General Objectives of Treatment of PKU
Objective 1: Provide family and child with understanding of PKU and skills for effective self-management
- Provide family with information about diagnosis and management
- Serve as a resource when family has questions about PKU
- 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
- Work with the family to develop a realistic food and formula pattern that fits the guidelines for PKU management
- Provide the family with information about normal nutrition guidelines, incorporating guidelines for PKU management
- 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
- Evaluate the food and formula pattern; estimate the amount of phenylalanine in the food pattern.
- Monitor response of serum phenylalanine levels to changes in the food and formula pattern.
- 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
- Monitor weight, height or length, weight for height or length (or BMI). Compare to CDC percentiles and to child’s usual growth pattern.
- Reassess protein, energy, and vitamin and mineral needs annually, using growth data and established recommendations (DRIs).
- Adjust recommendations for intake as appropriate.
- Arrange for psychometric testing.
- If psychometric testing indicates need for further assessment or additional services, provide family with appropriate referrals.