Night ventilation by body respirators for patients in chronic respiratory failure due to late stage Duchenne muscular dystrophy

Abstract

A program of respiratory muscle sparing in 9 patients with late stage Duchenne muscular dystrophy is presented. Results indicate that regular efficient night-time support by body respirator provides a constant significant improvement in daytime gas exchange for periods averaging up to 2 years following the occurrence of moderately severe respiratory failure. Until now, the longest survival is more than 4 years at the age of 28. the average PaCO2 and PaO2 before night ventilation were 60.8mmHg and 59.3mmHg, respectively. The levels after the program was begun were 45.5 (PaCO2) and 74.6 (PaO2). These arterial blood gas levels are acceptable for good cardiopulmonary homeostasis. There have been no deaths or episodes of cardiac failure since the program began. The ease of integrating the program into the patient's lifestyle and home are important advantages. Cost of equipment is discussed. The view that these patients are terminal must be qualified. A case is also made for early aggressive conservative measures including body respirators, postural drainage, chest physiotherapy with abdominal assisted coughing, intratracheal suctioning and bronchoscopy to combat respiratory infection, and decreasing the need of tracheostomy and volume ventilators which can be so disabling to the chronic restrictive lung disease (CRLD) patient. Research and development into better design of body respirators is vitally needed. Acute care hospitals must prepare themselves for such patients who now attain more independent living and longer lifespan. For this reason there is a growing need for these facilities to acquire tank ventilators for handling such patients who may need hospitalization for acute medical complications.