Respiratory muscle aids to avert respiratory failure and tracheostomy: A new patient management paradigm

Abstract

The purpose of this article is to describe the use of noninvasive inspiratory and expiratory muscle aids to prevent ventilatory insufficiency and failure, and to permit the extubation and tracheostomy tube decanulation of "unweanable" patients. Noninvasive airway pressure aids can provide up to continuous ventilator support for patients with little or no vital capacity and can provide for effective cough flows for patients with severely dysfunctional expiratory muscles. An April 2010 consensus of clinicians from 20 centers in 14 countries reported over 1500 spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis (ALS) patients who survived using continuous ventilatory support without tracheostomy tubes. Four of the centers routinely extubated unweanable DMD patients so that none of their over 250 such patients has undergone tracheotomy. This approach is now making inroads into Canada via centers in Ottawa and Montreal.