Determinants of usage and nonadherence to noninvasive ventilation in children and adults with Duchenne muscular dystrophy

Understanding the experiences of lung volume recruitment among boys with Duchenne muscular dystrophy: A multicenter qualitative study

“…Our study identified that perceived benefits had a strong influence on perceptions of the utility of LVR and motivation to use it. While lack of symptom improvement has not been previously identified as a barrier to cough augmentation technique use, 12,25,27,28 greater disease severity, more symptoms at baseline, and symptom improvement following treatment, have been associated with greater adherence in both children and adults with neuromuscular disease 26,29,30 . Similarly, children in our study who met the threshold of adherence had a lower FVC at baseline and were more likely to be wheelchair‐assisted, which may imply greater respiratory dysfunction.…”

“…While lack of symptom improvement has not been previously identified as a barrier to cough augmentation technique use, 12,25,27,28 greater disease severity, more symptoms at baseline, and symptom improvement following treatment, have been associated with greater adherence in both children and adults with neuromuscular disease. 26,29,30 Similarly, children in our study who met the threshold of adherence had a lower FVC at baseline and were more likely to be wheelchair-assisted, which may imply greater respiratory dysfunction. Clinicians need to be cautious to balance the need to introduce LVR early enough to give the family time to familiarize with it, while ensuring that families are not being asked to use LVR unnecessarily.…”

Reliability and Validity of the Korean Version of the Duchenne Muscular Dystrophy Functional Ability Self-Assessment Tool

“…In fact, some patients use only mouthpiece ventilation during the day, whereas others prefer nasal interfaces for ventilator support and alternate them throughout the day [ 25 ]. Additionally, rarely, some patients prefer ventilator support only when they are awake, so they don’t use the ventilator during the night [ 26 ]. According to the current DMDSAT items, it is difficult to evaluate such patients because they are not included in any given categories.…”

Non‐invasive ventilation usage and adherence in children and adults with Duchenne muscular dystrophy: A multicenter analysis

“…There were no clinical/biological predictors of NIV adherence mentioned. Our recently published single‐location (San Diego) retrospective review of 33 children and adults with DMD (age range 13–39 y) evaluating determinants of NIV usage demonstrated the mean ± SD percentage of non‐adherent nights was 13 ± 30% and nightly NIV usage was 7.4 ± 3.8 h 17 . The current study showed that most individuals with DMD were using NIV; however, some patients (26 of 59, 44.1%) were not prescribed corticosteroid/deflazacort therapy limiting the generalizability of this study.…”

“…To date, there are a limited number of studies reporting on NIV adherence in children and adults with NMD 13–16 . Two cross‐sectional, single‐center studies of NIV adherence in individuals with DMD have thus far been published 12,17 . Multicenter studies are needed to enhance the external validity of the findings of these studies.…”