WIC Case Study

Background:
Phenylketonuria (PKU) is a genetic mutation that causes a lack or shortage of the enzyme
that's required to process the amino acid phenylalanine (Phe). When a person with PKU eats
foods that are high in protein, such as dairy, soybeans, nuts or meat a high amount of
phenylalanine can develop and lead to serious health problems (1). When Phe accrues over time
patients may experience concentration, confusion and mood problems, as well as eczema and
other signs.
The best proven treatment for PKU patients is a phenylalanine-restricted diet
supplemented by a formula containing amino acids and other nutrients. The low-phe diet needs
two linked dietary adjustments to regulate optimal Phe levels. First, natural foods, such as animal
protein, beans and nuts, are severely restricted to limit protein intake (2).This usually includes a
limited intake of bread, pasta, rice and some vegetables. Low-protein breads and pasta products,
made from starch, are used to provide needed energy and to increase variation in a PKU patients
diet. Second, eating amino acid-based, Phe-free formula or amino acid medical food is required
to provide sufficient protein, vitamins, minerals and energy due to the constraint in other natural
foods (2).
Normal blood phenylalanine level is about 1 mg/dl. In classic PKU, levels may range
from 6 to 80 mg/dl, but are usually greater than 30 mg/dl (4). The goal of PKU management is to
maintain the blood level of phenylalanine between 2 and 10 mg/d (4). Regular phenylalanine
blood level measurement, and the supervision of a dietitian and other members of a health care
team, allow individuals and their families to work toward consistently sustaining the blood level
in the desirable range.
With proper planning, breastfeeding a child with PKU is possible. There is consensus
among European countries and the U.S. that any newborn infant with a plasma Phe concentration
of greater than 400600 M should be started on a low-phe diet as soon as possible, which is
easily achieved in infancy and compatible with limited breastfeeding (3). To begin, your baby
will need to be given a Phe-free milk formula that contains all required protein, vitamins and
minerals your child needs for growth (5). During this time youll need to pump milk to continue
the supply. Once Phe levels have reached the target level, you will be able to begin breastfeeding
along with the Phe-free formula, breastfeeding after formula has been given (5). This way your
baby will breastfeed only sparingly, but still take on all the valuable antibodies and enzymes
from your breast milk (5). It is important to be giving your baby an equal feeding of low-phe
formula and breastmilk. Mothers may want to pump their milk instead of having baby latch to
them to get a more accurate measurement on how much they are eating.

CRD 3.1 a: Assess the nutrition status of individuals, groups and populations in a variety of settings
where nutrition care is or can be delivered.
I evaluated patient A during my time at the Southeast Health District Health Department & WIC and my
observations are listed below.
Patient A:

High risk patient, diagnosed with PKU, treated by doctor at local hospital
6 months old, male
Patient is mostly breastfed by mother
Patient does receive low-phe formula, Periflex Early Years
Normal growth and normal weight
Breastfeeding infant of a woman at nutritional risk

CRD 3.1 b: Diagnose nutrition problems and create problem, etiology, signs and symptoms (PES)
statements.
1. Impaired nutrient utilization (NC-2.1) related to PKU and the infants inability to break down
Phenylalanine, thus requiring a low Phe diet; as evidenced by blood test conducted at childs
birth.
CRD 3.1 c: Plan and implement nutrition interventions to include prioritizing and nutrition diagnosis,
formulating a nutrition prescription, establishing goals and selecting and managing intervention.
1. Nutrition Education: Discussed infant nutrition and breastfeeding with patients mother. Trained
and imparted knowledge to the parent on importance of equally supplying low-phe formula and
breast milk to baby. Counsel parent on a low protein diet and use of special formulas.
2. Limit patients consumption of breastmilk to be equally equivalent to low-phe formula.
CRD 3.1 d: Monitor and evaluate problems, etiologies, signs, symptoms and the impact of interventions
on the nutrition diagnosis.
1. Encourage the mother to pump breastmilk to get accurate feeding for baby rather than having the
baby latch and overfed.
2. Monitor the knowledge gained by the parent by discussing with the parent about how many times
a day the patient is receiving low-phe formula and breast milk at the next visit.
3. Continue to visit doctor at local hospital for monitoring and evaluation of phenylalanine levels,
changes in mental health status and alertness.
CRD: 3.1 e: Complete documentation that follows professional guidelines, guidelines required by health
care systems and guidelines required by the practice setting.

References:
1. Phenylketonuria (PKU). (n.d.). Retrieved February 03, 2016, from
http://www.mayoclinic.org/diseases-conditions/phenylketonuria/basics/definition/con20026275
2. Macleod, E. L., & Ney, D. M. (2010). Nutritional Management of Phenylketonuria.
Annales Nestl (English Ed.) Ann Nestl [Engl], 68(2), 58-69.
3. Recommendations on the dietary management of phenylketonuria. Report of Medical
Research Council Working Party on Phenylketonuria. (1993). Archives of Disease in
Childhood, 68(3), 426-427.
4. PHENYLKETONURIA (PKU). (n.d.). Retrieved February 03, 2016, from
http://www.medhelp.org/lib/pku.htm
5. Breastfeeding. (n.d.). Retrieved February 03, 2016, from
http://www.pku.com/en/Living_life_to_the_full/breastfeeding/breastfeeding.html