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LetterCorrespondence

Noninvasive Respiratory Care Received by Individuals With Duchenne Muscular Dystrophy Since 1979

John Bach, Miguel Goncalves, Michael Chiou, Nicholas Hart and Michel Toussaint
Respiratory Care August 2017, 62 (8) 1120-1121; DOI: https://doi.org/10.4187/respcare.05635
John Bach
Department of Physical Medicine and Rehabilitation Rutgers New Jersey Medical School Newark, New Jersey
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Miguel Goncalves
Department of Pulmonology University Hospital of S. Joao Faculty of Medicine University of Porto, Portugal
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Michael Chiou
Department of Physical Medicine and Rehabilitation Rutgers New Jersey Medical School Newark, New Jersey
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Nicholas Hart
St Thomas' Hospital London, United Kingdom
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Michel Toussaint
Ziekenhuis Inkendaal Rehabilitation Hospital Brussels, Belgium
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To the Editor:

The authors of a recent review of Duchenne muscular dystrophy (DMD) management1 nicely pointed out that Dr Ishikawa's group reported survival for DMD subjects to a mean age of 39.6 y, but neither that 38 of them were dependent on continuous noninvasive ventilatory support nor that 8 had been extubated and 2 decannulated of tracheostomy tubes to continuous noninvasive ventilatory support despite having no ventilator-free breathing ability. None of the 17 who died did so from respiratory complications. There are currently > 80 who are continuous noninvasive ventilatory support-dependent. Before eliminating tracheotomies in 1995, Dr Ishikawa's trached patients died at a mean age of 29 y. Today, there are > 20 centers worldwide that manage DMD by continuous noninvasive ventilatory support and mechanical insufflation-exsufflation without ever resorting to tracheotomy for extubation failure, including the centers of the authors of this letter.2–5 In considering centers in multiple states, the authors of this review1 included no medical directors from them. The authors pointed out that Bach et al6 reported successful first-attempt extubation for 95% of 149 subjects with neuromuscular disease, but they overlooked that 20 had been continuous noninvasive ventilatory support-dependent with DMD. Indeed, the one who failed an initial extubation attempt subsequently succeeded, and none underwent tracheotomy.

The authors also overlooked the Respiratory Care follow-up paper7 on 96 more subjects successfully extubated to continuous noninvasive ventilatory support and mechanical insufflation-exsufflation as needed, including 12 more with DMD and no ventilator-free breathing ability. In 2013, a review of continuous noninvasive ventilatory support management by 6 of the > 20 centers that provide it reported 40 consecutive successful extubations on “unweanable” subjects with DMD.8 Today, that figure is > 73. Despite this, rather than “organize a support system of comprehensive instruction, equipping, and training in noninvasive management,”8 this review unfortunately continues to imply that tracheotomies must eventually become necessary for DMD, especially when conventional extubations fail. Indeed, they noted that 18 of 29 tracheostomies were performed due to acute respiratory illnesses and that 86% were performed before 21 y of age, so clearly the continuous noninvasive ventilatory support extubation protocol was not used, and the 11 who underwent elective tracheotomy did not benefit from continuous noninvasive ventilatory support and mechanical insufflation-exsufflation either. Their review cited noninvasive ventilation, which has become synonymous with low spans of bi-level or continuous positive airway pressure, and mechanical insufflation-exsufflation without giving settings for either. We use full noninvasive ventilatory support settings, not low bi-level spans, and mechanical insufflation-exsufflation at 50–70 cm H2O pressures, as was originally described to be effective.9

Their review concludes that “there have been few changes in pulmonary clinical practice”1 and perpetuates unnecessarily invasive care, although no DMD patients would prefer it over noninvasive care.10 It is also important to point out that with optimal noninvasive management, many if not most DMD patients become continuous noninvasive ventilatory support-dependent not only without being intubated or trached, but also without being hospitalized.8 Rather than evaluate and treat patients with DMD for sleep disordered breathing when, in reality, they have severe respiratory muscle dysfunction, should not a review of management include up to continuous noninvasive ventilatory support as well as vital mechanical insufflation-exsufflation, as cited in other consensuses?8

The following might also be pointed out: Although the review cited the need for cough flows and end-tidal CO2 monitoring, these are not routinely performed by pulmonary function testing, so it is unclear why the latter should be recommended; noninvasive ventilation has not only been available since the late 1980s, continuous noninvasive ventilatory support for DMD was described by Alexander and Johnson in 1979,11 by Bach et al in 1981,12 and subsequently others. Finally, this letter is fully sanctioned by 28 medical director authors of publications cited in a recent consensus on noninvasive management.8

Footnotes

  • The authors have disclosed no conflicts of interest.

  • Copyright © 2017 by Daedalus Enterprises

References

  1. 1.↵
    1. Andrews JG,
    2. Soim A,
    3. Pandya S,
    4. Westfield CP,
    5. Ciafaloni E,
    6. Fox DJ,
    7. et al
    . Respiratory care received by individuals with Duchenne muscular dystrophy from 2000 to 2011. Respir Care 2016;61(10):1349–1359.
    OpenUrlAbstract/FREE Full Text
  2. 2.↵
    1. Ishikawa Y,
    2. Miura T,
    3. Ishikawa Y,
    4. Aoyagi T,
    5. Ogata H,
    6. Hamada S,
    7. Minami R
    . Duchenne muscular dystrophy: survival by cardio-respiratory interventions. Neuromusc Disord 2011;21(1):47–51.
    OpenUrlCrossRefPubMed
  3. 3.
    1. McKim DA,
    2. Griller N,
    3. LeBlanc C,
    4. Woolnough A,
    5. King J
    . Twenty-four hour noninvasive ventilation in Duchenne muscular dystrophy: a safe alternative to tracheostomy. Can Respir J 2013;20(1):e5–e9.
    OpenUrlPubMed
  4. 4.
    1. Villanova M,
    2. Brancalion B,
    3. Mehta AD
    . Duchenne muscular dystrophy: life prolongation by noninvasive ventilatory support. Am J Phys Med Rehabil 2014;93(7):595–599.
    OpenUrl
  5. 5.↵
    1. Bach JR,
    2. Martinez D
    . Duchenne muscular dystrophy: prolongation of survival by noninvasive interventions. Respir Care 2011;56(6):744–750.
    OpenUrlAbstract/FREE Full Text
  6. 6.↵
    1. Bach JR,
    2. Gonçalves MR,
    3. Hamdani I,
    4. Winck JC
    . Extubation of patients with neuromuscular weakness: a new management paradigm. Chest 2010;137(5):1033–1039.
    OpenUrlCrossRefPubMed
  7. 7.↵
    1. Bach JR,
    2. Sinquee DM,
    3. Saporito LR,
    4. Botticello AL
    . Efficacy of mechanical insufflation-exsufflation in extubating unweanable subjects with restrictive pulmonary disorders. Respir Care 2015;60(4):477–483.
    OpenUrlAbstract/FREE Full Text
  8. 8.↵
    1. Bach JR,
    2. Gonçalves MR,
    3. Hon A,
    4. Ishikawa Y,
    5. De Vito EL,
    6. Prado F,
    7. Dominguez ME
    . Changing trends in the management of end-stage respiratory muscle failure in neuromuscular disease: current recommendations of an international consensus. Am J Phys Med Rehabil 2013;92(3):267–277.
    OpenUrlCrossRefPubMed
  9. 9.↵
    1. Bach JR
    . Update and perspectives on noninvasive respiratory muscle aids: part 2–the expiratory muscle aids. Chest 1994;105(5):1538–1544.
    OpenUrlCrossRefPubMed
  10. 10.↵
    1. 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.
    OpenUrlCrossRefPubMed
  11. 11.↵
    1. Alexander MA,
    2. Johnson EW,
    3. Petty J,
    4. Stauch D
    . Mechanical ventilation of patients with late stage Duchenne muscular dystrophy: management in the home. Arch Phys Med Rehabil 1979;60(7):289–292.
    OpenUrlPubMed
  12. 12.↵
    1. Bach J,
    2. Alba A,
    3. Pilkington LA,
    4. Lee M
    . Long-term rehabilitation in advanced stage of childhood onset, rapidly progressive muscular dystrophy. Arch Phys Med Rehabil 1981;62(7):328–231.
    OpenUrlPubMed
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Respiratory Care: 62 (8)
Respiratory Care
Vol. 62, Issue 8
1 Aug 2017
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Noninvasive Respiratory Care Received by Individuals With Duchenne Muscular Dystrophy Since 1979
John Bach, Miguel Goncalves, Michael Chiou, Nicholas Hart, Michel Toussaint
Respiratory Care Aug 2017, 62 (8) 1120-1121; DOI: 10.4187/respcare.05635

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Noninvasive Respiratory Care Received by Individuals With Duchenne Muscular Dystrophy Since 1979
John Bach, Miguel Goncalves, Michael Chiou, Nicholas Hart, Michel Toussaint
Respiratory Care Aug 2017, 62 (8) 1120-1121; DOI: 10.4187/respcare.05635
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