Treatment Guidelines

General Objectives of Treatment of PKU


Objective 1: Provide family and child with understanding of PKU and skills for effective self-management


  1. Provide family with information about diagnosis and management
  2. Serve as a resource when family has questions about PKU
  3. Provide education to child and family to manage PKU, according to developmental stage


Objective 2: Establish and maintain an appropriate formula prescription and food pattern


  1. Work with the family to develop a realistic food and formula pattern that fits the guidelines for PKU management
  2. Provide the family with information about normal nutrition guidelines, incorporating guidelines for PKU management
  3. Support the family when problems occur. Provide guidelines about the development of usual food behaviors.


Objective 3: Maintain serum phenylalanine levels between 1-6 mg/dL


  1. Evaluate the food and formula pattern; estimate the amount of phenylalanine in the food pattern.
  2. Monitor response of serum phenylalanine levels to changes in the food and formula pattern.
  3. In the event the serum phenylalanine levels are too high or too low, modify nutrition care plan and communicate to family.


Objective 4: Assure proper growth and development


  1. Monitor weight, height or length, weight for height or length (or BMI). Compare to CDC percentiles and to child’s usual growth pattern.
  2. Reassess protein, energy, and vitamin and mineral needs annually, using growth data and established recommendations (DRIs).
  3. Adjust recommendations for intake as appropriate.
  4. Arrange for psychometric testing.
  5. If psychometric testing indicates need for further assessment or additional services, provide family with appropriate referrals.