Low Protein Cooking For Phenylketonuria

Phenylketonuria (PKU) is a rare congenital disease in which a baby is born unable to break down the amino acid phenylalanine. Phenylalanine plays a role in the production of melanin, which is responsible for skin and hair color. As such, infants born the condition will often have lighter skin, hair and eyes than their siblings. Delayed development, mental retardation and musty breath are other symptoms of the disease. Treatment involves a diet that is extremely low in phenylalanine, which is mostly found in protein-rich foods, particularly when the child is growing. Women with PKU should follow a strict low-phenylalanine diet both before becoming pregnant and throughout the pregnancy. Those with this condition need monitoring by a doctor to determine how much phenylalanine is safe to consume daily.

Step 1

Feed your infant a special formula created for phenylketonuria, which contains the necessary amino acids. Your doctor can re-calculate formula requirements as the baby grows. Oftentimes, the formula will contain a small amount phenylalanine, which is mandatory for your baby to grow.

Step 2

Replace foods that contain low and moderate amounts of phenylalanine, such as pasta, rice, bread, cookies and certain fruits and vegetables, with other items, such as baked or mashed sweet potatoes, tarot or yucca, which make suitable substitutes due to their high carbohydrate content, when you prepare meals. Sweet potatoes can also double as a dessert item as they are naturally sweet. Appropriate fruits and vegetables to include in your meal plan are apples, apricots, cherries, beets, asparagus, squash, prunes, plums and grapefruit. It is best to avoid cooking with foods that are relatively low in phenylalanine, approximately 0.3 g per 100 g, whenever possible because if you eat them in excess, you could be consuming a dangerous amount. Your doctor can advise you on how much of these foods you can safely consume.

Step 3

Replace all high-protein foods to prevent complications of phenylketonuria. Pregnant women and phenylketonuria sufferers should not eat dairy items, soybeans, beans, beef, fish, chocolate, peas or nuts. Instead, cook protein-alternative items that are filling such as mushrooms, pumpkins and potatoes. You can use a large portabella mushroom as a beef patty alternative, while still giving a meaty texture. You can mash pumpkins and potatoes together for a side dish and use fresh herbs to provide extra flavor.

Step 4

Wrap sandwich items such as tomatoes, onions, alfalfa sprouts and mayonnaise-free potato salad in a leafy vegetable, such as romaine lettuce. You can still use bread but if you've reached your limit for the day, use a leafy alternative.

Step 5

Eliminate the artificial sweetener aspartame from your cooking and baking. Aspartame is not suitable for phenylketonuria sufferers as it contains phenylalanine.

Step 6

Bake without eggs by substituting with applesauce, pureed bananas or mashed strawberries. You can replace one large egg with about ¼ cup of these items.

Tips and Warnings

Pregnant women may be tested for the phenylketonuria recessive gene for early detection and treatment.
Due to the severity of this condition, you should always consult your doctor before removing or adding any items to your diet.