title: Satisfaction with life in adults with Marfan syndrome (MFS): associations with health-related consequences of MFS, pain, fatigue, and demographic factors.
abstract: The objective with this study was to explore satisfaction with life (SWL) among adults with Marfan syndrome (MFS) compared to the general Norwegian population and other patient groups and further to examine the associations between SWL and demographic factors, contact with social and health services, MFS-related health problems, chronic pain, and fatigue.
  
  
  
  This is a cross-sectional study with postal questionnaire, including the Satisfaction with Life Scale (SWLS), questions on demographic factors, health-related aspects of MFS, and validated instruments measuring chronic pain (Standardized Nordic Questionnaire) and fatigue (Fatigue Severity Scale). One hundred and seventeen adults with MFS were invited to participate, and 73 (62 %) participated.
  
  
  
  The SWLS mean score in adults with MFS was significantly lower than that reported for the general Norwegian population, but similar to or higher than that reported for other patient groups. Only fatigue, aortic dissection, and having regular contact with psychologist showed significant unique contribution to the SWLS score in the hierarchical multiple linear analyses. The total variance explained by the model was 45.2 % p ≤ 0.000, confirming that the combination of independent variables significantly predicted SWLS.
  
  
  
  The results reflect that MFS influences people's SWL and that particularly severe fatigue, aortic dissection, and psychological aspects are associated with lower SWL. This is important to take into account in the clinical work with people with MFS. Further investigation is needed, especially on larger sample groups. Studies with combination of qualitative and quantitative approaches are recommended to obtain more comprehensive and accurate knowledge about the consequences of MFS on satisfaction with life.
authors.affiliation: TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, 1450, Nesoddtangen, Norway. gry.velvin@sunnaas.no.|Department of Social Work, Child Welfare and Social Policy, Faculty of Social Sciences, Oslo and Akershus University College of Applied Sciences, Oslo, Norway. gry.velvin@sunnaas.no.
authors.forename: Gry
authors.initials: G
authors.lastname: Velvin
authors.affiliation: TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, 1450, Nesoddtangen, Norway.
authors.forename: Trine
authors.initials: T
authors.lastname: Bathen
authors.affiliation: TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, 1450, Nesoddtangen, Norway.|Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
authors.forename: Svend
authors.initials: S
authors.lastname: Rand-Hendriksen
authors.affiliation: Department of Social Work, Child Welfare and Social Policy, Faculty of Social Sciences, Oslo and Akershus University College of Applied Sciences, Oslo, Norway.
authors.forename: Amy Østertun
authors.initials: AØ
authors.lastname: Geirdal
mesh_terms: D000328:Adult; D000368:Aged; D017545:Aortic Aneurysm, Thoracic / Q000473:pathology / Q000523:psychology*; D059350:Chronic Pain / Q000150:complications*; D003430:Cross-Sectional Studies; D003710:Demography; D005221:Fatigue / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000473:pathology / Q000523:psychology*; D008875:Middle Aged; D009664:Norway; D010549:Personal Satisfaction; D011788:Quality of Life / Q000523:psychology*; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: Health symptoms and demographic factors; Marfan syndrome; Satisfaction with life