0929 THE MANAGEMENT OF SLEEP DISORDERS IN CHILDREN WITH EHLERS-DANLOS SYNDROME

Abstract

Introduction: Ehlers-Danlos Syndrome (EDS) is a clinically and genetically rare heterogeneous group of inherited connective tissue disorders. There is evidence for a high frequency of sleep problems in this population based on limited data in adults, specifically high rates of obstructive sleep apnea (OSA), fatigue, low sleep quality and periodic leg movement disorder (PLMD). No data exist regarding OSA and other sleep disorders as well as their management in children with EDS. The aim of this study was to describe the management for various sleep disorders in children with EDS. Methods: A retrospective review was performed in children with EDS evaluated in the sleep center at CCHMC between July 2009 and August 2016. Children younger than 18 years-old diagnosed with EDS were included in our study. Demographic information and medical history with particular emphasis on clinical management were reviewed. Results: Sixty-one children met the criteria for entry into analysis (64% females, 91% Caucasian). The average age at presentation and average age at onset of symptoms were 13.1 ± 4.0 years old, and 9.6 ± 4.0 years old, respectively. 22 (36%) were prescribed melatonin and 11 (18%) were recommended cognitive behavioral therapy for insomnia and circadian rhythm disorders. 7 (11%) were prescribed a benzodiazepine mainly for parasomnia or PLMD. 15 subjects were prescribed iron sulfate for possible RLS and PLMD. Intranasal corticosteroids, monteleukast, or anti-histamine were used in 11 (18%) of subjects with mild OSA. One patient was referred to ENT for moderate OSA. None were prescribed CPAp. 6 patients with narcolepsy and 4 patients with idiopathic hypersomnia were treated with modafinil, stimulants or both. Improving sleep hygiene was recommended in 13 (21%) subjects. Conclusion: Most children with EDS seen in our sleep clinic received medical or behavioral therapy for sleep-onset insomnia, behavioral sleep problems, circadian rhythm sleep disorders, PLMD, and mild OSA. As opposed to adults with EDS, none required CPAP. Our study emphasizes the importance of screening for other sleep disorders. Further study is needed to assess the effect of sleep management on clinical outcomes and quality of life in children with EDS.