Respiratory Medicine 145 (2018) 239–240
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Respiratory Medicine
journal homepage: www.elsevier.com/locate/rmed
Correspondence
Letter to the Editor: Impact of invasive ventilation on survival when non-invasive ventilation is
ineffective in patients with Duchenne muscular dystrophy: A prospective cohort
A R T I C LE I N FO
Keywords:
Continuous non-invasive ventilatory support
Duchenne muscular dystrophy
Mechanical insufflation-exsufflation
Dear Editor,
We read with interest the article conducted by Boussaid G. and
colleagues that was published in Respiratory Medicine in 2016 [1]. The
study included DMD patients from 1997 to 2014, however, no me-
chanical in-exsufflation (MIE) devices were available in France until
2004. Indeed, the authors never even mentioned MIE but assumed
“NIV” ineffectiveness, rather than airway clearance failure, necessitated
tracheotomy. They also never mentioned ventilator parameters such as
those used by centers that permit dependence on continuous non-
invasive ventilatory support (CNVS) and that routinely extubate ven-
tilator “unweanable DMD” patients without ever resorting to tra-
cheotomy [2–5].
In 2010, centers in 16 countries that exclusively use NVS (www.
breatheNVS.com) rather than TIV reported 385 consecutive DMD
patients who became dependent on CNVS for over 1100 patient-years
with none ever requiring tracheotomy [2]. Eight of the centers con-
secutively extubated 61 ventilator “unweanable” DMD patients to
CNVS and MIE without a single failure or need for TIV. Boussaid et al.
reported that DMD TIV users survived to a mean age of 29, which is
the same age reported for 21 Japanese TIV users until 1993 when
tracheotomies were permanently discontinued there in favor of NVS/
CNVS at full ventilatory support settings [3]. Subsequent to 1993,
DMD survival for their 88 reported patients, and now well over 100,
have lived to a mean age of 40 or 10 years longer than with TIV.
Boussaid et al. reported that 21% of their patients underwent TIV as a
result of an episode of respiratory failure for which NVS and MIE were
not even attempted to avoid intubation and no attempt was made to
extubate them to it to it as has been described [4,5]. They reported
risk of “NIV failure” high when vital capacity (VC) was < 20%
whereas NVS center patients' CNVS users typically had VCs less than
4% or 100 ml. Since they reported “risk factor for death” and NIV
https://doi.org/10.1016/j.rmed.2018.08.010
Received 20 August 2018; Accepted 21 August 2018
Available online 22 August 2018
0954-6111/ © 2018 Elsevier Ltd. All rights reserved.
T
failure associated with “swallowing disorders,” they should explain
why few if any patients with Wernig-Hoffman's disease, now over 20
years of age with 0 ml of VC, can be managed by CNVS rather than TIV
indefinitely even though they have no bulbar or skeletal muscle
function at all [6]. While Boussaid et al. reported that 35% of the TIV
users died from “respiratory complications,” no respiratory deaths
have been reported for CNVS plus MIE users in the centers that ex-
tubate them without resort to tracheotomy [2]. We strongly suggest
that no one claim that “NIV” fails without considering the need for
MIE to expulse airway debris and no patient with DMD should fail
extubation to CNVS and MIE [4,5].
References
[1] G. Boussaïd, F. Lofaso, D.B. Santos, I. Vaugier, S. Pottier, H. Prigent, S. Bahrami,
D. Orlikowski, Impact of invasive ventilation on survival when non-invasive venti-
lation is ineffective in patients with Duchenne muscular dystrophy: a prospective
cohort, Respir. Med. 115 (Jun) (2016) 26–32 https://doi.org/10.1016/j.rmed.2016.
04.009.
[2] M.R. Gonçalves, J.R. Bach, Y. Ishikawa, L.R. Saporito, J.C. Winck, Continuous non-
invasive ventilatory support outcomes for neuromuscular disease: a multicenter
collaboration and literature review, Pulmonol 24 (2018) (Epub ahead of print).
[3] Y. Ishikawa, T. Miura, Y. Ishikawa, T. Aoyagi, H. Ogata, S. Hamada, R. Minami,
Duchenne muscular dystrophy: survival by cardio-respiratory interventions,
Neuromuscul. Disord. 21 (2011) 47–51 https://doi.org/10.1016/j.nmd.2010.09.
006.
[4] J.R. Bach, M.R. Gonçalves, I. Hamdani, J.C. Winck, Extubation of unweanable pa-
tients with neuromuscular weakness: a new management paradigm, Chest 137 (5)
(2010) 1033–1039 https://doi.org/10.1378/chest.09-2144.
[5] J.R. Bach, D. Sinquee, L.R. Saporito, A.L. Botticello, Efficacy of mechanical insuf-
flation-exsufflation in extubating unweanable subjects with restrictive pulmonary
disorders, Respir. Care 60 (4) (2015) 477–483 https://doi.org/10.4187/respcare.
03584.
[6] J.R. Bach, Point: is Non-invasive ventilation always the most appropriate manner of
long-term ventilation for infants with Spinal Muscular Atrophy Type 1? Yes, almost
always, Chest 151 (5) (2016) 962–965 https://doi.org/10.1016/j.chest.2016.11.043.
Correspondence Respiratory Medicine 145 (2018) 239–240

John R. Bach∗ Building F1559, 183 South Orange Ave, Newark, NJ, 07103, USA
Department of Physical Medicine and Rehabilitation, Rutgers University - E-mail address: np424@njms.rutgers.edu
New Jersey Medical School, Behavioral Health Sciences Building F1559,
Yuka Ishikawa
183 South Orange Ave, Newark, NJ, USA
Yakumo Byoin National Sanatorium, Department of Paediatrics, 123
E-mail address: bachjr@njms.rutgers.edu
Miyazono-cho, Yamakoshi-gun Yakumo, Hokkaido, 049-3198, Japan
Susan B. Liu E-mail address: yuka@msa.biglobe.ne.jp
Rutgers University - New Jersey Medical School, Behavioral Health Sciences
Miguel R. Gonçalves
Building F1559, 183 South Orange Ave, Newark, NJ, 07103, USA
Noninvasive Ventilatory Support Unit, Pulmonology Department, Emergency
E-mail address: susan.bliu@gmail.com
and Intensive Care Medicine Department, São João University Hospital,
Nikhil Potpally Faculty of Medicine, University of Porto, Portugal
Rutgers University - New Jersey Medical School, Behavioral Health Sciences E-mail address: goncalvesmr@gmail.com

∗
Corresponding author.

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