Abstracts

Rationale: Patients with Dravet syndrome (DS) and their caregivers must navigate a complex process upon transitioning from pediatric to adult healthcare settings. This process has been shown to be challenging both from a caregiver and provider standpoint. Our study further examines the current state of transition of patients with DS in the U.S.

Methods: An electronic survey was disseminated to caregivers of adult patients with DS (ie, older than 18 years of age) and residing in the US through the Dravet Syndrome Foundation. The survey consisted of 34 questions that evaluated patient demographics, clinical features, and details on the process of transition of care, if applicable. 

Results: Fifty-five responses were received, 9 of which were excluded due to patients not meeting inclusion criteria. Forty-six responses were ultimately included in the analysis. Patients’ current mean age was 24 years (range 18-37 years) and the mean age at diagnosis was 12 years (range 0-37 years). Diagnosis was mostly made through genetic testing (83%) vs. clinical observation (15%). Twenty-nine patients (63%) did not undergo transition of care because they were still followed by pediatric providers (69%) or because they entered the adult healthcare setting directly (ie, without undergoing a process of transition) (21%). Seventeen patients (37%) had undergone/were currently undergoing transition of care. They were seen by an adult provider for the first time between 18 and 28 years of age (mean 22 years), and they started discussing about transition with their pediatric teams between the ages of 16 and 27 years (mean 19 years). Most of these patients’ caregivers (65%) reported that the adult team never, rarely, or sometimes addressed that many patients with DS are not capable of self-advocacy. Similarly, the majority of these caregivers (82%) stated that the adult team never, rarely, or sometimes addressed caregiver concerns related to legal guardianship and end-of-life decision-making or included a multidisciplinary team in their care. Nonetheless, most caregivers reported that the adult team is at least moderately attentive and available (69%) and inclusive of caregivers in the decision-making process (94%). Moreover, most caregivers reported that the adult team is knowledgeable about DS, particularly in adults, (75%) and about caring for patients with intellectual disability and/or behavioral issues (81%). Overall, most caregivers rated the transition process as good, very good, or excellent (63%).

Conclusions: Almost two-thirds of adults with DS in this study did not undergo transition of care - mostly because they were still followed by pediatric providers. Caregivers of the roughly one-third of adults with DS who underwent transition of care reported that their receiving adult teams failed to consistently address concerns about legal guardianship, end-of-life decision-making, and the fact that many patients with DS are not capable of self-advocacy. Further, most adult care teams were not multidisciplinary. We believe that patients with DS and their caregivers would benefit from more accessible transition programs, which would be ideally equipped to deliver care tailored to these patients’ needs.

Funding: None