Objective: To identify factors associated with health-related quality of life (HRQOL) in midlife and older women with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorders (hEDS/HSD).
Design: Online, cross-sectional survey.
Setting: Online survey was administered to participants in Ehlers-Danlos syndrome Facebook groups.
Participants: Women ages 40 years and older with a diagnosis of hEDS/HSD.
Methods: Online survey including demographic and health history; 36-Item Short-Form Health Survey (SF-36) to measure HRQOL; and free-text questions related to things that reduced and improved well-being.
Results: Sixty-six women (mean age, 55 years) participated in the survey. Symptom onset was in childhood, but treatment was not sought until their late 20s. The mean age at diagnosis was the mid-40s; thus, the diagnosis was delayed by more than 18 years from the time participants first sought treatment. Comorbidities were common, with 62% of participants reporting seven or more comorbid diagnoses. The SF-36 scores indicated a high symptom burden and poor HRQOL, with worse mean scores in the physical domains compared to the mental domains. Symptom severity, treatment restrictions, environmental factors, comorbidities, and interactions with health care providers influenced HRQOL.
Conclusion: Nursing has an emerging role in research and education related to genetic syndromes and heritable connective tissue disorders, including hEDS/HSD, in midlife and older women. Future research should address the complex relationships among physical, social, personal, and environmental factors to maximize HRQOL in this population.
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