Peak cough flow in children and young people with spinal muscular atrophy types II and III

ABSTRACT Spinal muscular atrophy is a neurodegenerative disorder, which may be associated with progressive respiratory failure. Our aim is to describe the peak cough flow of children and young people with spinal muscular atrophy types II and III. This is a descriptive, cross-sectional study conducted at a neuropediatrics outpatient clinic between March 2011 and May 2012, with patients with spinal muscular atrophy types II and III, and aging more than 5 years. Out of the 53 eligible patients, 21 participated in the research. The measurement of peak cough flow was carried out through the peak flow meter, with patients sitting and lying down. After taking three measures, we selected the one with the highest value among them. Type-III individuals reached peak cough flow values higher than those of type-II individuals. Measures taken in the sitting position (SMA II 159.4 l/min; SMA III 287.9 l/min) were higher than those measured in the lying position (SMA II 146.9 l/min; SMA III 257.5 l/min), with significant difference (p-value=0.008 in sitting position, and p=0.033 in lying position). We concluded that individuals with SMA III manifest higher PCF, especially when sitting, in comparison with SMA II.