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LGS and Dietary Therapies

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Marian Roan, RD, speaks about dietary therapies for Lennox-Gastaut Syndrome

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LGS and Dietary Therapies

  1. 1. LGS & Dietary Therapies Marian Roan MPH, RD, CSP, CNSC Clinical Dietitian November 12, 2017
  2. 2. Disclosures None
  3. 3. Agenda/Objectives Defining & Comparing Diets • Initiation Protocol Differences • Home strategies • Resources Choosing the Right Diet: • What does the research say? • What is best for my child and my family?
  4. 4. Ketogenic Diet Background • History • Ketone basics
  5. 5. Diet History: Ancient Medicine “Let food be thy medicine and medicine be thy food” – Hippocrates. •Fasting to achieve seizure control
  6. 6. Diet History: Modern Medicine • Wilder (1921): KD reproduces fasting and decreases seizures in children with epilepsy • 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 KD • With increasing AEDs developed and available, the use of the KD decreased. Ketones
  7. 7. Diet History: Revitalization • Charlie Abrams, 1990s • National Media Attention • The Charlie Foundation
  8. 8. Koko the Keto Kitty
  9. 9. Checking Ketones Urine acetoacetate 80-160 3-4+ Large Breath acetone Blood Beta hydroxybuterate 2-8 mmol/L
  10. 10. 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 how. • Blood sugar levels are also decreased. Lower blood sugar may also help decrease seizures.
  11. 11. Types of Dietary Therapy • Classic Ketogenic Diet • Modified Atkins Diet (MAD) • Medium Chain Triglyceride Oil Diet (MCT diet) • Low Glycemic Index Treatment (LGIT)
  12. 12. 60% 30% 10% How do these diets compare? Classic Low Glycemic Modified Atkins Fat Protein Carbs 90% 6%4% 77% 17% 6% Kossoff, EH, et al (2011). | Pfeifer, HH. (2012) ~4-8 g net* carb 45-60 g total carb 10 g net carb
  13. 13. Initiation Protocols • 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
  14. 14. 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
  15. 15. The KD Ratio Prescription Fat grams Protein grams Carb grams Amount for growthTypically 1-4 x more Calculated to meet ratio 4:1, 3:1, 2:1, 1:1
  16. 16. 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
  17. 17. 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
  18. 18. KD Products • 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 free formula – 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.
  19. 19. Modular Products • A variety of modular products may need to be added to ensure nutrient needs are met and ketogenic ratios are correct • Lipid – 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
  20. 20. Modular Products • Carbohydrate – Solcarb (solace) – carbohydrate powder – maltodextrin – 3.75 kcal/g – Polycal (nutricia) – carbohydrate powder – maltodextrin – 3.84 kcal/g • Protein – Beneprotein (Nestle) – whey protein powder – 6 g/7 g powder – Complete AA mix (Nutricia) – 100% AA – 8.2 g/10 g powder • Combination – Super Soluble DuoCal (nutricia) – Fat + Carbohydrate - hydrolyzed cornstarch, refined vegetables including coconut oil
  21. 21. Home Strategies & Side Effect Management • Constipation – Increase fluid – Increase fiber & probiotic foods – Medications: miralax, milk of magnesia, senna tablets – GI referral
  22. 22. Home Strategies & Side Effect Management • Sick Days (e.g dehydration from vomiting, diarrhea, inadequate fluids) – 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 pediatrician
  23. 23. 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
  24. 24. Resources to Learn More Websites Support Groups Books www.thecharliefoundation.org www.mathewsfriends.com www.myketocal.com www.epilepsy.org Keto Hope Foundation
  25. 25. Choosing the Child for the Diet and the Diet for the Child • What does the research say about KD and LGS? • What is best for my child and my family?
  26. 26. Research/Evidence Expert Opinion on the Management of Lennox–Gastaut Syndrome: Treatment Algorithms and Practical Considerations 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)
  27. 27. Treatment Algorithm The VNS may be synergistic with KD TPM may be synergistic with KD, but acidosis and risk for kidney stones VPA LGT RUF CLBTPM FLB Keto Diet VNS Resective Surg Callosotomy KD can amplify side effects of VPA Cross et al 2017
  28. 28. Selection Criteria • Patient is safe for the diet – can use fat for energy • Family is committed to a trial of the diet for 3 months. • Family can attend follow up appointments and obtain lab tests when requested.
  29. 29. 2014Processed Carb & high Glycemic Index Carb
  30. 30. Minimally Processed, Low Glycemic Index Carbs
  31. 31. What Diet is Best for Us? • Child’s food preferences • Child’s willingness to participate with diet restrictions • Oral feeding skills/safety • Food Allergies/intolerances • Daily schedule • Caregiver availability to prepare meals & cooking skill set • Hospital vs Home Start
  32. 32. Future Research in KDs • The future of KD research is in cognitive and behavior development. • Unknown if the KD in any form could have an effect cognitively or behaviorally even if seizures do not change for children with LGS.

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