Respirator use in progressive neuromuscular diseases

A survey was conducted to acquire information on the current pattern of respiratory device usage for patients with progressive neuromuscular diseases. Questionnaires were sent to 240 directors of Muscular Dystrophy Association (MDA) clinics. Of the 132 respondents, 32 (24%) physicians provide no respiratory support systems, 44 (33%) prescribe such systems routinely, and 56 (42%) provide the devices under specialized circumstances. A wide variety of negative and positive pressure ventilators are employed for patients having diseases such as amyotropic lateral sclerosis, Duchenne muscular dystrophy, spinal muscular atrophy. In the MDA clinics responding, 495 patients were found to be receiving some form of assisted ventilation. Of that number, 214 (43%) have permanent tracheostomies. We conclude that ventilators are being supplied to individuals with progressive neuromuscular disorders throughout the USA. However, there appear to be no standardized patient selection process or established protocol for respirator use in such cases.