Mi Ri Suh1, Won Ah Choi1, Dong Hyun Kim2, Jang Woo Lee3, Eun Young Kim1, Seong-Woong Kang4. 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.
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.
Authors: Mi Ri Suh; Dong Hyun Kim; Jiho Jung; Bitnarae Kim; Jang Woo Lee; Won Ah Choi; Seong-Woong Kang Journal: Medicine (Baltimore) Date: 2019-05 Impact factor: 1.817