blood.
Angina may feel like pressure or squeezing in your chest. You also may feel it in your shoulders, arms, neck, jaw, or back. Angina pain may even feel like indigestion. The pain tends to get worse with activity and go away with rest. Emotional stress also can trigger the pain.
Other symptoms of CHD are shortness of breath and arrhythmias (ah-RITH-me-ahs). Arrhythmias are problems with the rate or rhythm of the heartbeat.
Plaque also can form in the heart's smallest arteries. This disease is called coronary microvascular disease (MVD). Symptoms of coronary MVD include angina, shortness of breath, sleep problems, fatigue (tiredness), and lack of energy.
Carotid Arteries
The carotid arteries supply oxygen-rich blood to your brain. If plaque narrows or blocks these arteries (a disease called carotid artery disease), you may have symptoms of a stroke. These symptoms may include:
Sudden weakness
Paralysis (an inability to move) or numbness of the face, arms, or legs, especially on one side of the body
Confusion
Trouble speaking or understanding speech
Trouble seeing in one or both eyes
Problems breathing
Dizziness, trouble walking, loss of balance or coordination, and unexplained falls
Loss of consciousness
Sudden and severe headache
Peripheral Arteries
Plaque also can build up in the major arteries that supply oxygen-rich blood to the legs, arms, and pelvis (a disease calledperipheral artery disease).
If these major arteries are narrowed or blocked, you may have numbness, pain, and, sometimes, dangerous infections.
Renal Arteries
The renal arteries supply oxygen-rich blood to your kidneys. If plaque builds up in these arteries, you may develop chronic kidney disease. Over time, chronic kidney disease causes a slow loss of kidney function.
Early kidney disease often has no signs or symptoms. As the disease gets worse it can cause tiredness, changes in how you urinate (more often or less often), loss of appetite, nausea (feeling sick to the stomach), swelling in the hands or feet, itchiness or numbness, and trouble concentrating.
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Atherosclerosis
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How to diagnose Atherosclerosis ?
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Your doctor will diagnose atherosclerosis based on your medical and family histories, a physical exam, and test results.
Specialists Involved
If you have atherosclerosis, a primary care doctor, such as an internist or family practitioner, may handle your care. Your doctor may recommend other health care specialists if you need expert care, such as:
A cardiologist. This is a doctor who specializes in diagnosing and treating heart diseases and conditions. You may go to a cardiologist if you haveperipheral artery disease(P.A.D.)or coronary microvascular disease (MVD).
A vascular specialist. This is a doctor who specializes in diagnosing and treating blood vessel problems. You may go to a vascular specialist if you have P.A.D.
A neurologist. This is a doctor who specializes in diagnosing and treating nervous system disorders. You may see a neurologist if you've had a stroke due to carotid artery disease.
A nephrologist. This is a doctor who specializes in diagnosing and treating kidney diseases and conditions. You may go to a nephrologist if you have chronic kidney disease.
Physical Exam
During the physical exam, your doctor may listen to your arteries for an abnormal whooshing sound called a bruit (broo-E). Your doctor can hear a bruit when placing a stethoscope over an affected artery. A bruit may indicate poor blood flow due to plaque buildup.
Your doctor also may check to see whether any of your pulses (for example, in the leg or foot) are weak or absent. A weak or absent pulse can be a sign of a blocked artery.
Diagnostic Tests
Your doctor may recommend one or more tests to diagnose atherosclerosis. These tests also can help your doctor learn the extent of your disease and plan the best treatment.
Blood Tests
Blood tests check the levels of certain fats, cholesterol, sugar, and proteins in your blood. Abnormal levels may be a sign that you're at risk for atherosclerosis.
EKG (Electrocardiogram)
An EKG is a simple, painless test that detects and records the heart's electrical activity. The test shows how fast the heart is beating and its rhythm (steady or irregular). An EKG also records the strength and timing of electrical signals as they pass through the heart.
An EKG can show signs of heart damage caused by CHD. The test also can show signs of a previous or current heart attack.
Chest X Ray
A chest x ray takes pictures of the organs and structures inside your chest, such as your heart, lungs, and blood vessels. A chest x ray can reveal signs of heart failure.
Ankle/Brachial Index
This test compares the blood pressure in your ankle with the blood pressure in your arm to see how well your blood is flowing. This test can help diagnose P.A.D.
Echocardiography
Echocardiography (echo) uses sound waves to create a moving picture of your heart. The test provides information about the size and shape of your heart and how well your heart chambers and valves are working.
Echo also can identify areas of poor blood flow to the heart, areas of heart muscle that aren't contracting normally, and previous injury to the heart muscle caused by poor blood flow.
Computed Tomography Scan
A computed tomography (CT) scan creates computer-generated pictures of the heart, brain, or other areas of the body. The test can show hardening and narrowing of large arteries.
A cardiac CT scan also can show whether calcium has built up in the walls of the coronary (heart) arteries. This may be an early sign of CHD.
Stress Testing
During stress testing, you exercise to make your heart work hard and beat fast while heart tests are done. If you can't exercise, you may be given medicine to make your heart work hard and beat fast.
When your heart is working hard, it needs more blood and oxygen. Plaque-narrowed arteries can't supply enough oxygen-rich blood to meet your heart's needs.
A stress test can show possible signs and symptoms of CHD, such as:
Abnormal changes in your heart rate or blood pressure
Shortness of breath or chest pain
Abnormal changes in your heart rhythm or your heart's electrical activity
As part of some stress tests, pictures are taken of your heart while you exercise and while you rest. These imaging stress tests can show how well blood is flowing in various parts of your heart. They also can show how well your heart pumps blood when it beats.
Angiography
Angiography (an-jee-OG-ra-fee) is a test that uses dye and special x rays to show the inside of your arteries. This test can show whether plaque is blocking your arteries and how severe the blockage is.
A thin, flexible tube called a catheter is put into a blood vessel in your arm, groin (upper thigh), or neck. Dye that can be seen on an x-ray picture is injected through the catheter into the arteries. By looking at the x-ray picture, your doctor can see the flow of blood through your arteries.
Other Tests
Other tests are being studied to see whether they can give a better view of plaque buildup in the arteries. Examples of these tests include magnetic resonance imaging (MRI) and positron emission tomography (PET).
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Atherosclerosis
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What are the treatments for Atherosclerosis ?
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Treatments for atherosclerosis may include heart-healthy lifestyle changes, medicines, and medical procedures or surgery. The goals of treatment include:
Lowering the risk of blood clots forming
Preventing atherosclerosis-related diseases
Reducing risk factors in an effort to slow or stop the buildup of plaque
Relieving symptoms
Widening or bypassing plaque-clogged arteries
Heart-Healthy Lifestyle Changes
Your doctor may recommend heart-healthy lifestyle changes if you have atherosclerosis. Heart-healthy lifestyle changes include heart-healthy eating, maintaining a healthy weight, managing stress, physical activity and quitting smoking.
Heart-Healthy Eating
Your doctor may recommend heart-healthy eating, which should include:
Fat-free or low-fat dairy products, such as skim milk
Fish high in omega-3 fatty acids, such as salmon, tuna, and trout, about twice a week
Fruits, such as apples, bananas, oranges, pears, and prunes
Legumes, such as kidney beans, lentils, chickpeas, black-eyed peas, and lima beans
Vegetables, such as broccoli, cabbage, and carrots
Whole grains, such as oatmeal, brown rice, and corn tortillas
When following a heart-healthy diet, you should avoid eating:
A lot of red meat
Palm and coconut oils
Sugary foods and beverages
Two nutrients in your diet make blood cholesterol levels rise:
Saturated fatfound mostly in foods that come from animals
Trans fat (trans fatty acids)found in foods made with hydrogenated oils and fats, such as stick margarine; baked goods, such as cookies, cakes, and pies; crackers; frostings; and coffee creamers. Some trans fats also occur naturally in animal fats and meats.
Saturated fat raises your blood cholesterol more than anything else in your diet. When you follow a heart-healthy eating plan, only 5 percent to 6 percent of your daily calories should come from saturated fat. Food labels list the amounts of saturated fat. To help you stay on track, here are some examples:
1,200 calories a day
8 grams of saturated fat a day
1,500 calories a day
10 grams of saturated fat a day
1,800 calories a day
12 grams of saturated fat a day
2,000 calories a day
13 grams of saturated fat a day
2,500 calories a day
17 grams of saturated fat a day
Not all fats are bad. Monounsaturated and polyunsaturated fats actually help lower blood cholesterol levels. Some sources of monounsaturated and polyunsaturated fats are:
Avocados
Corn, sunflower, and soybean oils
Nuts and seeds, such as walnuts
Olive, canola, peanut, safflower, and sesame oils
Peanut butter
Salmon and trout
Tofu
Sodium
You should try to limit the amount of sodium that you eat. This means choosing and preparing foods that are lower in salt and sodium. Try to use low-sodium and no added salt foods and seasonings at the table or while cooking. Food labels tell you what you need to know about choosing foods that are lower in sodium. Try to eat no more than 2,300 milligrams of sodium a day. If you have high blood pressure, you may need to restrict your sodium intake even more.
Dietary Approaches to Stop Hypertension
Your doctor may recommend the Dietary Approaches to Stop Hypertension (DASH) eating plan if you have high blood pressure. The DASH eating plan focuses on fruits, vegetables, whole grains, and other foods that are heart healthy and low in fat, cholesterol, and sodium and salt.
The DASH eating plan is a good heart-healthy eating plan, even for those who dont have high blood pressure. Read more about DASH.
Alcohol
Try to limit alcohol intake. Too much alcohol will raise your blood pressure and triglyceride levels, a type of fat found in the blood. Alcohol also adds extra calories, which may cause weight gain.
Men should have no more than two drinks containing alcohol a day. Women should have no more than one drink containing alcohol a day. One drink is:
12 ounces of beer
5 ounces of wine
1 ounces of liquor
Maintaining a Healthy Weight
Maintaining a healthy weight is important for overall health and can lower your risk for coronary heart disease. Aim for a Healthy Weight by following a heart-healthy eating plan and keeping physically active.
Knowing your body mass index (BMI) helps you find out if youre a healthy weight in relation to your height and gives an estimate of your total body fat. To figure out your BMI, check out the National Heart, Lung, and Blood Institutes online BMI calculator or talk to your doctor. A BMI:
Below 18.5 is a sign that you are underweight.
Between 18.5 and 24.9 is in the normal range.
Between 25.0 and 29.9 is considered overweight.
A BMI of 30.0 or higher is considered obese.
A general goal to aim for is a BMI of less than 25. Your doctor or health care provider can help you set an appropriate BMI goal.
Measuring waist circumference helps screen for possible health risks. If most of your fat is around your waist rather than at your hips, youre at a higher risk for heart disease and type2 diabetes. This risk may be high with a waist size that is greater than 35 inches for women or greater than 40 inches for men. To learn how to measure your waist, visit Assessing Your Weight and Health Risk. For more information about losing weight or maintaining your weight, visit Aim for a Healthy Weight.
If youre overweight or obese, try to lose weight. A loss of just 3 percent to 5 percent of your current weight can lower your triglycerides, blood glucose, and the risk of developing type 2 diabetes. Greater amounts of weight loss can improve blood pressure readings, lower LDL cholesterol, and increase HDL cholesterol.
Managing Stress
Learning how to manage stress, relax, and cope with problems can improve your emotional and physical health. Consider healthy stress-reducing activities, such as:
A stress management program
Meditation
Physical activity
Relaxation therapy
Talking things out with friends or family
Physical Activity
Regular physical activity can lower many atherosclerosis risk factors, including LDL or bad cholesterol, high blood pressure, and excess weight. Physical activity also can lower your risk for diabetes and raise your HDL or good cholesterol, which helps prevent atherosclerosis.
Everyone should try to participate in moderate-intensity aerobic exercise at least 2 hours and 30 minutes per week or vigorous aerobic exercise for 1 hour and 15 minutes per week. Aerobic exercise, such as brisk walking, is any exercise in which your heart beats faster and you use more oxygen than usual. The more active you are, the more you will benefit. Participate in aerobic exercise for at least 10minutes at a time spread throughout the week.
Talk with your doctor before you start a new exercise plan. Ask your doctor how much and what kinds of physical activity are safe for you. Read more about physical activity at:
Physical Activity and Your Heart
U.S. Department of Health and Human Services, 2008 Physical Activity Guidelines for Americans
Quitting Smoking
If you smoke or use tobacco, quit. Smoking can damage and tighten blood vessels and raise your risk for atherosclerosis. Talk with your doctor about programs and products that can help you quit. Also, try to avoid secondhand smoke. If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking.
For more information about how to quit smoking, visit Smoking and Your Heart.
Medicines
Sometimes lifestyle changes alone arent enough to control your cholesterol levels. For example, you also may need statin medications to control or lower your cholesterol. By lowering your blood cholesterol level, you can decrease your chance of having a heart attack or stroke. Doctors usually prescribe statins for people who have:
Coronary heart disease, peripheral artery disease, or had a prior stroke
Diabetes
High LDL cholesterol levels
Doctors may discuss beginning statin treatment with people who have an elevated risk for developing heart disease or having a stroke. Your doctor also may prescribe other medications to:
Lower your blood pressure
Lower your blood sugar levels
Prevent blood clots, which can lead to heart attack and stroke
Prevent inflammation
Take all medicines regularly, as your doctor prescribes. Dont change the amount of your medicine or skip a dose unless your doctor tells you to. You should still follow a heart healthy lifestyle, even if you take medicines to treat your atherosclerosis.
Medical Procedures and Surgery
If you have severe atherosclerosis, your doctor may recommend a medical procedure or surgery.
Percutaneous coronary intervention (PCI), also known as coronary angioplasty, is a procedure thats used to open blocked or narrowed coronary (heart) arteries. PCI can improve blood flow to the heart and relieve chest pain. Sometimes a small mesh tube called a stent is placed in the artery to keep it open after the procedure.
Coronary artery bypass grafting (CABG) is a type of surgery. In CABG, arteries or veins from other areas in your body are used to bypass or go around your narrowed coronary arteries. CABG can improve blood flow to your heart, relieve chest pain, and possibly prevent a heart attack.
Bypass grafting also can be used for leg arteries. For this surgery, a healthy blood vessel is used to bypass a narrowed or blocked artery in one of the legs. The healthy blood vessel redirects blood around the blocked artery, improving blood flow to the leg.
Carotid endarterectomy is a type of surgery to remove plaque buildup from the carotid arteries in the neck. This procedure restores blood flow to the brain, which can help prevent a stroke.
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Atherosclerosis
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How to prevent Atherosclerosis ?
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Taking action to control your risk factors can help prevent or delay atherosclerosis and its related diseases. Your risk for atherosclerosis increases with the number of risk factors youhave.
One step you can take is to adopt a healthy lifestyle, which can include:
Heart-Healthy Eating. Adopt heart-healthy eating habits, which include eating different fruits and vegetables (including beans and peas), whole grains, lean meats, poultry without skin, seafood, and fat-free or low-fat milk and dairy products. A heart-healthy diet is low in sodium, added sugar, solid fats, and refined grains. Following a heart-healthy diet is an important part of a healthy lifestyle.
Physical Activity. Be as physically active as you can. Physical activity can improve your fitness level and your health. Ask your doctor what types and amounts of activity are safe for you. Read more about Physical Activity and Your Heart.
Quit Smoking. If you smoke, quit. Smoking can damage and tighten blood vessels and raise your risk for atherosclerosis. Talk with your doctor about programs and products that can help you quit. Also, try to avoid secondhand smoke. Read more about Smoking and Your Heart.
Weight Control. If youre overweight or obese, work with your doctor to create a reasonable weight-loss plan. Controlling your weight helps you control risk factors foratherosclerosis.
Other steps that can prevent or delay atherosclerosis include knowing your family history of atherosclerosis. If you or someone in your family has an atherosclerosis-related disease, be sure to tell your doctor.
If lifestyle changes arent enough, your doctor may prescribe medicines to control your atherosclerosis risk factors. Take all of your medicines as your doctor advises.
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Atherosclerosis
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What is (are) Sickle Cell Disease ?
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Espaol
The term sickle cell disease (SCD) describes a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells.
Hemoglobin is a protein in red blood cells that carries oxygen throughout the body.
Inherited means that the disease is passed by genes from parents to their children. SCD is not contagious. A person cannot catch it, like a cold or infection, from someone else.
People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genes causes a persons body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD.
Hemoglobin SC disease and hemoglobin S thalassemia (thal-uh-SEE-me-uh) are two other common forms of SCD.
Some Forms of Sickle Cell Disease
Hemoglobin SS
Hemoglobin SC
Hemoglobin S0 thalassemia
Hemoglobin S+ thalassemia
Hemoglobin SD
Hemoglobin SE
Overview
Cells in tissues need a steady supply of oxygen to work well. Normally, hemoglobin in red blood cells takes up oxygen in the lungs and carries it to all the tissues of the body.
Red blood cells that contain normal hemoglobin are disc shaped (like a doughnut without a hole). This shape allows the cells to be flexible so that they can move through large and small blood vessels to deliver oxygen.
Sickle hemoglobin is not like normal hemoglobin. It can form stiff rods within the red cell, changing it into a crescent, or sickle shape.
Sickle-shaped cells are not flexible and can stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen cant reach nearby tissues.
Normal Red Cells and Sickle Red Cells
The lack of tissue oxygen can cause attacks of sudden, severe pain, called pain crises. These pain attacks can occur without warning, and a person often needs to go to the hospital for effective treatment.
Most children with SCD are pain free between painful crises, but adolescents and adults may also suffer with chronic ongoing pain.
The red cell sickling and poor oxygen delivery can also cause organ damage. Over a lifetime, SCD can harm a persons spleen, brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin.
Sickle cells cant change shape easily, so they tend to burst apart or hemolyze. Normal red blood cells live about 90 to 120 days, but sickle cells last only 10 to 20 days.
The body is always making new red blood cells to replace the old cells; however, in SCD the body may have trouble keeping up with how fast the cells are being destroyed. Because of this, the number of red blood cells is usually lower than normal. This condition, called anemia, can make a person have less energy.
Outlook
Sickle cell disease is a life-long illness. The severity of the disease varies widely from person to person.
In high-income countries like the United States, the life expectancy of a person with SCD is now about 4060 years. In 1973, the average lifespan of a person with SCD in the United States was only 14 years. Advances in the diagnosis and care of SCD have made this improvement possible.
At the present time, hematopoietic stem cell transplantation (HSCT) is the only cure for SCD. Unfortunately, most people with SCD are either too old for a transplant or dont have a relative who is a good enough genetic match for them to act as a donor. A well-matched donor is needed to have the best chance for a successful transplant.
There are effective treatments that can reduce symptoms and prolong life. Early diagnosis and regular medical care to prevent complications also contribute to improved well-being.
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Sickle Cell Disease
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What causes Sickle Cell Disease ?
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Abnormal hemoglobin, called hemoglobin S, causes sickle cell disease (SCD).
The problem in hemoglobin S is caused by a small defect in the gene that directs the production of the beta globin part of hemoglobin. This small defect in the beta globin gene causes a problem in the beta globin part of hemoglobin, changing the way that hemoglobin works. (See Overview.)
How Is Sickle Cell Disease Inherited?
When the hemoglobin S gene is inherited from only one parent and a normal hemoglobin gene is inherited from the other, a person will have sickle cell trait. People with sickle cell trait are generally healthy.
Only rarely do people with sickle cell trait have complications similar to those seen in people with SCD. But people with sickle cell trait are carriers of a defective hemoglobin S gene. So, they can pass it on when they have a child.
If the childs other parent also has sickle cell trait or another abnormal hemoglobin gene (like thalassemia, hemoglobin C, hemoglobin D, hemoglobin E), that child has a chance of having SCD.
Example of an Inheritance Pattern
In the image above, each parent has one hemoglobin A gene and one hemoglobin S gene, and each of their children has:
A 25 percent chance of inheriting two normal genes: In this case the child does not have sickle cell trait or disease. (Case 1)
A 50 percent chance of inheriting one hemoglobin A gene and one hemoglobin S gene: This child has sickle cell trait. (Cases 2 and 3)
A 25 percent chance of inheriting two hemoglobin S genes: This child has sickle cell disease. (Case 4)
It is important to keep in mind that each time this couple has a child, the chances of that child having sickle cell disease remain the same. In other words, if the first-born child has sickle cell disease, there is still a 25 percent chance that the second child will also have the disease. Both boys and girls can inherit sickle cell trait, sickle cell disease, or normal hemoglobin.
If a person wants to know if he or she carries a sickle hemoglobin gene, a doctor can order a blood test to find out.
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Sickle Cell Disease
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Who is at risk for Sickle Cell Disease? ?
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In the United States, most people with sickle cell disease (SCD) are of African ancestry or identify themselves as black.
About 1 in 13 African American babies is born with sickle cell trait.
About 1 in every 365 black children is born with sickle cell disease.
There are also many people with this disease who come from Hispanic, southern European, Middle Eastern, or Asian Indian backgrounds.
Approximately 100,000 Americans have SCD.
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Sickle Cell Disease
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What are the symptoms of Sickle Cell Disease ?
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Early Signs and Symptoms
If a person has sickle cell disease (SCD), it is present at birth. But most infants do not have any problems from the disease until they are about 5 or 6 months of age. Every state in the United States, the District of Columbia, and the U.S. territories requires that all newborn babies receive screening for SCD. When a child has SCD, parents are notified before the child has symptoms.
Some children with SCD will start to have problems early on, and some later. Early symptoms of SCD may include:
Painful swelling of the hands and feet, known as dactylitis
Fatigue or fussiness from anemia
A yellowish color of the skin, known as jaundice, or whites of the eyes, known as icteris, that occurs when a large number of red cells hemolyze
The signs and symptoms of SCD will vary from person to person and can change over time. Most of the signs and symptoms of SCD are related to complications of the disease.
Major Complications of Sickle Cell Disease
Acute Pain (Sickle Cell or Vaso-occlusive) Crisis
Pain episodes (crises) can occur without warning when sickle cells block blood flow and decrease oxygen delivery. People describe this pain as sharp, intense, stabbing, or throbbing. Severe crises can be even more uncomfortable than post-surgical pain or childbirth.
Pain can strike almost anywhere in the body and in more than one spot at a time. But the pain often occurs in the
Lower back
Legs
Arms
Abdomen
Chest
A crisis can be brought on by
Illness
Temperature changes
Stress
Dehydration (not drinking enough)
Being at high altitudes
But often a person does not know what triggers, or causes, the crisis. (See acute pain management.)
Chronic Pain
Many adolescents and adults with SCD suffer from chronic pain. This kind of pain has been hard for people to describe, but it is usually different from crisis pain or the pain that results from organ damage.
Chronic pain can be severe and can make life difficult. Its cause is not well understood. (See chronic pain management.)
Severe Anemia
People with SCD usually have mild to moderate anemia. At times, however, they can have severe anemia. Severe anemia can be life threatening. Severe anemia in an infant or child with SCD may be caused by:
Splenic sequestration crisis. The spleen is an organ that is located in the upper left side of the belly. The spleen filters germs in the blood, breaks up blood cells, and makes a kind of white blood cell. A splenic sequestration crisis occurs when red blood cells get stuck in the spleen, making it enlarge quickly. Since the red blood cells are trapped in the spleen, there are fewer cells to circulate in the blood. This causes severe anemia. A big spleen may also cause pain in the left side of the belly. A parent can usuallypalpate or feel the enlarged spleen in the belly of his or her child.
A big spleen may also cause pain in the left side of the belly. A parent can usuallypalpate or feel the enlarged spleen in the belly of his or her child.
Aplastic crisis. This crisis is usually caused by a parvovirus B19 infection, also called fifth disease or slapped cheek syndrome. Parvovirus B19 is a very common infection, but in SCD it can cause the bone marrow to stop producing new red cells for a while, leading to severe anemia.
Splenic sequestration crisis and aplastic crisis most commonly occur in infants and children with SCD. Adults with SCD may also experience episodes of severe anemia, but these usually have other causes.
No matter the cause, severe anemia may lead to symptoms that include:
Shortness of breath
Being very tired
Feeling dizzy
Having pale skin
Babies and infants with severe anemia may feed poorly and seem very sluggish. (See anemia management.)
Infections
The spleen is important for protection against certain kinds of germs. Sickle cells can damage the spleen and weaken or destroy its function early in life.
People with SCD who have damaged spleens are at risk for serious bacterial infections that can be life-threatening. Some of these bacteria include:
Pneumococcus
Hemophilus influenza type B
Meningococcus
Salmonella
Staphylococcus
Chlamydia
Mycoplasma pneumoniae
Bacteria can cause:
Blood infection (septicemia)
Lung infection (pneumonia)
Infection of the covering of the brain and spinal cord (meningitis)
Bone infection (osteomyelitis)
(See how to prevent infections and infection management.)
Acute Chest Syndrome
Sickling in blood vessels of the lungs can deprive a persons lungs of oxygen. When this happens, areas of lung tissue are damaged and cannot exchange oxygen properly. This condition is known as acute chest syndrome. In acute chest syndrome, at least one segment of the lung is damaged.
This condition is very serious and should be treated right away at a hospital.
Acute chest syndrome often starts a few days after a painful crisis begins. A lung infection may accompany acute chest syndrome.
Symptoms may include:
Chest pain
Fever
Shortness of breath
Rapid breathing
Cough
(See acute chest syndrome management.)
Brain Complications
Clinical Stroke
A stroke occurs when blood flow is blocked to a part of the brain. When this happens, brain cells can be damaged or can die. In SCD, a clinical stroke means that a person shows outward signs that something is wrong. The symptoms depend upon what part of the brain is affected. Symptoms of stroke may include:
Weakness of an arm or leg on one side of the body
Trouble speaking, walking, or understanding
Loss of balance
Severe headache
As many as 24 percent of people with hemoglobin SS and 10 percent of people with hemoglobin SC may suffer a clinical stroke by age 45.
In children, clinical stroke occurs most commonly between the ages of 2 and 9, but recent prevention strategies have lowered the risk. (See Transcranial Doppler (TCD) Ultrasound Screening) and Red Blood Cell Transfusions.)
When people with SCD show symptoms of stroke, their families or friends should call 9-1-1 right away. (See clinical stroke management.)
Silent Stroke and Thinking Problems
Brain imaging and tests of thinking (cognitive studies) have shown that children and adults with hemoglobin SS and hemoglobin S0 thalassemia often have signs of silent brain injury, also called silent stroke. Silent brain injury is damage to the brain without showing outward signs of stroke.
This injury is common. Silent brain injury can lead to learning problems or trouble making decisions or holding down a job. (See Cognitive Screening and silent stroke management.)
Eye Problems
Sickle cell disease can injure blood vessels in the eye.
The most common site of damage is the retina, where blood vessels can overgrow, get blocked, or bleed. The retina is the light-sensitive layer of tissue that lines the inside of the eye and sends visual messages through the optic nerve to the brain.
Detachment of the retina can occur. When the retina detaches, it is lifted or pulled from its normal position. These problems can cause visual impairment or loss. (See Eye Examinations.)
Heart Disease
People with SCD can have problems with blood vessels in the heart and with heart function. The heart can become enlarged. People can also develop pulmonary hypertension.
People with SCD who have received frequent blood transfusions may also have heart damage from iron overload. (See transfusion management.)
Pulmonary Hypertension
In adolescents and adults, injury to blood vessels in the lungs can make it hard for the heart to pump blood through them. This causes the pressure in lung blood vessels to rise. High pressure in these blood vessels is called pulmonary hypertension. Symptoms may include shortness of breath and fatigue.
When this condition is severe, it has been associated with a higher risk of death. (See screening for pulmonary hypertension.)
Kidney Problems
The kidneys are sensitive to the effects of red blood cell sickling.
SCD causes the kidneys to have trouble making the urine as concentrated as it should be. This may lead to a need to urinate often and to have bedwetting or uncontrolled urination during the night (nocturnal enuresis). This often starts in childhood. Other problems may include:
Blood in the urine
Decreased kidney function
Kidney disease
Protein loss in the urine
Priapism
Males with SCD can have unwanted, sometimes prolonged, painful erections. This condition is called priapism.
Priapism happens when blood flow out of the erect penis is blocked by sickled cells. If it goes on for a long period of time, priapism can cause permanent damage to the penis and lead to impotence.
If priapism lasts for more than 4 hours, emergency medical care should be sought to avoid complications. (See priapism management.)
Gallstones
When red cells hemolyze, they release hemoglobin. Hemoglobin gets broken down into a substance called bilirubin. Bilirubin can form stones that get stuck in the gallbladder. The gallbladder is a small, sac-shaped organ beneath the liver that helps with digestion. Gallstones are a common problem in SCD.
Gallstones may be formed early on but may not produce symptoms for years. When symptoms develop, they may include:
Right-sided upper belly pain
Nausea
Vomiting
If problems continue or recur, a person may need surgery to remove the gallbladder.
Liver Complications
There are a number of ways in which the liver may be injured in SCD.
Sickle cell intrahepatic cholestasis is an uncommon, but severe, form of liver damage that occurs when sickled red cells block blood vessels in the liver. This blockage prevents enough oxygen from reaching liver tissue.
These episodes are usually sudden and may recur. Children often recover, but some adults may have chronic problems that lead to liver failure.
People with SCD who have received frequent blood transfusions may develop liver damage from iron overload.
Leg Ulcers
Sickle cell ulcers are sores that usually start small and then get larger and larger.
The number of ulcers can vary from one to many. Some ulcers will heal quickly, but others may not heal and may last for long periods of time. Some ulcers come back after healing.
People with SCD usually dont get ulcers until after the age of 10.
Joint Complications
Sickling in the bones of the hip and, less commonly, the shoulder joints, knees, and ankles, can decrease oxygen flow and result in severe damage. This damage is a condition called avascular or aseptic necrosis. This disease is usually found in adolescents and adults.
Symptoms include pain and problems with walking and joint movement. A person may need pain medicines, surgery, or joint replacement if symptoms persist.
Delayed Growth and Puberty
Children with SCD may grow and develop more slowly than their peers because of anemia. They will reach full sexual maturity, but this may be delayed.
Pregnancy
Pregnancies in women with SCD can be risky for both the mother and the baby.
Mothers may have medical complications including:
Infections
Blood clots
High blood pressure
Increased pain episodes
They are also at higher risk for:
Miscarriages
Premature births
Small-for-dates babies or underweight babies
(See pregnancy management.)
Mental Health
As in other chronic diseases, people with SCD may feel sad and frustrated at times. The limitations that SCD can impose on a persons daily activities may cause them to feel isolated from others. Sometimes they become depressed.
People with SCD may also have trouble coping with pain and fatigue, as well as with frequent medical visits and hospitalizations. (See living with emotional issues.)
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Sickle Cell Disease
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How to diagnose Sickle Cell Disease ?
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Screening Tests
People who do not know whether they make sickle hemoglobin (hemoglobin S) or another abnormal hemoglobin (such as C, thalassemia, E) can find out by having their blood tested. This way, they can learn whether they carry a gene (i.e., have the trait) for an abnormal hemoglobin that they could pass on to a child.
When each parent has this information, he or she can be better informed about the chances of having a child with some type of sickle cell disease (SCD), such as hemoglobin SS, SC, S thalassemia, or others.
Newborn Screening
When a child has SCD, it is very important to diagnose it early to better prevent complications.
Every state in the United States, the District of Columbia, and the U.S. territories require that every baby is tested for SCD as part of a newborn screening program.
In newborn screening programs, blood from a heel prick is collected in spots on a special paper. The hemoglobin from this blood is then analyzed in special labs.
Newborn screening results are sent to the doctor who ordered the test and to the childs primary doctor.
If a baby is found to have SCD, health providers from a special follow-up newborn screening group contact the family directly to make sure that the parents know the results. The child is always retested to be sure that the diagnosis is correct.
Newborn screening programs also find out whether the baby has an abnormal hemoglobin trait. If so, parents are informed, and counseling is offered.
Remember that when a child has sickle cell trait or SCD, a future sibling, or the childs own future child, may be at risk. These possibilities should be discussed with the primary care doctor, a blood specialist called a hematologist, and/or a genetics counselor.
Prenatal Screening
Doctors can also diagnose SCD before a baby is born. This is done using a sample of amniotic fluid, the liquid in the sac surrounding a growing embryo, or tissue taken from the placenta, the organ that attaches the umbilical cord to the mothers womb.
Testing before birth can be done as early as 810 weeks into the pregnancy. This testing looks for the sickle hemoglobin gene rather than the abnormal hemoglobin.
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Sickle Cell Disease
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What are the treatments for Sickle Cell Disease ?
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Health Maintenance To Prevent Complications
Babies with sickle cell disease (SCD) should be referred to a doctor or provider group that has experience taking care of people with this disease. The doctor might be a hematologist (a doctor with special training in blood diseases) or an experienced general pediatrician, internist, or family practitioner.
For infants, the first SCD visit should take place before 8 weeks of age.
If someone was born in a country that doesnt perform newborn SCD screening, he or she might be diagnosed with SCD later in childhood. These people should also be referred as soon as possible for special SCD care.
Examining the person
Giving medicines and immunizations
Performing tests
Educating families about the disease and what to watch out for
Preventing Infection
In SCD, the spleen doesnt work properly or doesnt work at all. This problem makes people with SCD more likely to get severe infections.
Penicillin
In children with SCD, taking penicillin two times a day has been shown to reduce the chance of having a severe infection caused by the pneumococcus bacteria. Infants need to take liquid penicillin. Older children can take tablets.
Many doctors will stop prescribing penicillin after a child has reached the age of 5. Some prefer to continue this antibiotic throughout life, particularly if a person has hemoglobin SS or hemoglobin S0 thalassemia, since people with SCD are still at risk. All people who have had surgical removal of the spleen, called a splenectomy, or a past infection with pneumococcus should keep taking penicillin throughout life.
Immunizations
People with SCD should receive all recommended childhood vaccines. They should also receive additional vaccines to prevent other infections.
Pneumococcus. Even though all children routinely receive the vaccine against pneumococcus (PCV13), children with SCD should also receive a second kind of vaccine against pneumococcus (PPSV23). This second vaccine is given after 24 months of age and again 5 years later. Adults with SCD who have not received any pneumococcal vaccine should get a dose of the PCV13 vaccine. They should later receive the PPSV23 if they have not already received it or it has been more than 5 years since they did. A person should follow these guidelines even if he or she is still taking penicillin.
Influenza. All people with SCD should receive an influenza shot every year at the start of flu season. This should begin at 6 months of age. Only the inactivated vaccine, which comes as a shot, should be used in people with SCD.
Meningococcus. A child with SCD should receive this vaccine (Menactra or Menveo) at 2, 4, 6, and 1215 months of age. The child should receive a booster vaccine 3 years after this series of shots, then every 5 years after that.
Screening Tests and Evaluations
Height, Weight, Blood Pressure, and Oxygen Saturation
Doctors will monitor height and weight to be sure that a child is growing properly and that a person with SCD is maintaining a healthy weight.
Doctors will also track a persons blood pressure. When a person with SCD has high blood pressure, it needs to be treated promptly because it can increase the risk of stroke.
Oxygen saturation testing provides information about how much oxygen the blood is carrying.
Blood and Urine Testing
People with SCD need to have frequent lab tests.
Blood tests help to establish a persons baseline for problems like anemia. Blood testing also helps to show whether a person has organ damage, so that it can be treated early.
Urine testing can help to detect early kidney problems or infections.
Transcranial Doppler (TCD) Ultrasound Screening
Children who have hemoglobin SS or hemoglobin S0 thalassemia and are between the ages of 2 and 16 should have TCD testing once a year.
This study can find out whether a child is at higher risk for stroke. When the test is abnormal, regular blood transfusions can decrease the chances of having a stroke.
The child is awake during the TCD exam. The test does not hurt at all. The TCD machine uses sound waves to measure blood flow like the ultrasound machine used to examine pregnant women.
Eye Examinations
An eye doctor, or ophthalmologist, should examine a persons eyes every 12 years from the age of 10 onwards.
These exams can detect if there are SCD-related problems of the eye. Regular exams can help doctors find and treat problems early to prevent loss of vision. A person should see his or her doctor right away for any sudden change in vision.
Pulmonary Hypertension
Doctors have different approaches to screening for pulmonary hypertension. This is because studies have not given clear information as to when and how a person should receive the screening. People with SCD and their caretakers should discuss with their doctor whether screening makes sense for them.
Cognitive Screening
People with sickle cell disease can develop cognitive (thinking) problems that may be hard to notice early in life.
Sometimes these problems are caused by silent strokes that can only be seen with magnetic resonance imaging (MRI) of the brain.
People with SCD should tell their doctors or nurses if they have thinking problems, such as difficulties learning in school, slowed decision making, or trouble organizing their thoughts.
People can be referred for cognitive testing. This testing can identify areas in which a person could use extra help.
Children with SCD who have thinking problems may qualify for an Individualized Education Program, or IEP. An IEP is a plan that helps students to reach their educational goals. Adults may be able to enroll in vocational rehabilitation programs that can help them with job training.
Education and Guidance
Doctors and other providers will talk with people who have SCD and their caretakers about complications and also review information at every visit.
Because there are a lot of things to discuss, new topics are often introduced as a child or adult reaches an age when that subject is important to know about.
Doctors and nurses know that there is a lot of information to learn, and they dont expect people to know everything after one discussion. People with SCD and their families should not be afraid to ask questions.
Topics that are usually covered include:
Hours that medical staff are available and contact information to use when people with SCD or caretakers have questions
A plan for what to do and where to get care if a person has a fever, pain, or other signs of SCD complications that need immediate attention
How SCD is inherited and the risk of having a child with SCD
The importance of regular medical visits, screening tests, and evaluations
How to recognize and manage pain
How to palpate (feel) a childs spleen. Because of the risk of splenic sequestration crisis, caretakers should learn how to palpate a childs spleen. They should try to feel for the spleen daily and more frequently when the child is ill. If they feel that the spleen is bigger than usual, they should call the care provider.
Transitioning Care
When children with SCD become adolescents or young adults, they often need to transition from a pediatric care team to an adult care team. This period has been shown to be associated with increased hospital admissions and medical problems. There seem to be many reasons for this.
Some of the increased risk is directly related to the disease. As people with SCD get older, they often develop more organ damage and more disabilities.
The shift in care usually occurs at the same time that adolescents are undergoing many changes in their emotional, social, and academic lives. The transition to more independent self-management may be difficult, and following treatment plans may become less likely.
When compared with pediatrics, there are often fewer adult SCD programs available in a given region. This makes it more difficult for a person with SCD to find appropriate doctors, particularly those with whom they feel comfortable.
To improve use of regular medical care by people with SCD and to reduce age-related complications, many SCD teams have developed special programs that the make transition easier. Such programs should involve the pediatric and the adult care teams. They should also start early and continue over several years.
Managing Some Complications of SCD
Acute Pain
Each person with SCD should have a home treatment regimen that is best suited to their needs. The providers on the SCD team usually help a person develop a written, tailored care plan. If possible, the person with SCD should carry this plan with them when they go to the emergency room.
When an acute crisis is just starting, most doctors will advise the person to drink lots of fluids and to take a non-steroidal anti-inflammatory (NSAID) pain medication, such as ibuprofen. When a person has kidney problems, acetaminophen is often preferred.
If pain persists, many people will find that they need a stronger medicine.
Combining additional interventions, such as massage, relaxation methods, or a heating pad, may also help.
If a person with SCD cannot control the pain at home, he or she should go to an SCD day hospital/outpatient unit or an emergency room to receive additional, stronger medicines and intravenous (IV) fluids.
Some people may be able to return home once their pain is under better control. In this case, the doctor may prescribe additional pain medicines for a short course of therapy.
People often need to be admitted to the hospital to fully control an acute pain crisis.
When taken daily, hydroxyurea has been found to decrease the number and severity of pain episodes.
Chronic Pain
Sometimes chronic pain results from a complication, such as a leg ulcer or aseptic necrosis of the hip. In this case, doctors try to treat the complication causing the pain.
While chronic pain is common in adults with SCD, the cause is often poorly understood. Taking pain medicines daily may help to decrease the pain. Some examples of these medicines include:
NSAID drugs, such as ibuprofen
Duloxetine
Gabapentin
Amitriptyline
Strong pain medicines, such as opiates
Other approaches, such as massage, heat, or acupuncture may be helpful in some cases. Chronic pain often comes with feelings of depression and anxiety. Supportive counseling and, sometimes, antidepressant medicines may help. (See coping and emotional issues.)
Severe Anemia
People should see their doctors or go to a hospital right away if they develop anemia symptoms from a splenic sequestration crisis or an aplastic crisis. These conditions can be life-threatening, and the person will need careful monitoring and treatment in the hospital. A person also usually needs a blood transfusion.
People with SCD and symptoms of severe anemia from other causes should also see a doctor right away.
Infections
Fever is a medical emergency in SCD. All caretakers of infants and children with SCD should take their child to their doctor or go to a hospital right away when their child has a fever. Adults with SCD should also seek care for fever or other signs of infection.
All children and adults who have SCD and a fever (over 38.50 C or 101.30 F) must be seen by a doctor and treated with antibiotics right away.
Some people will need to be hospitalized, while others may receive care and follow-up as an outpatient.
Acute Chest Syndrome
People with SCD and symptoms of acute chest syndrome should see their doctor or go to a hospital right away.
They will need to be admitted to the hospital where they should receive antibiotics and close monitoring. They may need oxygen therapy and a blood transfusion.
When taken daily, the medicine hydroxyurea has been found to decrease the number and severity of acute chest events.
Clinical Stroke
People with SCD who have symptoms of stroke should be brought to the hospital right away by an ambulance. If a person is having symptoms of stroke, someone should call 9-1-1.
Symptoms of stroke may include:
Weakness of an arm or leg on one side of the body
Trouble speaking, walking, or understanding
Loss of balance
Severe headache
If imaging studies reveal that the person has had an acute stroke, he or she may need an exchange transfusion. This procedure involves slowly removing an amount of the persons blood and replacing it with blood from a donor who does not have SCD or sickle cell trait. Afterward, the person may need to receive monthly transfusions or other treatments to help to prevent another stroke.
Silent Stroke and Cognitive Problems
Children and adults with SCD and cognitive problems may be able to get useful help based upon the results of their testing. For instance, children may qualify for an IEP. Adults may be able to enroll in vocational, or job, training programs.
Priapism
Sometimes, a person may be able to relieve priapism by:
Emptying the bladder by urinating
Taking medicine
Increasing fluid intake
Doing light exercise
If a person has an episode that lasts for 4 hours or more, he should go to the hospital to see a hematologist and urologist.
Pregnancy
Pregnant women with SCD are at greater risk for problems. They should always see an obstetrician, or OB, who has experience with SCD and high-risk pregnancies and deliveries.
The obstetrician should work with a hematologist or primary medical doctor who is well informed about SCD and its complications.
Pregnant women with SCD need more frequent medical visits so that their doctors can follow them closely. The doctor may prescribe certain vitamins and will be careful to prescribe pain medicines that are safe for the baby.
A pregnant woman with SCD may need to have one or more blood transfusions during her pregnancy to treat complications, such as worsening anemia or an increased number of pain or acute chest syndrome events.
Hydroxyurea
What Is Hydroxyurea?
Hydroxyurea is an oral medicine that has been shown to reduce or prevent several SCD complications.
This medicine was studied in patients with SCD because it was known to increase the amount of fetal hemoglobin (hemoglobin F) in the blood. Increased hemoglobin F provides some protection against the effects of hemoglobin S.
Hydroxyurea was later found to have several other benefits for a person with SCD, such as decreasing inflammation.
Use in adults. Many studies of adults with hemoglobin SS or hemoglobin S thalassemia showed that hydroxyurea reduced the number of episodes of pain crises and acute chest syndrome. It also improved anemia and decreased the need for transfusions and hospital admissions.
Use in children. Studies in children with severe hemoglobin SS or S thalassemia showed that hydroxyurea reduced the number of vaso-occlusive crises and hospitalizations. A study of very young children (between the ages of 9 and 18 months) with hemoglobin SS or hemoglobin S thalassemia also showed that hydroxyurea decreased the number of episodes of pain and dactylitis.
Who Should Use Hydroxyurea?
Since hydroxyurea can decrease several complications of SCD, most experts recommend that children and adults with hemoglobin SS or S0 thalassemia who have frequent painful episodes, recurrent chest crises, or severe anemia take hydroxyurea daily.
Some experts offer hydroxyurea to all infants over 9 months of age and young children with hemoglobin SS or S0 thalassemia, even if they do not have severe clinical problems, to prevent or reduce the chance of complications. There is no information about how safe or effective hydroxyurea is in children under 9 months of age.
Some experts will prescribe hydroxyurea to people with other types of SCD who have severe, recurrent pain. There is little information available about how effective hydroxyurea is for these types of SCD.
In all situations, people with SCD should discuss with their doctors whether or not hydroxyurea is an appropriate medication for them.
Pregnant women should not use hydroxyurea.
How Is Hydroxyurea Taken?
To work properly, hydroxyurea should be taken by mouth daily at the prescribed dose. When a person does not take it regularly, it will not work as well, or it wont work at all.
A person with SCD who is taking hydroxyurea needs careful monitoring. This is particularly true in the early weeks of taking the medicine. Monitoring includes regular blood testing and dose adjustments.
What Are the Risks of Hydroxyurea?
Hydroxyurea can cause the bloods white cell count or platelet count to drop. In rare cases, it can worsen anemia. These side effects usually go away quickly if a person stops taking the medication. When a person restarts it, a doctor usually prescribes a lower dose.
Other short-term side effects are less common.
It is still unclear whether hydroxyurea can cause problems later in life in people with SCD who take it for many years. Studies so far suggest that it does not put people at a higher risk of cancer and does not affect growth in children. But further studies are needed.
Red Blood Cell Transfusions
Doctors may use acute and chronic red blood cell transfusions to treat and prevent certain SCD complications. The red blood cells in a transfusion have normal hemoglobin in them.
A transfusion helps to raise the number of red blood cells and provides normal red blood cells that are more flexible than red blood cells with sickle hemoglobin. These cells live longer in the circulation. Red blood cell transfusions decrease vaso-occlusion (blockage in the blood vessel) and improve oxygen delivery to the tissues and organs.
Acute Transfusion in SCD
Doctors use blood transfusions in SCD for complications that cause severe anemia. They may also use them when a person has an acute stroke, in many cases of acute chest crises, and in multi-organ failure.
A person with SCD usually receives blood transfusions before surgery to prevent SCD-related complications afterwards.
Chronic Transfusion
Doctors recommend regular or ongoing blood transfusions for people who have had an acute stroke, since transfusions decrease the chances of having another stroke.
Doctors also recommend chronic blood transfusions for children who have abnormal TCD ultrasound results because transfusions can reduce the chance of having a first stroke.
Some doctors use this approach to treat complications that do not improve with hydroxyurea. They may also use transfusions in people who have too many side effects from hydroxyurea.
What Are the Risks of Transfusion Therapy?
Possible complications include:
Hemolysis
Iron overload, particularly in people receiving chronic transfusions (can severely impair heart and lung function)
Infection
Alloimmunization (can make it hard to find a matching unit of blood for a future transfusion)
All blood banks and hospital personnel have adopted practices to reduce the risk of transfusion problems.
People with SCD who receive transfusions should be monitored for and immunized against hepatitis. They should also receive regular screenings for iron overload. If a person has iron overload, the doctor will give chelation therapy, a medicine to reduce the amount of iron in the body and the problems that iron overload causes.
Hematopoietic Stem Cell Transplantation
At the present time, hematopoietic stem cell transplantation (HSCT) is the only cure for SCD. People with SCD and their families should ask their doctor about this procedure.
What Are Stem Cells?
Stem cells are special cells that can divide over and over again. After they divide, these cells can go on to become blood red cells, white cells, or platelets.
A person with SCD has stem cells that make red blood cells that can sickle. People without SCD have stem cells that make red cells that usually wont sickle.
What Stem Cells Are Used in HSCT?
In HSCT, stem cells are taken from the bone marrow or blood of a person who does not have sickle cell disease (the donor). The donor, however, may have sickle cell trait.
The donor is often the persons sister or brother. This is because the safest and most successful transplants use stem cells that are matched for special proteins called HLA antigens. Since these antigens are inherited from parents, a sister or brother is the most likely person to have the same antigens as the person with SCD.
What Happens During HSCT?
First, stem cells are taken from the donor. After this, the person with SCD (the recipient) is treated with drugs that destroy or reduce his or her own bone marrow stem cells.
The donor stem cells are then injected into the persons vein. The injected cells will make a home in the recipients bone marrow, gradually replacing the recipients cells. The new stem cells will make red cells that do not sickle.
Which People Receive HSCT?
At the present time, most SCD transplants are performed in children who have had complications such as strokes, acute chest crises, and recurring pain crises. These transplants usually use a matched donor.
Because only about 1 in 10 children with SCD has a matched donor without SCD in their families, the number of people with SCD who get transplants is low.
HSCT is more risky in adults, and that is why most transplants are done in children.
There are several medical centers that are researching new SCD HSCT techniques in children and adults who dont have a matched donor in the family or are older than most recipients. Hopefully, more people with SCD will be able to receive a transplant in the future, using these new methods.
What Are the Risks?
HSCT is successful in about 85 percent of children when the donor is related and HLA matched. Even with this high success rate, HSCT still has risks.
Complications can include severe infections, seizures, and other clinical problems. About 5 percent of people have died. Sometimes transplanted cells attack the recipients organs (graft versus host disease).
Medicines are given to prevent many of the complications, but they still can happen.
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Sickle Cell Disease
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How to prevent Sickle Cell Disease ?
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People who do not know whether they carry an abnormal hemoglobin gene can ask their doctor to have their blood tested.
Couples who are planning to have children and know that they are at risk of having a child with sickle cell disease (SCD) may want to meet with a genetics counselor. A genetics counselor can answer questions about the risk and explain the choices that are available.
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Sickle Cell Disease
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What is (are) Alpha-1 Antitrypsin Deficiency ?
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Alpha-1 antitrypsin (an-tee-TRIP-sin) deficiency, or AAT deficiency, is a condition that raises your risk for lung disease (especially if you smoke) and other diseases.
Some people who have severe AAT deficiency develop emphysema (em-fi-SE-ma)often when they're only in their forties or fifties. Emphysema is a serious lung disease in which damage to the airways makes it hard to breathe.
A small number of people who have AAT deficiency develop cirrhosis (sir-RO-sis) and other serious liver diseases.
Cirrhosis is a disease in which the liver becomes scarred. The scarring prevents the organ from working well. In people who have AAT deficiency, cirrhosis and other liver diseases usually occur in infancy and early childhood.
A very small number of people who have AAT deficiency have a rare skin disease called necrotizing panniculitis (pa-NIK-yu-LI-tis). This disease can cause painful lumps under or on the surface of the skin.
This article focuses on AAT deficiency as it relates to lung disease.
Overview
Alpha-1 antitrypsin, also called AAT, is a protein made in the liver. Normally, the protein travels through the bloodstream. It helps protect the body's organs from the harmful effects of other proteins. The lungs are one of the main organs that the AAT protein protects.
AAT deficiency occurs if the AAT proteins made in the liver aren't the right shape. They get stuck inside liver cells and can't get into the bloodstream.
As a result, not enough AAT proteins travel to the lungs to protect them. This increases the risk of lung disease. Also, because too many AAT proteins are stuck in the liver, liver disease can develop.
Severe AAT deficiency occurs if blood levels of the AAT protein fall below the lowest amount needed to protect the lungs.
AAT deficiency is an inherited condition. "Inherited" means it's passed from parents to children through genes.
Doctors don't know how many people have AAT deficiency. Many people who have the condition may not know they have it. Estimates of how many people have AAT deficiency range from about 1 in every 1,600 people to about 1 in every 5,000 people.
Outlook
People who have AAT deficiency may not have serious complications, and they may live close to a normal lifespan.
Among people with AAT deficiency who have a related lung or liver disease, about 3percent die each year.
Smoking is the leading risk factor for life-threatening lung disease if you have AAT deficiency. Smoking or exposure to tobacco smoke increases the risk of earlier lung-related symptoms and lung damage. If you have severe AAT deficiency, smoking can shorten your life by as much as 20 years.
AAT deficiency has no cure, but treatments are available. Treatments often are based on the type of disease you develop.
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Alpha-1 Antitrypsin Deficiency
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What causes Alpha-1 Antitrypsin Deficiency ?
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Alpha-1 antitrypsin (AAT) deficiency is an inherited disease. "Inherited" means it's passed from parents to children through genes.
Children who have AAT deficiency inherit two faulty AAT genes, one from each parent. These genes tell cells in the body how to make AAT proteins.
In AAT deficiency, the AAT proteins made in the liver aren't the right shape. Thus, they get stuck in the liver cells. The proteins can't get to the organs in the body that they protect, such as the lungs. Without the AAT proteins protecting the organs, diseases can develop.
The most common faulty gene that can cause AAT deficiency is called PiZ. If you inherit two PiZ genes (one from each parent), you'll have AAT deficiency.
If you inherit a PiZ gene from one parent and a normal AAT gene from the other parent, you won't have AAT deficiency. However, you might pass the PiZ gene to your children.
Even if you inherit two faulty AAT genes, you may not have any related complications. You may never even realize that you have AAT deficiency.
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Alpha-1 Antitrypsin Deficiency
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Who is at risk for Alpha-1 Antitrypsin Deficiency? ?
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Alpha-1 antitrypsin (AAT) deficiency occurs in all ethnic groups. However, the condition occurs most often in White people of European descent.
AAT deficiency is an inherited condition. "Inherited" means the condition is passed from parents to children through genes.
If you have bloodline relatives with known AAT deficiency, you're at increased risk for the condition. Even so, it doesn't mean that you'll develop one of the diseases related to the condition.
Some risk factors make it more likely that you'll develop lung disease if you have AAT deficiency. Smoking is the leading risk factor for serious lung disease if you have AAT deficiency. Your risk for lung disease also may go up if you're exposed to dust, fumes, or other toxic substances.
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Alpha-1 Antitrypsin Deficiency
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What are the symptoms of Alpha-1 Antitrypsin Deficiency ?
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The first lung-related symptoms of alpha-1 antitrypsin (AAT) deficiency may include shortness of breath, less ability to be physically active, and wheezing. These signs and symptoms most often begin between the ages of 20 and 40.
Other signs and symptoms may include repeated lung infections, tiredness, a rapid heartbeat upon standing, vision problems, and weight loss.
Some people who have severe AAT deficiency develop emphysema (em-fi-SE-ma)often when they're only in their forties or fifties. Signs and symptoms of emphysema include problems breathing, wheezing, and a chronic (ongoing) cough.
At first, many people who have AAT deficiency are diagnosed with asthma. This is because wheezing also is a symptom of asthma. Also, people who have AAT deficiency respond well to asthma medicines.
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Alpha-1 Antitrypsin Deficiency
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How to diagnose Alpha-1 Antitrypsin Deficiency ?
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Alpha-1 antitrypsin (AAT) deficiency usually is diagnosed after you develop a lung or liver disease that's related to the condition.
Your doctor may suspect AAT deficiency if you have signs or symptoms of a serious lung condition, especially emphysema, without any obvious cause. He or she also may suspect AAT deficiency if you develop emphysema when you're 45 years old or younger.
Specialists Involved
Many doctors may be involved in the diagnosis of AAT deficiency. These include primary care doctors, pulmonologists (lung specialists), and hepatologists (liver specialists).
To diagnose AAT deficiency, your doctor will:
Ask about possible risk factors. Risk factors include smoking and exposure to dust, fumes, and other toxic substances.
Ask about your medical history. A common sign of AAT deficiency is if you have a lung or liver disease without any obvious causes or risk factors. Another sign is if you have emphysema at an unusually early age (45 years or younger).
Ask about your family's medical history. If you have bloodline relatives who have AAT deficiency, you're more likely to have the condition.
Diagnostic Tests
Your doctor may recommend tests to confirm a diagnosis of AAT deficiency. He or she also may recommend tests to check for lung- or liver-related conditions.
A genetic test is the most certain way to check for AAT deficiency. This test will show whether you have faulty AAT genes.
A blood test also may be used. This test checks the level of AAT protein in your blood. If the level is a lot lower than normal, it's likely that you have AAT deficiency.
Lung-Related Tests
If you have a lung disease related to AAT deficiency, your doctor may recommend lung function tests and high-resolution computed tomography (to-MOG-rah-fee) scanning, also called CT scanning.
Lung function tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. These tests may show how severe your lung disease is and how well treatment is working.
High-resolution CT scanning uses x rays to create detailed pictures of parts of the body. A CT scan can show whether you have emphysema or another lung disease and how severe it is.
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Alpha-1 Antitrypsin Deficiency
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What are the treatments for Alpha-1 Antitrypsin Deficiency ?
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Alpha-1 antitrypsin (AAT) deficiency has no cure, but its related lung diseases have many treatments. Most of these treatments are the same as the ones used for a lung disease called COPD (chronic obstructive pulmonary disease).
If you have symptoms related to AAT deficiency, your doctor may recommend:
Medicines called inhaled bronchodilators (brong-ko-di-LA-tors) and inhaled steroids. These medicines help open your airways and make breathing easier. They also are used to treat asthma and COPD.
Flu and pneumococcal (noo-mo-KOK-al) vaccines to protect you from illnesses that could make your condition worse. Prompt treatment of lung infections also can help protect your lungs.
Pulmonary rehabilitation (rehab). Rehab involves treatment by a team of experts at a special clinic. In rehab, you'll learn how to manage your condition and function at your best.
Extra oxygen, if needed.
A lung transplant. A lung transplant may be an option if you have severe breathing problems. If you have a good chance of surviving the transplant surgery, you may be a candidate for it.
Augmentation (og-men-TA-shun) therapy is a treatment used only for people who have AAT-related lung diseases. This therapy involves getting infusions of the AAT protein. The infusions raise the level of the protein in your blood and lungs.
Not enough research has been done to show how well this therapy works. However, some research suggests that this therapy may slow the development of AAT deficiency in people who don't have severe disease.
People who have AAT deficiency and develop related liver or skin diseases will be referred to doctors who treat those diseases.
Future Treatments
Researchers are working on possible treatments that will target the faulty AAT genes and replace them with healthy genes. These treatments are in the early stages of development.
Researchers also are studying therapies that will help misshapen AAT proteins move from the liver into the bloodstream. They're also studying a type of augmentation therapy in which the AAT protein is inhaled instead of injected into a vein.
If you're interested in new treatments, ask your doctor about ongoing clinical trials for AAT deficiency.
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Alpha-1 Antitrypsin Deficiency
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How to prevent Alpha-1 Antitrypsin Deficiency ?
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You can't prevent alpha-1 antitrypsin (AAT) deficiency because the condition is inherited (passed from parents to children through genes).
If you inherit two faulty AAT genes, you'll have AAT deficiency. Even so, you may never develop one of the diseases related to the condition.
You can take steps to prevent or delay lung diseases related to AAT deficiency. One important step is to quit smoking. If you don't smoke, don't start.
Talk with your doctor about programs and products that can help you quit smoking. If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking.
For more information about how to quit smoking, go to the Health Topics Smoking and Your Heart article and the National Heart, Lung, and Blood Institute's "Your Guide to a Healthy Heart." Although these resources focus on heart health, they include basic information about how to quit smoking.
Also, try to avoid secondhand smoke and places with dust, fumes, or other toxic substances that you may inhale.
Check your living and working spaces for things that may irritate your lungs. Examples include flower and tree pollen, ash, allergens, air pollution, wood burning stoves, paint fumes, and fumes from cleaning products and other household items.
If you have a lung disease related to AAT deficiency, ask your doctor whether you might benefit from augmentation therapy. This is a treatment in which you receive infusions of AAT protein.
Augmentation therapy raises the level of AAT protein in your blood and lungs. (For more information, go to "How Is Alpha-1 Antitrypsin Deficiency Treated?")
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Alpha-1 Antitrypsin Deficiency
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What is (are) Primary Ciliary Dyskinesia ?
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Primary ciliary (SIL-e-ar-e) dyskinesia (dis-kih-NE-ze-ah), or PCD, is a rare disease that affects tiny, hair-like structures that line the airways. These structures are called cilia (SIL-e-ah).
Cilia move together in wave-like motions. They carry mucus (a slimy substance) toward the mouth to be coughed or sneezed out of the body. The mucus contains inhaled dust, bacteria, and other small particles.
If the cilia don't work well, bacteria stay in your airways. This can cause breathing problems, infections, and other disorders. PCD mainly affects the sinuses, ears, and lungs. Some people who have PCD have breathing problems from the moment of birth.
Sperm cells have structures that are like cilia. In men who have PCD, these structures also may not work well. This can cause fertility problems. "Fertility" refers to the ability to have children.
Fertility problems also occur in some women who have PCD. These problems likely are due to faulty cilia in the fallopian tubes. (The fallopian tubes carry eggs from the ovaries to the uterus.)
Overview
PCD is an inherited disease. "Inherited" means the disease is passed from parents to children through genes. With PCD, this process is very complex. Researchers are still learning how the disease is inherited and which genes are involved.
Generally, a child must inherit faulty genes from both parents to have PCD. These genes affect how cilia grow and function. Faulty genes may cause the cilia to be the wrong size or shape or move in the wrong way. Sometimes the cilia are missing altogether.
No single faulty gene causes all cases of PCD. Rather, many genes are associated with the disease.
If a child inherits a faulty gene (or genes) from only one parent, he or she may be a "PCD carrier." Carriers usually have no symptoms of PCD. However, carriers can pass faulty PCD genes on to their children.
The symptoms and severity of PCD vary from person to person. If you or your child has the disease, you may have serious sinus, ear, and/or lung infections. If the disease is mild, it may not show up until the teen or adult years.
The symptoms and severity of PCD also vary over time. Sometimes you may have few symptoms. Other times, your symptoms may become more severe.
Some people who have PCD have a condition called situs inversus (SI-tus in-VER-sus). This is a condition in which the internal organs (for example, the heart, stomach, spleen, liver, and gallbladder) are in opposite positions from where they normally are.
A correct and early diagnosis of PCD is very important. It will allow you or your child to get the proper treatment to keep your airways and lungs as healthy as possible. An early diagnosis and proper treatment also can prevent or delay lung damage.
Outlook
Many people who have PCD have normal lifespans. However, about 25 percent of people who have the disease may develop respiratory failure, a life-threatening condition. A small number of people who have PCD need lung transplants.
Scientists continue to study the faulty genes that cause PCD. Further studies of the disease will likely lead to earlier diagnoses, better treatments, and improved outcomes.
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Primary Ciliary Dyskinesia
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What causes Primary Ciliary Dyskinesia ?
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Primary ciliary dyskinesia (PCD) is a rare, inherited disease. "Inherited" means the disease is passed from parents to children through genes. With PCD, this process is very complex. Researchers are still learning how the disease is inherited and which genes are involved.
Generally, a child must inherit faulty genes from both parents to have PCD. These genes affect how cilia grow and function. Cilia are tiny, hair-like structures that line the airways.
The airways include your nose and linked air passages; mouth; larynx (LAR-ingks), or voice box; trachea (TRA-ke-ah), or windpipe; and tubes called bronchial tubes or bronchi, and their branches.
Cilia move mucus (a slimy substance) through your airways and toward your mouth to be coughed or sneezed out of your body. The mucus contains inhaled dust, bacteria, and other small particles.
Faulty genes may cause the cilia to be the wrong size or shape or move in the wrong way. Sometimes the cilia are missing altogether. If the cilia don't work well, bacteria stay in your airways. This can cause breathing problems, infections, and other disorders.
Primary Ciliary Dyskinesia
No single faulty gene causes all cases of PCD. Rather, many genes are associated with the disease.
If a child inherits a faulty gene (or genes) from only one parent, he or she may be a "PCD carrier." Carriers usually have no symptoms of PCD. However, carriers can pass faulty PCD genes on to their children.
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Primary Ciliary Dyskinesia
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