Quality of life in patients with Fabry's disease: a cross-sectional study of 86 adults.
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ABSTRACT: Background
Fabry disease (FD) is a multi-organ 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.
SUBMITTER: Andonian C
PROVIDER: S-EPMC9412216 | biostudies-literature | 2022 Aug
REPOSITORIES: biostudies-literature
Publications
Quality of life in patients with Fabry's disease: a cross-sectional study of 86 adults.
Andonian Caroline C Beckmann Jürgen J Mayer Oliver O Ewert Peter P Freiberger Annika A Huber Maximilian M Kaemmerer Harald H Kurschat Christine C Lagler Florian F Nagdyman Nicole N Pieper Lars L Regenbogen Claudia C Freilinger Sebastian S
Cardiovascular diagnosis and therapy 20220801 4
<h4>Background</h4>Fabry disease (FD) is a multi-organ 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.<h4>Methods</h4>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 ...[more]
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