570 Comparing NIV Adherence in Early versus Advanced Stage Sleep Disordered Breathing for Children with Neuromuscular Disease

Abstract

Abstract Introduction Children with neuromuscular disease (NMD) typically develop progressive sleep disordered breathing (SDB), including obstructive sleep apnea (OSA), nocturnal hypoxemia and/or hypoventilation, due to loss of upper airway muscle tone and weakness of respiratory muscles. Commonly, the SDB initially presents during rapid eye movement (REM) sleep, as this stage is associated with physiological muscle atonia, but then progresses to non-REM (NREM) sleep and ultimately daytime respiratory insufficiency. Non-invasive ventilation (NIV) is currently the treatment of choice for children with NMD and SDB. However, the use of NIV in REM-related SDB is less demonstrated and adequate therapy adherence is unclear. The aim of this study is to determine differences in NIV adherence in children with early (REM) versus advanced (non-REM) SDB. Methods Children (0–18 years) diagnosed with NMD and using NIV for the past 10 years were included. Demographic, clinical, technology-related, and sleep study data were collected from medical charts and polysomnography reports. Adherence data (mean hours of NIV use and % days NIV was used >4hrs) were collected from NIV machine downloads. Children were categorized into two groups based on based on their apnea-hypopnea index (AHI) ratio between REM and NREM sleep. Children with REM-SDB were defined as a REM/NREM AHI ratio of ≥ 2. Children with NREM-SDB were defined as a REM/NREM AHI ratio < 2. Results A total of 14 children (9 REM-SDB and 5 NREM-SDB) were included in the analysis. Both groups were comparable with respect to demographic, clinical, and technology-related characteristics. A total of 24 adherence reports were available for the cohort (16 REM-SDB and 8 NREM-SDB). The mean hours of NIV use per night was comparable between the REM-SDB and NREM-SDB groups (9.2±1.3hrs vs. 9.0±0.4hrs respectively), but the percent days NIV was used >4hrs was higher in the NREM-SDB group (68.7±9.6 vs. 93.0±2.7, p=0.03). Conclusion NIV adherence was high for children with both REM-SDB and NREM-SDB. While hours of NIV use were comparable between both groups, suggesting good NIV tolerance through the night, children with REM-SDB had a lower percentage of days with NIV use >4hr, suggesting less willingness to use the therapy. Support (if any):