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ADULTS WITH LOEYS-DIETZ SYNDROME AND VASCULAR EHLERS-DANLOS SYNDROME: A CROSS-SECTIONAL STUDY OF LIFE SATISFACTION

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 National...

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Autores principales: JOHANSEN, Heidi, VELVIN, Gry, FUGL-MEYER, Kerstin, LIDAL, Ingeborg Beate
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Foundation for Rehabilitation Information 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8638722/
https://www.ncbi.nlm.nih.gov/pubmed/34705050
http://dx.doi.org/10.2340/jrm.v53.572
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author JOHANSEN, Heidi
VELVIN, Gry
FUGL-MEYER, Kerstin
LIDAL, Ingeborg Beate
author_facet JOHANSEN, Heidi
VELVIN, Gry
FUGL-MEYER, Kerstin
LIDAL, Ingeborg Beate
author_sort JOHANSEN, Heidi
collection PubMed
description 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 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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spelling pubmed-86387222022-02-08 ADULTS WITH LOEYS-DIETZ SYNDROME AND VASCULAR EHLERS-DANLOS SYNDROME: A CROSS-SECTIONAL STUDY OF LIFE SATISFACTION JOHANSEN, Heidi VELVIN, Gry FUGL-MEYER, Kerstin LIDAL, Ingeborg Beate J Rehabil Med Original Report 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 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. Foundation for Rehabilitation Information 2021-10-27 /pmc/articles/PMC8638722/ /pubmed/34705050 http://dx.doi.org/10.2340/jrm.v53.572 Text en © 2021 Journal of Rehabilitation Medicine https://creativecommons.org/licenses/by-nc/4.0/This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (https://creativecommons.org/licenses/by-nc/4.0/) .
spellingShingle Original Report
JOHANSEN, Heidi
VELVIN, Gry
FUGL-MEYER, Kerstin
LIDAL, Ingeborg Beate
ADULTS WITH LOEYS-DIETZ SYNDROME AND VASCULAR EHLERS-DANLOS SYNDROME: A CROSS-SECTIONAL STUDY OF LIFE SATISFACTION
title ADULTS WITH LOEYS-DIETZ SYNDROME AND VASCULAR EHLERS-DANLOS SYNDROME: A CROSS-SECTIONAL STUDY OF LIFE SATISFACTION
title_full ADULTS WITH LOEYS-DIETZ SYNDROME AND VASCULAR EHLERS-DANLOS SYNDROME: A CROSS-SECTIONAL STUDY OF LIFE SATISFACTION
title_fullStr ADULTS WITH LOEYS-DIETZ SYNDROME AND VASCULAR EHLERS-DANLOS SYNDROME: A CROSS-SECTIONAL STUDY OF LIFE SATISFACTION
title_full_unstemmed ADULTS WITH LOEYS-DIETZ SYNDROME AND VASCULAR EHLERS-DANLOS SYNDROME: A CROSS-SECTIONAL STUDY OF LIFE SATISFACTION
title_short ADULTS WITH LOEYS-DIETZ SYNDROME AND VASCULAR EHLERS-DANLOS SYNDROME: A CROSS-SECTIONAL STUDY OF LIFE SATISFACTION
title_sort adults with loeys-dietz syndrome and vascular ehlers-danlos syndrome: a cross-sectional study of life satisfaction
topic Original Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8638722/
https://www.ncbi.nlm.nih.gov/pubmed/34705050
http://dx.doi.org/10.2340/jrm.v53.572
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