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

INTRODUCTION: The purpose of this study was to investigate the 5-year outcomes of noninvasive ventilation (NW) application in different neuromuscular disease (NMD) groups. METHODS: We categorized 180 subjects who had initiated NW between March 2001 and August 2009 into 4 groups and followed them for > 5 y. The NW maintenance rate and average duration, applying time, and forced vital capacity (FVC) were investigated at the time NW was initiated and 5 y after NW 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 NW over time were 22.5%, 89.4%, and 913%, respectively, and the average NW 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: NW 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.