The clinical, economic, and humanistic burden of Dravet syndrome – A systematic literature review

Sullivan, J., Deighton, A. M., Vila, M. C., Szabo, S. M., Maru, B., Gofshteyn, J. S., James, E. S., Rico, S. and Zuberi, S. M. (2022) The clinical, economic, and humanistic burden of Dravet syndrome – A systematic literature review. Epilepsy and Behavior, 130, 108661. (doi: 10.1016/j.yebeh.2022.108661) (PMID:35334258)

Abstract

Dravet syndrome (DS) is a developmental and epileptic encephalopathy with evolving disease course as individuals age. In recent years, the treatment landscape of DS has changed considerably, and a comprehensive systematic review of the contemporary literature is lacking. Here we synthesized published evidence on the occurrence of clinical impacts by age, the economic and humanistic (health-related quality-of-life [HRQoL]) burden, and health state utility. We provide an evidence-based, contemporary visualization of the clinical manifestations, highlighting that DS is not limited to seizures; non-seizure manifestations appear early in life and increase over time, contributing significantly to the economic and humanistic burden of disease. The primary drivers of HRQoL in DS include seizure severity, cognition, and motor and behavioral problems; in turn, these directly affect caregivers through the extent of assistance required and consequent impact on activities of daily living. Unsurprisingly, costs are driven by seizure-related events, hospitalizations, and in-home medical care visits. This systematic review highlights a paucity of longitudinal data; most studies meeting inclusion criteria were cross-sectional or had short follow-up. Nonetheless, available data illustrate the substantial impact on individuals, their families, and healthcare systems and establish the need for novel therapies to address the complex spectrum of DS manifestations.

• Item Type: Articles
• Keywords: Clinical evolution, Health-related quality of life, Caregiver burden, Economic burden, Dravet syndrome
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Zuberi, Dr Sameer
• Authors: Sullivan, J., Deighton, A. M., Vila, M. C., Szabo, S. M., Maru, B., Gofshteyn, J. S., James, E. S., Rico, S., and Zuberi, S. M.
• College/School: College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing
• Journal Name: Epilepsy and Behavior
• Publisher: Elsevier
• ISSN: 1525-5050
• ISSN (Online): 1525-5069
• Published Online: 22 March 2022
• Copyright Holders: Copyright © 2022 The Authors
• First Published: First published in Epilepsy and Behavior 130: 35334258
• Publisher Policy: Reproduced under a Creative Commons licence