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H O H O

H3N C C N C C O- + H20

CH3 H CH2

OH

Urea Cycle DietsTranslating Laboratory Information into

Food Choices

Learning ObjectivesLearning ObjectivesUnderstand– Why

• Importance of urea cycle diet

– What• Components of urea cycle diet

– How• How urea cycle diet is manipulated

Why is the diet modified?Why is the diet modified?

Decrease nitrogenous wastePromote growthMaintain health

Important ConsiderationsImportant Considerations

Energy for the bodyProtein in the diet

Major Fuel SourcesCarbohydrate

FatProtein

SugarFat

Protein

Don’t Throw All Dietary ProteinDon’t Throw All Dietary ProteinOut the DoorOut the Door

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Consideration For Dietary Protein: Consideration For Dietary Protein: Amino AcidsAmino Acids

•Building blocks of protein

Consideration For Dietary Protein: Consideration For Dietary Protein: Amino AcidsAmino Acids

•Essential amino acids •Dietary source

•Non-essential amino acids•Dietary source

•Endogenous source (our body makes them)

Components of the Urea Cycle DietComponents of the Urea Cycle Diet

Protein foods– Natural sources of protein– Metabolic Formula

Protein FREE foods– Natural foods– Synthetic foods

Protein Sources in UCD DietProtein Sources in UCD Diet

50% protein from natural foods– Fruits, vegetables, starches– Provide essential and nonessential

amino acids50% protein from metabolic formula– Provide essential amino acids

High Protein FoodsHigh Protein Foods

– Meat (beef, chicken, pork, lamb, fish)– Eggs– Cheese– Dairy (milk and dairy products)– Legumes– Beans

These are eliminated from the diet

Moderate Protein FoodsModerate Protein FoodsMust calculate the amount of

protein in these foods

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Metabolic Metabolic FormulasFormulas

Metabolic FormulasMetabolic 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 FoodsProtein Free FoodsNatural– Sugary candy– Oils & fats– Fruits (extremely low in protein)

Synthetic/ manufactured foods– Carbohydrate

• Moducal– Fat

• Microlipids– Carbohydrate & Fat

• Pro phree• Duocal

– Medical Foods

Modules Modules

Special Medical Foods

•Low protein pasta and rice

•Low protein chocolate

•Low protein breads

•Low protein chips and cookies

Low Protein CookingLow Protein Cooking

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Medical Food CompaniesMedical Food Companies

www.ener-g.com

www.medicalfood.com

www.shsna.com

www.cambrookefoods.com

Dietary Specialtieswww.dietspec.com

Importance of Medical FoodsImportance of Medical Foods

Short term (first 5 years)– Important to acquire taste for products– Not needed for metabolic control during 1st

five years of lifeLong 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 FoodsCost of Special Medical Foods

$12.00 per loaf1 loaf of low protein bread

$2.00 to 4.00 per loaf1 loaf of regular bread

$ 4.00 per serving1 serving of low protein pasta

$0.60 per serving1 serving of regular pasta

Insurance ReimbursementInsurance ReimbursementTerminology – Call the foods “medical foods”

Prescription– Have physician write a prescription for the

foodsLetter of medical necessity – Must state and describe diagnosis– Describe what will happen if medical foods are

not used

Urea Cycle DietDiet of Fruits, Vegetables,

Medical Foods, and Metabolic Formula

How is Diet Manipulated ? How is Diet Manipulated ?

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Diet PrescriptionDiet Prescription

Laboratory dataGrowthOverall healthPhysical appearance Enzyme activity

Diet Prescription=

Enzyme ActivityEnzyme Activity

Dimmer switch– Not just “off” or “on”Light switch

Diet PrescriptionDiet Prescription

Metabolic Formula ProgramRegular Food Program– Protein Prescription– Calorie Prescription

• Enough to promote growth

Formula RulesFormula Rules

•Drink 100% of formula including the foam

•Estimate amount of formula vomited and replace later inthe day

•Add the recommendedamount of water.

Formula RulesFormula Rules

•Storage•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 RulesProtein Rules

•Spread dietary protein through out the day•Thought to spread out nitrogenous waste

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Amino Acid Budget10 grams of protein per day

Or10 protein dollars per day

Protein Free FoodsProtein Free Foods

Needed to provide calories for growthMay 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 ConsiderationsOther Considerations

Eat your colorsAntioxidantsEssential fatty acidsUse of whole foods when possible

ConstipationConstipation

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

Problem SolvingProblem Solving

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Diet Histories are important !

Food Composition is Important

Correct Measurements Do Matter

Too much nitrogenous wasteToo much nitrogenous waste

Too much dietary proteinNot enough dietary proteinNot enough calories

Good Growth DataGood Growth Data

Growth charts– Well maintained– Numerous

measurements– Accurately plotted

Proper Equipment

Problem SolvingProblem Solving

Elevated ammonia– Too much dietary protein– Not enough dietary protein– Not enough calories

Poor growth– Not enough calories– Not enough zinc or other

nutrientsToo much weight gain– Too many calories

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Growth Chart

Solving Problems Solving Problems

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 Programs1. Keep out of the hospital if possible

2. Increase protein free calories

3. Modify formula

Sick Day Protocol: Sick Day Protocol: FormulaFormulaDilute

• Increase water to dilute formula• Use pedialyte or gatorade instead of water

Reduce• Remove or eliminate non-metabolic formula

– Examples: similac, pediasureSpread

• Space formula evenly through out daySlow Down

• Give formula slowly• Give smaller amounts of formula at each feeding• Consider continuous drip

Sick Day Protocol: Sick Day Protocol: DietDiet

Reduce– Reduce or eliminate foods which

contain proteinIncrease– Increase protein free calories

• Juice, popsicles, pedialyte, gatorade

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Sick Day Protocol: Sick Day Protocol: HospitalHospital

Goal:– Stop catabolism– RehydrateMany options– Intravenous (IV)– Enteral (tube) – Oral (mouth)

Child Life IssuesChild Life Issues

Medical Foods Cooking ClassesMedical Foods Cooking Classes School LunchSchool Lunch

American Disability Act– Child’s legal right to have a

modified school lunchProtection of Rights– School Foodservice will not

mention this right– Advocacy

• Clinic must advocate for family• Clinic provides State Modified

School Lunch form

School FormPeriods of RebellionPeriods of Rebellion

Kindergarten– May be first time child meets children

who don’t have their conditionAdolescence– Rebellion is normal part of development

and separation– Allow adolescent to rebel in another

way• Example: purple hair

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Education on

Inheritance

Dietary Management of Urea Cycle Dietary Management of Urea Cycle DisordersDisorders