Noninvasive ventilation (NIV) has been shown to improve daytime arterial blood gases and the symptoms associated with respiratory failure for neuromuscular disease patients.
In amyotrophic lateral sclerosis patients, NIV improves quality of life and sleep-related symptoms.
In patients without severe bulbar dysfunction, 1 NIV prolongs survival by 7 months.
In Duchenne muscular dystrophy (DMD) patients - a retrospective review of 100 consecutive patients born between 1970 and 1990 found that NIV improved survival. Spinal surgery did not increase forced vital capacity, but in combination with nocturnal ventilation, further improved median survival.
NIV is associated with a significant reduction in hospital in-patient days.
Bourke SC, et al. Noninvasive ventilation in ALS: Indications and effect on quality of life. Neurology 2003;
Eagle M, Bourke J, Bullock R, et al. Managing Duchenne muscular dystrophy—the additive effect of spinal surgery and home nocturnal ventilation in improving survival. Neuromuscular Disorders 2007;
eger P, Bedicam JM, Cornette A et al. Nasal intermittent positive pressure ventilation. Long term follow up in patients with severe chronic respiratory insufficiency.