Living beyond respiratory failure with neuromuscular disease - an open, prospective 6-year trial on the long-term effects and complications of home ventilation

A growing number of patients with neuromuscular disease have been treated w ith home mechanical ventilation during the past 15 years. We prospectively examined the long-term effects and complications of this method, particular ly with regard to noninvasive positive pressure ventilation (NPPV). Thirty- one patients with amyotro phic lateral sclerosis (ALS, n=20) or other slowl y progressive neuromuscular diseases (NMD, n=11) were observed for 17,517 h ome ventilation days (almost 48 ventilation years). The mean observed venti lation time was 565 days (min/max: 30/2930). Twenty-five patients were vent ilated noninvasively with different masks. The calculated mean survival wit h NPPV ventilation (criteria: death, tracheostomy, or patient deciding to b reak off) was 2052 (SE: +/- 317.8) days in the NMD group, 248 days (+/-35.7 ) for ALS patients without bulbar symptoms, and 82 days (+/-27.4) with bulb ar paralysis. Complications with the need for intervention were observed si x times more frequently with ALS than with NMD. NPPV is effective for years in patients with slowly progressing NMD. Those ALS patients without bulbar symptoms can profit for up to a year from NPPV, while those with bulbar pa ralysis can have some symptom relief. Complications of every kind are much more frequent in ALS patients.