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

Show Author's information John R Bach1,2,3( )Anokhi D Mehta2
Department of Physical Medicine and Rehabilitation,
Department of Neurosciences, Rutgers-New Jersey Medical School, Newark, NJ, USA
Center for Ventilator Management Alternatives, University Hospital, Newark, NJ, USA

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 patient-years 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.

Keywords:

glossopharyngeal breathing, assisted cough, neuromuscular disease, Duchenne muscular dystrophy, spinal muscular atrophy, amyotrophic lateral sclerosis
Published: 21 February 2014 Issue date: December 2014
References(50)
1.
Bach JR, Bianchi C, Aufiero E. Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest. 2004;126(5):1502–1507.
2.
Bach JR, Gupta K, Reyna M, Hon A. Spinal muscular atrophy type 1: prolongation of survival by noninvasive respiratory aids. Pediatr Asthma Allergy Immunol. 2010;22(4):151–162.
3.
Bach JR. Amyotrophic lateral sclerosis: prolongation of life by noninvasive respiratory AIDS. Chest. 2002;122(1):92–98.
4.
Bach JR, Alba AS. Management of chronic alveolar hypoventilation by nasal ventilation. Chest. 1990;97(1):52–57.
5.
Bach JR, Saporito LR. Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure. A different approach to weaning. Chest. 1996;110(6):1566–1571.
6.
Kang SW, Bach JR. Maximum insufflation capacity. Chest. 2000;118(1):61–65.
7.
Currie DC, Munro C, Gaskell D, Cole PJ. Practice, problems and compliance with postural drainage: a survey of chronic sputum producers. Br J Dis Chest. 1986;80(3):249–253.
8.
Williams AJ, Yu G, Santiago S, Stein M. Screening for sleep apnea using pulse oximetry and a clinical score. Chest. 1991;100(3):631–635.
9.
Bach JR, Alba AS, Bodofsky E, Curran FJ, Schultheiss M. Glossopharyngeal breathing and noninvasive aids in the management of post-polio respiratory insufficiency. Birth Defects Orig Artic Ser. 1987;23(4):99–113.
10.
Bach JR. New approaches in the rehabilitation of the traumatic high level quadriplegic. Am J Phys Med Rehabil. 1991;70(1):13–19.
11.
Dail C, Rodgers M, Guess V, et al. Glossopharyngeal Breathing. Downey, CA: Rancho Los Amigos Department of Physical Therapy; 1979.
12.
Dail CW, Affeldt JE. Glossopharyngeal Breathing [video]. Los Angeles, CA: Department of Visual Education, College of Medical Evangelists; 1954.
DOI
13.
Webber B, Higgens J. Glossopharyngeal breathing: what, when and how? [video]. West Sussex, UK: Aslan Studios Ltd; 1999.
14.
Bach JR, Bianchi C, Vidigal-Lopes M, Turi S, Felisari G. Lung inflation by glossopharyngeal breathing and “air stacking” in Duchenne muscular dystrophy. Am J Phys Med Rehabil. 2007;86(4):295–300.
15.
Bach JR, Alba AS. Noninvasive options for ventilatory support of the traumatic high level quadriplegic patient. Chest. 1990;98(3):613–619.
16.
Bach JR, Kang SW. Disorders of ventilation: weakness, stiffness, and mobilization. Chest. 2000;117(2):301–303.
17.
Bach JR, Mahajan K, Lipa B, Saporito L, Goncalves M, Komaroff E. Lung insufflation capacity in neuromuscular disease. Am J Phys Med Rehabil. 2008;87(9):720–725.
18.
Bach JR, Baird JS, Plosky D, Navado J, Weaver B. Spinal muscular atrophy type 1: management and outcomes. Pediatr Pulmonol. 2002;34(1):16–22.
19.
Bach JR, Alba AS, Shin D. Management alternatives for post-polio respiratory insufficiency. Assisted ventilation by nasal or oral-nasal interface. Am J Phys Med Rehabil. 1989;68(6):264–271.
20.
Bach JR, Alba AS. Intermittent abdominal pressure ventilator in a regimen of noninvasive ventilatory support. Chest. 1991;99(3):630–636.
21.
Bach JR, Robert D, Leger P, Langevin B. Sleep fragmentation in kyphoscoliotic individuals with alveolar hypoventilation treated by NIPPV. Chest. 1995;107(6):1552–1558.
22.
Allen J. Pulmonary complications of neuromuscular disease: a respiratory mechanics perspective. Pediatr Respir Rev. 2010;11(1):18–23.
23.
Ishikawa Y. Manual for the Care of Patients using Noninvasive Ventilation. Matsudo, Japan: Japan Planning Center; 2005.
24.
Bach JR, Alba AS, Saporito LR. Intermittent positive pressure ventilation via the mouth as an alternative to tracheostomy for 257 ventilator users. Chest. 1993;103(1):174–182.
25.
Richards GN, Cistulli PA, Ungar RG, Berthon-Jones M, Sullivan CE. Mouth leak with nasal continuous positive airway pressure increases nasal airway resistance. Am J Respir Crit Care Med. 1996;154(1):182–186.
26.
Bach JR, Gonzalez M, Sharma A, Swan K, Patel A. Open gastrostomy for noninvasive ventilation users with neuromuscular disease. Am J Phys Med Rehabil. 2010;89(1):1–6.
27.
Bach JR. Noninvasive respiratory management of high level spinal cord injury. J Spinal Cord Med. 2012;35(2):72–80.
28.
Gomez-Merino E, Bach JR. Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing. Am J Phys Med Rehabil. 2002;81(6):411–415.
29.
Guérin C, Bourdin G, Leray V, et al. Performance of the CoughAssist insufflation-exsufflation device in the presence of an endotracheal tube or tracheostomy tube: a bench study. Respir Care. 2011;56(8):1108–1114.
30.
Bach JR, Gonçalves MR, Hamdani I, Winck JC. Extubation of patients with neuromuscular weakness: a new management paradigm. Chest. 2010;137(5):1033–1039.
31.
Fishburn MJ, Marino RJ, Ditunno JF. Atelectasis and pneumonia in acute spinal cord injury. Arch Phys Med Rehabil. 1990;71(3):197–200.
32.
Garstang SV, Kirshblum SC, Wood KE. Patient preference for in-exsufflation for secretion management with spinal cord injury. J Spinal Cord Med. 2000;23(2):80–85.
33.
Bach JR, Smith WH, Michaels J, et al. Airway secretion clearance by mechanical exsufflation for post-poliomyelitis ventilator-assisted individuals. Arch Phys Med Rehabil. 1993;74(2):170–177.
34.
Barach AL, Beck GJ. Exsufflation with negative pressure; physiologic and clinical studies in poliomyelitis, bronchial asthma, pulmonary emphysema, and bronchiectasis. AMA Arch Intern Med. 1954;93(6):825–841.
35.
Bach JR. Mechanical insufflations-exsufflation. Comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest. 1993;104(5):1553–1562.
36.
Waldhorn RE, Herrick TW, Nguyen MC, O’Donnell AE, Sodero J, Potolicchio SJ. Long-term compliance with nasal continuous positive airway pressure therapy of obstructive sleep apnea. Chest. 1990;97(1):33–38.
37.
Bach JR. Conventional approaches to managing neuromuscular ventilation failure. In: Bach JR, editor. Pulmonary Rehabilitation: The Obstructive and Paralytic Conditions. Philadelphia, PA: Hanley & Belfus; 1996:285–301.
38.
Bach JR, Rajaraman R, Ballanger F, et al. Neuromuscular ventilatory insufficiency: effect of home mechanical ventilator use v oxygen therapy on pneumonia and hospitalization rates. Am J Phys Med Rehabil. 1998;77(1):8–19.
39.
Toussaint M, Steens M, Wasteels G, Soudon P. Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. Eur Respir J. 2006;28(3):549–555.
40.
Bach JR, Tuccio MC, Khan U, Saporito LR. Vital capacity in spinal muscular atrophy. Am J Phys Med Rehabil. 2012;91(6):487–493.
41.
Schroth MK. Special considerations in the respiratory management of spinal muscular atrophy. Pediatrics. 2009;123 Suppl 4:S245–S249.
42.
Kohler M, Clarenbach CF, Böni L, Brack T, Russi EW, Bloch KE. Quality of life, physical disability, and respiratory impairment in Duchenne muscular dystrophy. Am J Respir Crit Care Med. 2005;172(8):1032–1036.
43.
McKim DA, Griller N, LeBlanc C, Woolnough A, King J. Twenty-four hour noninvasive ventilation in Duchenne muscular dystrophy: a safe alternative to tracheostomy. Can Respir J. 2013;20(1):e5–e9.
44.
Villanova M, Brancalion B, Mehta AD. Duchenne muscular dystrophy: life prolongation by noninvasive ventilatory support. Am J Phys Med Rehabil. In press.
45.
Ishikawa Y, Miura T, Ishikawa Y, et al. Duchenne muscular dystrophy: survival by cardio-respiratory interventions. Neuromuscul Disord. 2011;21(1):47–51.
46.
Mills B, Bach JR, Zhao C, Saporito L, Sabharwal S. Posterior spinal fusion in children with flaccid neuromuscular scoliosis: the role of noninvasive positive pressure ventilatory support. J Pediatr Orthop. 2013;33(5):488–493.
47.
Mahajan KR, Bach JR, Saporito L, Perez N. Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis/motor neuron disease. Muscle Nerve. 2012;46(6):851–855.
48.
Bolikal P, Bach JR, Goncalves M. Electrophrenic pacing and decannulation for high-level spinal cord injury: a case series. J Spinal Cord Med. 2012;35(3):170–174.
49.
Bach JR. A comparison of long-term ventilatory support alternatives from the perspective of the patient and care giver. Chest. 1993;104(6):1702–1706.
50.
Bach JR, Gonçalves MR, Hon A, et al. Changing trends in the management of end-stage neuromuscular respiratory muscle failure: recommendations of an internal consensus. Am J Phys Med Rehabil. 2013;92(3):267–277.
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Published: 21 February 2014
Issue date: December 2014

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© 2014 The Author(s).

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Patient consent was obtained for the use of the patient photos.

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© 2014 Bach and Mehta. This work is published by Dove Medical Press Limited, and licensed under Creative Commons Attribution – Non Commercial (unported, v3.0) License. The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. Permissions beyond the scope of the License are administered by Dove Medical Press Limited. Information on how to request permission may be found at: http://www.dovepress.com/permissions.php

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