General Guidelines - Treatment Protocol - For Health Care Providers - PKU Clinic - University of Washington, Seattle

Skip to main content.

Treatment GuiDelines: Toddlers

Objective 1: Provide family with understanding of PKU and skills for effective PKU management
Objective	Methods	Materials
Parents will become familiar with the self management timeline and identify tasks during 2-3 years of age .	Discuss the self-management timeline. Continue to discuss PKU diagnosis and management during clinic visits.	Self-Management Timeline Curriculum materials
Child will be introduced to the concept of "yes" and "no" foods at meals, in the grocery store, etc. Family, including older siblings, will talk about "yes" and "no" foods.	Discuss the concept of "yes" and "no" foods with parents and child at clinic visit and follow-up phone calls	Games That Teach Chef Lo-Phe's Phenominal Cookbook
Child will be asked to identify "yes" and "no" foods.		Food models, pictures of foods
Objective 2: Establish and maintain an appropriate formula prescription and food pattern
Parents will continue to provide child with prescribed formula and food pattern and to record food and formula intake.	Discuss child's intake and family's formula preparation methods during clinic visits and telephone consultations.	Food record sheets Low Protein Foods List by VE Schuett
Parents will emphasize "formula first" and communicate that formula intake is not negotiable.	Review food records with family. Address issues around formula acceptance: taste, flavor, when to change. Emphasize importance of weighing formula: allows child to participate in own management.	Article: Living with PKU--dealing with "formula battles", National PKU News 1993; 5:2:4-5
Parents will understand typical toddler eating behaviors and will understand family and pper influences on food habits. Family will include child in clude child in cooking experiences.	Discuss typical toddler eating behaviors: food "strikes" or "jags" changes in food preferences and appetite refusal of food indicates increasing independence Recommend books about parenting practices	Child of Mine and How to Get Your Kid to Eat by Ellyn Satter. Chef Lophe's Phe-Nominal Cookbook
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
Family will track serum phenylalanine levels and make prescribed changes to formula and food pattern.	Report serum phenylalanine levels to child and 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
Child will grow appropriately.	Measure weight, height and head circumference at initial clinic visit and follow-up visits. Plot growth on appropriate CDC charts. Evaluate estimated energy and protein intake. Evaluate estimated micronutrient intake annually.	CDC growth charts Food Records
Child will receive psychometric testing by 3 years of age.	Arrange for administration of tests, according to neuropsychological testing guidelines.	Psychologist Birth to 3 Center referral, if needed Neuropsychological Testing FAQ