Adults with Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome: a cross-sectional study of patient experiences with physical activity. Issue 10 (8th May 2022)

• Record Type: Journal Article
• Authors: Johansen, Heidi; Velvin, Gry; Lidal, Ingeborg
• Is Part Of: Disability and rehabilitation. Volume 44:Issue 10(2022)
• Page Start: 1968
• Page End: 1975
• Publication Date: 2022-05-08
• Subjects: Adults -- Loeys-Dietz syndrome -- physical activity -- postal questionnaire -- vascular Ehlers-Danlos syndrome; People with disabilities -- Periodicals; Rehabilitation -- Periodicals; 617.03
• DOI: 10.1080/09638288.2020.1815874
• Languages: English
• ISSNs: 0963-8288

Abstract

Abstract: Purpose: To study patient experiences with physical activity among persons with Loeys-Dietz- or vascular Ehlers-Danlos syndrome. Materials and methods: A postal questionnaire survey in 2018. Seventy adults with molecularly verified Loeys-Dietz syndrome types 1-4, or vascular Ehlers-Danlos syndrome recruited through a National Resource Centre for Rare Disorders in Norway. Results: The response rate was 74%, (Loeys-Dietz n = 34, vascular Ehlers-Danlos n = 18), aged 18 to 68 years, 58% women. In total, 88.5% reported they had received advice regarding physical activity and most (77%) had modified their habits. Fifty percent had an appropriate- and 40% a low mean physical activity sum score. Another 10% had too high physical activity levels due to high intensity scores. Lower scores with the Fatigue Severity Scale ( p = 0.033) and the anxiety subscale of the Hospital Anxiety and Depression Scale ( p = 0.021), were associated with high physical activity levels. About a third reported unmet rehabilitation needs. Conclusion: Many adults with Loeys-Dietz- or vascular Ehlers-Danlos syndrome may have a potential to reach more favorable physical activity levels by increasing the frequency and duration of activities. Future directions should include evaluation of effects of professional-led practical and safe physical activity sessions as well as customized multidisciplinary rehabilitation programs for these patient groups.