Abstract
An April 2010 consensus of clinicians from 22 centers in 18 countries reported 1,623 spinal muscular atrophy type 1, Duchenne muscular dystrophy, and amyotrophic lateral sclerosis noninvasive intermittent positive pressure ventilatory support users, of whom 760 developed continuous dependence that prolonged their survival by more than 3,000 patient-years without tracheostomies. Four of the centers routinely extubated unweanable patients with Duchenne muscular dystrophy, so that none of their more than 250 such patients has undergone tracheotomy. This article describes the manner in which this is accomplished; that is, the use of noninvasive inspiratory and expiratory muscle aids to prevent ventilatory failure and to permit the extubation and tracheostomy tube decannulation of patients with no autonomous ability to breathe (ie, who are “unweanable” from ventilator support). Noninvasive airway pressure aids can provide up to continuous ventilatory support for patients with little or no vital capacity and can provide for effective cough flows for patients with severely dysfunctional expiratory muscles.