OBJECTIVE 1: Provide family with understanding of PKU and skills for effective PKU management.

Objective

Methods

Materials

Parents will be able to explain what PKU is and how PKU affects their infant.

Discuss PKU diagnosis and management during initial clinic visit.

• Living with PKU video
• New Parents’ Guide to PKU*
• A Baby sitter’s Guide to PKU*

OBJECTIVE 2: Establish and maintain an appropriate formula prescription and food pattern.

Parents will be able to accurately mix and properly store formula.

Demonstrate proper formula mixing (weigh powder, add water to specified volume) and storage during initial clinic visit.

Emphasize importance of weighing formula:

• Weighing is more accurate, especially important when metabolic balance is goal

• Gram scale
• Formula
• Printed formula mixing instructions

Parents will provide infant with prescribed formula and record formula intake

Discuss infant’s intake and family’s formula preparation methods during clinic visits and telephone consultations.

Review food records with family.

• Food record sheets

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

Serum phenylalanine and tyrosine will be measured as prescribed:

• Monthly blood draw when serum phe levels are between 1-6 mg/dL and tyr levels are between 0.8-1.8 mg/dL
  
• Weekly blood draw when serum phe levels are <1 or >6 mg/dL

Provide family with resources to obtain serum phenylalanine level:

• Standing order at local clinic/hospital or with PMD
• Home visit from public health nurse

• Filter papers
• Lancets
• Monitoring Blood Phenylalanine Levels at Home*
• Article: Tips for dealing with blood draws at different ages, National PKU News 1991;3:1:4-5

Parents will track serum phenylalanine levels and make prescribed changes to formula.

Report serum phenylalanine levels to family within 24 hours of report from lab.

As needed:

• Make adjustments to formula prescription
• Request for follow-up blood draw

• Chart (notebook) for family to track serum phe levels and growth
• Follow-up note or postcard to family documenting phe level, changes to formula prescription.

OBJECTIVE 4: Assure proper growth and development.

Infant will grow appropriately.

Measure weight, length, and head circumference at initial clinic visit and follow-up visits. Plot growth on appropriate CDC charts.

Evaluate estimated energy and protein intake.

• CDC growth charts
• Food Records