The Ketogenic Diet for PDCD

What is the Ketogenic Diet?

First, it’s important to note the difference between the keto diet as a fad diet vs. the keto diet as a medical diet. Most people probably think of the keto diet as a fad diet that relies heavily on red meat and other fatty, processed, and salty foods that are notoriously unhealthy. But a true ketogenic diet has actually been around for close to 100 years to treat drug resistant epilepsy in children. It switches the body’s energy source to fats rather than using glucose.

In addition to epilepsy, the keto diet is especially likely to be effective to treat GLUT-1 deficiency, Pyruvate Dehydrogenase Complex Deficiency (that’s us!), infantile spasms, and Doose syndrome. Studies have also shown that it benefits patients with some types of cancer, dementia and migraine

The ketogenic diet was designed to simulate the metabolism of fasting. When a fasting person has burned up all glucose stores for energy, they then begin to burn fat. Unlike fasting, by providing the body with exogenous fat, the ketogenic diet allows a person to maintain a fat burning metabolism for an extended period of time. In the absence of glucose, the fat is not burned completely but leaves a residue in the form of ketone bodies, which build up in the bloodstream and can cross the blood brain barrier. This provides patients with PDCD an alternative source of energy that their brain can use and bypasses the breakdown in their metabolism. 

References: Kossoff, Eric, et al. Ketogenic Diet Therapies for Epilepsy and Other Conditions. 2020.

By: Sarianne Madsen, RD CSP. Ketogenic Dietitian at UCSF San Francisco

A Breakdown of Ketogenic Formulas

As the popularity and usage of the ketogenic diet for refractory epilepsy and other medically indicated conditions has grown, so have the list of available commercially prepared formula suitable for those on a medical ketogenic diet. With so many options it can be confusing to choose which one may be the best fit. Your keto team (RD, MD, NP) can help find and develop the right product and plan for each individual in order to achieve the best success on the ketogenic diet. For educational purposes here is a list of the most commonly known keto formula products and their main attributes.

Ketocal

Made by Nutricia

Ketocal has now been around for over 20 years! It was the first commercially prepared product made specifically for those on a medical ketogenic diet. It comes in both powder and liquid and can be helpful for:

  1. Sole source nutrition with either oral formula feeding or tube feeding

  2. Addition to a mostly food based keto diet plan to help boost fat intake

  3. Ingredients for specially formulated Ketocal based recipes such as pancakes, muffins, smoothies, soups, or pizza. Check out their website for all of the recipes they have developed to be used with Ketocal. Unlike other recipes found online for keto, these are designed for high ratios and can help lower carbohydrate and increase fat on both classic and modified keto diets

Products available:

  •  Ketocal 4:1 liquid in both unflavored or in 237ml shelf stable cartons

  •  Ketocal 2.5:1 ratio vanilla liquid 237ml in shelf stable cartons (with MCT oil)

  •  Ketocal 3:1 ratio powder unflavored

  •  Ketocal 4:1 powder vanilla

Ketovie

Made by Cambrooke and Ajinomoto

Ketovie started with only a couple products and has now expanded to 6 different liquid keto formulas. Their standard formulas are whey based protein and they offer a peptide-based formula for people with food allergies and sensitivities to intact proteins. Their latest product is a plant based one for people that are dairy free. All of their products also contain MCT oil (which is helpful for boosting ketosis), Carnitine, and Selenium to help ensure adequacy of these nutrients while on this diet. They also have a line of ready to eat keto food products, called Ketovie Café that can be a handy and easy addition to an oral keto diet plan. Similar to the Ketocal brand their formula products can be useful for:

  1. Sole source nutrition with either oral formula feeding or tube feeding

  2. Addition to a mostly food based keto diet plan to help boost fat intake, especially the chocolate and vanilla standard options

  3. Components for specially formulated KetoVie based recipes such as mousse and smoothies

Products available:

  • KetoVie 4:1 liquid in Vanilla, Chocolate, and Unflavored with MCT oil in 250ml shelf stable cartons

  • KetoVie 3:1 liquid Unflavored in 250ml cartons

  • KetoVie 4:1 Plant Based liquid in 250ml cartons

  • KetoVie 4:1 Peptide Unflavored liquid in 250ml cartons

  • KetoVie Café--Food

Keto Peptide

Made by Functional Formularies

The keto formula made by functional formularies is the first organic food based keto formula to come onto the market. It’s gluten, soy, dairy, and corn free and with a ratio of 2.4:1 and can be mixed with additional fat sources to achieve a higher ratio. It also utilizes peptide proteins and overall may be helpful for improved digestion, constipation, and reflux. It is not intended to be used for sole source nutrition, though an expert keto team can devise a diet plan with it that ensures all macro and micro nutrient goals are being met. Functional formularies, working with Dawn Martenz, have also developed many food-based recipes using this formula product which can be found on their website. They have also made an oral pouch called Keto Sustain that can be helpful for snacking on the go, or in school lunches for oral keto diet plans. Their formula product is most useful for:

  1. Adding food based, blended feeds to a tube feeding regimen

  2. Utilizing for recipes designed for an oral ketogenic food plan

Products available:

  • Keto Peptide, Organic Food based formula, 2.4:1 ratio, 227gm (8oz) pouch

  • Keto Sustain, 4oz Food based oral pouch at a 2.7:1 ratio

Keto Diet Q and A

By: Sarianne Madsen, RD CSP. Ketogenic Dietitian at UCSF San Francisco

  • Ketosis is what occurs when carbohydrates are limited and the body’s metabolism shifts into burning fat as a fuel source. It is a fed, mimicked, starvation state that is a delicate balance. Ketosis provides energy for the brain, but in excess it can contribute to side effects including too much acid in the body. Typically, therapeutic ketosis starts at 3mmol/L and can go up to 5 or 6mmol/L without seeing side effects. There is some data to support that levels around the 3-3.5mmol/L provides benefit to patients with PDCD, though it is important to note that ketones naturally fluctuate from morning to evening so a range of 3-4.5mmol/L would be ideal. Working with your keto diet team is important to consistently hit these target ranges—by either strengthening the diet to promote more ketosis or lightening the diet to reduce levels. There are also quick fixes to help treat acute over-ketosis that can happen while ill or if missing meals. Your keto team can provide you with some ideas.

  • Theoretically once on a stable, planned, and controlled medical keto diet there should not be wide fluctuations on blood ketones outside of the typical daily variance. The diet provides a steady amount of fat, carbohydrates, and protein to result in consistent ketosis levels. However, if more carbohydrates (and to a lesser extent protein) are introduced than what is prescribed, the blood sugar levels will rise shortly after the meal, and then the ketone levels will fall in the next few hours after that.

  • Moving from a keto formula to a blenderized diet can be beneficial for many reasons. It can result in improved gastrointestinal symptoms and overall better tolerance, while providing a way to still cook for your child and have them enjoy different foods even if tube fed. There are some challenges to work through in order to be successful such as, tube size, thickness of blend, amount of water, bolus versus pump and rate, food variety (vegetables/fruit colors, protein, and different fat sources), and ensuring that all micronutrient and macronutrient needs are met. An experienced keto team can help guide you through this process.

  • Every keto team has different guidelines in how they practice with body care products and there is no one right answer. In my clinical experience I have found that it is not typically necessary to worry about a small amount of body care product that may have some carbohydrates (such as shampoo). I try to use low carb products in general for daily use items such as toothpaste and lotion (Charlie Foundation has a great list), and then follow ketosis as clues for any need to further reduce dietary carbs or hunt down unintended carbohydrate contributors.

  • Keto can be challenging at times for both the patients and the families, but with the support of a good team and network of other keto families it is both achievable and manageable and there are so many creative recipes and products available now that can make keto fun! I am in awe of the power that changing a diet can have on the clinical outcomes for patients—it makes it all worth it.