What is PKU?

Phenylketonuria is a birth defect which causes an amino acid called phenylalanine to buildup in the body. With PKU, you can’t breakdown protein and phenylalanine. It is tested via the Guthrie or ““heel-prick” test.

Foods to consume consist of low protein and low phenylalanine.

• Diet should be low in phenylalanine foods, which are foods that are low in protein. Foods include:

• Some fruit contain more Phe than others, so you need to be careful which fruit you choose.
• You can eat these fruits freely on a PKU diet: Fruit including apples, bananas, mango, berries, pineapple, peaches.
• Vegetables that you can eat freely include: Cabbage, carrots, onions, tomatoes, lettuce, and cucumber, celery.
• Fats, such as olive oil, whipped butter, coconut oil, avocado oil.
• Ricotta cheese
• Low protein pasta and breads
• Cassava flour, arrowroot, cornstarch
• Certain dairy alternatives that consist of almond, coconut or rice milk such as vegan cheese.

Foods to Restrict consist of high protein and high phenylalanine.

 These foods include:

• Animal-based proteins, meat, poultry, fish, eggs, bacon, turkey, chicken, steak

• Beans, nuts, soy products, seeds, peanut butter.

• Dairy such as cheese and milk, greek yogurt

• Certain grains including oats, barley, quinoa, wheat, and rye, toast, whole wheat toast, cereal.

• Gelatin

• Beer

•Drinks or products that contain aspartame. Aspartame releases Phe when it is digested. Be careful of diet sodas, flavored drink mixes, and energy drinks.

•Be aware of certain fruits and veggies that contain more Phe than others to ensure you don’t consume too much. Including broccoli, asparagus, kale, potatoes, spinach, dried banana, dried mango.

Supplements Recommended:

Tyrosine: Supplementing Tyrosine can prevent deficiency caused by the PKU diet and improve behavior. Phenylalanine converts to tyrosine.

Tips to Help Maintain the PKU Diet:

• Mix up the flavors by using different herbs and spices.

• Use different kitchen tools to cut your veggies and fruit into various shapes, such as using a spiraliger to make spaghetti squash noodles or a blender to make smoothies.