Ask the Keto Dietitian Series: Dravet Syndrome

dravet-syndrome

A Q&A with ketogenic dietitian Robyn Blackford, RDN, LDN. For more posts in our “Ask the Keto Dietitian” series, click here.

Can you tell us a little bit about yourself and your work as a ketogenic dietitian?

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 15 years. Our center manages close to 300 patients on the medical ketogenic diet. I believe that the medical ketogenic diet has 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 the medical ketogenic diet 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

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 the medical ketogenic diet. Since patients with Dravet Syndrome tend to be in the intractable epilepsy category, meaning that they do not respond to first- and second-line medications, the medical 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 the medical ketogenic diet helpful in managing seizures for patients with Dravet Syndrome?

Yes, the medical 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 the medical ketogenic diet helpful in managing seizures for patients with Dravet Syndrome?

Yes, there is research supporting the use of the medical ketogenic diet for patients with Dravet Syndrome. The Dravet Syndrome Foundation (DSF) website has a lot of information on past and current research studies for those with Dravet Syndrome. Included on the site is the Patient-Centered Outcomes Research (PCOR), which provides patients and their clinicians with evidence-based information in order to help them make more informed health care decisions and answer questions about the potential benefits and risks of different care options. The medical ketogenic diet is included in the PCOR.

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

Do your patients with Dravet Syndrome typically remain on their antiepileptic drugs (AEDs) while they are following the medical ketogenic diet?

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

Is there a specific type of medical ketogenic diet that is recommended for patients with Dravet Syndrome (for example, classical ketogenic diet, modified Atkins diet, etc)?

No, there is not one specific type of medical ketogenic diet that is recommended. Any of these versions of the diet 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 neurology and/or keto provider.

How long do patients with Dravet Syndrome typically stay on the medical 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 the safety of the patient for the duration of the diet. Often times patients are on the diet for a few years before attempting a wean of the diet or simply going to a less restrictive version of the diet (such as the modified Atkins diet) for maintenance. If the medical 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 the medical ketogenic diet?

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

References:

Laux L, Blackford R. The ketogenic diet in Dravet syndrome. J Child Neurol. 2013;28(8):1041-1044. doi:10.1177/0883073813487599.

Wu, E., et. al. (2015). Incidence of Dravet Syndrome in a US Population. Pediatrics 136(5): 1310-e1315. doi: 10.1542/peds.2015-1807.

Hailey’s Story: Epilepsy & The Medical Ketogenic Diet

ketogenic-diet-for-seizures

About Me:

My name is Hailey, a twenty-four-year-old college student happily living on the coast with my adventurous boyfriend of five years and our goofy husky German shepherd. I am a student yoga teacher and am pursuing my bachelor’s degree in Science Education. While at first glance I may look like a typical, healthy, 20-something college student, people are usually shocked to hear what I’m going through medically. You see, I have uncontrolled seizures, also known as intractable epilepsy, along with a neuro-cardiac condition called Dysautonomia. My seizures started mid-summer when I was thirteen years old. From there, my health rapidly declined. At my worst, I was having over forty seizures daily. I had to wear a helmet and was wheelchair bound. We tried several different anti-seizure medications, but unfortunately, nothing stopped my seizures.

Worsening Seizures:

When I was eighteen, in a dental office, I had a seizure and my breathing did not return. Thanks to the quick action of the dental office and first responders, I survived and recovered after just three days on a ventilator in the Intensive Care Unit (ICU). During my time there, my doctors recommended that I start eating healthier and exercising.  As a kid my diet was terrible, eating lots of junk food. However, I was a healthy weight, so my parents never worried. With low expectations, but desperate, I “cold-turkey” changed my diet, eating mostly whole foods and cutting drastically back on processed foods. I started feeling much better. I joined the swim team, and fell in love with yoga, rock climbing and jiu jitsu (sorry mom). 

The Medical Ketogenic Diet:

ketoegnic-diet-for-seizures

Although exercising and eating healthier made me feel better, I was still having one to two seizures per month. This meant that I still couldn’t drive. Therefore, I became motivated to reduce my seizures further. I began researching and stumbled upon the Charlie Foundation and the medical ketogenic diet. I was eager to give the medical ketogenic diet a try. However, my doctors initially tried to steer me away from it, warning that it was extremely difficult and limiting. I explained to them that my seizures were very difficult and limiting too! Ultimately, we mutually agreed to try it, with the help of a medical ketogenic dietitian who I found on the Charlie Foundation’s Website. The first week or two, the diet was very difficult. I experienced flu-like symptoms and suddenly had insomnia. I would lie in bed crying because all I wanted was a box of bread rolls. I never thought I’d cry over food. 

As time went on, the ketogenic diet got a lot easier and I learned what I could and couldn’t eat, and how much. My dietitian helped me obtain a prescription for KetoCal 4:1 LQ formula to reduce the stress of meal planning since I was attending work, school, and swim team. For the first time since my seizures began, I went two months seizure free. While the diet wasn’t completely effective, it did make a big difference on the frequency of my seizures! I was on the ketogenic diet for just over a year and a half when doctors purposely had me cheat to induce seizures for testing. After that, I lost motivation to continue the diet and fell off track for a few years. However, my seizures became more frequent, so I decided it was time to return to the ketogenic diet. I have been back on the medical ketogenic diet since July of 2019 and this time, with a better mindset and motivation. 

My Life Today:

Since being on the medical ketogenic diet the second time, my new seizure-free record to beat is about three and a half months, or 110 days. I have had a few accidental carb ingestions that have lowered my ketones enough to cause breakthrough seizures. While unfortunate, these instances motivate me to continue the diet and are great examples of how powerful the diet is, at least for me! My mind feels clearer and less fuzzy on the medical ketogenic diet. I also find that it is easier to maintain a healthy weight because getting into bad eating habits is not an option when you’re on the medical ketogenic diet. 

We live in a society where we want a quick fix. However, if you’re reading this, I’m willing to bet that your quick fixes are not working. I’m learning that the medical ketogenic diet really isn’t too difficult. In a pinch, I can walk into a gas station and purchase nuts, salami and cheese. I can find an avocado just about anywhere if I need a snack. If I’m feeling lazy, I can just eat some spinach mixed with tuna and mayo. At most restaurants I can order plant protein with fats and veggies. If I’m really in a hurry, I just grab a KetoCal 4:1 LQ from the fridge. KetoCal also takes the stress off traveling. If I’m in the mood for pancakes, bagels, or sweets, I can make recipes that are ketogenic, using KetoCal 4:1 powdered formula, coconut flour, or almond flour. 

You can follow my ketogenic diet story on my Instagram page at https://www.instagram.com/keto4seizurefreedom/.

Advice for Other Adults Considering the Medical Ketogenic Diet for Epilepsy Management:

My advice to other adults who are considering the medical ketogenic diet for seizures? Don’t let anyone tell you doing the ketogenic diet for epilepsy is too hard. Also, don’t get too down on yourself if you accidentally get kicked out of ketosis. We try our best but accidental ingestions happen. 

– Hailey

I was paid by Nutricia for my time to write this blog post, however, my opinions are my own.

KetoCal is a medical food for the dietary management of intractable epilepsy and is intended for use under medical supervision.

Please note that this is one individual’s report of her experience with the medical ketogenic diet and may not be the experience of others. Individual results will vary. Please discuss with your healthcare provider if the medical ketogenic diet is right for you.

To learn more about the medical ketogenic diet for the management of intractable epilepsy, visit www.ketogenicdietforepilepsy.com.

A Dietitian’s Tips for Celebrating Easter on the Medical Ketogenic Diet

Lindsey Thompson, MS, RD, CSP, LD

We are pleased to welcome back Lindsey Thompson, MS, RD, CSP, LD.  Lindsey is a ketogenic dietitian and Keto Ambassador from Kansas City. In today’s guest blog post, Lindsey shares her tips and recipe ideas for families celebrating Easter with a child on the medical ketogenic diet for the management of intractable epilepsy. Thank you for sharing your wisdom with us, Lindsey!


One of the challenges for children on specialized diets is that food is often the focal point of holidays. Easter is a holiday that many families celebrate together and is no exception. If you have a family member on the medical ketogenic diet, including them in your family meal is imperative, but will require some extra planning.

Easter Meals

On Easter many families feature meals with ham or lamb, pies, breads and dishes with eggs. Consider incorporating the type of meat you are serving into a ketogenic meal for your child, such as ham or lamb with a side of fruit or vegetable and cream or other fats. In addition, here are a few keto-friendly dishes you might consider for your family member’s Easter meal:

KetoCal® Cheese and Ham Tart

KetoCal Cheesy Tomato Tart

KetoCal Apple Crumble with Whipped Cream

Ketogenic Deviled Eggs:

Use the following ingredients to put into your child’s ketogenic diet planner to calculate his or her recipe:

  • Egg white- cooked
  • Egg yolk- cooked
  • Mayonnaise
  • Butter- softened
  • Small amounts of mustard, Worcestershire sauce and hot sauce (if your family member likes spicy foods)
  • Salt, pepper and paprika to taste

Instructions:

  1. Hard boil eggs. Slice eggs in half and remove yolks.
  2. Place the cooked egg yolks in a bowl & mash them up with a fork.
  3. Stir in the mayonnaise, mustard, Worcestershire sauce, hot sauce. Sprinkle with salt and pepper.
  4. Scoop the filling into the halved egg whites.
  5. Sprinkle with paprika.

Easter Egg Hunts

In addition to family feasts, many families will attend or have Easter egg hunts for the Easter holiday. Children often hunt and discover plastic eggs filled with treats or goodies. Here are some tips to help include your family member on the ketogenic diet in your family’s Easter Egg Hunt:

Easter is a special holiday for families. With a little extra planning, your child on the medical ketogenic diet can enjoy and feel included as a part of your family’s Easter traditions.

Happy Easter to all!

-Lindsey

 I have been paid by Nutricia for my time to write this blog post but my opinions are my own. 

KetoCal is a medical food for the management of intractable epilepsy and is intended for use under medical supervision. Talk with your healthcare provider about whether KetoCal is right for you.

The medical ketogenic diet for epilepsy should be used under medical supervision.