Ask A Keto Dietitian: Dravet Syndrome

Hello! My name is Robyn Blackford, and I am one of the ketogenic dietitians at Lurie Children’s Hospital in Chicago. I have been a full-time ketogenic dietitian in pediatrics for over 20 years. Our center manages close to 350 patients on a medical ketogenic diet. I believe that medical ketogenic diets have incredible possibilities and that all patients have a right to try the diet and be carefully followed by an expert medical ketogenic diet center.

I am also one of the Keto Ambassadors for Nutricia in North America, where I spend time mentoring and teaching medical ketogenic diets to healthcare professionals.

On a more personal note, I have been married to my husband, Jason, for many years and am the mom of 2 teenagers.

What is Dravet Syndrome?

Dravet Syndrome is a rare, catastrophic, lifelong form of epilepsy that begins in the first year of life with frequent and/or prolonged seizures. This epilepsy diagnosis affects 1 in every 15,700 individuals, and 80% will have a mutation in the SCN1A gene.

What are the symptoms of Dravet syndrome?

A study published in 2015 describes how patients with Dravet syndrome typically have at least 4 of the 5 following symptoms:

• Normal or near-normal cognitive and motor development before seizure onset
• Two or more seizures with or without fever before 1 year of age
• Seizure history consisting of myoclonic, hemi-clonic, or generalized tonic-clonic seizures
• Two or more seizures lasting longer than 10 minutes
• Failure to respond to first-line antiepileptic drug therapy with continued seizures after 2 years of age

Medical Ketogenic Diets

Do you have patients with Dravet syndrome who are following a medical ketogenic diet?

Yes, our Epilepsy Center has a vast number of patients with Dravet syndrome and many of them are on a ketogenic diet. Since patients with Dravet syndrome tend to be in the drug-resistant epilepsy category, meaning that they do not respond to first- and second-line medications, a ketogenic diet is often suggested as part of their next-line options.  Our Epilepsy Center has been responsible for many research studies involving those with Dravet syndrome and their families.

Is a medical ketogenic diet helpful in managing seizures for patients with Dravet syndrome?

Yes, a ketogenic diet can be quite helpful in managing seizures in those with this epilepsy syndrome. Since this diagnosis can be made early in life, I like to use the diet as part of their regimen as early as possible. I have seen many times where an early diagnosis and precise management can lead to successful seizure control and positive outcomes for our patients.

Is there any research on ketogenic diets for patients with Dravet syndrome?

Yes, there is research supporting the use of ketogenic diets for patients with Dravet syndrome. The Dravet Syndrome Foundation (DSF) website has a lot of information on funded research and current clinical trials for those with Dravet syndrome. The DSF was involved in the International Consensus Panel Study (2022) recommendations, that includes medical ketogenic diets as part of the treatment options.

In 2013, Dr. Linda Laux and I published a paper that found 65% of children with Dravet syndrome at our center following a ketogenic diet experienced a greater than 50% reduction in seizure frequency.

Do your patients with Dravet syndrome typically remain on their antiseizure medications (ASMs) while they are following a ketogenic diet?

Yes, many patients with Dravet syndrome remain on some ASMs. The wonderful team of professionals with whom I work always aim for the least number of seizures with the least side effects possible. So, they are always looking for ways to decrease medications without losing the seizure control patients have achieved. They look to balance quality of life with the seizures. I feel like a ketogenic diet is a great addition to a seizure management plan, especially when it is taking the place of a sedating medication that is not helpful to a patient’s quality of life.

Is there a specific type of ketogenic diet that is recommended for patients with Dravet syndrome?

No, there is not one specific type of ketogenic diet that is recommended. Any variation of a ketogenic diet (classic ketogenic diet, modified Atkins, etc.) can be helpful for seizure control. The preference for diet options may depend, for example, on the age and feeding differences between patients. So, please discuss these issues with your child’s neurologist and/or keto provider.

How long do patients with Dravet syndrome typically stay on a ketogenic diet?

Patients can be on the diet for as long as they need to be to help manage seizures. The keto team will require intermittent visits for follow-up and blood work to assess patient safety for the diet. Often patients are on the diet for a few years before trying to wean off or go to a less restrictive version (such as the modified Atkins diet) for maintenance. If a ketogenic diet fails to be beneficial, then it would be stopped more quickly according to the plan from your keto team.

Where can families of Dravet patients find more information about ketogenic diets?

Here are some of my favorite websites to visit for more information:

• Dravet Syndrome Foundation
• Charlie Foundation
• Lurie Children’s Ketogenic Diet Program