Physical management of patients with neuromuscular diseases

Abstract

In the current economic climate in which it is no longer practical for a patient with neuromuscular disease to routinely be evaluated by specialists in orthopedics, cardiology, pulmonary medicine, neurology, nutrition, physical and occupational therapy, speech and language pathology, and physiatry, a neuromuscular disease practitioner needs to be capable of understanding and managing 90 % of these domains. Patients' ambulation can often be prolonged by judiciously eliminating lower limb contractures, repositioning muscles, targeted physical therapeutic interventions, and medical therapy. Children with spinal muscular atrophy type 1 (SMA1) can live into adulthood without tracheostomy tubes despite requiring continuous ventilatory support from 4 months of age. Patients with Duchenne muscular dystrophy (DMD) can live into their late 40s without tracheostomy tubes despite continuous ventilator dependence for over 25 years. Twenty-five to over 40 % of amyotrophic lateral sclerosis (ALS) patients can survive for an average of 1 and up to 8 years without tracheostomy tubes despite requiring continuous ventilatory support. All of these advances require knowledge of skeletal and respiratory muscle interventions. While critical for quality of life and to prolong life, they are little understood by the medical community.