Abstracts

Rationale: Dravet syndrome (DS) is a severe developmental and epileptic encephalopathy caused mostly by mutations in the SCN1A gene and characterized by intractable seizures that typically onset within the first year of life. Seizures in DS are commonly precipitated by vaccination, likely related to subsequent fever and/or immune activation. Vaccination against the SARS-CoV-2 virus has emerged as a primary tool in combating the current global corona virus disease (COVID-19) pandemic. However, nothing is known about how individuals with DS respond to these 'COVID-19 vaccinations'. To address this gap in knowledge and to better inform the decision making process for families and providers, this study used a survey to collect information from caregivers on the experiences of individuals with DS surrounding COVID-19 vaccination.

Methods: A survey was distributed to emails in the Dravet Syndrome Foundation’s (DSF) Family Network and posted to the Dravet Parent & Caregiver Support Group on Facebook between May and August 2021. Deidentified information including demographics and vaccination status of the individual with DS were collected. For those who had not yet received a COVID-19 vaccination, caregivers were asked about future vaccination plans and any reasons behind choices not to vaccinate. Caregivers of vaccinated individuals were asked questions about vaccination manufacturer, the timing and presence of side effects, preventative measures taken, and incidence of seizures proximal to vaccination.

Results: Responses from 120 caregivers indicated that COVID-19 vaccination was generally well-tolerated by individuals with DS (mean age 21 years), and more than 50% of respondents reported no side effects. Seizures proximal to vaccination were reported in 13% of all individuals, but no patients experienced status epilepticus. Side effects generally occurred within 24 to 48 hours of a vaccine dose, with lethargy and injection site soreness being the most commonly reported symptoms. An additional 158 responses represented unvaccinated individuals with DS (mean age 7 years). Of these, 58% (n=91) indicated they did not plan for the individual with DS to receive a COVID-19 vaccination once eligible. Reasons for COVID-19 vaccine-hesitancy were primarily related to fear of increased seizure activity with vaccination (78%), but there were also significant concerns related to safety of the vaccines in general.

Conclusions: This timely study addresses a significant gap in our knowledge surrounding the effects of COVID-19 vaccination on individuals with DS. Our results indicate that COVID-19 vaccination is generally well-tolerated by patients with DS, with only 13% reporting increased seizure activity proximal to vaccination. One of the primary concerns related to vaccine hesitancy was reported as fear of increased seizure activity, which importantly, is also directly addressed by the data collected here. This study provides valuable insight for caregivers and healthcare providers to make decisions about COVID-19 vaccination for individuals with DS.

Funding: Please list any funding that was received in support of this abstract.: This study did not received public or private funding.