A survey conducted in conjunction with the Dravet Syndrome Foundation suggests that many patients with DS do not undergo the transition of care from pediatric to adult neurology providers, with caregivers of those who did expressing some concerns about the process.
The findings of an electronic survey of adult individuals with Dravet syndrome (DS) and their caregivers suggest that a large number of these patients do not undergo a transition of care from pediatric physicians to adult physicians, highlighting a possible detriment to the optimal care of these individuals.1
Ultimately, of the 46 survey responses that were included in the analysis, almost two-thirds (n = 29; 63%) of the adults with DS did not undergo the transition to adult care, and most were still being followed by their pediatric physicians (n = 20; 71%). Other reasons for a lack of transition of care included discharge from the pediatric clinic (n = 5; 18%), diagnosis by an adult neurologist or epileptologists (n = 2; 7.1%), or other (n = 1; 3.6%).
Of the individuals who had undergone, or were undergoing, the transition of care (n = 17; 37%), the average age of patients when the conversations about transition began with the pediatric care team was 19 years (range, 16-27). These patients first saw an adult neurology physician at an average age of 21.6 years (range, 18-28). Of those who transitioned care, 29% (n = 5) were being seen by both their pediatric and adult provider simultaneously, for a range of 4 to 24 months, with a single respondent still being seen by both at the time of the survey.
The data were compiled in conjunction with the Dravet Syndrome Foundation and presented at the 2022 American Epilepsy Society Annual Meeting, held December 2 to 6, in Nashville, Tennessee, by Fábio A. Nascimento, MD, an assistant professor of neurology in the Division of Epilepsy, and the director of the Adult Epilepsy Genetics Program, Epilepsy Transition Program, and Adult Tuberous Sclerosis Clinic at Washington University in St. Louis. “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,” Nascimento and colleagues concluded.
“Caregivers of over half of adults with DS who underwent transition of care reported that the receiving adult team 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,” they wrote.
All told, more than half of the 17 surveyed caregivers who underwent a transition of care noted that these challenges were either never or rarely addressed. The only item that was indicated as something that always or often occurred was the addressing of patients’ self-advocacy capabilities—still only reported by fewer than half of the caregivers.
“While many caregivers rated the transition process as good or better, a substantial number still found the process to be only fair or poor, highlighting the need for further supports and improvements,” Nascimento et al wrote. Overall, 63% of caregivers rated the transition process as either good, very good, or excellent, with most rating it as very good.
The caregiver perceptions of adult neurology providers concerning a number of aspects of clinical care were also assessed, including their attentiveness and ability to accommodate patients with behavioral/cognitive challenges, their knowledge about caring for patients with intellectual disability, their knowledge about the inclusion/collaboration with caregivers in clinical decision making, and their knowledge about DS in adults, in addition to their general attentiveness and availability. Most caregivers responded that their adult providers were very much or moderate at all of these surveyed items. Only 1 item, knowledge about DS in adults, received a response of not at all, from less than 2% of the respondents.
The patients included in the study were a mean age of 24.1 years (range, 18-37), with a mean age at diagnosis of 12 years (range, 1-37). The vast majority (n = 39; 84.8%) of patients had received a genetic diagnosis. Most of the patients (n = 24; 54.5%) were from suburban areas, followed by urban (n = 13; 29.5%), and rural (n = 7; 15.9%) areas. Responses were collected between February 17, 2022, and July 31, 2022.
“Transition of care from the pediatric to adult healthcare setting can be particularly challenging for patients with DS and their caregivers,” Nascimento and colleagues wrote. “Navigating the transition of care has been reported as one of the major concerns and stressors for caregivers to teenagers and adults with DS.”