healthy living
PKU (Phenylketonuria)
KAISER PERMANENTE
Please do not feed the
child with PKU any food
not allowed or approved
by the parents. Even
“little tastes” add up and
result in an elevation of
PHE in their blood. While
the PKU child’s curiosity
may be aroused by these
foods, he is accustomed
to doing without them.
Regional Metabolic Clinic/Oakland
A Teacher’s Guide to
How can teachers help?
PKU Basics
Phenylketonuria (PKU) is an Treat the child with PKU as a
inherited disorder of protein normal, healthy member of the
metabolism. Children with PKU class. Remember, he or she is
do not have a functioning no different from anyone else in
enzyme to metabolize or break terms of social, emotional,
down an amino acid called physical, and academic growth
phenylalanine, which is found in and development.
all food proteins.
Keep an open line of
Protein in foods is important for
communication with the child’s
building and repairing the body’s
parents to reinforce parent
tissues. All children need a
teaching and monitoring of food
certain amount of PHE for
eaten away from home. The
normal growth and tissue repair.
child’s parents are your closest
Because a child with PKU lacks
experts on PKU.
the enzyme which breaks down
the extra PHE, the extra PHE
Let the Parents know:
builds up in the body tissues
including the blood. This extra
…if the child has eaten any food
PHE can prevent normal brain not allowed.
development and result in
mental retardation.
…if the child does not eat foods
The good part is that nearly all that are sent from home.
states, including California, -
routinely check a newborn’s …if special occasions, such as
blood during the first week of life birthday or holiday parties, are
to see if the infant has PKU. The planned to make sure there is a
infant with PKU can then be put low PHE food for the child.
on a carefully controlled diet Parents may want to send a
which allows enough PHE for supply of low PHE treats that
growth but prevents the excess store well and can be available.
PHE which can interfere with
normal brain development. With
proper dietary control initiated
early in life, normal physical and
intellectual development can
proceed in a child with PKU.
ADA Review July 2018
 healthy living A Teacher’s Guide to
PKU (Phenylketonuria)
KAISER PERMANENTE
The PKU Diet
Since PHE is found in all foods that contain protein, PKU children must follow a strict low-protein diet. Foods which
contain high amount of protein are high in PHE and should not be eaten. Foods with small of amounts of protein
and PHE can be eaten only in controlled amounts. Some foods are “free” foods because they contain no protein
and are free of PHE. They are eaten to help boost the child’s intake of calories needed for energy.
A child with PKU has a special drink which has most of the protein, vitamins and minerals that a child needs for
growth with little or no PHE. The special drink provides almost all of the nutrients that other children get from their
food. This drink has a taste and smell that may seem objectionable to someone not used to it, but PKU children
have grown up with it and acquire the taste for it at an early age.
The amount of drink and food the child consumes in a day is carefully calculated by the child’s family and dietitian.
All foods must be carefully measured out to control the amount of PHE the child eats. The amounts are adjusted to
the child’s changing needs as he or she grows. A child with PKU learns at an early age that his or her diet is
restricted and to ask a parent if a new food is allowed. By school age, children with PKU know a good deal about
their own diet.

Parents have complete, detailed lists of foods to use in feeding their child with PKU.
Here are some examples:
NOT ALLOWED: ALLOWED BUT FREE FOODS
CONTROLLED
Meat Soda, Kool-Aid, Lemonade
Fruits
Fish Popsicles
Fruit and Vegetable juices
Chicken Jelly
Vegetables
Turkey Gum drops, suckers, hard
Breads candy
Milk
Cereals
Cheese
Crackers
Ice Cream
Potato chips
Yogurt
Popcorn Additional resources
Eggs
Special Low Protein Foods- Contact the Kaiser Permanente
Beans Regional Metabolic Clinic at:
some of these foods may be
Nuts sent to school as part of the (510) 752-5101
Peanut Butter child’s meal or snack. These
foods are important as they For additional information visit:
NutraSweet, Aspartame, Equal allow greater variety in the diet Texas Department of Health
without providing too much A Teacher’s Guide to PKU
PHE.

This information is not intended to diagnose health problems or to take the place of medical advice or care you receive from your physician
or other health care professional. If you have persistent health problems, or if you have additional questions, please consult with your
doctor. If you have questions or need more information about your medication, please speak to your pharmacist. Kaiser Permanente does
not endorse the medications or products mentioned. Any trade names listed are for easy identification only.

© 2015 Kaiser Permanente Regional Genetics Department. All rights reserved.

Adapted from Texas Department of Health - A Teacher’s Guide to PKU