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.

How is the Medical Ketogenic Diet for Epilepsy Different from the Keto Diet for Weight Loss?

The classical medical ketogenic diet for the management of intractable epilepsy was created in the 1920s at the Mayo Clinic. It became popularized in the 1990s thanks to the launch of the Charlie Foundation and the release of the movie First Do No Harm. With the recent popularity of the keto diet for weight loss purposes, many people may wonder how it is different from the medical ketogenic diet that is used to manage intractable epilepsy. While both have the same basic premise: low carbohydrates and high fat, there are many distinctions. In today’s post, we’ll describe the key ways that the medical ketogenic diet for the management of epilepsy is different from the mainstream keto diet that is used for weight loss.  

Medical Supervision

One major difference between the medical ketogenic diet and the keto diet for weight loss is that the medical ketogenic diet must be designed and monitored by a medical team, usually a neurologist and a ketogenic dietitian (a registered dietitian who is trained to manage medical ketogenic diets for epilepsy). Not only is the medical ketogenic diet typically initiated in the hospital, it is also monitored by the medical team on a regular basis to look out for side effects, to ensure nutritional needs are being met, and to assess the diet’s effect on seizure control. 

Diet Design

One of the key differences between the medical ketogenic diet for epilepsy and the keto diet for weight loss is the way the diets are designed. The classical medical ketogenic diet is designed according to the ketogenic ratio, that is, the ratio of the amount of fat (measured in grams) to the amount of combined carbohydrates and protein (also measured in grams). The exact amount of carbs, fat, and protein allowed on a classical medical ketogenic diet depends on:

  • The specific ketogenic ratio selected by the medical team
  • The patient’s specific daily calorie requirement
  • The amount of protein required to sustain the patient’s specific goal body mass

Therefore, no two patients on a medical ketogenic diet will have the exact same daily carb, protein, and fat requirements. In comparison, the keto diet for weight loss typically limits carbohydrates to a given amount or aims for a certain percentage of calories coming from carbs, protein, and fats. If you were to compare the two diets according to the number of daily carbs and protein allowed, the medical ketogenic diet will typically be much lower in carbs and protein, and much higher in fat. Note that there are less strict variations of the classical medical ketogenic diet, such as the modified Atkins diet, but even these versions of medical ketogenic diets will be much lower in carbs and higher in fat compared to a keto diet for weight loss.

Protein Restriction

Another difference between the two diets is that the classical medical ketogenic diet restricts protein in addition to carbohydrates. As described above, the amount of protein allowed in the medical ketogenic diet depends on the patient’s goal body mass. In other words, they are allowed just enough protein to sustain their body’s muscle mass but no extra. A keto diet for weight loss typically focuses more on limiting carbohydrates, with less restriction to protein intake.

Diet Initiation

Unlike the keto diet for weight loss, the medical ketogenic diet for epilepsy is generally initiated in the hospital under close medical supervision. Because of the very strict limitation of carbohydrates and protein, the level of ketosis reached on the medical ketogenic diet is much higher compared to a keto diet for weight loss. Therefore, it is essential that a medical ketogenic diet is initiated under medical supervision.

  • To ensure that the patient tolerates the selected ketogenic ratio
  • To monitor for and treat potential side effects like low blood glucose or vomiting
  • To allow the medical team to make adjustments to the diet as needed

This hospital time is also used to train the patient or their caregiver how to manage the diet once they go home.

Precision of Food Measurement

Another difference between the two diets is the precision with how foods must be measured. On the classical medical ketogenic diet, all foods and drinks must be measured to the tenth of a gram using a gram scale. For example, a meal may call for 29.2 grams of chicken breast, 50.5 grams of mayonnaise, etc. Every single bite of the meal must be consumed to ensure that the ketogenic ratio of the meal is accurate. With a keto diet for weight loss, foods can typically be measured with household measurements like cups, ounces, and tablespoons, and there is less concern about finishing the entire meal.

Sticking to the Diet

For patients following a medical ketogenic diet for epilepsy management, breaking the diet, even just a bite, can result in breakthrough seizures. Therefore, having a “cheat meal” or taking a week off for the holidays is simply not an option. Individuals following a keto diet for weight loss may lose weight-loss progress if they fall off the diet for a while, but the consequences are not so serious.

Accounting for Medications & Non-Food Products

With the medical ketogenic diet, the carbohydrate allowance is so specific that trace amounts of carbohydrates from medications need to be accounted for. Typically, when a patient starts the medical ketogenic diet, they are switched to variations of their anti-seizure medications with lower carb amounts (for example, switching from liquid form to tablet form). If there are still carbohydrates coming from medications, the carbohydrate allowance from foods will have to be adjusted to account for them. Oftentimes, the medical ketogenic diet team will recommend specific brands of self-care products that are lower or without carbohydrates (yes, the body can absorb trace amounts of carbs through the skin!). This may be especially important for items that go in the mouth (like toothpaste) or that are applied to large areas of the skin (like moisturizer or sunscreen). Individuals following a keto diet for weight loss generally don’t have to be this precise or careful about trace carbohydrates.

Length of Time

People who follow a keto diet for weight loss may follow the diet for a certain amount of time to lose weight or they may follow a keto lifestyle permanently as a method to maintain weight. On the other hand, patients on a medical ketogenic diet for epilepsy management will usually follow the diet for a specific period of time. In most cases, if a patient’s seizures are controlled on the medical ketogenic diet for 2 years, the medical team will recommend weaning off of the diet with the hope that they can come off the diet and maintain seizure control. However, with certain medical conditions, like Glut-1 Deficiency Syndrome, the medical ketogenic diet must be maintained for longer periods of time or even for life.

Hopefully this post gives you a better idea of what makes the medical ketogenic diet for epilepsy different from the more widespread keto diet used for weight loss purposes. For more information on the medical ketogenic diet for epilepsy, visit www.ketogenicdietforepilepsy.com.

– Mallory

This blog post is brought to you by Nutricia North America.

The medical ketogenic diet should be used under medical supervision.

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

Reagan’s Story: Epilepsy & the Medical Ketogenic Diet

Reagan eating her medical ketogenic meal at a restaurant

First Seizure

It is a day I won’t soon forget.  March 2013, Reagan and I took a girls’ road trip to Connecticut for my cousin’s baby shower.  Reagan seemed off, and I attributed it to traveling and not being in her own space.  She was restless and found it hard to sleep in a different crib.  I scooped her up and had her lay with me. I awoke to her making odd sounds, drooling and grey, her body seemed limp, and she was unresponsive.  An ambulance arrived soon after, and we were taken to a local hospital to hear the words no parent wants to hear: “Your child had a seizure”.  Here I was in a city several hours from home, with my 16-month-old child, without my husband, terrified of what had just happened.  Thankfully all of my family was in town for the baby shower, and my mom, aunt, and sisters sat with me while I ran through Reagan’s already extensive medical history with the pediatric neurologist. Was she born full term? No, she was considered a late preterm baby.  Was she meeting her milestones? No, she had fine and gross motor delays, as well as speech delays.  Is she followed by a neurologist? Yes, and recently had an MRI, which didn’t show anything abnormal.  

Epilepsy Diagnosis

Yet, here we were. Reagan had experienced her first of many seizures.  Reagan was transported by ambulance from the hospital in Connecticut to our hometown children’s hospital.   We were later discharged with emergency medication and instructions for what to do if another seizure were to happen.  The nights and weeks following I slept on Reagan’s floor next to her crib, terrified of another seizure. Four months later, another seizure followed, as well as another ambulance ride and hospital stay. Two months later, after a move to Pennsylvania, yet another. Then came the diagnoses: epilepsy, as well as cerebral palsy.  It was a shock to the system. What did this mean for my nearly two-year-old? How would we manage this? Would she have a full life?

Antiepileptic Medications

Reagan started a medication, and any parent knows giving a medication to your young child can be like pulling teeth.  But we soon learned the tricks of the trade, making sure she was getting the doses when needed.  The first few months came with sleepless nights, feeling on edge and always wondering when the next seizure would come.  And they came, almost like clockwork every four months.  That “honeymoon period” of a seizure every four months lasted about a year and a half.  Then the seizures started to pop up more frequently, when Reagan was sick, when she was tired, and when she was overheated.  A second medication was added onto the first, and we achieved eight months of seizure freedom.  With our neurologist, we started to wean Reagan from the initial medication.  Unfortunately, that put us into a downward spiral of seizures and medication side effects. A new medication was introduced, and seizures came every two weeks.  A new medication came shortly after, and it slowly helped to space the seizures out. After months of the medication being just okay, the neurologist suggested adding in another medication.  With that came side effects that included extreme fatigue and loss of cognition.  My little girl was hiding in plain sight, in a heavily medicated fog.  For several months we worked through finding the right dose and finally it came.  The combination of those two medications has bought us three solid months of seizure freedom, but the side effects were still there.  And while some would consider that success, we didn’t.  For us, success was being both seizure-free AND side effect free. 

Enter the Medical Ketogenic diet

 At the end of 2018, at a routine neurologist appointment, my husband and I expressed our concerns about the side effects these medications were having on Reagan.  She gave us three options: the medical ketogenic diet, VNS, or brain surgery.  We weren’t ready for surgery just yet and given that I had some knowledge of the medical ketogenic diet and its success rate with children living with epilepsy, we knew that would be our next step. The months leading up to Reagan’s inpatient stay to begin the medical ketogenic diet, we prepared Reagan for her new lifestyle.  We explained to her this special diet could help make her feel better. We also went over the list of her approved foods and involved her in the appointments with the dietitian.  By the time we entered the hospital, she was ready, even though both my husband and I were anxious. During the five-day-stay, Reagan acclimated well to her new diet and enjoyed visiting the hospital’s Kids’ Club House, watching movies and making friends with the nurses.  She even got to visit the kitchen with the dietitian to make KetoCal® pancakes and a smoothie.

Reagan preparing a KetoCal pancake recipe

Reagan Today

Today, Reagan has been on the medical ketogenic diet for almost five months.  She loves the food, knows her schedule and knows that she must complete each meal. She’s always been a little foodie and often asks me to contact the dietitian for new and exciting snacks and meals.  The first month was hard on our family. The constant weighing and measuring were time-consuming and a little emotional.  But now we are in a groove, even eating out at restaurants and managing holidays. Reagan maintains good seizure control, and we’ve seen so many additional positive changes.  A little bit of that medicated fog has lifted already, she has increased attention to tasks, and those skills she seemed to have lost are coming back. The best part? We just received the go-ahead from Reagan’s neurologist to wean her from one of the medications! Since this was our main motivation for trying the medical ketogenic diet, we are thrilled!

Thinking back to that Mom who was scared and wondered what this would mean for her then two-year-old, I would tell her that epilepsy is a part of Reagan, but it does not define her. I would tell her that you will manage this head-on and become a better, stronger person and an advocate not only for Reagan but for all kids living with epilepsy.  Does Reagan have a full life? Absolutely!  Does her life look different than the neurotypical child? Yes, but Reagan has faced each and every one of these challenges from MRIs, to frequent doctor and therapist appointments, to medication changes to the medical ketogenic diet, head-on with a smile on her face. And when asked about her life, her response is “it’s fun!”

Erin Monast lives in the Philadelphia suburbs with her husband Travis, their daughter Reagan and dog Tag.  She writes the blog, This Is Epilepsy and is inspired every day by the strength and perseverance of Reagan.

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.

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