TABLE TALK: MANAGING THE PKU DIET FOR KIDS IN SOCIAL SITUATIONS

Meg Stone, MS, RD, LD Jessica Williamson, MS, RD, LD Emory University, Department of Human Genetics Disclosures

• We have no disclosures to report Objectives

• Understand the 3 components of the PKU diet and their individual roles in PKU dietary management

• Highlight the importance of tracking daily dietary PHE/protein intake and identify resources for both parents and children to aid in counting

• Discuss the purpose of PHE level monitoring and how results are used to tailor dietary recommendations

• Identify possible barriers affecting dietary compliance in social settings and explore possible solutions DISCUSSION: PKU DIET IN SOCIAL SETTINGS What works well and not so well? 3 COMPONENTS OF PKU DIET

Formula

Fruits Vegetables

Low-protein modified , grains, starches, etc 1. Formula/Medical

• Role of formula in PKU diet: • Provides majority of protein in diet while supplementing tyrosine and restricting phenylalanine • May provide vitamins and minerals (not all formulas provide these) • Provides calories for energy

• Importance of drinking formula: • Helps control plasma phenylalanine concentrations • Essential for brain function and cognitive development • Prevents protein malnutrition due to dietary protein restriction

• Types of formula: • Powdered • Ready to (RTD) • Combination of both powdered and RTD 2. Low-Protein Modified Foods (LPMF)

• Role of LPMF in PKU diet: • Promotes satiety • Provides calories to meet child’s energy needs • Does not contribute a substantial amount of PHE to child’s PHE allowance

• LPMF in Social Settings: • Many LPMF options are similar to regular starchy foods, making substitutions easier • Breads & buns • Tortillas & pitas • Baking & all purpose mix • Regular & hot cereals • Pancakes & waffles • Cookie dough • Pasta • Rice • Pizza crust 3. Other Foods Low in Protein

• What are other foods low in protein? • Primarily vegetables and fruits • Also includes gluten-free foods and dairy-free products • Inclusion of these foods varies depending on PHE goal • These are the foods counted toward daily PHE goal • Daily dietary PHE intake should not exceed prescribed daily PHE goal • Can usually be found within your local grocery store or supermarket

• Role of other foods: • Provide some phenylalanine needed to promote growth • Provides vitamins, minerals, and fiber EMPOWERING YOUR CHILD TO MONITOR PHE INTAKE Daily PHE/Protein Counting

• Resources: 1. HowMuchPhe (subscription required) 2. Applications (Accugo, MyFitnessPal) 3. Books/Pocket Guides (PKU Food List, HowMuchPhe) 4. Nutrition Facts Labels (available on all packaged foods, unavailable on produce) Grocery Store Scavenger Hunt

• Have your child help compile a list of food items <1 gram of protein per serving found in the local supermarket or grocery store

• Tackle an aisle at a time to make collecting information less overwhelming

• Use this list as a reference guide for your family and friends providing

in various social settings Emory Genetics, 2016 Stoplight Game for Counting PHE in Foods

“Go” Foods “Caution” Foods “Stop” Foods Rule of thumb: foods low in Rule of thumb: need to be Rule of thumb: contain a large protein/PHE and typically make up consumed in limited quantities amount of protein; should not be the bulk of the diet consumed as they are not suitable for the PKU diet

Disclaimer: May still need to be Disclaimer:Typically contain >1 Disclaimer: Inclusion dependent counted toward PHE goal gram of protein/serving and always upon individual PHE tolerance and if eaten in large quantities need to be counted toward PHE response to concurrent PKU drug goal therapies

Disclaimer: inclusion of “caution” foods dependent upon individual PHE allowance Stoplight Game for Counting PHE in Foods

“Go” Foods “Caution” Foods “Stop” Foods • Fruit: Apples, berries, clementines, grapes, • Potato products • Meat: beef, poultry, pork, etc. grapefruit, jack fruit, kiwi fruit, lemons, • Spinach • Fish limes, mango, melon, nectarines, papaya, • Spring greens • Eggs peaches, pears, pineapple, plum, • Peas & beans • Dairy products pomegranate, rhubarb, raisins. • Corn & popcorn • Cheese products, including • Vegetables: Artichoke, baby corn, green • Kale spreads beans, cabbage, carrots, cassava, celery, • Asparagus • Nuts & seeds cucumber, garlic, lettuce, mushrooms, • Broccoli • Flour-based foods: bread, onion, all herbs, peppers, pumpkin, • Brussels Sprouts biscuits, buns, cake squash, sweet potato, tomato • Cauliflower • Tofu • Fats: butter, vegetable fat and oils • Plantain • Aspartame : E951, E962, • Misc: sugar, icing (except chocolate), jam, • Bananas Aspartame-Acesulfame-K honey, syrup, marmalade • Figs (dried) & dates • LPMF Stoplight Challenge Activity

Jessica’s Stoplight Foods • Step 1: Pick out 20 most frequently consumed foods “Go Foods” “Caution” Foods “Stop” Foods Apple Banana No stop foods • Step 2: Have your child look up protein or PHE Orange Asparagus allowed; PHE content of each food using a trusted resource Pineapple Broccoli goal = 400 mg Cabbage French fries • Step 3: Categorize foods into 1 of the 3 Cucumber Gluten-free bread stoplight categories Grapes Gluten-free Jam waffles • Step 4: Update as necessary if new foods are Syrup Peas incorporated into the diet TEACHING YOUR CHILD THE IMPORTANCE OF MONITORING PHE LEVELS Monitoring

• Regular PHE levels are needed to adjust diet appropriately • When should we submit blood work? • As recommended by your local clinics (weekly, monthly, more often with levels outside treatment range) • Also when you observe changes in behavior, concentration, etc. (create open dialogue with child to discuss feelings toward school work load; observe child completing homework if possible)

• What if my child doesn’t like providing samples? • Set up appointment with local outpatient lab to have blood drawn as scheduled

• Are food records really necessary? • Yes. Food records allow your child’s dietitian to identify potential foods contributing to high PHE levels TIPS FOR PKU DIET MANAGEMENT IN SOCIAL SETTINGS Birthday Parties

• Prepare • Reach out to the host ahead of time and discuss a plan to serve alternative PKU diet friendly snacks or • Review the “stoplight” system with your child • Provide your child with a notebook so that he or she can write down what is consumed

• Execute • Depending on what is being served, prepare low protein modified cupcakes or cake to be served alongside the regular

• Resources • http://www.cambrooke.com/recipes/ • NPKUA “PKU Information for Adult Friends of the Family” • NPKUA “PKU Information for Friends of a Child with PKU” • NPKUA “PKU Information for Daycare Providers and Teachers” Dining Out

• Prepare • Speak with management ahead of time to discuss items, preparation details, and available substitutions • Have your child review the menu online and determine what items are PKU diet-friendly • Decide what items will need to be substituted with LPMF

• Execute • Bring unprepared or prepared low protein modified rice, tortilla, or pasta to pair with PKU- friendly dishes or a combination of sides • If is unable to prepare LPMF, bring prepared in microwavable containers to restaurant and ask for it to be served on the child’s plate with the rest of the

• Resources • http://www.cambrooke.com/recipes/ • NPKUA “My PKU Binder: Chapter 10 Dining Out and Other Tips” Sporting Events

• Prepare • Discuss your child’s dietary restrictions with other parents responsible for providing snacks • Have your child help prepare a list of PKU diet-friendly post-game snacks, such as fruits, applesauce, etc. (“Green Light Go Foods”) • Review the “stoplight” system with your child

• Execute • If all or part of the scheduled is not PKU diet-friendly, pack a low PHE snack in your child’s sporting bag • Pack formula in an insulated bottle for longer games or tournaments

• Resources • http://www.cambrooke.com/recipes/ • NPKUA “PKU Information for Adult Friends of the Family” • NPKUA “PKU Information for Friends of a Child with PKU” Sleepovers

• Prepare • Reach out to the host ahead of time and discuss a plan to serve alternative PKU diet friendly dishes and snacks • Suggest that can be easily modified to reduce PHE intake , such as build-you-own personal pizza • Review the “stoplight” system with your child and appropriate portions of “yellow light” foods • Provide your child with a notebook so that he or she can write down what is consumed

• Execute • Depending on what is being served for , prepare or send LPMF, such as pizza crust, shredded cheese, mixquick, tortillas, etc. • Depending on what is being served for breakfast, prepare or send fruit and LPMF, such as mixquick, for pancakes or waffles

• Resources • http://www.cambrooke.com/recipes/ • NPKUA “PKU Information for Adult Friends of the Family” • NPKUA “PKU Information for Friends of a Child with PKU” GAME TIME Using the scenario on your table, create a PKU-diet friendly alternative to prepare for the described social situation THANK YOU! Resources

• NPKUA “My PKU Binder”- https://npkua.org/portals/0/pdfs/pkubinder/PKU%20Binder%202011.pdf • National Centre for Inherited Metabolic Disorders- http://metabolic.ie/wp- content/uploads/2015/05/Aspastame-and-other-Sweetener-Information-for-PKU.pdf • National Society for Phenylketonuria- http://www.nspku.org/sites/default/files/publications/Dietary%20Information%20Booklet%202013_0.pdf • CambrookeTherapeutics- http://www.cambrooke.com/ • Nutricia North America- http://www.medicalfood.com/ • Vitaflo- https://www.nestlehealthscience.us/vitaflo-usa • Abbott- https://abbottnutrition.com/ • Emory Metabolic Genetics and Nutrition Program- http://genetics.emory.edu/patient-care/metabolic- genetics-nutrition/educational-materials.html • HowMuchPhe- https://howmuchphe.org/