A defining criterion of Tourette is symptoms-involuntary tics-that start between age 2 and age 15. Anyone who has Tourette first got it as a child, probably during school, when peers can be ruthless to anyone out of the mainstream. Recent studies show that the greatest frequency and severity of tics occurs for most children around middle school. It's a terrible time to be twitching and barking.
Yet the roughly 1 percent of students who have Tourette syndrome are, aside from their tics, no different than other students. The condition has no impact on intelligence or athletic ability. Even tics themselves are not unique; some 20 to 25 percent of children develop a physical tic or tics during their school years that, in most cases, go away. When the symptoms last more than a year, and audible tics join the physical ones, it's probably Tourette syndrome-TS to the cognoscenti.
By E.H. Santiago in the Long Island Press. Used by permission.
Tourette syndrome is probably the comedy world's all-time favorite affliction, because while obscenity is always funny, involuntary obscenity is hilarious.
The world's first known case, first described back in 1825, involved a fancy and influential French noblewoman, the Marquise de Dampierre, who lived in horror of her own eruptions into vulgar blasphemy among high society. Comedy gold. Right?
terrible time to be twitching and barking.
Of four children with Tourette syndrome contacted for this story, only one was willing to be named-even though three of the four are "out" among their peers, to varying degrees. One parent of two children with TS says that both were open in talking about the condition with their peers in grade school, but that "around fifth grade they got more private."
Matthew Hirsch, of Massapequa, is 10 years old. He plays baseball and basketball and football. He enjoys video games. He also makes funny noises.
Diagnosed at a relatively early age, Matt is intimately aware of his Tourette ups and downs.
"In first, second grade it started out pretty bad, I'd get most of the attention and it felt kind of weird," he says. "It was harder to make friends and I had a little trouble with that, but it's okay now."
Matt's TS could hardly be more public. "The whole grade now knows, they're used to it," he says. He was featured this month in an HBO special called "I Have Tourette's But Tourette's Doesn't Have Me." He notes with some chagrin that his part was relatively small. He had been up for the narrator's role, but at the time of filming he "wasn't tic-ing all that much"-not enough for the producers, at least.
The Hirschs, according to his mother Cheryl, were "extremely fortunate" to find "wonderful, very supportive" staff at Unqua Elementary School. "My son has had a string of great teachers," she says, confirming that Lucille Marandino, who Matt singled out, was wonderful. But not every school district has the resources or understanding needed to handle TS students.
Jane Smith (not her real name), as her father Tom describes her, was an ordinary kid right up until the summer before starting sixth grade.
"She went to bed one night and woke up tic-ing away," Tom Smith says. In retrospect, he remembered that there had been episodes of blinking and stuttering when Jane was 7 years old, but they had passed.
Ever since then, at the beginning of each school year, the Smiths put together information packets on Tourette for each of their daughter's teachers. Still, the school experience has been a mixed bag. There is some affectionate teasing, but there has also been taunting and bullying. And that's just the students.
"The kids are better than some teachers," Tom says. The lack of support from the adults they're supposed to turn to can be more confounding to TS children than teasing itself. At the same time, when it comes to TS, ignorance prevails.
"We feel very sorry for teachers," says Judit Ungar, president of the Tourette Syndrome Association, Inc. (TSA) "They have 30 to 35 kids in a class, many with special needs, and they don't often have the knowledge they need to deal with Tourette syndrome."
Whether or not comedians and the media are at fault, Tourette is widely misunderstood. Outbursts of obscenity like the Marquise's, technically called coprolalia, is one of the rarest symptoms.
Many adults believe that the behaviors can be controlled, that the student can "just stop" if she or he wants to. One Long Island teen, who we'll call Tina because her classmates do not know about her condition, was at a camp event one summer, and her head was twitching. A counselor came over and told her to stop because it was distracting to the kids behind her. When she did not, the counselor repeated the instruction.
"I can't," the girl replied, "I have Tourette syndrome."
"No you don't," said the counselor, grabbing her head to hold it still. "Stop making fun of disabled people."
Not just the lay public, but even doctors are confused. Tourette syndrome is easily misdiagnosed. There is no lab test or diagnostic image that reveals it. Patients are often able to temporarily suppress a tic until the doctor looks away or even until they get home. Some patients have learned coping mechanisms, such as disguising tics with an apparently purposeful movement, such as brushing aside hair. Tics that involve sniffing or blinking may suggest allergies. And tics are characteristic of many conditions, including mental retardation, autism and even simple nervousness.
If the world is ill informed, Long Island is headquarters of the effort to change that. In 1972, TSA was incorporated in Bayside, Queens. TSA is a national nonprofit that promotes both public and scientific understanding of Tourette syndrome through educational outreach, publishing, political lobbying and funding research. It was founded at the kitchen table of Eleanor and Bill Perle by three Long Island families who had children with Tourette and who figured they couldn't be the only ones. At the grassroots level, Jen Zwilling of Brookville is co-founder of the TSA's National Youth Ambassador Program, which sends students with Tourette to other schools to talk about their condition. Long Island writer Marcia Byalick, who has a friend whose child has TS, tried to reach the student audience with a young adult novel about a teen with TS. And parents are right behind the TSA in promoting awareness.
"My personal mission is to educate people about this," says one mother. "I worry that he'll always be 'that weird kid.'"
Tourette syndrome is characterized by multiple physical and audible tics that first appear during childhood or the early adolescent years, wax and wane, and last more than a year. Typically, it starts with a simple facial twitch, which is replaced or supplemented by other, perhaps more complex, twitches involving the body. Auditory tics typically start later, and can begin with something as innocuous as coughing, eventually progressing to barking, full sentences or coprolalia.
Tourette spasms aren't constant. They come and go over time in fractal patterns. Although they often sleep badly, TS patients don't normally tic in their sleep. In 1995, Manhattan neurologist Oliver Sacks described a Canadian surgeon with severe TS, whose tics disappeared in the operating theater. Hence the tics can, to a degree, be controlled.
"Sometimes I try to hold it in," says Matt Hirsch. "If I'm really focused and concentrating then it doesn't happen."
Adult TS patients describe the approach of a tic as an urge that grows more compelling, like a tickling or itch that demands to be scratched-and the only relief is the tic. While many patients, especially as they mature, are able to control their tics temporarily, eventually they've got to release the building tension. Teens who spend all day suppressing their involuntary spasms may come home and explode with all the day's tics at once.
Just like toddlers have to learn the signs that mean potty time, children with TS are often not aware of the sensation that precedes the tic. They may not even be aware that they act in an unusual way at all. Hence younger children have a much harder time disguising their symptoms.
reading, reading." When Tina was still tic-ing a year later, her neurosurgeon made the diagnosis of TS.
In her grade school days, Tina's peers were not kind. They would say, "What are you doing? Stop that." As she grew older, Mary became more aware of her tics and better able to control or disguise them. Now an adolescent, most of her tics are invisible-spasms in the stomach or body parts that are covered by clothes. She is a poised, articulate, athletic, social middle schooler-and none of her peers know she's got TS. But they do sometimes joke about Tourette, usually with some "%$*##," in the lunchroom.
The "typical" case doesn't begin to address the enormous range of TS, which is the most severe of tic disorders. In addition to coprolalia, there is echolalia, the repeating of another's words, and echopraxia, the aping of another's movements. And some tics are far from the simple eyebrow spasm. Jane Zwilling, vice chair of the L.I. chapter of the TSA, recalls one young middle-school boy (boys suffer tic disorders at least three times as much as girls) whose TS induced him to periodically stand up in class and shout, "I've got a chicken in my pants!"
It's easy to see why some teachers might be inclined to see that as "acting up." Yet that boy's tic is no more voluntary than a simple eye blink, just more elaborate.
"There are always teachers who say, 'They could stop if they wanted to,'" Tina's mother says.
significant impact on their ability to lead normal lives. Other conditions, such as obsessive-compulsive behavior and sleep disorders, but particularly attention deficit hyperactivity (ADHD), often come with Tourette. Tics can interfere with studying; an eye tic, for example, can make it hard to read. The teasing and bullying of peers leaves many TS children with poor self-esteem, and many adults, like Tina's camp counselor, aren't much help. Dealing with the tics all day, even if they're not visible to others, can be physically exhausting.
Tourette is not one of the disabilities covered by the federal Individuals with Disabilities Education Act (IDEA), but the TSA and other advocates are working to change that. In the meantime, some states, including New York, do include TS in the category of "other health impaired." Tourette-afflicted children can also get some accommodations in school via Section 504, a federal civil rights law requiring that impaired students receive special education services.
"In some cases, a TS child could be better served by a 504," explains John Lynch, Executive Director of Pupil Services for the Northport-East Northport district, where each school has periodic "Disability Awareness Weeks" to promote sensitivity among the students. He notes that they have to be especially careful not to compromise the confidentiality of any one student.
"[TS] is not that prevalent, so you probably would have people who require some training," he says, comparing Tourette to autism a few years ago. "When [autism] first came out, no one really knew much about it. It's only in the last five to ten years that it has really come [into the public consciousness]."
That's why the TSA makes such strenuous efforts to spread the word. Each year, the group hosts a free, full-day conference for educators. This year's will be held on May 19 at the Long Island Marriott in Uniondale. The group also has touring educators who talk at schools across the country. The youth ambassador program is working to recruit even more students to speak about the condition. Even the medical community gets re-education from the TSA.
"Tourette syndrome is one of thousands of illnesses, disorders, syndromes [doctors] learn about," says Ungar. "So they don't get much time on each one."
What causes Tourette syndrome? Researchers still aren't certain-the word "syndrome" applies to a set of symptoms occurring together from a common cause-but they are closing in on some answers. A neurological disorder that is sometimes inherited, TS appears to interfere with the processing of dopamine and serotonin in the basal ganglia of the brain. Last October, Yale University researchers claimed to have identified a gene that causes Tourette when mutated. But even that is considered only a partial answer, since it appears to apply only to some cases of TS.
Given the confusing nature of TS manifestations, it's no surprise that just about every treatment imaginable has been tried. Georges Gilles de la Tourette, the 19th century French physician whose descriptions of patients with these symptoms led to the condition being named for him, was an early practitioner of what we now call alternative medicine, such as suspension, vibration and hypnotherapy. More recent treatments for TS have included, most commonly, psychotherapy, group therapy, hypnosis, bio-feedback, chiropractic, diets, vitamins, herbs, and, of course, many, many drugs.
Most of these treatments are useless, although some drugs have proven effective in easing symptoms-often by as much as 90 percent, according to the TSA. Haloperidol (Haldol) works for 60 to 90 percent of patients, the group says. Similar, less well-known pharmaceuticals such as clonidine, guanfacine, risperidone, ziprasidone, clonazepam, and selective serotonin reuptake inhibitors (SSRIs) can also reduce tics. Even Botox has proven useful.
But none of these drugs was developed for TS. All are for other ailments-depression, high blood pressure or anxiety. Furthermore, the drugs used to treat TS aren't specifically approved for use in children. Dosage is more art than science, with even children of similar age and weight having different reactions.
Even so, Dr. Samuel H. Zinner, assistant professor of pediatrics and developmental-behavioral pediatrician at the University of Washington Center on Human Development and Disability, recommends that physicians treating Tourette periodically taper off and/or discontinue medication. It's both empowering and reassuring for the children and their families, he believes, and, because the tics come and go over time, it makes sense from a medical standpoint.
Tom Smith says that his daughter takes anti-hypertensive medication, but in a very low dose. "I'm not really a drug person," he says. "And the tics wax and wane naturally, so you don't even know if the medications are helping."
Just this month, researchers at Britain's University of Nottingham published results that suggest Tourette patients have more, not less, neurological control than the general population. Lead researcher Dr. Georgina Jackson hypothesized that this is because TS patients develop exceptional skills in suppressing involuntary movements through sheer brain power.
Tourette isn't fatal, it can be treated, and it doesn't have to interfere with normal life.
"It's not as much of a challenge as it used to be," says Matt Hirsch, who seems more mature than your usual 10-year-old. "All my friends, when I talk about it now, they say 'Oh yeah, I forgot you have that.'"
The TSA (718-225-2999; www.tsa-usa.org) has volumes of information, and video clips showing some common tics. Parents who suspect their child has Tourette should consult a pediatric neurologist.
By E.H. Santiago in the Long Island Press. Used by permission. Article courtesy of the Long Island Press. Long Island Press & LongIslandPress.com, All rights reserved.