Thank Christine for the honor of allowing me to speak here at this conference.
Ask the audience: How many here are MDs, RNs or NPs? How many here are RDs that work with the ketogenic diet? Work with children? Adults? How many are parents? How many are parents of a keto kid/families on the keto diet? How many have tried the keto diet in the past? How many were at the last conference in Denver and attended the presentation of ketogenic diets?
Disclaimer: although there are consensus guidelines regarding ketogenic dietary treatment, implementation and management varies slightly among center and child given the uniqueness of each individual and their response to the diet and treatment. Please discuss any dietary changes or treatments with your medical team prior to initiating them.
Photo from Epilepsy kids cartoon.
Not so much food, as the lack of food.
The Transfiguration is the last painting by the Italian High Renaissance master Raphael. Here the apostles cannot stop the boy’s seizures (demon possesion narrative at the time) until Jesus comes and tells them Scene of Jesus saying that this type of fits come out with prayer and fasting.
Livingston (1972) : treated > 1000 children on the ketogenic diet: 52% had complete control of seizures, 27% had improved control.
At the discovery of phyenytoin in 1938, the attention of the physicians and researchers shifted focus from the MOA and efficacy of the KD to new AEDs. In a new era
A type of ketogenic diet therapy even is used for our pets!
***Ketonix ? What ketone does urine check. Glucose monitor
Beta-hydroxybutyrate stays in blood if it is not used as energy. Acetone (which Ketonix indicates) is processed in the liver and expelled in the breath, e.g the concentration decreases over time. The beta-hydroxybutyrate level can be rapidly decreased by ingesting sugar, the acetone level will not react as quick. Example, eating a high protein meal can produce a result of low beta-hydroxybutyrate while still having acetone.
When glycogen stores are used up, body loses water storage. Hydration is very important.
A net carb is your total carb grams – fiber grams (do NOT subtract sugar alcohols). Some centers use net carbs in calculating the classic ketogenic diet others use total carb.
Classic Ketogenic 4:1 ratio or 3:1 ratio Older children, tend to respond at a lower ratio About 4-8 g of carbohydrate per 1000 calories Weigh with food scale. Modified Atkins ~1:1 ratio; 10 g carbohydrate per 1000 calories; but NO limit on protein. Use measuring cups & spoons Option to use scale Low glycemic index ~1:1 ratio; 40-60 g of carbohydrate per day. Use exchange lists for low glycemic load/index foods Food does not need to be weighed on a scale
MCT diet Use MCT oil for 40-60% of fat intake; consume carbohydrates per list exchanges; weigh out food on scale.
*Coconut oil is mostly C-12 MCT oil is mostly C-10 C-8 chain in mouse models has been found to be anti-epileptic
-There is an OTC designer MCT oil that is higher in C-8 …
Ed point about coconut oil - ~ 60% is MCT oil and of that it is C-12 chain. MCT oil is mostly C-10 chain but can be designed/manufactured to be high in c-8, which in some mouse models has been found to be particularly anti-epileptic. There are multiple mechanisms of the ketogenic diet – not just anti seizure, but also can somehow change the natural history of the progression of epilepsy and prevent further
This tells us the dose of the classic ketogenic diet.
Does the KD look like this? Or
What is on the market – A LOT – prescribed by your keto team. A medical food. Must be approved and monitored by MD and RD. Though can get them for $$ on the “grey” amazon and craig’s list market. (no gaurentee of quality or exp. Date!) Keto is popular now. GT feeders, oral eaters. Each formula option has it’s +/- You need to know your formulas, know what medi-cal covers, what insurance covers, the cost, the ingredients, the tolerance of the patient, and the ability to mix of the family. Powder vs liquid (GT = liquid) Still use a scale for liquid for exact measurements – or provide a graduated cylinder – but difficult to clean. 1 mL = 1 g
Duocal – not commonly used by RDs, but I like to use it in RCF because it provides fat and carb – so I can use more than just the solcarb or polycal. Also it decreases the products that we have on our formulary, so that is easier for everyone.
Recommend not giving senna tea (do not have a dose), caution with magnesium Calm drink for young children. We need to know doses.
4 cups water, ½ tsp baking soda, ½ tsp morton lite salt, flavoring w crystal lite or lemon/citrus zest. Diluted pedialyte Need the bicarb, need the sodium, in some cases need the dextrose that is in pedialyte. Confer with your keto team about the specifics of your sick plan.
But don’t be scared!! This is why you are followed closely!! And why the RD and MD make all those crazy recommendations. Bone density: yearly bone density (DEXA scans). Referral to endocrinologist for bisphosphonate treatment Kidney stones: yearly ultra sounds. Referral to urologist or nephrologist.
Photos of other books on here. ketovie
Find photo of Cross Et Al
Success of the ketogenic may allow AEDs to be tapered and some discontinued. Response rate occures within 3 months of goal diet dose but for evaluating how long the child is to be on the diet – look at the risk/benefit We do not have evidence for the MCT but the MCT makes ketones like the CKD and the MAD, so it will likely work as well. The LGIT may be a place to start the diet for families that have a highly processed diet, and then gradually adjust to a classic or MAD diet.
Half respond with 50% reduction of seizures to KD or MAD. Diet can be started along with 1st or 2nd AED. Possible that LGIT and MCT diet will have same response rate. Compliance depends on management of adverse effects and seizure response.
VPA – valproic acid – first line therapy LGT – lamotrigine –us as an adjunct RUF – second line adjunctive – try to discontinue VPA or LGT
There are some positive and troublesome but not contraindicatory indications between medications. Weight gain with VPA – a lower sugar diet can be beneficial, even if not a keto, LGIT, MAD ~50% of children with LGS respond to the classic or modified ketogenic diet therapy within 3 months with a decrease of 50% frequency in seizures. Recent recommendations are to implement the diet as early as the first AED is started if desired and especially if two AEDs are not providing benefit. It is possible that the LGIT and MCT diets may provide similar treatment responses, though the studies have not been done. Compliance to the diet depends on management of adverse effects and seizure response.
How do we get from here to
Here…for carb choices? Sometimes we have to take time to get ready for these changes. Sometimes we have the child and parents on board but we have to get the grandparents! You know, the kid comes home and had grahm crackers, a bag of gold fish, extra juice, cookies, cake, suddenly they are bouncing off walls and won’t eat dinner? Wait a minute, when did this change, when grandma was mom, there was no sugar in the house? Food as love – trips, time together, games,
Are there any modifications that need to be implemented first? Pros/Cons for each diet?
LGS and Dietary Therapies
LGS & Dietary Therapies
Marian Roan MPH, RD, CSP, CNSC
November 12, 2017
Defining & Comparing Diets
• Initiation Protocol Differences
• Home strategies
Choosing the Right Diet:
• What does the research say?
• What is best for my child and my family?
Ketogenic Diet Background
• Ketone basics
Diet History: Ancient Medicine
“Let food be thy
medicine and medicine
be thy food”
•Fasting to achieve seizure control
Diet History: Modern Medicine
• Wilder (1921): KD reproduces fasting and
decreases seizures in children with
• Between 1941 and 1980, all child epilepsy
text books reported on the ketogenic diet.
• Discovery of phenytoin (1938)– focus
shifted to developing new AEDs.
• Livingston (1972) : treated > 1000 children
• With increasing AEDs developed and
available, the use of the KD decreased.
Diet History: Revitalization
• Charlie Abrams, 1990s
• National Media Attention
• The Charlie Foundation
How does it work?
• Stimulates starvation.
• Body burns carbohydrate (glycogen) supply and burns
fat as the primary energy source.
• Ketones are made when fat is broken down.
• Ketones circulating in the body and brain can result in
improved seizure control although we still do not know
• Blood sugar levels are also decreased. Lower blood
sugar may also help decrease seizures.
Types of Dietary Therapy
• Classic Ketogenic Diet
• Modified Atkins Diet (MAD)
• Medium Chain Triglyceride Oil Diet (MCT diet)
• Low Glycemic Index Treatment (LGIT)
How do these diets compare?
Classic Low Glycemic Modified Atkins
Fat Protein Carbs
Kossoff, EH, et al (2011). | Pfeifer, HH. (2012)
~4-8 g net* carb 45-60 g total carb 10 g net carb
• Classic Ketogenic diet
– hospitalization from 2-5 days
– Some with 24 hr fasting start
– Some with decreased calories to start
– Some gradually increase diet ratio (dose)
• MAD, MCT, LGIT
– Home diet start after 1-2 hour+ clinic education
MCT Oil: Medium Chain Triglycerides
• Derived from coconut oil
(~50-60% MCT oil)
• Absorbed directly from
the gut into the liver
• Makes more ketones per
gram than LCT
• Does not contain
essential fatty acids
• Has gut side effects if
intake is too high too fast
The KD Ratio Prescription
Amount for growthTypically 1-4 x more Calculated to meet ratio
4:1, 3:1, 2:1, 1:1
General Medication Principles
• Tablets – YES!
• Gel Caps – YES!
• Liquids – NO!
• Chewables – NO!
• Suspensions – NO!
Disclaimer – sometimes we do prescribe liquids, chewables, and suspensions…if there are no
other options (only 2/50 children are currently on “sugar free” suspensions currently in my
practice – not low carb!)
Key Keto Point
Home Strategies & Side Effect Management
• Picky eating / Feeding difficulties
– Meal schedule, division of responsibility around
feeding – Ellyn Satter www.ellynsatterinstitute.org
– Formulas, modulars, pre-prepared keto food products
– “all in one meals”
– Feeding therapy referral
• Formulas that may be utilized for the ketogenic diet include:
• KetoCal (Nutricia)
– KetoCal 3:1 Powder (unflavored)
– KetoCal 4:1 Powder (vanilla)
– KetoCal 4:1 LQ Liquid (vanilla & unflavored)
• RCF (Abbott) – Ross carbohydrate free formula – soy based carbohydrate
– Used in milk protein allergy
– Used when carbohydrates must be very limited due to low caloric needs
• KetoVolve (Nutr-e-volution)
– Mild flavored powder; also chocolate flavor
• KetoVie 4:1 (Cambrooke) & KetoVie Peptide
– Chocolate & Vanilla –
– Peptide not on market yet
Also baking products and pre-made food available from Cambrooke. Baking flour from Solace.
• A variety of modular products may need to be added to
ensure nutrient needs are met and ketogenic ratios are
– Microlipid (Nestle) – safflower oil emulsion at 4.5 kcal/mL
– MCT oil (Nestle) – fractionated coconut oil at 7.7 kcal/mL
– Liquigen (Nutricia)- MCT emulsion at 4.5 kcal/mL
– Betaquik (Vitaflo) – MCT emulsion at 1.89 kcal/mL
– Carbzero (Vitaflo) – LCT emulsion at 1.8 kcal/mL
– Retail Oils (Olive Oil, coconut oil) – variable caloric density
– Solcarb (solace) – carbohydrate powder – maltodextrin – 3.75 kcal/g
– Polycal (nutricia) – carbohydrate powder – maltodextrin – 3.84 kcal/g
– Beneprotein (Nestle) – whey protein powder – 6 g/7 g powder
– Complete AA mix (Nutricia) – 100% AA – 8.2 g/10 g powder
– Super Soluble DuoCal (nutricia) – Fat + Carbohydrate - hydrolyzed
cornstarch, refined vegetables including coconut oil
Home Strategies & Side Effect Management
– Increase fluid
– Increase fiber & probiotic foods
– Medications: miralax, milk of magnesia, senna tablets
– GI referral
Home Strategies & Side Effect Management
• Sick Days (e.g dehydration from vomiting, diarrhea,
– TABLET MEDICATIONS for pain and fever
– # 1 HYDRATION
• Electrolyte beverage
• fluids 2-4 oz every hour
– Broth + 1-2 saltines
– Meals with half added fat
– Close communication with ketogenic diet team and
Long Term Side Effect Management
• Decreased bone density
– Weight bearing activity
– Vitamin D, Calcium & Phosphorus
• Kidney stone risk
– Drink a lot of fluids
– May need alkalizing agent e.g. cytra k crystals, baking soda
• Vitamin & Mineral deficiency
– Multivitamin & mineral, calcium, vitamin D, carnitine
• High blood lipids (cholesterol, triglycerides)
– Fat type adjusted – increase MCT, decrease sat fats, increase
unsat fats, add omega 3 rx
– Fiber increased and ratio decreased
Resources to Learn More
Keto Hope Foundation
Choosing the Child
for the Diet and the
Diet for the Child
• What does the research say about KD and
• What is best for my child and my family?
Expert Opinion on the Management of Lennox–Gastaut
Syndrome: Treatment Algorithms and Practical
Authors: J. Helen Cross,1,* Stéphane Auvin,2 Mercè Falip,3 Pasquale Striano,4 & Alexis Arzimanoglou5,6
Reviewed 18 studies that included data on LGS patients
and found 47% experienced >50% reduction in seizure
frequency for 3-36 months on the classic KD (1 MAD)
The VNS may
TPM may be synergistic
with KD, but acidosis
and risk for kidney stones
Cross et al 2017
• Patient is safe for the diet
– can use fat for energy
• Family is committed to a trial of the diet for 3
• Family can attend follow up appointments and
obtain lab tests when requested.
What Diet is Best for Us?
• Child’s food preferences
• Child’s willingness to participate with diet
• Oral feeding skills/safety
• Food Allergies/intolerances
• Daily schedule
• Caregiver availability to prepare meals & cooking
• Hospital vs Home Start
Future Research in KDs
• The future of KD research is in
cognitive and behavior
• Unknown if the KD in any
form could have an effect
cognitively or behaviorally
even if seizures do not change
for children with LGS.