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Management of Nutritional Issues Associated with Alternating Hemiplegia of Childhood Maria Raspolic MS, RD Santa Clara Valley Medical Center

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Page 1: Maria Raspolic MS, RD Santa Clara Valley Medical Center

Management of Nutritional Issues Associated with Alternating

Hemiplegia of ChildhoodMaria Raspolic MS, RD

Santa Clara Valley Medical Center

Page 2: Maria 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

Page 3: Maria Raspolic MS, RD Santa Clara Valley Medical Center

Poor growth Dehydration Constipation Decreased bone mineral density

Common Nutritional Issues Associated with AHC

Page 4: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Poor Growth

Page 5: Maria Raspolic MS, RD Santa Clara Valley Medical Center

Growth Assessment

Page 6: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Page 7: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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.

Page 8: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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.

Page 9: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Page 10: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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?

Page 11: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Recomendation

Page 12: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Constipation

Page 13: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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.

Page 14: Maria Raspolic MS, RD Santa Clara Valley Medical Center

If additional help needed:Milk of magnesiaLactuloseMiralaxBenefiber

Constipation con’t.

Page 15: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Page 16: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

How Much Calcium is Needed?

Page 17: Maria Raspolic MS, RD Santa Clara Valley Medical Center

Food sources/ table

Food Source Serving SizeAmount of Calcium per

Servingalmonds 3 ounces 210 milligrams (mg)

blackstrap molasses 1 tablespoon 170 mg

broccoli (cooked) 1 cup 60 mg

canned salmon (with bones)

3 ounces 180 mg

canned sardines (with bones)

3 ounces 325 mg

collards 1 cup 265 mg

cottage cheese 1 cup 155 mghard cheese (cheddar, swiss)

1 ounce 225 mg

kale (cooked) 1 cup 95 mg

milk 1 cup 300 mg

mozzarella cheese 1 ounce 200 mg

rhubarb (cooked) 1 cup 345 mg

ricotta cheese 1⁄2 cup 335 mg

spinach (cooked) 1 cup 245 mg

yogurt 8 ounces 425 mg

Calcium Sources in Food

Page 18: Maria Raspolic MS, RD Santa Clara Valley Medical Center

Calcium CarbonateViactiv, Tumbs, Caltrate

Calcium CitrateCitracal

Oyster Shell, Bone Meal

Calcium Supplements

Page 19: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Page 20: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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.

Page 21: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Vitamin D con’t.

Page 22: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Page 23: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Ketogenic Diet

Page 24: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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?

Page 25: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Page 26: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Page 27: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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.

Page 28: Maria Raspolic MS, RD Santa Clara Valley Medical Center

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

Page 29: Maria Raspolic MS, RD Santa Clara Valley Medical Center

ANY QUESTIONS OR COMMENTS?