Amyotrophic lateral sclerosis. Communication status and survival with ventilatory support

Abstract

The use of ventilatory support via an indwelling tracheostomy tube in the management of advanced amyotrophic lateral sclerosis patients demands the commitment of enormous resources. The use of noninvasive respiratory aids can facilitate and simplify home management, decrease expense and prepare patients and families for decision making regarding tracheostomy if and when this becomes necessary. The purposes of this study were to: describe the utility of noninvasive respiratory aids, determine to what extent survival might be expected to increase by the use of mechanical ventilation, and explore the consequences of patient disposition and communication status on survival. Eighty-nine patients survived a mean of 4.4 +/- 3.9 yr (range = 1 month to 26.5 yr) using respiratory support. This included 37 patients who were still alive. The up to 24 h use of noninvasive intermittent positive pressure ventilation (IPPV) methods delayed or eliminated tracheostomy for 25 patients. Survival was comparable for patients maintained at home or in chronic care facilities. The maintenance of effective communication appeared to favor patients remaining in the community. It could not be shown to affect survival, but it greatly increased quality of life. The use of noninvasive respiratory muscle aids can eliminate the need for "crisis" decision making regarding tracheostomy for many individuals with ALS.