0803 Sleep Patterns in Patients with Congenital Central Hypoventilation Syndrome and Their Parents

Abstract Introduction Congenital central hypoventilation syndrome (CCHS) is a rare genetic disease affecting control of breathing necessitating lifelong assisted ventilation (AV) via noninvasive positive pressure (NPPV), diaphragm pacing (DP), or tracheostomy (PPV-T). Owing to abnormal ventilatory responses, patients require monitoring during sleep by caregiver. The aims this study were assess patterns in the patient-parent dyads using actigraphy and diary. Methods Patients with CCHS their parents completed Epworth Sleepiness Scale for Children Adolescents (ESS-CHAD), (ESS), diaries, used wrist 7 days. analyzed data included AV modality, ESS, ESS-CHAD scores, outcomes— latency (SL), efficiency (SE), total time (TST), wake after onset (WASO), frequency. Results median (IQR) ages 11 who 11(8-13.5) 41(39-47.5) years, respectively. modalities NPPV (n=3), DP (n=2), PPV-T (n=6). ESS scores patient 8(2-9) 6(3.5-7), respectively indicating higher normal daytime sleepiness. In CCHS, showed SL 14.6(10.1-19.6) minutes, decreased SE 78.5% (72.5%-84%), TST 7.2(6.6-8.1) hours, elevated WASO 1.7(1.1-2.3) frequent awakenings 22.2(18.4-23.6) per night. parents, 7(3.6-10.9) 81.6% (80%-86%), 6.7(6-6.8) 1.1(1-1.3) 19.3(15.4-22.4) Two separate slept < 6 hours day. Among 2 nighttime home nursing, had >85%, ≥7.3 reported better quality. Conclusion are at risk disturbances. Specifically, SE, TST. Both night wakings disruptions. Nighttime nursing may improve parental sleep. Apart from vigilant management clinicians should also evaluate quality parents. Support (if any)