infants, Children, Adolescents - Treatment Protocol - For Health Care Providers - PKU Clinic - University of Washington, Seattle

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Treatment Guidelines:

Infant Age 6-12 months

Objective 1: Provide family with understanding of PKU and skills for effective PKU management
Objective	Methods	Materials
Parents will become familiar with self-management timeline and identify tasks during the 6-12 months age	Discuss the self-management timeline Continue to discuss PKU diagnosis and management during clinic visits	Self-Management Timeline Sick day protocol
Parents will develop plan for babysitter, other care providers	Discuss plan developed by parents, provide input, as appropriate	Babysitter's Guide to PKU
Objective 2: Establish and maintain an appropriate formula prescription and food pattern
Parents will continue to 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
Parents will be able to add pureed and solid foods based on energy needs and physiologic readiness Parents will record food intake Parents will introduce foods with increasing texture, as appropriate Parents will introduce finger foods, as appropriate Parents will introduce cup	Discuss introduction of solid foods with parents at clinic visit Typical progression of feeing skills Appropriate foods and portion sizes, including introduction of new foods one at a time, to observe tolerance Appropriate and inappropriate foods Use of low protein products Importance of careful monitoring of intake Calculations of phenylalanine content of foods Follow-up with family via telephone call one week after introduction of solids	Low Protein Foods List by V E Schuett Sample menus for 15, 30, and 45 mg phe from food Food record sheets Self-Management Timeline List of low protein foods and suppliers Look-Alike Foods Finger Foods are Fun
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	Continue to 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 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
If concerns exist, child will receive psychometric testing	Administer: Bayley Scales of Infant Development MacArthur Communicative Development Inventory Share results with family Provide family with necessary referrals, if needed	Psychologist Birth to 3 Center referral FAQ About Neuropsychological Testing