"Daring to speak of SUDEP" - caregiver and neurologist perspectives on the risk for premature mortality in Dravet Syndrome

Abstract

Objective: There is limited data regarding provision of information and managing risk of premature mortality in Dravet Syndrome (DS). We aimed to investigate the perspectives of caregivers and neurologists on receiving and providing information and managing risk in relation to premature mortality in DS.

Methods: Semi-structured interviews were undertaken with caregivers of children (n = 12) and adults (n = 4) with DS and neurologists (n = 9). Caregiver interviews focused on perceptions of risk of premature mortality, views on information received and managing risk. Neurologist interviews focused on how to inform caregivers about the risk and on how to support caregivers in living with the risk. Data was analyzed via thematic analysis.

Results: Caregiver themes regarding perception of risk and information received included: Limited information from healthcare providers, SUDEP is scary, Navigating Uncertainty, Delivery is Important and Optimal timing varies. Neurologist themes regarding informing included: Inform early, Verbal is best, An individualized approach, Emotional issues to consider and Inform along with other risks. Caregiver themes regarding management of risk included: Use of surveillance methods, Need for better seizure control, Managing anxiety related to the risk, and Need for both emotional and practical support to manage risk. Neurologist themes included: Focus on practical steps such as use of surveillance while avoiding overprotectiveness and Need for psychological support for caregivers.

Significance: Caregivers want to be informed about the risk of premature mortality in DS in a physical meeting, even though it is 'scary'. When the neurologist fails to inform, information is found via other sources. Neurologists may prefer to give verbal information to facilitate adapting to the individual context, but complementary written information is important for caregivers. The multidisciplinary team is crucial for providing support for the caregivers to manage and live with the risk of premature mortality.

Keywords: Dravet Syndrome; Information; Mortality; Risk management; Sudden unexpected death in epilepsy.