PMC4153236_01
Patient with typical spinal muscular atrophy type 1 using continuous noninvasive ventilatory support via nasal interface.
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Currently, at age 18, she has a VC of 120 ml (2% of normal), no measurable cough flows, and requires high span bi-level PAP continuously via nasal interfaces (Figure 1) with less than 2 minute periods free from it before respiratory distress and blood gas derangement necessitate return to ventilatory support.