Treatment Guidelines
Infants: Age 0-6 months
Objective 1: Provide family with understanding of PKU and skills for effective PKU management |
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| 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.
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Living with PKU video |
Objective 2: Establish and maintain an appropriate formula prescription and food pattern |
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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
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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 |
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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 with resources to obtain serum phenylalanine level:
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Filter papers Lancets
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 child and family within 24 hours of report from lab. As needed:
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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 |
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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. |
Food Records |
New Parent's Guide to PKU