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.

Respiratory Involvement in Patients with Neuromuscular Diseases: A Narrative Review

“…However, other authors have criticized this strategy as inadequate to effect complete respiratory muscle rest. ese experts prefer the term "noninvasive ventilatory support (NVS)" to emphasize the role of this modality in substituting the respiratory muscles in the work of breathing [5,6]. eir alternative approach does not employ sleep studies (e.g.…”

“…polysomnography) for NIV initiation and titration. In fact, it consists of initially implementing NVS during sleep based on reduced supine VC and symptoms of sleep hypoventilation (fatigue, morning headaches, somnolence etc) using either tidal volumes at the range of 0.8-1.5 L in the volume-preset mode or high-span drive pressures institution of nocturnal NIV [6,16,20,88]. Apart from hypoventilation, the detection of upper airway obstructive events before or a er the initiation of NIV might also be of prognostic significance in certain diseases, e.g.…”

“…In addition, patients with NMDs are prone to developing various other respiratory complications such as infections, atelectasis, and aspiration syndromes [1,2]. e introduction of ventilatory support in the early stages of symptomatic sleep hypoventilation, especially in the form of noninvasive ventilation (NIV), as well as various other advances in respiratory care have contributed signi cantly to the improvement of survival and the quality of life of patients with NMDs [3][4][5][6].…”

Is “Noninvasive Ventilation” the Way to Prevent Respiratory Failure in Amyotrophic Lateral Sclerosis?

“…It noted that noninvasive ventilation, which has come to mean continuous positive airway pressure and bilevel positive airway pressure at less than ventilatory support settings, relieves symptoms and improves quality of life without even considering up to continuous noninvasive ventilatory support (CNVS) to prolong life for patients with ALS for, in some cases, over 10 years of CNVS. 2,3 It stated that "patients die, on average, within 3 to 5 years of diagnosis, unless they choose to undergo tracheotomy, in which case they may live, on average, two additional years" ignoring the fact that 25% to 45% of patients with ALS can survive using up to CNVS for an average of over 1 year. 3,4 This was true for 113 patients with ALS in one center alone, with more than one-half becoming CNVS dependent without even being hospitalized.…”

“…Better than vital capacity, MIE expiratory flows (MIE-EFs) correlate with tracheostomy-free survival such that only patients with predominantly upper motor neuron ALS whose MIE-EFs decrease to about 100 L/min require tracheotomies. 2 Indeed, we have now removed the tracheostomy tubes of 10 patients with ALS predominantly nonbulbar disease, despite them being up to continuously respirator dependent with little to no ventilator free breathing ability and vital capacities as low as 200 mL, to up to CNVS dependence.…”