Ventilator management in Duchenne muscular dystrophy and postpoliomyelitis syndrome: twelve years' experience

The ventilator management protocol followed over the last 12 years in 23 patients with Duchenne muscular dystrophy (DMD) and six polio survivors with chronic respiratory failure (CRF) secondary to the late effects of poliomyelitis or postpolio syndrome (PPS) is reviewed. After the onset of respiratory failure, patients with DMD continued to show a classic course of progressive, generalized muscle weakness and a steadily declining vital capacity from an average of 482mL to 336mL. The DMD group required an average increase of 0.95 hours in their daily use of assisted ventilation per year. Their overall average length of survival was increased from 19 years 9 months to 25 years 9 months. Members of the postpolio group, to date, have shown no significant decrease in muscle strength nor have they needed more than nocturnal ventilation. Recommended evaluation and pulmonary follow-up for patients with CRF secondary to neuromuscular disease is outlined. Most of these patients can be managed for a number of years with body ventilators before a tracheotomy is necessary.