@article {Suhrespcare.05475, author = {Mi Ri Suh and Won Ah Choi and Dong Hyun Kim and Jang Woo Lee and Eun Young Kim and Seong-Woong Kang}, title = {Five-Year Follow-Up and Outcomes of Noninvasive Ventilation in Subjects With Neuromuscular Diseases}, elocation-id = {respcare.05475}, year = {2018}, doi = {10.4187/respcare.05475}, publisher = {Respiratory Care}, 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){\textendash}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 {\textpm} 19.26 months, 55.22 {\textpm} 11.47 months, and 57.48 {\textpm} 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{\textendash}congenital myopathy, respectively. FVC decreased significantly after 5 y except in the group with combined SMA{\textendash}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.}, issn = {0020-1324}, URL = {https://rc.rcjournal.com/content/early/2018/01/03/respcare.05475}, eprint = {https://rc.rcjournal.com/content/early/2018/01/03/respcare.05475.full.pdf}, journal = {Respiratory Care} }