National Kidney Month – Managing PKU

For many years, Mum Mum’s has been dedicated to providing healthy, allergen free foods to help keep families healthy. Mum Mum’s has their own registered dietitian, who is also a Certified Kid’s Nutrition Specialist and celiac who serves as the Mum Mum’s nutrition expert.  Mums often have the same question, so this blog also serves as a place to answer some of those questions.

This month, in honor of National Kidney Month, it seems a good time to discuss a question we receive often: PKU – or phenylketonuria. PKU is a rare genetic condition in which a baby is born without the ability to breakdown the amino acid phenylalanine. Essentially there is a mutation in the gene that produces the enzyme needed to break down phenylalanine so too much phenylalanine builds up in the body causing health problems ranging from mild to severe.

Amino acids are the building blocks of protein, so babies and adults with PKU need to be very mindful of their protein intake.  Thankfully, it is routine to test a newborn immediately for PKU, and it is usually the result of both parents carrying the gene for PKU. It is also important that women who have PKU that are expecting or plan to become pregnant maintain a low phenylalanine diet both prior to and during pregnancy, and let their doctors know they have PKU.

Since phenylalanine is found in high protein foods, the following foods should be avoided:

• Eggs
• Milk
• Cheese
• Nuts
• Soybeans
• Beans
• Chicken
• Steak and other beef products
• Pork
• Fish
• Chocolate candy
• Peas
• Beer
• desserts

It is also critical to avoid foods with Aspartame, found in many artificial sweeteners, because aspartame releases phenylalanine when digested. Even fruits and vegetables contain lower amounts of protein and should be properly calculated into the PKU diet, and infants with PKU will require a phenylalanine free infant formula.

Of course, it is necessary to remain under the care of a physician when diagnosed with PKU for regular check-ups and this information should not take the place of medical advice.  Parents of babies with PKU will also be referred to a dietitian for nutrition education on how manage a PKU diet.

Low protein foods like crackers and pasta are often ok to consume without calculating the phenylalanine, but many of our mums wanted to know the exact macronutrient breakdown of a Baby Mum Mum’s so we are providing the answer:

Total carbohydrates:      .92g/1g

Total protein:                     .062g/1g

Total fat:                              .009g/1g

This information can be given to your dietitian to help you calculate your baby’s diet, and know that with a little savvy and the help of your medical team PKU can be healthfully managed.