Role of respiratory characteristics in treatment adherence with noninvasive home mechanical ventilation in myotonic dystrophy type 1, a retrospective study.

Autor: Vosse BAH; Department of Pulmonary Diseases and Home Mechanical Ventilation, Maastricht University Medical Centre+, P.O. Box 5800 6202 AZ Maastricht, the Netherlands. Electronic address: bettine.vosse@mumc.nl., Horlings CGC; Department of Neurology, Medical University Innsbruck, Austria., Joosten IBT; Department of Neurology and School for Mental Health and Neuroscience, Maastricht University Medical Centre+, P.O. Box 5800 6202 AZ Maastricht, the Netherlands., Cobben NAM; Department of Pulmonary Diseases and Home Mechanical Ventilation, Maastricht University Medical Centre+, P.O. Box 5800 6202 AZ Maastricht, the Netherlands., van Kuijk SMJ; Department of Clinical Epidemiology and Medical Technology Assessment, Maastricht University Medical Centre+, P.O. Box 5800 6202 AZ Maastricht, the Netherlands., Wijkstra PJ; Department of Pulmonary Diseases and Home Mechanical Ventilation, University Medical Centre Groningen, P.O. Box 30.001 Groningen, the Netherlands., Faber CG; Department of Neurology and School for Mental Health and Neuroscience, Maastricht University Medical Centre+, P.O. Box 5800 6202 AZ Maastricht, the Netherlands.
Jazyk: angličtina
Zdroj: Neuromuscular disorders : NMD [Neuromuscul Disord] 2023 Sep; Vol. 33 (9), pp. 57-62. Date of Electronic Publication: 2023 Aug 09.
DOI: 10.1016/j.nmd.2023.08.004
Abstrakt: Chronic respiratory insufficiency is common in patients with myotonic dystrophy type 1 (DM1) and can be treated with noninvasive home mechanical ventilation (HMV). HMV is not always tolerated well resulting in low treatment adherence. We aimed to analyze if baseline respiratory characteristics such as pulmonary function, level of pCO 2 and presence of sleep apnea are associated with HMV treatment adherence in DM1 patients. Pulmonary function testing, polysomnography and blood gas measurement data of DM1 patients were retrospectively collected. Initiation of HMV and treatment adherence after one year was documented. Patients with low treatment adherence (average daily use of HMV <5 h) were grouped with patients that discontinued HMV and compared with patients with high treatment adherence (average daily use of HMV >5 h). HMV was initiated in 101 patients. After one year, 58 patients had low treatment adherence. There were no differences between the low and high treatment adherence group regarding the respiratory characteristics. None of the included predictors (gender, age, body mass index, cytosine-thymine-guanine repeat length, FVC, daytime pCO 2 , bicarbonate, nighttime pCO 2 , nighttime base excess, apnea-hypopnea index and mean saturation during sleep) was able to significantly predict high treatment adherence. In conclusion, the respiratory characteristics are not associated with treatment adherence with HMV in DM1 patients and cannot be used to identify patients at risk for low HMV treatment adherence.
Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests of personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2023. Published by Elsevier B.V.)
Databáze: MEDLINE
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