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Pediatr Pulmonol. 2019 Dec;54(12):2035-2043. doi: 10.1002/ppul.24484. Epub 2019 Sep 1.

Understanding adherence to noninvasive ventilation in youth with Duchenne muscular dystrophy.

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Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Division of Behavioral Medicine and Clinical Psychology, Center for Adherence and Self-Management, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.


Duchenne muscular dystrophy (DMD) is an X-linked, progressive neuromuscular disorder that results in chronic respiratory insufficiency and subsequently failure requiring noninvasive ventilation (NIV). Adherence to NIV in neuromuscular disorders and related barriers are poorly described. The aim of the current study was to assess NIV adherence, adherence barriers, and identify psychosocial predictors of adherence in young boys with early DMD-related sleep disordered breathing and recommended nocturnal NIV. This cross-sectional study included 42 youth with DMD with prescribed nocturnal NIV, and their caregivers. Caregivers and youth completed questionnaires assessing adherence barriers, psychosocial symptoms (eg, anxiety and depressive symptoms), and stress. Medical information pertinent to cardiopulmonary health and neurologic status at both enrollment and initiation of NIV was reviewed. Adherence to NIV, defined as percent days used and days used ≥4 hours/day was 56.1 ± 38.7% and 46.2 ± 40.6%, respectively. Average duration of use on days worn was 5.61 ± 4.23 hours. NIV usage was correlated with the severity of obstructive sleep apnea but not cardiopulmonary variables. Mask discomfort was the most commonly reported adherence barrier followed by behavioral barriers (eg, refusing to use). Multiple regression analyses revealed that internalizing behaviors (eg, anxiety and depressive symptoms) and total adherence barriers significantly predicted NIV adherence. Adherence to NIV in DMD is poor and similar to other pediatric chronic diseases. Our data suggest interventions targeting adherence barriers and patient internalizing symptoms may improve adherence to NIV in DMD.


Duchenne muscular dystrophy; adherence; barriers; bilevel positive airway pressure; children; noninvasive ventilation

[Available on 2020-06-01]

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