Psychosocial Aspects in Adults with Familial Thoracic Aortic Aneurysm/Dissection (FTAAD): A Cross-sectional Study on Quality of Life, Challenges in Physical Activity, Education, Work, and Family Life
Project leader
Gry Velvin, senioradvisor (MSW, cand.polit. Ph.D.), TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital.
Background
Familial thoracic aortic aneurysms and aortic dissections (FTAAD) refer to a group of inherited conditions affecting the main artery (aorta) and other major blood vessels (arteries). This group is at high risk for severe medical events/consequences and requires close monitoring. There is limited knowledge on how these diagnoses impact an individual's life situation and their relatives. Further research-based knowledge is essential for the care of individual patients, their families, and consultations to professionals.
Objective
The objective of this project is to assess health complaints in adults with FTAAD diagnoses and explore psychosocial factors such as quality of life, physical activity, education, work, and family life.
Methods and materials
The study is designed and conducted in collaboration with the Norwegian Marfan Association and the Norwegian EDS Association.
The study consists of three sub-studies:
Sub-study 1: Review of pertinent research and literature: Systematic review on the quality of life in FTAAD, Systematic review on fatigue in FTAAD, Literature review on physical activity with FTAAD.
Sub-study 2: Focus Group Interviews with a total of 47 adults (n=36 with FTAAD diagnose, n=11 relatives to people with FTAAD diagnosis). Participants were recruited through invitations to participants a family course on FTAAD diagnoses (patients with vEDS and LDS) at Frambu in 2017, and participants from the Marfan Association's meeting in 2018 (patients with MFS). The main primary topics were: i) physical activity with a FTAAD diagnosis and ii) psychosocial factors and quality of life iii) being a relative to people with a FTAAD diagnosis. The focus groups were audio-recorded and transcribed verbatim and analysed by content analysis.
Sub-study 3: Quantitative questionnaire survey: All patients registered with a LDS and vEDS diagnoses at TRS National Resource Centre were invited to participate (52 of 70 participated). Participants were asked to fill out a study-specific questionnaire that covers demographic information, specific health complaints related to the diagnosis, physical activity, as well as validated questionnaires for quality of life, psychological factors, chronic pain, and fatigue. The study-specific part of the questionnaire was based on questionnaires used in analogous studies.
The tools (interview-guide and the questionnaire) were developed in close cooperation with the user- representatives. The tools were tested in a pilot study with staff and user representatives and revised based on these feedbacks.
User Participation in the Study
Two adult diagnosed with FTAAD diagnoses and one relative to a person with FTAAD have been engaged as members of a reference group to ensure the quality, relevance, recruitment, analysis, dissemination and presentation of the results.
Results
To date, 10 scientific articles have been published in international and national journals, and one scientific paper is currently under review in a journal. The results from the study have been presented at several international/national conferences and user meetings. Additionally, the results from the study have been used as foundational material for information about FTAAD on the TRS website, and in collaborations and consultations with the health- and social services, the users and their relatives.
Gry Velvin (Phd), TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital
Heidi Johansen (MSc), TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital.
Ingeborg Lidal (MD, Ph.D.), TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital
Trine Bathen (MSc), TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital.
Amy Østertun Geirdal (Professor I, Ph.D), Department of Social Work, Child Welfare and Social Policy, Faculty of Social Science, Oslo Metropolitan University, Norway
Kerstin Fugl-Meyer (Professor I, Ph.D.), Department of Neurobiology, Care Sciences and Society, Division of Family Medicine and Primary Care, Karolinska Institutet, Stockholm, Sweden
Ethical approvals
REK: 2017/745
Funding
No
Time period
Ends 31.12.25
Hva er TRS?
TRS er et nasjonalt kompetansesenter for sjeldne, medfødte skjelett- og bindevevstilstander, ryggmargsbrokk og dysmeli.
Målgruppene er de som har en av diagnosene, pårørende, helsepersonell og andre aktuelle fagpersoner.
Senteret er en del av Nasjonal kompetansetjeneste for sjeldne diagnoser og ligger på Sunnaas sykehus på Nesodden utenfor Oslo.
