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

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 patientyears 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.

Sleep Transcutaneous vs. End-Tidal CO2 Monitoring for Patients with Neuromuscular Disease

“…When pulmonary function is essentially normal, symptoms may also be attributed to sleep-disordered breathing for which continuous or bilevel positive airway pressure may be adequate rather than noninvasive ventilatory support. 18 Diurnal CO 2 was normal and sleep PetCO 2 maximums were less than 49 mm Hg, yet sleep PtcCO 2 was greater than 49 mm Hg for the 19 patients of group 2. Therefore, these 19 (48.7%) patients would not have been considered to have possibly been hypercapnic had only PetCO 2 monitoring been performed.…”

Physical Medicine Interventions to Avoid Acute Respiratory Failure and Invasive Airway Tubes

“…This noninvasive ventilation was accomplished through use of simple mouthpieces attached to the tubing of portable positive pressure ventilators. Many of these patients have now been using diurnal mouthpiece noninvasive intermittent positive pressure ventilatory support (NVS) along with nasal or lip cover NVS for sleep for continuous NVS (CNVS) for more than 60 years with no respiratory hospitalizations [6]. Indeed, 30% of our patients with Duchenne muscular dystrophy (DMD) have become dependent on CNVS for up to 30 years without being hospitalized [7].…”

Mouthpiece ventilation and complementary techniques in patients with neuromuscular disease: A brief clinical review and update

“…Its requirement for NMD patients without bulbar impairment is unproven regardless of severity of ventilatory failure. [28][29][30][31] Rationale for the use of ventilatory support with a mouthpiece Nasal and oronasal interfaces are the most commonly used interfaces for NVS. [14][15][16] They allow ventilation through the nose and/or nose and mouth, and they are suitable interfaces if the patient is not claustrophobic or has any facial pressure sores.…”

“…However, its effectiveness in improving long-term survival has been documented in a series of more than 700 NMD patients who required continuous ventilatory support. 29,34 There are no published evidence-based guidelines concerning mouthpiece ventilation (MPV). Its application is mainly based on the experience of few centers.…”