1. Role of respiratory characteristics in treatment adherence with noninvasive home mechanical ventilation in myotonic dystrophy type 1, a retrospective study.
- Author
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Vosse, Bettine Anna Hildegard, Horlings, Corinne Gosewina Cornelia, Joosten, Isis Bep Theodora, Cobben, Nicolle Andrée Marie, van Kuijk, Sander Martijn Job, Wijkstra, Peter Jan, and Faber, Catharina Gerritdina
- Subjects
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PATIENT compliance , *MYOTONIA atrophica , *ARTIFICIAL respiration , *PULMONARY function tests , *RESPIRATORY insufficiency - Abstract
• Myotonic dystrophy type 1 patients often present with respiratory insufficiency. • Sleep apnea is very common in myotonic dystrophy type 1 patients. • Home mechanical ventilation is frequently needed in myotonic dystrophy patients. • Low compliance with home mechanical ventilation often occurs in myotonic dystrophy. • Respiratory features cannot predict home mechanical ventilation treatment failure. 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. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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