What Is the Treatment for Dravet Syndrome?

I’m terrified and at my wits end. My beautiful little girl has been just diagnosed with Dravet Syndrome, a childhood epilepsy disorder, and I’m in the middle of a crash course on this disease. My research has not made me feel less scared.

I had a healthy pregnancy and my little girl was healthy. Then, she got a regular cold virus when she was about seven or eight months old, and her dad and I noticed a weird shivering we attributed to the fever. We know now that was her first seizure, called a febrile seizure because a fever triggered it.

At 10 months, she was at a family birthday party and started seizing for five minutes after a bunch of people took flash photos of her and another girl – seizures induced by photic stimuli, I think it’s called.

Doctors at the time couldn’t find anything on the EEG, but last week, my baby had five febrile seizures in three days during another cold virus. After more testing, her neurologist finally told us she was having generalized seizures and myoclonic jerks, and a genetic mutation was responsible.

Our treatment team put her on a bunch anti-epilepsy drugs (AEDs), but she is still having a few seizures a day. The doctors say there’s no cure, but maybe something new is out there? What is the treatment for Dravet syndrome?

Dravet syndrome is a rare, but it’s one of a less-rare family of myoclonic seizure disorders beginning in early childhood. “Myoclonic” describes the main types of seizures associated with Dravet, which are jerking spasms of muscle groups that do not cause loss of consciousness. Your child may experience other types of seizures too, including generalized ones. You should have a pediatric neurologist to consult.

Sadly, there is no cure.

The severity of Dravet symptoms ranges from mild to profound, as do the resulting developmental disabilities. Up to 20% of infants diagnosed with Dravet die before adulthood, and most who live are reliant on caretakers throughout their lives, according to the U.S. National Institutes of Health.

• The mainstays of medical therapy for myoclonic epilepsy are valproic acid (sodium valproate), ethosuximide, or benzodiazepines (clonazepam or clobazam).
• Phenobarbital, lamotrigine, vigabatrin, and carbamazepine may worsen the seizures in some cases, so they shouldn’t be used continuously.
• Combination therapy with valproic acid and benzodiazepines is often helpful.
• Stiripentol is indicated for treatment of seizures associated with Dravet syndrome in patients aged 2 years or older who are taking clobazam, but isn’t useful on its own.
• The ketogenic diet – a diet extremely low in carbohydrates and high in fats and protein – may be useful in children with particularly refractory epilepsy.
  • Be careful when putting your child on this diet that they get the right nutrients; consult with your pediatrician and maybe a nutritionist.
  • Pay particular attention to the possibility of dehydration.
• Caution should be used in children with drop attacks, as they may fall and injure themselves. A helmet can be protective. Routine seizure precautions are applicable.

Two drugs first approved in 2018 and 2020 specifically for Dravet Syndrome have helped improve the prognosis, however.

• A purified form of cannabidiol derived from the cannabis plant called Epidiolex is FDA-approved for seizures associated Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), or tuberous sclerosis complex in patients aged 1 year or older.
  • Doctors don’t know how it works, but compared to conventional epilepsy drugs and a placebo, cannabidiol consistently showed a 35% to 40% drop in the number of seizures for the Epidiolex groups in several international studies.
• Fenfluramine (Fintepla) is indicated for treatment of seizures associated with Dravet syndrome in patients two years and older.