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Cited by 15Connective tissue disorders researchMusculoskeletal pain and rehabilitationPsychological Well-being and Life Satisfaction

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

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Johansen, Heidi, Velvin, Gry, Fugl-Meyer, Kerstin, Lidal, Ingeborg Beate·2021·Journal of Rehabilitation Medicine
DOI 10.2340/jrm.v53.572PMC PMC8638722
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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.

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  • Types of pain in hEDS patients were described as chronic back pain (78.6%), chronic arthralgias/joint pain (83.3% of 99%), chronic myalgias (85.7%), and recurrent headaches (76.3%).

    “Types of pain in hEDS patients were described as chronic back pain (78.6%), chronic arthralgias/joint pain (83.3% of 99%), chronic myalgias (85.7%), and recurrent headaches (76.3%).30,46”

    From Role of Anxiety in Individuals with Generalized Joint Hypermobility: A Systematic Review (2025) · cites this paper as [46]

    The Results (Demographic and clinical characteristics; page not available) report chronic musculoskeletal pain in 79% of 52 participants with LDS or vEDS, not hEDS. The Methods (Questionnaire; page not available) measure musculoskeletal pain generally; searches for the quoted percentages and for hEDS-specific pain types found no passage reporting the claimed breakdown. (LLM, conf=0.76)

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Sorted by citations

Authors
Johansen, H., Velvin, G., Fugl-Meyer, K., & Lidal, I. B.
Affiliations
  • Sunnaas sykehus (NO)
  • OsloMet – Oslo Metropolitan University (NO)
Corresponding author
Johansen, Heidi
ORCID
Venue
Journal of Rehabilitation Medicine
Publisher
MJS Publishing, Medical Journals Sweden AB
Published
2021-10-27
Volume
53
Issue
11 (November)
Pages
jrm00236-jrm00236
ISSN
  • 1650-1977 (ISSN-L)
  • 1651-2081
DOI
10.2340/jrm.v53.572
PMC
PMC8638722
OpenAlex
W3209574689
Cited by
15
References
3
Citations by year
2025: 6 · 2024: 4 · 2023: 2 · 2022: 3
FWCI
1.27
Type
journal article
Language
en
Open access
Licence
Locations
  • Journal of Rehabilitation Medicine (publishedVersion, cc-by-nc)
  • PubMed Central (publishedVersion, cc-by-nc)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
  • Health Sciences › Medicine › Pharmacology › Musculoskeletal pain and rehabilitation
  • Social Sciences › Psychology › Social Psychology › Psychological Well-being and Life Satisfaction
Keywords
Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, life satisfaction, fatigue, anxiety, depression, sexual satisfaction, job satisfaction, physical health, disease burden, physical activity
MeSH terms
Adolescent, Adult, Aged, Cross-Sectional Studies, Ehlers-Danlos Syndrome, Female, Humans, Middle Aged, Personal Satisfaction, Surveys and Questionnaires, Young Adult, Loeys-Dietz Syndrome

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