Universität zu Köln

Andonian, Caroline, Beckmann, Juergen, Mayer, Oliver, Ewert, Peter, Freiberger, Annika, Huber, Maximilian, Kaemmerer, Harald, Kurschat, Christine, Lagler, Florian, Nagdyman, Nicole, Pieper, Lars, Regenbogen, Claudia and Freilinger, Sebastian . Quality of life in patients with Fabry?s disease: a cross-sectional study of 86 adults. Cardiovisc. Diagn. Ther.. SHATIN: AME PUBLISHING COMPANY. ISSN 2223-3660

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Abstract

Background: Fabry disease (FD) is a multi-org an disorder associated with severe physical and psychological impairments, particularly in adulthood. To date, comprehensive data on the psychological burden of FD are lacking. The present study assessed quality of life (QOL) in a representative cohort of adults with FD.Methods: Patient-reported outcome measures were retrospectively analyzed in 86 adults with FD (49.6 +/- 16.6 years; 62.8% female) and compared to adults with congenital heart defects (ACHD) which is another lifelong disease and affliction. QOL was assessed using the European Quality of Life 5 Dimensions 5 Levels questionnaire (EQ-5D-5L).Results: Subjects affected by FD reported an overall reduced QOL (EQ-VAS: 71.8 +/- 20.0). Most frequently reported complaints occurred within the dimensions pain/discomfort (69.7%), daily activities (48.9%) and anxiety/depression (45.4%). Compared to ACHD, individuals with FD scored significantly lower in the areas of pain/discomfort, usual activities and mobility (all P<0.05). Older age and female sex were particularly associated with diminished QOL (P=0.05).Conclusions: Patients with FD are at high risk for impaired QOL. They require additional support to cope with disease-related challenges. Increased attention should be directed towards improving their subjective well-being to potentially increase their QOL and long-term health outcomes.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Andonian, Caroline UNSPECIFIED UNSPECIFIED UNSPECIFIED Beckmann, Juergen UNSPECIFIED UNSPECIFIED UNSPECIFIED Mayer, Oliver UNSPECIFIED UNSPECIFIED UNSPECIFIED Ewert, Peter UNSPECIFIED UNSPECIFIED UNSPECIFIED Freiberger, Annika UNSPECIFIED UNSPECIFIED UNSPECIFIED Huber, Maximilian UNSPECIFIED UNSPECIFIED UNSPECIFIED Kaemmerer, Harald UNSPECIFIED UNSPECIFIED UNSPECIFIED Kurschat, Christine UNSPECIFIED UNSPECIFIED UNSPECIFIED Lagler, Florian UNSPECIFIED UNSPECIFIED UNSPECIFIED Nagdyman, Nicole UNSPECIFIED UNSPECIFIED UNSPECIFIED Pieper, Lars UNSPECIFIED UNSPECIFIED UNSPECIFIED Regenbogen, Claudia UNSPECIFIED UNSPECIFIED UNSPECIFIED Freilinger, Sebastian UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-685735
DOI:	10.21037/cdt-22-215
Journal or Publication Title:	Cardiovisc. Diagn. Ther.
Publisher:	AME PUBLISHING COMPANY
Place of Publication:	SHATIN
ISSN:	2223-3660
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language DIAGNOSIS; DEPRESSION; MANAGEMENT; FEMALES; IMPACT; RECOMMENDATIONS; MANIFESTATIONS; POPULATION; COHORT; MALES Multiple languages Cardiac & Cardiovascular Systems Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/68573