Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes : A mixed-method study
Baeza-Velasco, Carolina (Université Paris Cité. Laboratoire de Psychopathologie et Processus de Santé)
Rodriguez, Nuria (Health and Social Projects (with Heart))
Parra, Laura (Université Paris Cité. Laboratoire de Psychopathologie et Processus de Santé)
Gutiérrez Rosado, Teresa (Universitat Autònoma de Barcelona. Departament de Psicologia Clínica i de la Salut)

Fecha:	2023
Resumen:	Vascular 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. Sixteen 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. Compared 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. Our 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.
Derechos:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Lengua:	Anglès
Documento:	Article ; recerca ; Versió publicada
Materia:	Ehlers-Danlos sydromes ; Loeys-Dietz syndrome ; Psychosocial adjustment ; Quality of life ; Anxiety ; Depression ; Rare diseases ; Mixed-method
Publicado en:	Frontiers in psychology, Vol. 14 (february 2023) , ISSN 1664-1078

DOI: 10.3389/fpsyg.2023.1019863
PMID: 36925590

10 p, 408.3 KB

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