The role of the respiratory physiotherapist in the management of Duchenne muscular dystrophy.

Abstract

Patients with Duchenne muscular dystrophy (DMD) are living longer, mostly due to the combined contribution of corticosteroids and advances in cardiopulmonary interventions. However, respiratory complications are a major cause of morbidity and unplanned hospital admissions. While the widespread use of noninvasive ventilation has reduced respiratory mortality, it remains a major contributor to death in DMD, alongside cardiac causes. There is an expanding pipeline of therapies aimed at restoring dystrophin function or compensating for dysfunction in development; however, there is currently no curative treatment for DMD. Respiratory failure is inevitable as this disease progresses; therefore, maintaining respiratory health is essential to prolonging survival and quality of life in this patient population. Respiratory physiotherapy should be considered if there are concerns about cough strength, ability to manage respiratory secretions or frequent chest infections. Techniques such as breathstacking and lung volume recruitment can help to offset the effects of inspiratory muscle weakness, and assisted coughing can help to correct the effects of expiratory muscle weakness. Mechanical insufflation-exsufflation can maximise cough effectiveness in the presence of both inspiratory and expiratory muscle weakness. Treatments should be anticipatory and proactive in approach to slow the decline of respiratory muscle function and aim to prevent respiratory morbidity. Given the time spent with patients assessing and introducing therapies, physiotherapists are uniquely placed to work with patients and their families and caregivers in developing positive relationships between them and the interventions employed. It is imperative that patients are empowered to be involved in the introduction of these therapies, as adherence with noninvasive ventilation and airway clearance strategies needs to be lifelong and is essential to their success.