1. Maria Raspolic MS, RD Santa Clara Valley Medical Center

2. Objectives: Review most common nutritional issues in AHC Provide recommendations to minimize above issues Review ketogenic diet as treatment for seizures

3. Poor growth Dehydration Constipation Decreased bone mineral density

4. Inadequate nutrient intakes Decreased muscle tone Oral motor dysfunction Limited growth potential Frequent illness, fatigue, infections

6. 1. Provide food of high nutritional quality Breakfast: oatmeal, eggs, cream of wheat, yogurt, Carnation Instant Breakfast, meat Lunch/Dinner: refried beans/cheese, lentils, tofu, almond/nut butter, avocados, fatty fish, meat, sweet potatoes, fried rice/mex rice Snacks: milk shakes/smoothies, cheese stick

7. 2. Liquid supplements Concentrate infant formula to 24-30 kca/oz Pediasure, Boost Kids Essential, Nutren Jr Ensure, Nutren, Boost Carnation Instant Breakfast 1.5 and 2 cal/cc formulas

8. 3. Supplemental tube feeding Child not able to gain weight adequately Excessive time needed to feed Difficult decision for parents Goal: improved quality of life Dramatic improvement in nutritional status

9. Inadequate fluid intakes Excessive fluid loss Need for thickened liquids Result in constipation, decreased appetite Kidney stones, UTI, thickening of secretions Monitor number of diapers, UA

10. 100 cc/kg of body wt for the first 10 kg 50 cc/kg for the second 10 kg 20 cc/kg for the additional kgs 44 lbs :2.2 = 22 kg 1000 + 500 + 40 = 1540 cc 1540: 30 = 51 oz

11. Provide hi nutritional value liquids: Milk, soy, rice, almond, coconut Smoothies, milkshakes ? juice

12. Multifactorial cause Poor intakes of fluids and solids Low muscle tone/ GI motility Low activity levels Low fiber diet

13. Contributes to poor appetite Abdominal distention/discomfort Irritability Adequate fluid intakes and fiber Hi fiber foods: cereal (5 gr/serving), legumes Sweet potatoes, fruits/vegetables Prune or pear juice

14. If additional help needed: Milk of magnesia Lactulose Miralax Benefiber

15. Limited ambulation Inadequate intakes of Ca, Phos, Vit D Anticonvulsant therapy Limited sun exposure If untreated may lead to osteoporosis, bone deformities and fractures

16. 1-3 years: 500mg 4-8 years 800 mg 9-18 years 1300 mg

17. Food sources/ table Food SourceServing SizeAmount of Calcium per Serving almonds3 ounces210 milligrams (mg) blackstrap molasses1 tablespoon170 mg broccoli (cooked)1 cup60 mg canned salmon (with bones) 3 ounces180 mg canned sardines (with bones) 3 ounces325 mg collards1 cup265 mg cottage cheese1 cup155 mg hard cheese (cheddar, swiss) 1 ounce225 mg kale (cooked)1 cup95 mg milk1 cup300 mg mozzarella cheese1 ounce200 mg rhubarb (cooked)1 cup345 mg ricotta cheese12 cup335 mg spinach (cooked)1 cup245 mg yogurt8 ounces425 mg Calcium Sources in Food

18. Calcium Carbonate Viactiv, Tumbs, Caltrate Calcium Citrate Citracal Oyster Shell, Bone Meal

19. Sunshine Vitamin Sunblock use prevents Vit D production Anticonvulsant meds (Phenobarb, Dilantin) Decreased absorption of Calcium Limited food sources: fish liver oil, fatty fish, egg yolk, mushrooms, milk (fortified)

20. Recommend to check blood levels yearly Goal: 30-60 mmol/dl Supplement 1000 IU/day 50 000 IU/ week Vit D3 (cholecalciferol) in the skin by sun expo Vit D2 (ergocalciferol) synthesized by plants 15 min sun exposure prevent Vit D deficiency

21. Borusiak et al, 2012 128 children receiving one AED 24 % hypocalcemia 25% hypophosphatemia 13% low vit D Phenobarb, Depakote, Trileptal, Dilantin

22. Common in children with AHC Complete MVI recommended Chewable tablet preferred Liquid/soft gummy vits less minerals Bugs Bunny, Flinstones, Scooby Doo, NanoVites

23. How does it work? Brain needs glucose from food 24 hours supply Breakdown of fat produces ketones ??? Prevention of seizures

24. 2-3 months trial 30% of the children seizure free 30% significant reduction in seizures, reduction in medication or no medication Reminder do not respond or find it to hard to continue

25. Dehydration-check urine daily with keto stick Constipation-MOM, Miralax Kidney Stones- UA, trace amount of blood Nutrient deficiency- complete MVI, ck blood levels ZN, Se, Vit D Decreased growth- adjust protein, kcal Hi Cholesterol- replace butter with olive oil, supplement with carnitine

26. Gradual decrease in CHO over one week 2-3 day hospital admission Fasting only in the AM At lunch time full keto meal Allow fluids to meet hydration need Spec. gravity and ketones check with every void

27. Teach families how to calculate and prepare meals Keto meal planer Complexity of meals controlled by parents Ready to feed Ketogenic formula; Ketocal RCF for tube feeding

28. Reduction of the ratio over couple of months Most parents find diet easier than anticipated Ketogenic diet is the most effective available treatment for intractable epilepsy today Atkins diet