Abstract
Hood V, Berg AT, Knupp KG, Koh S, Laux L, Meskis MA, Zulfiqar-Ali Q, Perry MS, Scheffer IE, Sullivan J, Wirrell E, Andrade DM. Epilepsia. 2022;63(7):1778-1786. doi:10.1111/epi.17250 Vaccination against the SARS-CoV-2 virus is a primary tool to combat the COVID-19 pandemic. However, vaccination is a common seizure trigger in individuals with Dravet syndrome (DS). Information surrounding COVID-19 vaccine side effects in patients with DS would aid caregivers and providers in decisions for and management of COVID-19 vaccination. A survey was emailed to the Dravet Syndrome Foundation’s Family Network and posted to the Dravet Parent & Caregiver Support Group on Facebook between May and August 2021. Deidentified information obtained included demographics and vaccination status for individuals with DS. Vaccine type, side effects, preventative measures, and changes in seizure activity following COVID-19 vaccination were recorded. For unvaccinated individuals, caregivers were asked about intent to vaccinate and reasons for their decision. Of 278 survey responses, 120 represented vaccinated individuals with DS (median age = 19.5 years), with 50% reporting no side effects from COVID-19 vaccination. Increased seizures following COVID-19 vaccination were reported in 16 individuals, but none had status epilepticus. Of the 158 individuals who had not received a COVID-19 vaccination, 37 were older than 12 years (i.e., eligible at time of study), and only six of these caregivers indicated intent to seek vaccination. The remaining 121 responses were caregivers to children younger than 12 years, 60 of whom indicated they would not seek COVID-19 vaccination when their child with DS became eligible. Reasons for vaccine hesitancy were fear of increased seizure activity and concerns about vaccine safety. These results indicate COVID-19 vaccination is well tolerated by individuals with DS. One main reason for vaccine hesitancy was fear of increased seizure activity, which occurred in only 13% of vaccinated individuals, and none had status epilepticus. This study provides critical and reassuring insights for caregivers and health care providers making decisions about the safety of COVID-19 vaccinations for individuals with DS.Objective:
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Commentary
The Covid-19 pandemic worsened mortality rates worldwide. A population mortality study from Hungary found that patients who died of Covid-19 younger than 50 years were 10 to 20 times more likely to have epilepsy (and intellectual disability) than what would have been expected. 1 Twelve percent of hospitalized Covid patients younger than 22 years had severe neurological manifestations, including a multisystem inflammatory syndrome; 25% died, and 40% had neurological sequelae, with a higher risk in patients with epilepsy and other neurological disorders. 2
We need to protect our vulnerable patients in current and future pandemics. Covid-19 vaccination prevented 19.8 million excess deaths around the world in the first year of vaccination so is highly effective at reducing mortality. 3
Hesitancy toward vaccines is still prevalent in Dravet syndrome (DS), from both a historical context, with false attribution of vaccination with causation, 4 and current fears around vaccine reactions or fever worsening seizures or causing status epilepticus.
Recent United States outbreaks of polio and measles in unvaccinated individuals show how vaccine hesitancy has led to re-emergence of diseases once thought almost abolished, at least in developed countries.
Hood et al collected survey data from families with DS on their experiences and outcomes of Covid 19 vaccination, to guide more effective strategies at promoting vaccination. 5 We tend to champion scientific controlled studies, but we can still glean highly relevant information from well-designed surveys in the real world and from listening to our patients and their families. This is relevant in rare diseases like DS, where large-scale prospective study recruitment is unrealistic and where patients with disabilities like in DS are regrettably excluded from vaccine clinical trials.
Two hundred seventy-eight families completed the survey, designed by the DS Foundation and Clinician Advisory Board, and distributed from the DS foundation database of 2316 emails. Ninety five percent of patients had a confirmed SCN1A gene mutation. One hundred nineteen (76%) patients with DS had undergone Covid 19 vaccination. They also obtained open opinions from those not electing to vaccinate or whose family member was younger than 12 years (the vaccine being only approved for older than 12 years at that time).
The main side effects of the Covid-19 vaccine were lethargy or soreness at the injection site, and occurred within 6 to 24 hours. Fever occurred in 5% after dose 1 and 19% after dose 2 of the vaccine. Having side effects after dose 1 predicted side effects after dose 2.
Eleven (9%) patients had seizures after the first dose. No patients had status epilepticus, a considerable concern beforehand.
The use of antipyretics or bridge therapy (a temporary benzodiazepine course) around the time of vaccination did not prevent seizures. In fact a seizure was more likely associated with antipyretic or bridge therapy, but possibly as a result of more severe epilepsy or its use being a reactive measure.
Of the 158 who were not vaccinated, 120 were younger than 12 years and half of this subgroup were going to get vaccinated upon reaching the age limit of approval. Six of the remaining 38 patients older than 12 years were planning to get vaccinated. For the other 32 patients, the most common reasons for not getting a vaccine were a prior vaccine associated seizure or concern about seizures or side effects. Some felt the vaccine was not necessary or was unsafe due to inadequate testing or lack of full FDA approval. Five patients declined vaccination for reasons not based on scientifically accurate information.
The study provides important insights into vaccine uptake or hesitancy in DS in the Covid-19 pandemic, but it is also relevant for future pandemics or vaccine indications. The low incidence of seizures and lack of status epilepticus is reassuring. Clearly, the risk of Covid induced complications and seizures is higher than any risk from the vaccine.
The study lacks data on Covid vaccination younger than 12 years and is a retrospective survey, with population and recall bias. However, DS is a rare disease and the authors are commended for having a large representative group of DS families.
Armed with this information, how can we improve vaccine uptake? Families are not reading scientific journals—we need to actively address vaccine hesitancy through patient education forums and public health initiatives. Most people get their information, and often health advice, from the internet—promoting factually correct online content from internet searches is of critical importance. 6
