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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Foundation for Rehabilitation Information
2021
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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. |
format | Online Article Text |
id | pubmed-8638722 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Foundation for Rehabilitation Information |
record_format | MEDLINE/PubMed |
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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