Is home mechanical ventilation really effective in patients with amyotrophic lateral scleroses

Introduction: Most patients with amyotrophic lateral scleroses (ALS) develop respiratory insufficiency in the advanced stage of their disease. Home mechanical ventilation (HMV) might be effective in reducing complaints of dyspnea, fatigue and poor sleep quality. Objectives: To assess whether the effect of HMV on gas exchange and quality of life (QOL) is different in patients with ALS versus without ALS. Methods: Post-hoc analyses were performed with data from an RCT in which all patients were instituted on HMV. Arterial blood gasses were assessed next to QOL using: Severe Respiratory Insufficiency (SRI), Maugeri Respiratory Failure and Short-From Health Survey 36. Independent-sample T-test for difference between groups and paired-sample T-test for difference within groups were performed. Results: 77 Patients started HMV, 30 with ALS and 47 without. Both groups showed significant improvements in blood gasses after 2 and 6 months. While QOL improved for almost all domains by all questionnaires in the non-ALS group, it decreased overall in the ALS group. The only domain that improved significantly in the ALS group was the domain attendant symptoms and sleep of the SRI questionnaire. Conclusion: This study shows that HMV improves gas exchange, both in patients with and without ALS. However in ALS patients, QOL did not improve with 6 months of HMV and even deteriorates significantly in some domains (physical functioning and social relationship). We believe that rapid progression of ALS is an important reason for this negative result. Before firm conclusions can be drawn about the impact of HMV on QOL in ALS patients, prospective studies are necessary, bearing in mind the ethical aspects.