Predictors of non-invasive ventilation tolerance in amyotrophic lateral sclerosis.

BACKGROUND

The most frequent cause of death in patients with Amyotrophic Lateral Sclerosis (ALS) is respiratory failure. Recently, it has been shown that non-invasive ventilation improves survival and quality of life in ALS patients with respiratory failure, but little is known about predictors of non-invasive ventilation adaptation and tolerance. In this study we evaluated the effect of a comprehensive information about non-invasive ventilation use and a prolonged and intensive monitoring on tolerance to this palliative care.

METHODS

We prospectively monitored all consecutive ALS patients with chronic respiratory failure and indication to non-invasive ventilation between January 2005 and December 2007. Non-invasive ventilation adaptation was always performed in a hospital setting.

RESULTS

Forty-four patients were considered eligible: six declined the non-invasive ventilation proposal and one was excluded due to severe fronto-temporal dementia. Non-invasive ventilation was offered to thirty-seven inpatients in our ALS Centre, thirty-two of whom presented with severe (n=9) or mild-moderate (n=23) bulbar impairment at non-invasive ventilation initiation. The mean time interval for adaptation to ventilation was 5±2 days, but patients remained in hospital for an average extended period of one week. Thirty-five of the 37 patients who started non-invasive ventilation, including those with severe bulbar impairment, remained tolerant at twelve months follow-up.

CONCLUSIONS

Our study shows that an intensive educational training and adaptation on non-invasive ventilation, when performed in a hospital multidisciplinary setting, increases compliance and tolerance over time, even in those patients with severe bulbar impairment. However, the design of our study, mainly based on a continuous monitoring and educational training of the patients, might not make it fully applicable to an outpatients setting.