Introduction: Dravet Syndrome (DS) is a severe epileptic encephalopathy, generally characterized by refractory seizures, cognitive impairment, and behavior problems. Health-related quality of life ...Show moreIntroduction: Dravet Syndrome (DS) is a severe epileptic encephalopathy, generally characterized by refractory seizures, cognitive impairment, and behavior problems. Health-related quality of life (HRQoL) assesses the impact of a chronic disease on an individual’s well-being. This study investigates the course of HRQoL in patients with DS between 2015 and 2021/2022, and whether the course of HRQoL can be predicted by age, the course of minor and major seizures, cognitive impairment and behavior problems. Additionally, this study aims to explore the relations between parental empowerment, parental distress, and the HRQoL in patients. Methods: This study is partly longitudinal (N=41) and cross-sectional (N=51), and uses data from three different studies. The outcome variable HRQoL was assessed with the Pediatric Quality of Life Inventory (PedsQL). Parental empowerment was measured with the Family Empowerment Scale (FES) and parental distress with the Distress Thermometer for Parents (DT-P). A multiple linear regression analysis was conducted to see which comorbidities significantly contributed to the prediction of the course of HRQoL. Moreover, a mediation analysis was performed to study the relationship between parental empowerment and parental distress, with the hypothesized mediating role of HRQoL in patients. Results: HRQoL scores improved significantly over time (t(40) = -3.40, p = .002, d = 0.53). Only predictive factors age (t(35) = 3.03, p = .005) and the course of behavior problems (t(35) = -2.46, p = .019) contributed significantly to the prediction of the course of HRQoL. Lastly, no relation was found between parental empowerment and parental distress, indicating no mediation. However, significant associations were found between parental empowerment and HRQoL in patients (r(51) = 0.31, p = .029), and parental distress and HRQoL in patients (r(51) = 0.54, p < .001). Conclusions: This study suggests an improvement of HRQoL is predicted by an increase of age and a decline of behavior problems. Therefore, it would be beneficial to involve multidisciplinary teams including psychologists to provide support for behavior problems. Lastly, this study emphasizes the crucial role of the well-being of the caregivers, and advises healthcare professionals to pay attention to the caregiver’s care needs.Show less
