Lung Insufflation Capacity in Neuromuscular Disease

Bach, John R.; Mahajan, Kedar R; Lipa, Bethany M.; Saporito, Lou; Gonçalves, Miguel R.; Komaroff, Eugene

doi:10.1097/phm.0b013e31817fb26f

Cited by 71 publications

(70 citation statements)

References 19 publications

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“…Thus we assessed the long-term effect of the PIC training for tracheostomized DMD patients. Similar to a recent report (8), PIC was improved in all patients with sustained training. In addition, febrile events were also reduced.…”

Section: Discussionsupporting

confidence: 78%

Lung Inflation Training Using a Positive End-expiratory Pressure Valve in Neuromuscular Disorders

et al. 2012

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Objective Respiratory muscle weakness causes alveolar hypoventilation and reduced lung compliance in neuromuscular disorders. Lung inflation is important to prevent secondary pulmonary complications however respiratory and laryngeal dysfunction often hamper lung inflation. There is a need for a convenient and lowcost device that enables effective lung inflation. We tested a lung inflation training method using a positive end-expiratory pressure (PEEP) valve. Methods Vital capacity (VC), maximum insufflation capacity (MIC) and peak cough flow (PCF) as well as PEEP lung inflation capacity (PIC) were assessed in 93 neuromuscular patients. Consecutive PIC training was done for 4 months in six tracheostomized Duchenne muscular dystrophy (DMD) patients and PIC was assessed before and after training. Results PIC training was practicable in all participants and no serious adverse events were detected. PIC was significantly higher than VC or MIC in all disorders, although MIC was higher than VC in DMD only. Patients with dysphagia showed lower MIC and PCF compared with non dysphagic patients. PIC was as low as 345±77 mL in tracheostomized DMD patients; however consecutive training increased it up to 619±205 mL. Conclusion The PEEP valve enabled effective lung inflation regardless of laryngeal function. Consecutive training can improve lung condition even in advanced cases. Early introduction of PIC training could be effective at preventing respiratory complications in patients with neuromuscular disorders.

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Section: Discussionsupporting

confidence: 78%

Lung Inflation Training Using a Positive End-expiratory Pressure Valve in Neuromuscular Disorders

et al. 2012

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“…However, when bulbar function is severely impaired and maximum insufflation capacity is equal to vital capacity, the chances of improving cough strength are poor. 45 It may be appropriate in these circumstances to consider a tracheostomy.…”

Section: Cough Augmentationmentioning

confidence: 99%

Hot Topics in Noninvasive Ventilation: Report of a Working Group at the International Symposium on Sleep-Disordered Breathing in Leuven, Belgium

et al. 2015

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During the last few decades, attention has increasingly focused on noninvasive ventilation (NIV) in the treatment of chronic respiratory failure. The University of Leuven and the University Hospitals Leuven therefore chose this topic for a 2-day working group session during their International Symposium on Sleep-Disordered Breathing. Numerous European experts took part in this session and discussed (1) NIV in amyotrophic lateral sclerosis (when to start NIV, NIV and sleep, secretion management, and what to do when NIV fails), (2) recent insights in NIV and COPD (high-intensity NIV, NIV in addition to exercise training, and NIV during exercise training), (3) monitoring of NIV (monitoring devices, built-in ventilator software, leaks, and asynchronies) and identifying events during NIV; and (4) recent and future developments in NIV (target-volume NIV, electromyography-triggered NIV, and autoregulating algorithms)

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“…The maximum passive insufflation volume can be termed the "lung insufflation capacity." 17 The primary objectives of lung expansion therapy are to increase the VC and to maximize CPF, to maintain or improve pulmonary compliance, to diminish atelectasis, and to master NVS. The deep lung volumes by air stacking also permit patients to raise voice volume and speak longer phrases.…”

Section: Intervention Objectivesmentioning

confidence: 99%

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

Bach¹,

Mehta²

2014

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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.

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Lung Insufflation Capacity in Neuromuscular Disease

Cited by 71 publications

References 19 publications

Lung Inflation Training Using a Positive End-expiratory Pressure Valve in Neuromuscular Disorders

Lung Inflation Training Using a Positive End-expiratory Pressure Valve in Neuromuscular Disorders

Hot Topics in Noninvasive Ventilation: Report of a Working Group at the International Symposium on Sleep-Disordered Breathing in Leuven, Belgium

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

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