Hi everybody, I'm Zahaba Turner, I am a pediatric dietitian and I work at the Johns Hopkins Children's Center. I'm going to be talking today about the Ketogenic Diet Therapy for Epilepsy. I have been working at Johns Hopkins Children's Center for 18 years, putting, placing children on the ketogenic diet. In full disclosures, I am a consultant for Nutritia. And what we're going to talk about today is the history of the ketogenic diet for epilepsy. We're going to go through the basics of how to actually implement the diet, and then talk about alternative diets that are available to the classical ketogenic diet. And here we have a timeline of the ketogenic diet, just, you know, talking about the history through the years, and in 400 BC you have Hippocrates, who talks about a man with body burns and seizures, and he mentions complete abstinence from food and water. And then, even in the New Testament, it's mentioned in the King James Version, it says, and he said unto them, this kind can come forth by nothing but by prayer and fasting. And prayer and fasting is mentioned many times in the Bible, but this was mentioned in reference to somebody with epilepsy. And then all the way up to the 1800s, where water diets, prolonged periods of fasting, was the means of treatment, all the way up to Galen in 1910, 1920s, with one of the first history reports of the diet, recorded and written in 1920. And the fasting diets were basically until the child can stand it, and we know that children can't fast for a very long period of time, but still, it was a means of treatment for epilepsy. And then, up until about 1938, where, that's a picture of dialysis, but really where medications became very popular, and each medication that came out was thought to be the cure for epilepsy. Now, in the 1920s, the ketogenic diet was a very popular treatment for both adults and pediatrics, and you see that in the papers that were written. But really, with the introduction of medications, that's when the diet started to become not so popular. And it wasn't really up until 1994, when the Charley Foundation was started, that the diet started to become popular again. And it was thanks to the Charley Foundation for really promoting the diet and research around the diet. And as you can see in that map, and that's from 2014, and since then it has evolved, the gray area is where the ketogenic diet is being done all over the world. And then, leading up to present day, so 2021 is the history, is the 100-year anniversary of the diet. And there are a lot of research papers and a lot of centers all over the United States and the entire world that are following and placing kids on the ketogenic diet. In 2009, there was an international consensus statement that was written, and it's been updated again in 2018. And it's a really helpful article. It talks about, the title is Optimal Clinical Management of Children Receiving Dietary Therapies for Epilepsy, and really updated recommendations from the international group of different ways, which patients who should go on the diet, who should not go on the diet, and a very helpful research paper that are available. All right, so now let's talk about an overview. Now, for many years, and even the way that I was taught, is of this youth fear of fat. If somebody put butter next to your bread, you would never put butter on your diet. And if you look at the Time, this is a famous thing, the Time article in 1984, you know how bad fat is for you. Avoid eggs, avoid bacon, and that's how a lot of us grew up. And then in 2014, outcomes, butter is good for you, and fat is good for you, and just a really confusing confusion for what is healthy and what people should eat. So when you have this fear of fat, leading up to maybe 10 years ago, I would say even less, people were scared to put their child or anybody on the ketogenic diet, which is very high in fat. And in comparison, a typical American diet, on the left is the carbohydrate, and what's recommended by the American Dietary Association is carbohydrate should be about 60% of your daily intake. Protein is in purple, and fat is about like 30% of your diet, 25 to 30% of your diet. And if you compare that to a classical ketogenic diet at a 4 to 1 ratio, which we'll talk about, that then becomes 90% of your diet. Protein is about 8%, and carbohydrates are 2% of the total diet. And that's a huge, huge adjustment for the types of foods that you're eating and how your body metabolizes what they're eating. Because instead of metabolizing carbohydrates and turning that into glucose, now the body is going to eat, now the patient is going to eat fat, and the body is going to metabolize it into ketone bodies. So who gets placed on the ketogenic diet? And it's typically patients who fail the traditional anti-convulsant therapy or are poor candidates for epilepsy surgery. So if a medication can help and cure and stop the seizures, then that's usually the first line of therapy. But patients who fail their medications, the traditional anti-convulsant therapy, should be placed on the diet. And it should be thought of sooner than later, not after the fifth medication is prescribed, but probably closer to the second, and then maybe, you know, definitely by the third. The diet can be used by all ages, from infants all the way through adulthood. And you're seeing that, we are seeing that, you know, more often, and a lot of research being done in both infancy all the way through adulthood. The average time on the diet, what's recommended, is one to two years, typically two years seizure-free, but there really is no maximum time on the diet. There are some diseases or diagnoses where people stay on the diet for life. So there really is no maximum time on the diet. In the consensus statement that I mentioned earlier, I placed there two tables that the international consensus recommends placing somebody on the ketogenic diet. So table one is patients that have a greater than 70% response rate. So that could be somebody with these diagnoses. So Angelman syndrome, complex one mitochondrial disorders, DOSA syndrome, epilepsy with myoclonic or tonic seizures, group one deficiency. Think about it with patients that are formula-fed, either through a G-tube or infants. Otahara syndrome, and you could see the rest of the list. FIRE syndrome. And then there's a table two of the list of that there's been one or two reports of a moderate benefit to going on the diet as well. There are confidence in indications the diet is not used for everybody, anybody with any kind of fat malabsorption or genetic chain like that. Acyl dehydrogenase deficiency or any kind of fat absorption deficiency should not be on the diet. And the main thing for me would be anybody who can't maintain adequate nutrition. So if it's a real struggle to get a patient to eat and they would not be somebody who could go on a diet unless they support therapy or have a long-term plan, like a G-tube place. Patient or parent or caregiver non-compliance is another reason. So what is the diet? Classical ketogenic diet consists of about 80 to 90% of the diet comes from fat. Adequate protein, meaning we meet the patient's needs for the protein. So in America, we eat a lot of protein, way more than what we technically need. So adequate means we're meeting those needs, but it seems a lot less than what a typical child would eat and very low in carbohydrates. All the foods on classical ketogenic diet are weighed on a grand scale and every meal and every formula and everything that is eaten is calculated into a ratio. So when we talk about calories, we start there and as a dietitian, and there is no calorie restriction. What that means is we use our clinical judgment when we estimate their nutrition needs and we use many different calculations that are appropriate for that child. But the main goal in children is to be adequate calories for them. Protein is the DRI for protein and we meet those numbers as well. And for fluid, it's 100% maintenance fluid is our goal. So a ratio is the grams of fat to the grams of protein and carbohydrate combined. So for example, a four to one ratio is four grams of fat to one gram of protein and carbohydrate combined. So the left side of the ratio is the fat. The higher the number, the higher the amount of fat and the right side of the ratio is the grams and protein. We typically do a three to one ratio for infants, teenagers and compromised patients and four to one is for everybody two years old and older. The higher the ratio, the lower the amount of protein and carbohydrate. This is a great picture from the Charley Foundation showing what typical meals look like with four to one versus three to one versus two to one versus one to one and four to one being the highest in fat. You could see the fat in the medicine shooter, medicine syringe or and in the with mayonnaise, a small amount of protein, the chicken and a small amount of broccoli. But once you get to the three to one, you see a little less fat, a drop more protein and a drop more of the vegetable. When you go down to a one to one, to me, that looks like what an Atkins diet look like, you know, when people go on the Atkins diet. So it's protein and vegetable with a little bit of fat. And this is a nice picture to actually see what the diet looks like. A typical diet starts with heavy whipping cream. Heavy whipping cream was originally created to take the place of milk. American kids are very big milk drinkers. So 50% of the prescribed fat usually comes from heavy whipping cream. And then the other 50% comes from the butter, mayo and oil, all of the above. There's a protein and then a fruit or vegetable. So a typical menu will look like this. It'll say 40 grams of 36% heavy whipping cream, 24 grams of chicken breast, 11 grams of broccoli, 21 grams of fat and 12 grams of nuts. And as the dietitian, we instruct and we teach and we educate the families how to prepare these recipes. And here's some pictures that families have sent me over the years of foods, just to show that the diet can be prepared and look normal, like the heart pancakes up on the top. It's just, you know, using cookie cutters with a little bit of whipped cream in the middle. There's plenty of like fat bomb, like chocolate with coconut oil recipes. But many recipes can be done creatively. I show this to parents all the time to show that this is regular foods, just rearranged. Like in the middle, I have a pepperoni with cheese on it. You know, the pancake recipe could then be put into the waffle maker to make it look like a waffle with a little bit of creativity. You know, the diet is deficient in all vitamins. Now, a lot of it has to do with the fact that there's very little fruits and vegetables, but also having to do with all of our grains. Well, our wheat products in the United States are enriched with B vitamins. So if you take away pastas and breads and all these enriched products and cereals, that's where a lot of kids get their vitamins from and they're no longer getting it from them. So the diet is deficient in B vitamin, vitamin C, calcium, zinc, phosphorus. It has a fiber, very little fiber, carnitine, selenium. So we supplement all patients with a carbohydrate free or a low carb vitamin that can be found and sometimes minerals. The universal recommendations from the consensus statement is a multivitamin with minerals and a calcium and vitamin D. And then there are some optional extra supplementations and that is per patient basis. If they use vitamin D or maybe a citra K or poly citra K to help prevent kidney stones, constipation is an issue on the diet. So they might need some kind of laxative and other additional minerals based on individual patients. All right, so there's how do you initiate the classical ketogenic diet? Is it done inpatient versus outpatient? There are pros and cons to both ways. Classic ketogenic diet is usually within admissions in the hospital. 80% of the consensus statement admits at Johns Hopkins Hospital, we admit patients for a three day admission. What we typically do is we admit multiple families at one time. It's a three day. They come in Monday and they leave on Wednesday. We do many daily classes. We check blood glucose and urine ketones checked as well. We initiate it slowly. It's typically an 18 to 24 hour fast, but more typically closer to an 18 hour and the only fast patients who are greater than two years old. Goal ratio after the fast, we start at half strength calories for 24 hours. That's either provided in an eggnog, which is really just liquid eggs and heavy whipping cream or a keto formula and it's at half calories. So if their goal calorie is 1000 calories for 24 hours, we only give them 500 calories. And then after that, we advance to full strength. So if it's 1000 calories, 1000 calories, let's say a four to one ratio, and it depends if they eat, then they'll get 1000 calories with food or divided into meals or they'll get 1000 calories with their formula. Many centers, and this is like a hot topic, discussing to fast a patient versus not fasting, the consensus statement, 68 centers still fast, but it is optional. No center felt that fasting was mandatory. At Hopkins, we typically discuss it for every single patient before they are admitted if we should fast them or not. And all centers agree that not to fast children under the age of two. Follow up generally every three months for the first year and in the but with one month first visit after starting the ketogenic diet. So once they start on the diet, we see them a month later and then three months after that. We do typically do labs at each visit unless the patient is very hard for them, then we might spread it out to every six months, but typically every three months. Typically, we don't change the medication during that first month of the diet. That first month of follow up. It's really just a check in and to see how are they doing. You know, are they tolerating the diet? Are they getting the formula? Any issues, you know, can come up in that first month and you could catch things to make the diet easier for them. We do a lot of frequent phone calls and email contacts in between each clinic visit. And we manage everything mostly with their local through our keto team. Now, the diet does have side effects. It's not a treatment with outside effects. The most common are usually GI related constipation, reflux, abdominal pain. It is high in fat. It can cause more reflux, abdominal pain a lot in the first couple of days, maybe week of starting the diet. And constipation is something that we definitely see. And, you know, we talk about ways to alleviate. Less common, we do see hyperlipidemia. Typically in the beginning of the diet and then cholesterol levels and definitely do come down. Kidney stones is rare, but at Hopkins, we put every patient on a citrate to help avoid kidney stones. Poor growth is something we see. And that is why we see patients often, like every three months, to make sure that their kidneys are following in their growth. And vitamin deficiencies, unfortunately, if they're not taking their vitamin, they could have a vitamin deficiency. All right. So alternative diets to the classic keto diet. So there are alternative diets that have been created mostly to try to be a little bit more flexible for some parents since the classical ketogenic diet can be hard on families. The modified Atkins diet was created when describing the ketogenic diet to somebody. They're like, oh, it's like Atkins. And they were like, oh. And then a patient was told to stop eating their pastas and their cereals and a lot of their high carb foods leading up to their admission for the ketogenic diet. And they started to see a decrease in seizures. And they're like, wait, maybe there's something here. So there is no calorie or protein restriction. We do not weigh any of the foods on a gram scale. We stress the importance of fat. So it is still a very high fat diet. So at every single meal, there has to be fat. And then we educate how to count carbohydrates. How do you figure out how many carbohydrates are in each meal? We show them apps and websites and everything on how to count the carbohydrate. We provide patients with the grams of carbohydrates per day. And the family then calculates it. So if it's 20 grams of carbohydrates per day, they typically figure about 4 grams of carbohydrates per meal, maybe 5 grams of carbohydrates per meal. And then the rest in snacks. Another diet with the low glycemic index diet, which was modeled after the South Beach diet and focusing on low glycemic index carbohydrates with a number less than 50. And the low glycemic index foods can be found online. Which foods are low versus which foods are high. It's basically your whole grain foods. Think of that like a white potato versus a sweet potato. But still, it is lower in carbs. And there should be a goal given to each patient. But they are allowed more carbohydrates than the Modified Atkins and definitely than the classical ketogenic diet. And still instructed to push fat and how important fat is at every single meal. And there's an MCT oil diet, which uses medium chain triglyceride oil, which is MCT oil, in place of the cream and butter. And it does allow for more carbohydrates and protein. And the way that the diet is figured out, it's based on a percentage of calories. So there's no ratio. So you would do about 30 to 60% of your calories from MCT oil, 10% from carbohydrates, 12% from protein, and then the rest from LCT, low chain triglyceride fat, and 15 calories are not restricted. So in practical applications, the ketogenic diet therapies for epilepsy, it's a well-established treatment for medication refractory epilepsy for both children and adults. Initiation of the ketogenic diet really does depend on the patient and the hospital or the center. So many centers can do it outpatient, they can do it inpatient, they can admit one patient, they can admit four patients at one time. It really depends on how the center has been established. And there's no wrong way to initiate within the means of what the consensus statement says. There's so many new foods available as well as recipe programs that have made the diet so much easier and much more palatable. The Keto Diet Calculator Program, the dietician and the parents can do it. And create recipes based on the child's preference. And I'd like to thank my Keto team at Hopkins, Dr. Kossoff, Dr. Hernandez, Megan who's a nurse, and Sophie who is a dietician with me. Thank you.

ketogenic diet

epilepsy treatment

pediatric dietitian

dietary supervision

Charley Foundation

Johns Hopkins Children's Center