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Respir Care. 2018 Mar;63(3):274-281. doi: 10.4187/respcare.05475. Epub 2018 Jan 3.

Five-Year Follow-Up and Outcomes of Noninvasive Ventilation in Subjects With Neuromuscular Diseases.

Author information

1
Department of Rehabilitation Medicine, Gangnam Severance Hospital, Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine, Seoul, South Korea.
2
Department of Rehabilitation Medicine, Kangdong Sacred Heart Hospital, Hallym University College of Medicine, Seoul, South Korea.
3
Department of Physical Medicine and Rehabilitation, National Health Insurance Service Ilsan Hospital, Goyang, South Korea.
4
Department of Rehabilitation Medicine, Gangnam Severance Hospital, Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine, Seoul, South Korea. kswoong@yuhs.ac.

Abstract

INTRODUCTION:

The purpose of this study was to investigate the 5-year outcomes of noninvasive ventilation (NIV) application in different neuromuscular disease (NMD) groups.

METHODS:

We categorized 180 subjects who had initiated NIV between March 2001 and August 2009 into 4 groups and followed them for > 5 y. The NIV maintenance rate and average duration, applying time, and forced vital capacity (FVC) were investigated at the time NIV was initiated and 5 y after NIV initiation in each group.

RESULTS:

In subjects with amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and spinal muscular atrophy (SMA)-congenital myopathy, the 5-year subjects who continued to use NIV over time were 22.5%, 89.4%, and 91.3%, respectively, and the average NIV maintenance durations were 21.53 ± 19.26 months, 55.22 ± 11.47 months, and 57.48 ± 8.34 months, respectively (P < .001). Median daily applying time changed from 8.0 h to 24.0 h (P < .001), from 8.0 h to 12.0 h (P < .001), and from 8.0 h to 9.0 h (P = .11) in subjects with ALS, DMD, and SMA-congenital myopathy, respectively. FVC decreased significantly after 5 y except in the group with combined SMA-congenital myopathy.

CONCLUSIONS:

NIV was tolerated long-term without significant increases in daily application time for most subjects with NMD. However, in individuals with ALS, development of severe bulbar symptoms can risk maintaining NIV.

KEYWORDS:

amyotrophic lateral sclerosis; congenital myopathy; duchenne muscular dystrophy; neuromuscular disease; noninvasive ventilation; spinal muscular atrophy

PMID:
29298901
DOI:
10.4187/respcare.05475
[Indexed for MEDLINE]
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