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.

Ask the Keto Dietitian Series – the Modified Atkins® Diet (MAD)

Staey Bessone

A Q&A with Ketogenic Dietitian Stacey Bessone, RDN, LDN. For more posts in our “Ask the Keto Dietitian” series, click here.

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

I am originally from Tampa, Florida and came back after finishing my education in Miami at Florida International University. I have always worked in pediatrics for my almost 24 years as a registered dietitian.  I worked in the same children’s hospital I work in now right out of school but left and came back in 2011 when I took a position to exclusively practice with the medical ketogenic diet. We developed our clinic and see patients from all over the state. We also do clinical research so we can study different aspects of the diet. About half of my patients are on the classical medical ketogenic diet and the other half are on the modified Atkins® diet (MAD). I enjoy teaching the modified Atkins® diet as it is outpatient and usually with adolescents.  I enjoy my job immensely and would never choose to do anything other than the medical ketogenic diet.  It is so rewarding and I have the best patients and families.

How long have you been a ketogenic dietitian?

I have practiced the medical ketogenic diet exclusively for the last nine years.

How many patients do you have on the medical ketogenic diet?

We usually have between 60 and 80 patients on one of the medical ketogenic diets at a time.  Often we are teaching new patients while other patients may be weaning the diet.

What is the modified Atkins® diet (MAD) for intractable epilepsy?

The modified Atkins® diet for epilepsy is a carbohydrate-controlled diet in which a patient counts carbs (minus fiber) and is encouraged to eat more fat to promote ketosis.  It is started as an outpatient (meaning the patient doesn’t need to stay in the hospital) and is simpler to begin than the classic medical ketogenic diet.

How is the MAD different from the classical medical ketogenic diet for epilepsy?

The MAD diet is different in that food is measured in household measurements like tablespoons and cups or by servings (versus using a gram scale for the classical medical ketogenic diet). Instead of focusing on the ketogenic ratio (as with the classical medical ketogenic diet), the focus is on counting carbohydrates throughout the day. Fat is encouraged to be added at every meal and snack.  There is no restriction on protein intake or foods with no carbohydrate. Patients learn to read food labels and can eat out at restaurants. It is a great option for adolescents and families that are on-the-go.

What types of patients are good candidates for the MAD?

Your healthcare professional will work with you to select the most appropriate diet option for your needs. In general, the modified Atkins® diet can be a suitable option for older children, teens, and adults who may find a restrictive diet more challenging.  Also, MAD is a good option for patients that need diet intervention immediately and may not be able to wait for a hospital admission to do the classical medical ketogenic diet. 

While not appropriate for all situations, the MAD can have a similar effect on seizure control as the classical ketogenic diet for some individuals. Most of the research shows that nearly 50% of patients on MAD have about a 50% or more reduction in seizures.

About what percentage of your keto patients are on the MAD?

About half of my patients are on the MAD.

How did the MAD come about? When was it started? Where did the idea come from?

In 2003, Dr. Eric Kossoff, a neurologist at Johns Hopkins Medicine in Baltimore, developed the novel form of the medical ketogenic diet after a patient on a classical medical ketogenic diet came for a follow up visit and had not been weighing his food and had been following the diet “loosely”.  Surprisingly, the child was still in ketosis and doing very well.  Following that patient, there was a young girl who was started on the Atkins® diet in advance of starting the classical diet and achieved ketosis with seizures stopping after three days. These patients had good seizure control so Dr. Kossoff investigated the less strict “modified Atkins® diet” with a formal study. He found that it was a viable option to manage epilepsy with similar results to the classical medical ketogenic diet.  I am happy to be part of the research team at Johns Hopkins with Dr. Kossoff (and dietitian Zahava Turner, RD, CSP, LD) for the past several years through my hospital’s partnership with Johns Hopkins Baltimore.

What are the biggest challenges for your patients following the MAD?

The biggest challenge for patients on MAD (or any other medical ketogenic diet) is sticking to the carb count and not eating any foods that are not allowed.  It can also be challenging for some patients to get enough fat. For some patients, a slip-up can lead to breakthrough seizures since the diet is helping to manage the seizures. 

Is there any research on using KetoCal® with the MAD?

Yes. There was a study in 2011 in children that found using KetoCal with the MAD increased both fat intake and the ketogenic ratio (1.8:1) in comparison with MAD alone (1.01:1). A follow-up study in adults in 2018 showed that starting the MAD diet using KetoCal during the first month in a randomized protocol had better long term compliance and higher fat intake compared to those who did not use the formula.

– Stacey

Thank you, Stacey, for your insights about the modified Atkins® diet for epilepsy!

Hear from patients who have tried the modified Atkins® diet:

Looking for a hospital or program that offers the medical ketogenic diet or modified Atkins® diet? Click here to find one near you.

Learn more about the medical ketogenic diet or modified Atkins® diet for epilepsy here.

Atkins® is a trademark of Atkins® Nutritionals, Inc and not affiliated or endorsed by Nutricia North America.

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. Talk with your healthcare provider to determine if KetoCal is right for you.