Respiratory care in neuromuscular disease

Abstract

A structured approach to respiratory care improves outcomes, including symptom control, quality of life, and survival, in patients with neuromuscular diseases such as amyotrophic lateral sclerosis and Duchenne muscular dystrophy. Assessment of respiratory function should include bulbar function, respiratory muscle function, and cough effectiveness. Chest physiotherapy techniques and cough-assist devices help in the clearance of secretions. Supportive treatments such as non-invasive ventilation and gastrostomy feeding improve symptoms, survival, and quality of life. Early involvement of palliative care clinicians assists with advance care planning and provision of optimal end of life care in a setting of choice including hospital, home, or hospice. Symptoms such as breathlessness, choking, and pain can be controlled using treatments such as oxygen, sputum clearance techniques, opioids, -benzodiazepines, and hyoscine.