P2.A003: Mental Health, Sleep Concern, and Quality of Life in Children with Hypermobile Ehlers-Danlos Syndrome.

Background: Hypermobile Ehlers-Danlos Syndrome (hEDS) is the most common heritable connective tissue disorder. Common features include joint hypermobility and tissue fragility. Anxiety, mood, and sleep disorders are prevalent and well-studied in the general population and adults with hEDS; but there are few studies in pediatric patients with hEDS. The objective of this study is to quantify psychiatric and sleep disorders in children with hEDS and evaluate their impact on quality of life (QoL).

Methods: Patients were included in the study if they had an hEDS diagnosis and were seen in the rheumatology clinic between May and December 2022. Demographics, psychiatric, and sleep history were collected. Patient-reported outcomes (PROs) were completed (Patient Reported Outcomes Measurement Information System Pediatric Profile Version 2 [PROMIS], Generalized Anxiety Disorder-7 [GAD-7], Adolescent Sleep Wake Scale [ASWS], and Pediatric Quality of Life Inventory Rheumatology Module [PEDSQLRheum]) that evaluate QoL, anxiety, and sleep. The PROMIS sub-scales T-Scores are reported, with higher values representing more of the domain of interest. GAD-7 is a summed score with higher values representing more severe anxiety. In the ASWS, higher scores indicate better sleep quality, and for the PEDSQLRheum, higher values indicate better QoL. The institutional review board approved this study (ID: 00001628).

Results: There were 123 patients that met inclusion criteria. The majority were female (92%) and Caucasian (92%) with a mean age of 15.8 years (SD = 2.71). Most patients (85%) had at least some psychiatric involvement with anxiety (80%) as the most common diagnosis followed by depression (42%). About half (47%) reported previous psychology evaluation, and half of patients (51%) were on psychiatric medication. Almost half (42%) reported a history of poor sleep, while 17% had a previous sleep medicine evaluation and 16% utilized sleep aid medication (Table 1). The GAD-7 mean was 10.54 (SD = 6.26) and ASWS was 2.28 (SD = 0.45). Significant correlations were seen for the GAD-7 and PROMIS Depression measure (r = 0.71, p = < 0.0001), ASWS and PROMIS Depression measure (r = 0.48, p = < 0.0001), ASWS and GAD-7 (r = 0.50, p = < 0.0001), sleep involvement and the GAD-7 (r = 0.22, p = 0.02), and sleep involvement and depression (r = 0.24, p = 0.02) (Table 2).

Conclusion: Psychiatric and sleep disorders are more prevalent in children with hEDS than in the general pediatric population, however, less than half the respondents had a previous psychology evaluation. Additionally, depression and poor sleep were associated with poorer QoL for the respondents. Mental health and sleep evaluation should be considered as part of the management of children with hEDS to address these prevalent issues and improve QoL for those with hEDS.