Abstract

Prolonged ventilatory support has been used to treat 51 patients with respiratory failure secondary to skeletal (22) or neuromuscular (29) disease. Symptomatic relief was achieved in five patients with rapidly progressive neurological disease who died within 27 months. The remaining 46 patients, aged 11-69 years at presentation, have been followed for more than two and a half years. All but 10 were treated with negative pressure ventilation from the outset, intermittent positive pressure ventilation being used initially in the others and continued at home in three. Nocturnal negative pressure ventilation has been used at home by 39 patients. A permanent tracheostomy has been maintained in 14, to facilitate positive pressure ventilation in three and to circumvent upper airway obstruction during sleep in 11. Sustained improvement in symptoms and arterial blood gas tensions has been maintained, independence and the capacity for gainful employment have been regained in those of an appropriate age, and the incidence of subsequent hospital admissions has been low. Neither the mode of presentation nor the aetiology of the restrictive ventilatory defect influenced outcome in patients with stable or only slowly progressive primary disease.