Authors: Veronica Southard, Arline Allera, Joanne DiFrancisco-Donoghue.
Background: Hypermobile Ehlers-Danlos, (hEDs), and Hypermobile Spectrum Disorder (HSD) are connective tissue diseases that are often clinically indistinguishable. The purpose of this study was to assess 3 outcomes for utility for persons with these diseases in terms of intrarater, interrater reliability and intertrial reliability as well as, the Health Assessment Questionnaire Disability Index (HAQ-DI) an assessment of participants perception of pain and quality of life. To assess change in vital signs and cortisol saliva measures before and after testing. Lastly, compare participants to normal.
Methods: Participants attended 3 sessions within 2 weeks. Two trained examiners assessed the reliability of the two-minute walk test, (2MWT), five times sit to stand (5XSTS), grip strength on five participants. There were 10 participants, each completed the self-report, (HAQ-DI). 10 trained assessors evaluated between 1-10 of the participants for the stability of the scores between three trials. Vital signs and rate of perceived exertion (RPE) were assessed pre and post 2MWT; saliva samples were taken.
Statistics: For 5 subjects the ICC (2,3) was computed for intra and interrater reliability. For 10 subjects (ICC3,3) intertrial consistency was done. Paired t tests assessed grip strength, change after 2MWT for vital signs, changes in cortisol, and assess the HAQ-DI between administrations. Pearson correlations were completed on HAQ-DI variables, RPE, saliva, and vital signs.
Results: All intra and interrater reliability, intertrial agreement was found to be good to excellent. Paired t tests indicated that the HAQ-DI was stable and indicated a moderate perception of pain and reductions in quality of life. These participants showed evidence of impairments relative to normal. There were no differences in saliva cortisol levels.
Conclusion: These tests appear to have utility in identification of impairments in bodily systems, activity levels and participation in these participants. Further investigation is necessary in order to establish the optimal management.
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