Management of Nutritional Issues Associated with Alternating

Hemiplegia of ChildhoodMaria Raspolic MS, RD

Santa Clara Valley Medical Center

Objectives: Review most common nutritional issues in

AHC Provide recommendations to minimize

above issues Review ketogenic diet as treatment for

seizures

Management of Nutritional Issues Associated with Alternating Hemiplegia of Childhood

Poor growth Dehydration Constipation Decreased bone mineral density

Common Nutritional Issues Associated with AHC

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

Poor Growth

Growth Assessment

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

Intervention

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

Intervention con’t.

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

Intervention con’t.

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

Dehydration

100 cc/kg of body wt for the first 10 kg50 cc/kg for the second 10 kg20 cc/kg for the additional kgs

44 lbs :2.2 = 22 kg1000 + 500 + 40 = 1540 cc1540: 30 = 51 oz

How much fluid?

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

Recomendation

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

Constipation

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

Constipation con’t.

If additional help needed:Milk of magnesiaLactuloseMiralaxBenefiber

Constipation con’t.

Limited ambulation Inadequate intakes of Ca, Phos, Vit D Anticonvulsant therapy Limited sun exposure If untreated may lead to osteoporosis,

bone deformities and fractures

Low Bone mineral Density

1-3 years: 500mg4-8 years 800 mg9-18 years 1300 mg

How Much Calcium is Needed?

Food sources/ tableFood Source Serving Size Amount of Calcium per

Servingalmonds 3 ounces 210 milligrams (mg)blackstrap molasses 1 tablespoon 170 mgbroccoli (cooked) 1 cup 60 mgcanned salmon (with bones) 3 ounces 180 mgcanned sardines (with bones) 3 ounces 325 mgcollards 1 cup 265 mgcottage cheese 1 cup 155 mghard cheese (cheddar, swiss) 1 ounce 225 mgkale (cooked) 1 cup 95 mgmilk 1 cup 300 mgmozzarella cheese 1 ounce 200 mgrhubarb (cooked) 1 cup 345 mgricotta cheese 1⁄2 cup 335 mgspinach (cooked) 1 cup 245 mgyogurt 8 ounces 425 mg

Calcium Sources in Food

Calcium CarbonateViactiv, Tumbs, Caltrate

Calcium CitrateCitracal

Oyster Shell, Bone Meal

Calcium Supplements

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)

Vitamin D

Recommend to check blood levels yearlyGoal: 30-60 mmol/dlSupplement 1000 IU/day

50 000 IU/ weekVit D3 (cholecalciferol) in the skin by sun expoVit D2 (ergocalciferol) synthesized by plants

15 min sun exposure prevent Vit D deficiency

Vitamin D con’t.

Borusiak et al, 2012128 children receiving one AED24 % hypocalcemia25% hypophosphatemia13% low vit DPhenobarb, Depakote, Trileptal, Dilantin

Vitamin D con’t.

Common in children with AHC Complete MVI recommended Chewable tablet preferred Liquid/soft gummy vits less minerals Bugs Bunny, Flinstone’s, Scooby Doo,

NanoVites

Vitamin and Mineral Deficiencies

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

Ketogenic Diet

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

Chances of success?

Dehydration-check urine daily with keto stickConstipation-MOM, MiralaxKidney Stones- UA, trace amount of bloodNutrient deficiency- complete MVI, ck blood

levels ZN, Se, Vit DDecreased growth- adjust protein, kcalHi Cholesterol- replace butter with olive oil,

supplement with carnitine

Side Effects of the diet

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

Initiation of the diet

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

Initiation of the Diet con’t.

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

Discontinuation of the diet

ANY QUESTIONS OR COMMENTS?