Treatment Guidelines

Recommended Assessment Schedule

The patient with PKU should be monitored regularly depending upon age. The following monitoring guidelines have been developed for medical/nutritional management of children with PKU:

Birth to 1 Year:

  • Serum phenylalanine and tyrosine levels (including report to family and appropriate adjustment of formula) – according to schedule established by New Infant Guidelines then once per month when levels are stable
  • RD contacts (nutritional assessment, including measurement of length, weight, head circumference, and review of diet and blood levels, and counseling) –once per month.
  • Hemoglobin or hematocrit measurement – once, at 6 months of age
  • PKU Clinic visit – once per month

1 - 3 Years:

  • Serum phenylalanine and tyrosine levels (including report to family and appropriate adjustment of formula and phenylalanine from food) – once per month; mid-month levels when serum phenylalanine is less than 1 mg/dL or greater than 6 mg/dL
  • RD contacts (nutritional assessment, including measurement of length, weight, head circumference, and review of diet and blood levels, and counseling) – once per month
  • Hemoglobin or hematocrit measurement – once per year
  • Albumin/prealbumin measurement – once every 1-2 years
  • PKU Clinic visit – once per month
  • Psychometric assessment – one evaluation at age 3, according to neuropsychological testing guidelines

3 - 5 Years:

  • Serum phenylalanine and tyrosine levels (including report to family and appropriate adjustment of formula and phenylalanine from food) – once per month; mid-month levels when serum phenylalanine is less than 1 mg/dL or greater than 6 mg/dL
  • RD contacts (nutritional assessment, including measurement of height and weight, review of diet and blood levels, and counseling) – once per month
  • Hemoglobin or hematocrit measurement – once per year
  • Albumin/prealbumin measurement – once every 1-2 years
  • PKU Clinic visit – once per month
  • Psychometric assessment – one evaluation every 3 years, according to neuropsychological testing guidelines

5 Years and Older:

  • Serum phenylalanine and tyrosine levels (including report to family and appropriate adjustment of formula and phenylalanine from food) – once per month; mid-month levels when serum phenylalanine is less than 1 mg/dL or greater than 6 mg/dL
  • RD contacts (nutritional assessment, including measurement of height and weight, review of diet and blood levels, and counseling) – once per month
  • Hemoglobin or hematocrit measurement – once per year
  • Albumin/prealbumin measurement – once every 1-2 years
  • PKU Clinic visit – once per month
  • Psychometric assessment – one evaluation every 3 years, according to neuropsychological testing guidelines