Approach to the Pediatric Patient with Respiratory Complications of Neuromuscular Disease

Childhood neuromuscular diseases, while heterogeneous in timing of onset and rate of progression, can lead to similar types of morbidity and mortality seen in adults with neuromuscular weakness. This chapter reviews how distinctive aspects of the developing respiratory system can exacerbate the effects of neuromuscular weakness, and how the presence of neuromuscular weakness alters growth and development of the respiratory system. The most common types of respiratory assessments for patients with neuromuscular disease are also examined, with special emphasis on the challenges and limitations of such testing in children. Usual and novel interventions for patients with neuromuscular disease are also assessed, highlighting the unique considerations required for children.