Pain Characteristics and Symptom Management in Children with Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder.

Aims: This study aims to investigate pediatric hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorder (HSD) pain features and management strategies.

Methods: This is a mixed-methods, cross-sectional study design using patient-reported outcomes in 21 children diagnosed with hEDS/HSD. Children who reported bothersome pain were interviewed for pain features. The Child Activity Limitation Interview-21, the Brief Pain Inventory pain interference items, and the Functional Disability Inventory were used to investigate pain interference. To evaluate psychological symptoms regarding pain, the pediatric version of the Survey of Pain Attitude and the child version of the Pain Catastrophizing Scale were used.

Results: Nineteen children had bothersome pain and of them, eight children reported constant pain. The most frequently reported regions of pain were at the ankle (mild pain) and the back (moderate-to-severe pain). Children reported mild-to-moderate pain interference and believed medications were beneficial for their pain management. Nineteen children sought treatment and of those 16 children used to exercise and acetaminophen and 13 visited physicians as a means of treatment. Parents were overall satisfied with their child's treatment (13 out of 19).

Conclusions: Sufficient awareness of pain-related symptoms and understanding of the treatment strategies in early childhood is needed to prevent deleterious consequences in adulthood.