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Abstract
BackgroundDespite the high prevalence of depressive symptoms in Fabry disease (FD), it is unclear which patient characteristics are important in relation to these symptoms. Additionally, the impact of coping styles in relation to depressive symptoms in FD has been unexplored. Determining the impact of different factors relating to depressive symptoms in FD can guide both prevention and treatment of these symptoms.MethodsDepressive symptoms (Center for Epidemiologic Studies Depression scale (CESD)) and coping styles (Utrecht Coping List) were assessed in a Dutch FD cohort. Other potentially important variables were identified from FD literature and assessed in this cohort. Relations were evaluated using multiple linear models.ResultsPotentially important variables in FD literature were: pain, unemployment, health perception, being single, comorbidities and stroke. Employed coping styles were “avoidance and brooding”, “positivity and problem solving” and “seeking social support”. Thirty-one of the 81 FD patients (38%) had depressive symptoms. CESD-scores were lower in patients with better health perception and more “positivity and problem solving” and higher in patients with more pain and “avoidance and brooding”. The best model explained 70% (95%CI: 54–76%) of observed variance of the CESD.ConclusionsDepressive symptoms in FD are related to pain, negative health perception and use of specific coping styles. Psychological interventions could be employed to alter coping behavior and alleviate depressive symptoms.
Highlights
Despite the high prevalence of depressive symptoms in Fabry disease (FD), it is unclear which patient characteristics are important in relation to these symptoms
The exploratory factor analysis (EFA) reduced the number of Utrecht Coping List (UCL) scales for the multiple regression analysis while providing a reflection of coping styles employed by FD patients
CESD Center for Epidemiologic Studies Depression scale, BPI Brief Pain Inventory, PSQI Pittsburgh Sleep Quality Index, 36-item Short Form Survey (SF-36) Short Form-36 Health Survey aPresence or absence of subjective cognitive complaints bpresence or absence of objective cognitive impairment. In this cross-sectional cohort study including more than half of the Dutch FD patients we found a high prevalence of depressive symptoms (38%), comparable to earlier work in FD patients [4]
Summary
Despite the high prevalence of depressive symptoms in Fabry disease (FD), it is unclear which patient characteristics are important in relation to these symptoms. The impact of coping styles in relation to depressive symptoms in FD has been unexplored. Determining the impact of different factors relating to depressive symptoms in FD can guide both prevention and treatment of these symptoms. Accumulation of globotriaosylceramide and related compounds occurs in various cell types due to deficiency of α-galactosidase A activity Accumulation of those substrates may result in damage of the kidneys, heart and brain [1]. Important predictors of symptoms and complications in FD are sex and phenotype [2].
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