Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes: A mixed-method study
BackgroundVascular Ehlers-Danlos (vEDS) and Loeys-Dietz syndromes (LDS) are hereditary disorders of connective tissue having severe vascular complications (HDCTv) which lead to an increased risk of premature death. Little is known about the impact of the disease in patient’s daily life.MethodSixteen...
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Frontiers Media S.A.
2023-02-01
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| Series: | Frontiers in Psychology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fpsyg.2023.1019863/full |
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| author | Carolina Baeza-Velasco Carolina Baeza-Velasco Carolina Baeza-Velasco Nuria Rodriguez Laura Parra Teresa Gutiérrez-Rosado |
| author_facet | Carolina Baeza-Velasco Carolina Baeza-Velasco Carolina Baeza-Velasco Nuria Rodriguez Laura Parra Teresa Gutiérrez-Rosado |
| author_sort | Carolina Baeza-Velasco |
| collection | DOAJ |
| description | BackgroundVascular Ehlers-Danlos (vEDS) and Loeys-Dietz syndromes (LDS) are hereditary disorders of connective tissue having severe vascular complications (HDCTv) which lead to an increased risk of premature death. Little is known about the impact of the disease in patient’s daily life.MethodSixteen HDCTv patients (vEDS = 9 and LDS = 7), 16 age and sex-matched hypermobile Ehlers-Danlos syndrome patients (hEDS) and 18 healthy subjects (HS), responded to self-questionnaires assessing psychosocial adjustment, quality of life (QoL), anxiety, depression, pain, fatigue and sleep problems. Patients with HDCTv were also interviewed in order to explore qualitatively their experience with the disease.ResultsCompared with HS, patients with HDCTv scored significantly higher on anxiety, depression, fatigue, sleep problems, and lower on QoL. Most HDCTv patients (93.8%) have optimal psychosocial adjustment. In addition, HDCTv patients scored higher on QoL and psychosocial adjustment, but lower in pain, fatigue, sleep problems, and depressive symptoms than hEDS patients. Four main themes were identified in qualitative analyses: living with HDCTv, knowledge/ignorance of the disease, health behaviors/self-care and coping strategies.ConclusionOur results suggest that despite the negative impact of HDCTv on the patients’ daily lives, overall, they present an optimal disease adjustment which points to appropriate coping strategies. More research in psychosocial aspects of people with these rare diseases are needed to confirm these results and better understand their needs. |
| first_indexed | 2024-04-10T06:58:32Z |
| format | Article |
| id | doaj.art-b6154740b86f4b6785aba298cac86997 |
| institution | Directory Open Access Journal |
| issn | 1664-1078 |
| language | English |
| last_indexed | 2024-04-10T06:58:32Z |
| publishDate | 2023-02-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Psychology |
| spelling | doaj.art-b6154740b86f4b6785aba298cac869972023-02-28T06:18:24ZengFrontiers Media S.A.Frontiers in Psychology1664-10782023-02-011410.3389/fpsyg.2023.10198631019863Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes: A mixed-method studyCarolina Baeza-Velasco0Carolina Baeza-Velasco1Carolina Baeza-Velasco2Nuria Rodriguez3Laura Parra4Teresa Gutiérrez-Rosado5Laboratoire de Psychopathologie et Processus de Santé (LPPS UR4057), Université Paris Cité, Boulogne-Billancourt, Paris, FranceDepartment of Emergency Psychiatry and Acute Care, CHU Montpellier, Montpellier, FranceInstitute of Functional Genomics, University of Montpellier, CNRS, INSERM, Montpellier, FranceHealth and Social Projects (with Heart), Barcelona, SpainLaboratoire de Psychopathologie et Processus de Santé (LPPS UR4057), Université Paris Cité, Boulogne-Billancourt, Paris, FranceDepartment of Clinical and Health Psychology, University Autonomous of Barcelona, Barcelona, SpainBackgroundVascular Ehlers-Danlos (vEDS) and Loeys-Dietz syndromes (LDS) are hereditary disorders of connective tissue having severe vascular complications (HDCTv) which lead to an increased risk of premature death. Little is known about the impact of the disease in patient’s daily life.MethodSixteen HDCTv patients (vEDS = 9 and LDS = 7), 16 age and sex-matched hypermobile Ehlers-Danlos syndrome patients (hEDS) and 18 healthy subjects (HS), responded to self-questionnaires assessing psychosocial adjustment, quality of life (QoL), anxiety, depression, pain, fatigue and sleep problems. Patients with HDCTv were also interviewed in order to explore qualitatively their experience with the disease.ResultsCompared with HS, patients with HDCTv scored significantly higher on anxiety, depression, fatigue, sleep problems, and lower on QoL. Most HDCTv patients (93.8%) have optimal psychosocial adjustment. In addition, HDCTv patients scored higher on QoL and psychosocial adjustment, but lower in pain, fatigue, sleep problems, and depressive symptoms than hEDS patients. Four main themes were identified in qualitative analyses: living with HDCTv, knowledge/ignorance of the disease, health behaviors/self-care and coping strategies.ConclusionOur results suggest that despite the negative impact of HDCTv on the patients’ daily lives, overall, they present an optimal disease adjustment which points to appropriate coping strategies. More research in psychosocial aspects of people with these rare diseases are needed to confirm these results and better understand their needs.https://www.frontiersin.org/articles/10.3389/fpsyg.2023.1019863/fullEhlers-Danlos sydromesLoeys-Dietz syndromepsychosocial adjustmentquality of lifeanxietydepression |
| spellingShingle | Carolina Baeza-Velasco Carolina Baeza-Velasco Carolina Baeza-Velasco Nuria Rodriguez Laura Parra Teresa Gutiérrez-Rosado Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes: A mixed-method study Frontiers in Psychology Ehlers-Danlos sydromes Loeys-Dietz syndrome psychosocial adjustment quality of life anxiety depression |
| title | Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes: A mixed-method study |
| title_full | Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes: A mixed-method study |
| title_fullStr | Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes: A mixed-method study |
| title_full_unstemmed | Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes: A mixed-method study |
| title_short | Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes: A mixed-method study |
| title_sort | adjustment to disease and quality of life in people with vascular ehlers danlos and loeys dietz syndromes a mixed method study |
| topic | Ehlers-Danlos sydromes Loeys-Dietz syndrome psychosocial adjustment quality of life anxiety depression |
| url | https://www.frontiersin.org/articles/10.3389/fpsyg.2023.1019863/full |
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