Breath stacking in children with neuromuscular disorders

Abstract

Respiratory muscle weakness in neuromuscular disorders (NMD) can lead to shallow breathing and respiratory insufficiency over time. Children with NMD often cannot perform maneuvers to recruit lung volume. In adults, breath stacking with a mask and one-way valve can achieve significantly increased lung volumes. To evaluate involuntary breath stacking (IBS) in NMD, we studied 23 children of whom 15 were cognitively aware and able to communicate verbally. For IBS, a one-way valve and pneumotachograph were attached to a face mask. Tidal volumes (Vt) and minute ventilation (VE ) were calculated from airflow over 30 sec before and after 15 sec of expiratory valve closure. Six cooperative male subjects with Duchenne muscular dystrophy (DMD) participated in a subsequent comparison of IBS with voluntary breath stacking (VBS) and supported breath stacking (SBS). The average Vt in those studied with IBS was 277 ml (range 29-598 ml). The average increase in volume by stacking was 599 ml (range -140 to 2,916 ml) above Vt . The average number of stacked breaths was 4.5 (range 0-17). VE increased on average by 18% after stacking (P < 0.05, paired t-test). Oxygen saturation did not change after stacking. Four of the 23 children did not breath stack. Compared to IBS, VBS achieved similar volumes in the six subjects with DMD but SBS was more successful in those with greatest muscle weakness. IBS may achieve breath volumes of approximately three times Vt and may be particularly useful in non-cooperative subjects with milder degrees of respiratory muscle weakness.