Major Fuel Sources Carbohydrate Fat Protein. Learning Objectives. Why is the diet modified? Important Considerations

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1 H O H O H 3 N C C N C C O - + H 2 0 CH 3 H CH 2 OH Urea Cycle Diets Translating Laboratory Information into Food Choices Learning Objectives Understand Why Importance of urea cycle diet What Components of urea cycle diet How How urea cycle diet is manipulated Why is the diet modified? Decrease nitrogenous waste Promote growth Maintain health Important Considerations Energy for the body Protein in the diet Major Fuel Sources Carbohydrate Fat Protein Don t Throw All Dietary Protein Out the Door Sugar Fat Protein

2 Consideration For Dietary Protein: Amino Acids Building blocks of protein Consideration For Dietary Protein: Amino Acids Essential amino acids Dietary source Non-essential amino acids Dietary source Endogenous source (our body makes them) Components of the Urea Cycle Diet Protein foods Natural sources of protein Metabolic Formula Protein FREE foods Natural foods Synthetic foods Protein Sources in UCD Diet 50% protein from natural foods Fruits, vegetables, starches Provide essential and nonessential amino acids 50% protein from metabolic formula Provide essential amino acids High Protein Foods These are eliminated from the diet Meat (beef, chicken, pork, lamb, fish) Eggs Cheese Dairy (milk and dairy products) Legumes Beans Moderate Protein Foods Must calculate the amount of protein in these foods

3 Metabolic Formulas Metabolic Formulas Complete Formulas WND-1 and WND-2 Cyclinex-1 and Cyclinex-2 Contain protein, fat, and carbohydrate Low Volume Formulas UCD-1 and UCD-2 Contain protein Contain very minimal amount of carbohydrate Protein Free Foods Modules Natural Sugary candy Oils & fats Fruits (extremely low in protein) Synthetic/ manufactured foods Carbohydrate Moducal Fat Microlipids Carbohydrate & Fat Pro phree Duocal Medical Foods Special Medical Foods Low Protein Cooking Low protein pasta and rice Low protein chocolate Low protein breads Low protein chips and cookies

4 Medical Food Companies Importance of Medical Foods Dietary Specialties Short term (first 5 years) Important to acquire taste for products Not needed for metabolic control during 1 st five years of life Long term (after 5 years of age) Will need medical foods to provide protein free calories Will need medical foods for metabolic control Need to introduce around 12 months of age Attend low protein cooking class Cost of Special Medical Foods Insurance Reimbursement 1 serving of regular pasta 1 serving of low protein pasta 1 loaf of regular bread 1 loaf of low protein bread $0.60 per serving $ 4.00 per serving $2.00 to 4.00 per loaf $12.00 per loaf Terminology Call the foods medical foods Prescription Have physician write a prescription for the foods Letter of medical necessity Must state and describe diagnosis Describe what will happen if medical foods are not used Urea Cycle Diet Diet of Fruits, Vegetables, Medical Foods, and Metabolic Formula How is Diet Manipulated?

5 Diet Prescription Enzyme Activity Laboratory data Growth Overall health Physical appearance Enzyme activity = Diet Prescription Dimmer switch Not just off or on Light switch Diet Prescription Metabolic Formula Program Regular Food Program Protein Prescription Calorie Prescription Enough to promote growth Formula Rules Formula Drink Rules 100% of formula including the foam Estimate amount of formula vomited and replace later in the day Add the recommended amount of water. Formula Rules Formula Storage Rules cans of formula in cool place opened cans in refrigerator for 10 days Throw away unused formula after 24 hours Don t microwave formula Formula Rules Protein Rules Spread dietary protein through out the day Thought to spread out nitrogenous waste

6 Amino Acid Budget 10 grams of protein per day Or 10 protein dollars per day Protein Free Foods Needed to provide calories for growth May be needed to provide vitamins and minerals for health and growth First Foods Foods must provide a specific amount of protein Metabolic formula must be adjusted to compensate for protein not consumed in foods Foods must be accurately measured or weighed Other Considerations Eat your colors Antioxidants Essential fatty acids Use of whole foods when possible Constipation Problem Solving Soften Stools & Increase Size Benefiber Fruit-eze Increase osmotic load Use juice instead of water when making formula Pear juice is high in fiber and fructose

7 Food Composition is Important Diet Histories are important! Too much nitrogenous waste Too much dietary protein Not enough dietary protein Not enough calories Correct Measurements Do Matter Good Growth Data Growth charts Well maintained Numerous measurements Accurately plotted Proper Equipment Problem Solving Elevated ammonia Too much dietary protein Not enough dietary protein Not enough calories Poor growth Not enough calories Not enough zinc or other nutrients Too much weight gain Too many calories

8 Solving Problems Growth Chart Refusal to drink formula Flavor formula Mocha mix Juice Flavor packets Entertainment around formula Sing and dance while shaking and making formula Use of stickers Gastrostomy tube Request feeding pump for times of illness Alternative Methods to Providing Formula Sick Day Programs 1. Keep out of the hospital if possible 2. Increase protein free calories 3. Modify formula Sick Day Protocol: Formula Dilute Increase water to dilute formula Use pedialyte or gatorade instead of water Reduce Remove or eliminate non-metabolic formula Examples: similac, pediasure Spread Space formula evenly through out day Slow Down Give formula slowly Give smaller amounts of formula at each feeding Consider continuous drip Sick Day Protocol: Diet Reduce Reduce or eliminate foods which contain protein Increase Increase protein free calories Juice, popsicles, pedialyte, gatorade

9 Sick Day Protocol: Hospital Goal: Stop catabolism Rehydrate Many options Intravenous (IV) Enteral (tube) Oral (mouth) Child Life Issues Medical Foods Cooking Classes School Lunch American Disability Act Child s legal right to have a modified school lunch Protection of Rights School Foodservice will not mention this right Advocacy Clinic must advocate for family Clinic provides State Modified School Lunch form School Form Periods of Rebellion Kindergarten May be first time child meets children who don t have their condition Adolescence Rebellion is normal part of development and separation Allow adolescent to rebel in another way Example: purple hair

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11 Education on Inheritance Dietary Management of Urea Cycle Disorders