Noninvasive Respiratory Management of Patients With Neuromuscular Disease

Abstract

This review article describes definitive noninvasive respiratory management of respiratory muscle dysfunction to eliminate need to resort to tracheotomy. In 2010 clinicians from 22 centers in 18 countries reported 1,623 spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis users of noninvasive ventilatory support (NVS) of whom 760 required it continuously (CNVS). The CNVS sustained their lives by over 3,000 patient-years without resort to indwelling tracheostomy tubes. These centers have now extubated at least 74 consecutive ventilator unweanable patients with DMD, over 95% of CNVS-dependent patients with SMA1, and hundreds of others with advanced neuromuscular disorders (NMDs) without resort to tracheotomy. Two centers reported a 99% success rate at extubating 258 ventilator unweanable patients without resort to tracheotomy. Patients with myopathic or lower motor neuron disorders can be managed noninvasively by up to CNVS, indefinitely, despite having little or no measurable vital capacity, with the use of physical medicine respiratory muscle aids. Ventilator-dependent patients can be decannulated of their tracheostomy tubes.

Keywords: Assisted cough; Glossopharyngeal breathing; Intermittent abdominal pressure ventilator; Mechanical insufflation-exsufflation; Neuromuscular diseases; Noninvasive ventilatory support.

Fig. 1. A 61-year-old man with ALS dependent on sleep nasal noninvasive ventilatory support since 2014 and on daytime NVS via 15 mm angled mouthpiece as well, seen here, since February 2016.

Fig. 2. A 68-year-old woman, spinal cord injured at birth, began sleep noninvasive ventilatory support via lipseal, seen here, in 1977 and began to require daytime mouthpiece NVS in 2004 and continuous NVS since 2010.

Fig. 3. Twenty- and 19-year-old brothers with severe spinal muscular atrophy type 1, continuously dependent on noninvasive ventilatory support since 8 and 4 months of age, respectively, with no measurable vital capacities. The patients have received all nutrition via nasogastric tubes placed at 4 months of age. They drool but do not aspirate.

Fig. 4. Changes in vital capacity (VC), maximum insufflation capacity, and glossopharyngeal maximum single breath capacity over a 20 year period for a patient with Duchenne muscular dystrophy. The GPB, added to his autonomous tidal volumes, permitted the patient to breathe without requiring full ventilatory support until 43 years of age despite having a VC of 20 mL from age 42. VC, vital capacity; MIC, maximum insufflation capacity; GPmaxSBC, glossopharyngeal maximum single breath capacity.

Fig. 5. The intermittent abdominal pressure ventilator (BachBelt, Dima Italia Inc., Bologna, Italy), seen here, is normally worn under clothing and, therefore, optimizes appearance.