Respiratory Issues in the Management of Children With Neuromuscular Disease

Most children with neuromuscular disease eventually require assistance with airway clearance and with breathing, especially during sleep. Techniques and devices for airway clearance and noninvasive ventilation that are commonly used in adults have been successfully adapted for use in infants and young children. Both physiological differences and small size of young patients with neuromuscular disease, however, can limit the applicability of such interventions or require special consideration. Measurements to identify the appropriate time to begin airway clearance assistance are lacking for young children, and the role of early introduction of noninvasive ventilation to preserve or enhance lung growth and chest-wall mobility remains to be elucidated. The paucity of nasal interfaces and headgear commercially made for small patients can reduce patient tolerance of noninvasive ventilation and exacerbate patient-ventilator dyssynchrony. Despite these issues, a greater number of children with neuromuscular diseases are living well past their second decade. Strategies to transition these patients to appropriate adult-care providers, to secure cost-effective health care for them, and to help integrate them into adult society must be developed.