Respiratory Care of the Individual with Muscular Dystrophy

This chapter pertains to respiratory care of the individual with muscular dystrophy. It discusses clinical presentation, disease progression, and available treatments for these conditions. Duchenne muscular dystrophy, the most common and best studied, is presented as the primary model. Cardiorespiratory problems are the primary causes of morbidity and mortality in muscular dystrophy. Pulmonary complications are related to respiratory muscle weakness, which results in a weak cough that leads to impaired airway clearance, predisposing to atelectasis and pneumonia, as well as sleep disordered breathing, with first nocturnal and then diurnal hypoventilation. Multi-system co-morbidities, including cardiac, musculoskeletal, nutritional, and orthopedic, can all further impact the respiratory system in muscular dystrophy, and therefore management by a multi-disciplinary team is needed to recognize and address these problems. Clinical assessment by history and physical examination is limited in its ability to identify pulmonary complications, and therefore a high index of suspicion is needed. Pulmonary function testing and polysomnography are the main diagnostic tools used to identify and monitor respiratory issues. Respiratory management consists of prevention with vaccinations, the use of assisted (usually non-invasive) ventilation to treat hypoventilation, and airway clearance techniques. These treatments have been shown to increase survival and well-being for individuals with muscular dystrophy.