Open Access

Susanne Schubert-Bast, Lara Kay, Andreas Simon, Geoffrey Wyatt, Rowena Holland, Felix Rosenow, Adam Strzelczyk

• Highlights • Prevalence of probable DS identified from German healthcare data: 4.7 per 100,000. • Healthcare costs: €11,048 per patient-year, mostly inpatient care 47%, medication 26%. • Costs and hospitalizations greater in patients with rescue medication than without. • Mean (SD) of 5.0 (2.5) different ASMs prescribed per patient over study period. • Patients with probable DS had significantly higher mortality risk vs. controls (11.88% vs. 1.19%). Abstract Objective: Ten-year retrospective study to assess burden of illness in patients with probable Dravet syndrome (DS) identified from German healthcare data. Methods: In the absence of an International Classification of Diseases code, patients with probable DS were identified using a selection algorithm considering diagnoses and drug prescriptions. Primary analyses were prevalence and demographics; secondary analyses included healthcare costs, annual hospitalization rate (AHR) and length of stay (LOS), medication use, and mortality. Results: In the final study year, 64 patients with probable DS (mean [range] age: 33.2 [3–82] years; male: 48%) were identified. Prevalence: 4.7 per 100,000 people. During the study, 160 patients with probable DS were identified and followed up for 1,261 patient-years. Mean cost of healthcare was €11,048 per patient-year (PPY), mostly attributable to inpatient care (47%), medication (26%), and services and devices (19%). Annual healthcare costs were significantly greater for those with prescribed rescue medication (15% of patient-years) vs. without (€16,123 vs. €10,125 PPY, p < 0.001). Mean (standard deviation [SD]) AHR and LOS were 1.1 (1.7) and 17.5 (33.5) days PPY. AHR was significantly greater in patients with prescribed rescue medication vs. without (1.6 [2.0] vs. 1.0 [1.6] PPY, p < 0.001). Mean (SD) number of antiseizure medications prescribed was 2.6 (1.2) PPY and 5.0 (2.5) over the entire observable time for each patient. Mortality rate was significantly higher for probable DS vs. matched controls (11.88% [19 events] vs. 1.19% [172 events], p < 0.001). Conclusion: Probable DS is associated with substantial healthcare costs in Germany.