Pulmonary complications of neuromuscular disease: A Respiratory mechanics perspective

Summary 

Paediatric neuromuscular disease compromises both the gas exchange and pump functions of the respiratory system. This can have profound implications for both growth and development of the respiratory system, as well as morbidity and mortality. Aspiration lung disease is common, and leads to increasingly restrictive pulmonary physiology over time. Abnormal lung and chest wall mechanics, and weak respiratory muscles, can combine to cause respiratory failure. Improving the balance between the work of breathing (by decreasing the respiratory load) and the respiratory pump (by improving respiratory muscle strength and decreasing respiratory muscle fatigue) can help prevent the onset of respiratory failure. Airway clearance techniques and non-invasive ventilation are two important tools in this effort. Better ways of assessing the respiratory pump, mechanical function, control and fatigue are needed especially in children.

Keywords: neuromuscular disease, respiratory mechanics, respiratory failure, lung compliance, chest wall compliance, diaphragm, respiratory muscle strength, respiratory muscle fatigue, pulmonary function tests, maximal respiratory pressures, tension-time index, respiratory muscle training, nocturnal ventilation