title: Is chronic ventilatory support really effective in patients with amyotrophic lateral sclerosis?
abstract: Most patients with amyotrophic lateral sclerosis (ALS) develop respiratory insufficiency in the advanced stage of their disease. Non-invasive ventilation (NIV) is commonly regarded to be a treatment that is effective in reducing these complaints. To assess whether the effect of NIV on gas exchange and quality of life (QOL) is different in patients with ALS versus without ALS. A post hoc analysis was done with data from a previously published trial, in which all patients were instituted on NIV. Arterial blood gasses were assessed next to QOL by generic as well as disease-specific questionnaires. 77 patients started NIV: 30 with ALS and 47 without. Both groups showed significant improvements in blood gasses after 2 and 6 months. Compared to the non-ALS group, the ALS group had significantly worse scores after 6 months in MRF-28, SRI, HADS and SF-36 than the non-ALS group. This study shows that NIV improves gas exchange, both in patients with and without ALS. QOL improves markedly more in patients without ALS than in those with ALS, in whom only some domains improve. Our observation of little or no effect in ALS patients warrants a large study limited to ALS patients only.
authors.affiliation: Department of Home Mechanical Ventilation, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. a.hazenberg@umcg.nl.|Department of Pulmonology and Tuberculosis, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. a.hazenberg@umcg.nl.|University Medical Center Groningen, GRIAC Research Institute, University of Groningen, Groningen, The Netherlands. a.hazenberg@umcg.nl.
authors.forename: A
authors.identifier: http://orcid.org/0000-0002-1596-3243
authors.initials: A
authors.lastname: Hazenberg
authors.affiliation: Department of Home Mechanical Ventilation, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.|Department of Pulmonology and Tuberculosis, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.|University Medical Center Groningen, GRIAC Research Institute, University of Groningen, Groningen, The Netherlands.
authors.forename: H A M
authors.initials: HA
authors.lastname: Kerstjens
authors.affiliation: TNO, The Netherlands Organization for Applied Scientific Research, The Hague, The Netherlands.
authors.forename: S C L
authors.initials: SC
authors.lastname: Prins
authors.affiliation: Department of Epidemiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: K M
authors.initials: KM
authors.lastname: Vermeulen
authors.affiliation: Department of Home Mechanical Ventilation, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.|Department of Pulmonology and Tuberculosis, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.|University Medical Center Groningen, GRIAC Research Institute, University of Groningen, Groningen, The Netherlands.
authors.forename: P J
authors.initials: PJ
authors.lastname: Wijkstra
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000523:psychology / Q000628:therapy*; D001784:Blood Gas Analysis; D016206:Databases, Bibliographic / Q000706:statistics & numerical data; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D063087:Noninvasive Ventilation / Q000379:methods* / Q000523:psychology; D011788:Quality of Life / Q000523:psychology; D016032:Randomized Controlled Trials as Topic; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy; D012720:Severity of Illness Index; D016896:Treatment Outcome
keywords: Amyotrophic lateral sclerosis; Non-invasive ventilation; Quality of life