Adults with Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome: A cross-sectional study of life satisfaction

Abstract

Objective: To explore life satisfaction among adults with Loeys-Dietz and those with vascular Ehlers-Danlos syndrome.
 Design: Postal survey in 2018.
 Participants and methods: Persons with molecularly verified Loeys-Dietz syndrome or vascular Ehlers-Danlos syndrome were recruited through the TRS [AQ2] National Resource Centre for Rare Disorders in Norway. The study used the Life Satisfaction Questionnaire 11, Hospital Anxiety and Depression Scale, Fatigue Severity Scale, and questions about physical activity and disease burden. Descriptive statistics were conducted with Bonferroni corrections.
 Results: The response rate was 74%, 52 participants, age range 18–68 years, and 58% were women. Only half of the participants were satisfied with their lives as a whole. Participants reported dissatisfaction with vocation, somatic health, and sexual life in particular. Participants with vascular Ehlers-Danlos syndrome (n = 18) were satisfied with more areas of life than those with Loeys-Dietz syndrome (n = 34). Low overall satisfaction was significantly associated with severe fatigue (p = 0.002) and symptoms of anxiety (p = 0.001).
 Conclusion: This study provides important information about living with Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome. Future studies should identify factors that reduce/increase life satisfaction. Professionals in welfare systems need more information about the unique challenges of living with these diagnoses. Guidelines for research and clinical measurements of life satisfaction should be updated.

Pain and fatigue in adults with Loeys–Dietz syndrome and vascular Ehlers–Danlos syndrome, a questionnaire‐based study

“…Pain limited most participants in leisure activities, fewer in housekeeping and in work life. This is consistent with findings on life satisfaction reported in an earlier article on the same study population (Johansen et al, 2021) which showed low satisfaction with the leisure domain. These findings might indicate that adaptation to leisure and housekeeping is more difficult than customizing employment.…”

“…This cross‐sectional postal questionnaire survey was part of a larger study on physical function and psychosocial aspects in adults with HTADs (Johansen et al, 2020a; Johansen et al, 2020b; Johansen et al, 2021; Velvin et al, 2019).…”

Education and employment status among adults with Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome in Norway, a questionnaire based study

“…In a previous paper, we showed that a considerable group of LDS and vEDS participants left work before retirement age [4], and many scored to be dissatisfied with their vocational situation [15]. With data collected from the same study group, the aim of the current paper was to present a more detailed description of educational level and issues related to work participation (degree of physical demands in work, and work challenges).…”

“…This survey was part of a larger study on physical function and psychosocial aspects in adults with HTADs [4,[15][16][17][18][19]. In this paper, we used data from a cross-sectional quantitative, questionnaire-based study of individuals with LDS and vEDS of working age (18-67 years) (n = 50).…”

Fysisk aktivitet og helsekompetanse for personer med familiære torakale aortaaneurismer og -disseksjoner

“…Mange har også et muskel-skjelett-system som er påvirket, slik som overbevegelige ledd og skjelettfeilstillinger (1, 8), kroniske smerter (8,9), og angst (10,11), alvorlig fatigue (10,12) eller redusert livskvalitet (11,13) er vanlig. Mange har også utfordringer i arbeidslivet (8,11,12) og har vansker med å nne gode former for fysisk aktivitet (5).…”