How to Live With PKU

The genetic disorder phenylketonuria, commonly called PKU, requires patients to severely limit intake of the amino acid phenylalanine, found mainly in protein. A person can live with PKU and not suffer from any of its complications if a special diet is implemented during infancy and followed for life.

Instructions

Difficulty: Moderately Challenging

Manage Childhood PKU

1. Understand that dietary restrictions are the most common and most effective method of helping PKU-positive people live with the disorder. While gene therapy treatments are in development, they will not be fit for medical application to human patients for quite some time.
2. Help your child by emphasizing togetherness, hobbies and activities rather than food, especially on occasions like Thanksgiving, when protein-rich turkeys make non-PKU sufferers' mouths water.
3. Ensure your child's school has complete and total awareness of the necessary dietary restrictions. While a doctor or dietitian makes a better source for specifics, in general, PKU patients must severely limit or eliminate protein-rich foods from their diet. These include meat, fish, eggs, nuts, legumes and dairy. The sugar substitute aspartame also creates phenylalanine when digested and must be avoided.
4. Allow your child to manage her own diet at the earliest possible age. This will help him feel more in control of his own PKU and will make coping easier.

Manage PKU Through Adulthood

5. Continue to follow a strict PKU diet into adulthood. While doctors at one time told PKU patients that the diet could be discontinued following adolescence, there is considerable disagreement within the modern medical community as to the wisdom of such advice. Err on the side of caution and stick with the diet.
6. Enlist the help of a dietitian to assist you as you live with PKU. A dietitian can help plan suitable menus for special occasions and can give you pointers on strategies for eating in restaurants. Usually, a phenylalanine "points" system is used that tracks the amount of daily phenylalanine intake. Adults must usually limit their intake to 400 to 500 mg per day.
7. Choose an insurance provider that will help shoulder the expense of protein-free foods and formula. Your doctor or dietitian will be able to suggest companies that offer such coverage.

Tips & Warnings

• Failure to detect or treat PKU in infancy will almost certainly result in the mental retardation of the child. A simple test administered shortly after birth can tell whether or not your baby is positive for PKU. Negative consequences of the disorder will begin to manifest at age two to three months, so early detection is of crucial importance.

Who Can Help: