title: Mechanical insufflation/exsufflation improves vital capacity in neuromuscular disorders.
abstract: Inherited neuromuscular disorders inevitably result in severe lung volume restriction associated with high morbidity and mortality. The aim of this retrospective study was to evaluate the long-term effects of the regular use of mechanical insufflation/exsufflation on the course of the vital capacity. This retrospective data analysis included 21 patients (16.1 ± 6.5 years) with neuromuscular disorders and severe lung volume restriction using nocturnal noninvasive ventilation. The patients were advised to regularly use the mechanical insufflation/exsufflation twice a day for 10 minutes applying sets of three insufflation/exsufflation breath via face mask irrespective of respiratory tract infection. Data on the course of vital capacity were collected 2 years prior and 2 years after the introduction of regular use of mechanical insufflation/exsufflation. Before the introduction of mechanical insufflation/exsufflation vital capacity decreased from 0.71 ± 0.38 L to 0.50 ± 0.24 L in the last year and from 0.88 ± 0.45 L to 0.71 ± 0.38 L in the next to last year. In the first year, after regular use of mechanical insufflation/exsufflation vital capacity significantly increased by 28% (from 0.50 L to 0.64 L)-after the second year the vital capacity increase remained stable (0.64 vs. 0.65 L). These data suggest that the regular use of mechanical insufflation/exsufflation improves vital capacity in patients with neuromuscular disorders and severe lung volume restriction.
authors.affiliation: Department of Pediatric Pulmonology and Sleep Medicine, University of Duisburg-Essen, Children's Hospital, Essen, Germany florian.stehling@uk-essen.de.
authors.forename: Florian
authors.initials: F
authors.lastname: Stehling
authors.affiliation: Department of Pediatric Pulmonology and Sleep Medicine, University of Duisburg-Essen, Children's Hospital, Essen, Germany.
authors.forename: Anastasios
authors.initials: A
authors.lastname: Bouikidis
authors.affiliation: Department of Pediatric Neurology, University of Duisburg-Essen, Children's Hospital, Essen, Germany.
authors.forename: Ulrike
authors.initials: U
authors.lastname: Schara
authors.affiliation: Department of Pediatric Pulmonology and Sleep Medicine, University of Duisburg-Essen, Children's Hospital, Essen, Germany.
authors.forename: Uwe
authors.initials: U
authors.lastname: Mellies
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000150:complications / Q000534:rehabilitation*; D020388:Muscular Dystrophy, Duchenne / Q000150:complications / Q000534:rehabilitation*; D009468:Neuromuscular Diseases / Q000150:complications / Q000534:rehabilitation; D012131:Respiratory Insufficiency / Q000209:etiology / Q000534:rehabilitation*; D012138:Respiratory Therapy / Q000379:methods*; D012189:Retrospective Studies; D016896:Treatment Outcome; D014797:Vital Capacity; D055815:Young Adult
keywords: Neuromuscular disorders; lung function; mechanical insufflation/exsufflation; vital capacity