International Symposium on Marfan, LDS and related conditions

The purpose of the project is to examine perceived health problems and how this affects quality of life, physical activity, education, work and family life, in adults with HTAADs. More research-based knowledge is important for the patients, their families and professionals.

 

The study is a combination of systematic reviews and a cross-sectional mixed method study of people with HTAADs in Norway.

 

The cross sectional mixed-method study: combing focus groups and quantitative questionnaire

 

In focus group interviews 47 adults participated: 11 with LDS, 11 with vEDS, 14 with MFS and 11 relatives of people with HTAADs.

 

In the questionnaire part of the study 52 adults participated: 34 with LDS and 18 with vEDS. Adults with MFS were not included in this part of the study, due to that

 

Velvin G, Wilhelmsen JE, Johansen H, Bathen T, Geirdal AØ. Systematic review of Quality of Life in persons with Hereditary Thoracic Aortic Aneurysm and Dissection diagnoses. Clinical Genetics. 2019; 95:661–676.

 

Johansen H, Velvin G, Lidahl I. Adults with Loeys–Dietz syndrome and vascular Ehlers–Danlos syndrome: A cross‐sectional study of health burden perspectives. American Journal of Medical Genetics part A, november 2019. https://doi.org/10.1002/ajmg.a.61396.

 

Velvin G, Johansen H, Vardeberg K, Sjögren Fugl-Meyer K, Wilhelmsen JE, Lidal I. Physical exercise for people with hereditable thoracic aortic disease. A study of patient perspectives. Disability and Rehabilitation, desember 2019. 17:1-8. doi: 10.1080/09638288.2019.1703145.

 

Johansen H, Velvin G, Lidal IB. Adults with Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome: a cross-sectional study of patient experiences with physical activity. Disability and Rehabilitation. 2020. DOI: 10.1080/09638288.2020.1815874.

 

Johansen H, Velvin G, Fugl-Meyer K, Lidal IB.  Adults with Loeys-Dietz syndrome and vascular Ehlers-danlos syndrome: A cross-sectional study of life satisfaction. J Rehabil Med 2021; 53: jrm00236.

 

Velvin G. & Johansen H. Fysisk aktivitet og trening for personer med familiære thorakale aortaaneurismer og aortadisseksjoner. En oversiktsartikkel. Fysioterapeuten Forskning. 2022, 1: 48-58. Article in Norwegian.

 

Johansen H. & Velvin G. Fysisk aktivitet og helsekompetanse for personer med familiære torakale aortaaneurismer og -disseksjoner. Sykepleien Forskning 2022;17(88791):e-8879. The article is also published in english translation in the same journal (Nursing Research): Physical activity and health literacy in people with familial thoracic aortic aneurysm and dissection .  

 

Johansen H. Velvin G & Lidal, I. B. (2022). Pain and fatigue in adults with Loeys–Dietz syndrome and vascular Ehlers–Danlos syndrome, a questionnaire-based study.​ American Journal of Medical Genetics Part A, 1– 12. https://doi.org/10.1002/ajmg.a.62858.