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Clinical characteristics, resource utilization, quality of life and care situation for patients with Dravet syndrome in Germany

Kalski M, Schubert-Bast S, Kieslich M, Leyer AC, Polster T, Herting A, Mayer T, Trollmann R, Neubauer BA, Bettendorf U, Bast T, Wiemer-Kruel A, Von Spiczak S, Kurlemann G, Wolff M, Kluger G, Carroll J, Macdonald D, Pritchard C, Irwin J, Klein KM, Rosenow F, Strzelczyk A, Kay L (2019)

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Publication Type: Journal article

Publication year: 2019

Journal

DOI: 10.1007/s10309-019-00287-7

Abstract

Introduction and objective Dravet syndrome (DS) is a rare epileptic and developmental encephalopathy associated with cognitive impairment and delayed development due to genetic mutations predominantly in the SCN1A gene. This multicenter, prospective survey-based study collected data about the clinical characteristics, health care resource utilization, costs and quality of life (QoL) of patients with DS and their caregivers in Germany. Methods A retrospective questionnaire covering the previous 3-12 months was used in addition to a prospective diary over 3 months to confirm the retrospective data and to obtain detailed information about indirect costs, QoL, medication, seizures and health care resource utilization. The questionnaires and the diary were handed out to the parents via Dravet-Syndrome e.& x202f;V. and participating centers. Results The questionnaire and diary were completed by 93 and 77 caregivers of DS patients, respectively. Patient mean age was 10 years (range: 15 months-33.7 years), whereby the time to diagnosis was significantly lower in the last two decades. In the previous 12 months, 95% of patients experienced at least one epileptic seizure. Fever (93.4%), excitement (56%), sleep deprivation (51.6%) and physical exertion (50.5%) were reported as the factors that triggered seizures. The lifetime prevalence of status epilepticus was 77%, with 28% having had at least one episode of status epilepticus within the last year. Patient QoL was lower compared with the general population, with 46% of the parents reporting symptoms of depression. The 3-month direct healthcare costs were 6043 & x202f;euro per patient, the largest components of which were inpatient costs (1702 & x202f;euro) followed by nursing care services (1130 & x202f;euro), anticonvulsant drugs (892 & x202f;euro) and ancillary treatment (559 & x202f;euro). The 3-month total indirect cost for mothers was 4399 & x202f;euro and for fathers 391 & x202f;euro. Conclusion The DS is associated with frequent and often treatment refractory seizures, including status epilepticus as well as various comorbidities. This study shows the substantial associated socioeconomic impact of the disease, with high direct and indirect costs and reduced QoL for patients and the caregivers. In order to achieve an improvement in the QoL for patients with DS and caregivers, new treatment and care concepts are necessary.

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APA:

Kalski, M., Schubert-Bast, S., Kieslich, M., Leyer, A.-C., Polster, T., Herting, A.,... Kay, L. (2019). Clinical characteristics, resource utilization, quality of life and care situation for patients with Dravet syndrome in Germany. Zeitschrift für Epileptologie. https://doi.org/10.1007/s10309-019-00287-7

MLA:

Kalski, Malin, et al. "Clinical characteristics, resource utilization, quality of life and care situation for patients with Dravet syndrome in Germany." Zeitschrift für Epileptologie (2019).

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