Improving Nocturnal Non‐Invasive Positive Pressure Support Adherence in Children and Young Adults With Duchenne Muscular Dystrophy

BACKGROUND: Patients with Duchenne muscular dystrophy (DMD) have progressive muscular weakness, respiratory decline and often have poor adherence to nocturnal non-invasive positive airway pressure ventilation (NIPPV). METHODS: In 2020, we sought to increase nocturnal NIPPV adherence (≥ 4 h use overnight on at least 65% of nights) from 33% to 75% by December 2024 at a pediatric neuromuscular center. We used quality improvement (QI) methods and identified key drivers (follow-up, education, staff engagement, consistent documentation). Interventions included: assuring timely follow-up (within ≤ 14 months), pre-visit planning, reminders to bring NIPPV equipment to clinic, addressing adherence barriers and partnering with psychologists. Improving overnight NIPPV adherence was the primary outcome measure. Process measures included the proportion of patients with: (1) NIPPV equipment in the clinic, (2) timely follow-up, and (3) a properly documented follow-up call. Measures were tracked on run charts. RESULTS: Baseline median adherence improved from 33% to 60% in 8 months, and subsequently to 74% (n = 181). The proportion of patients with NIPPV machines in the clinic increased (53%-82%), as did the proportion of patients with timely follow-up (81%-95%). Common patient-reported (n = 123) adherence barriers included: "sleeping well without NIPPV" (20%), "air pressure discomfort" (14%) and "falling asleep before putting it on" (14%). Common parent-reported barriers were: "forget to use" (17%) and "fall asleep before putting it on" (15%). CONCLUSION: Nocturnal NIPPV adherence improved in youth with DMD using QI methods. Tracking adherence automatically via equipment records, further investigating barriers and minimizing discomfort with NIPPV may further improve adherence.