Prevalence of Pain and Impact of Joint Strength on Perceived Physical Function in Pediatric Patients with Ehlers-Danlos Syndrome.

Abstract

ObjectiveTo identify how pediatric patients with hypermobile Ehlers Danlos syndrome (hEDS) present to physical therapy (PT) and to better understand their musculoskeletal dysfunction. DesignPatients were included in the study if they had a diagnosis of hEDS and at least one completed PT evaluation after diagnosis. A chart review was conducted to assess joint strength and pain characteristics at the initial PT assessment. Physical Function Mobility (PF-Mobility; four domains with 1-5 scale with higher score representative of better physical function) as part of Patient-Reported Outcome Measurement Information System and pain intensity score (visual analog scale, 0-10 scale where a lower number indicates less pain) was completed by participants. SettingMidwestern tertiary care center. ParticipantsSixty-nine participants were included in the study with an average age of 16.4 years (SD 2.8) at the initial PT visit and 96% were female. InterventionNot applicable. Main Outcome measuresJoint strength, pain intensity and location, and PF-Mobility. ResultsThe knee was the most reported location with pain (68%) followed by the shoulder (46%), hip (41%), and back (39%). Average pain intensity was 5.0 (SD 1.8), joint strength scores ranged from 8.2 (SD 0.9) to 9.9 (SD 0.4), and PF-Mobility mean was 3.3 (SD 0.6). No correlation was noted between joint pain intensity and strength at the joint. ConclusionWhile participants perceived poor mobility and significant pain, PT-assessed strength did not reveal significant muscle weakness. Additional work is needed to explore this discrepancy, including altered biomechanics, evaluation methods, and non-musculoskeletal factors such as neuropathic and psychological components,