Abstract | INTRODUCTION: 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.
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Authors | Mi Ri Suh, Won Ah Choi, Dong Hyun Kim, Jang Woo Lee, Eun Young Kim, Seong-Woong Kang |
Journal | Respiratory care
(Respir Care)
Vol. 63
Issue 3
Pg. 274-281
(Mar 2018)
ISSN: 1943-3654 [Electronic] United States |
PMID | 29298901
(Publication Type: Journal Article)
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Copyright | Copyright © 2018 by Daedalus Enterprises. |
Topics |
- Adolescent
- Adult
- Aged
- Amyotrophic Lateral Sclerosis
(complications, physiopathology)
- Female
- Follow-Up Studies
- Humans
- Male
- Middle Aged
- Muscular Atrophy, Spinal
(complications, congenital, physiopathology)
- Muscular Dystrophy, Duchenne
(complications, physiopathology)
- Noninvasive Ventilation
- Respiratory Insufficiency
(etiology, physiopathology, therapy)
- Survival Rate
- Time Factors
- Tracheostomy
- Vital Capacity
- Young Adult
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