Amyotrophic Lateral Sclerosis: Prolongation of Life by Noninvasive Respiratory AIDS

Study Objective: To describe prolongation of survival in patients with amyotrophic lateral sclerosis (ALS) by continuous noninvasive intermittent positive-pressure ventilation (NPPV) and mechanically assisted coughing (MAC) using oximetry as feedback.

Setting: A retrospective review of ALS patients visiting one center from 1990 to 2000.

Design: Patients were trained in mouthpiece and nasal NPPV when symptomatic for hypoventilation, and trained in MAC with oximetry feedback when assisted peak cough flow (PCF) levels decreased to < 270 L/min. Survival was considered to be prolonged when full-time NPPV was required with limited ventilator-free breathing tolerance.

Results: Of 101 patients who met the criteria for access to NPPV and MAC, 15 have not yet used them, and 11 patients with severe bulbar muscle dysfunction died without ever successfully using them. Three patients used NPPV full-time, and oximetry and MAC episodically, but did not yet require ongoing NPPV. Eighteen used NPPV part-time for a mean (+/- SD) duration of 3.8 +/- 4.1 months. Nineteen others underwent tracheotomy after 4.7 +/- 4.5 months of receiving part-time NPPV. Sixteen patients used part-time NPPV for 17.5 +/- 13.0 months (maximum, 25 months), then full-time NPPV for 14.1 +/- 12.6 months (maximum, 40 months) before undergoing tracheotomy. Nineteen patients used part-time and full-time NPPV for 25.2 +/- 19.8 months (maximum, 114 months) and 17.5 +/- 13.3 months (maximum, 87 months), respectively, without undergoing tracheotomy. Ten of these NPPV users died once bulbar dysfunction became severe.

Conclusion: We conclude that up to continuous use of NPPV, along with MAC when needed, can permit prolonged survival and delay the need for tracheotomy for a significant minority of ALS patients by > 1 year.