COVID-19 vaccination is safe in patients with Dravet syndrome, but vaccine hesitancy among caregivers has impacted vaccine uptake in this patient population.

“Patients with neurological conditions can experience worse manifestations of COVID-19,” Danielle Andrade, MD, MSc, FRCPC, CSCN (EEG), explains. Therefore, it is very important to vaccinate patients with epilepsy such as those with Dravet syndrome. However, in patients with Dravet syndrome, seizures can be triggered by fever and by vaccines.”

Previously, Dravet syndrome was thought to be caused in part by vaccines, according to Dr. Andrade, because vaccination in early childhood coincided with seizure onset in a third of patients. While more recent research has shown that, in most patients, Dravet syndrome is caused by loss-of-function mutations in the SCN1A gene, “we knew that parents and caregivers might be more concerned about pediatric vaccines, including the COVID-19 vaccine,” she notes.

For a study published in Epilepsia, Dr. Andrade and colleagues sought to obtain information about COVID-19 vaccination among patients with Dravet syndrome, including preventive measures that were being used to reduce the likelihood of seizures as well as side effects. The goal was to use this information to help guide the decision-making process among caregivers and healthcare providers and to understand the choices and reasons for reluctance of those caring for individuals with Dravet syndrome who had not yet been vaccinated against COVID-19, according to the study results.

Small Increase in Seizure Activity & No Reports of Status Epilepticus

Dr. Andrade and colleagues analyzed results from 278 surveys conducted among members of Dravet syndrome support groups from May-August 2021. The respondents included 120 individuals with Dravet syndrome who had been vaccinated against COVID-19 (median age, 19.5) and 158 individuals who had not received the vaccine. At the time the survey was conducted, the COVID-19 vaccine was only available to individuals aged 12 and older.

“Half of the vaccinated individuals reported no adverse effects,” Dr. Andrade says. “Among the 50% of vaccinated individuals who reported side effects, which were mild and included light-headedness, lethargy, and soreness at the injection site, only 13% of those patients experienced an increase in seizure activity from baseline.”

That 13% equates to “a very small number of patients in a population known to experience fever-induced seizures,” notes Dr. Andrade.

“The other important take-home message is that no patients went into status epilepticus,” she says. “That was one of the concerns reported by caregivers who chose not to get patients vaccinated against COVID-19.”

Among the 158 patients who had not been vaccinated against COVID-19, 37 were eligible for the vaccine during the study period, but only six of the caregivers of these patients said they planned to have the patient vaccinated. Additionally, among the 121 responses from caregivers to children younger than 12 years, 60 said they did not intend to have their child with Dravet syndrome vaccinated against COVID-19 when they were eligible. Reasons for vaccine hesitancy included concerns about heightened seizure activity and vaccine safety (Figure).

Strategies for COVID-19 Vaccination & Directions for Future Research

Overall, results of the present study “are very encouraging,” says Dr. Andrade. “Our findings show that the vaccine is safe for patients who have Dravet syndrome, even though a small portion of participants in our study had an increase in baseline seizure activity,” she explains.

She also notes that caregivers in their study used certain strategies, such as preventive anti-pyretic medications and bridge anti-seizure medications, to prevent seizures during the period of COVID-19 vaccination, when the patient is more susceptible to an increase in seizure activity. These are tools that healthcare providers can suggest to patients with Dravet syndrome, who are about to receive the COVID-19 vaccine, and their caregivers, according to the study results.

“We now need to understand responses to the COVID-19 vaccine in the youngest patients with Dravet syndrome,” Dr. Andrade says. “At the time we did this research, the vaccine was only available to those aged 12 and older. We need to understand if the response in younger patients with Dravet syndrome is similar to what we’ve seen in the older population.”