Phenylketonuria (PKU)
is a genetic disorder caused by a deficiency in phenylalanine hydroxylase (PAH) enzyme resulting in high phenylalanine (Phe) concentrations in the blood and brain.Since the 1960’s a Phe-restricted and Tyr-supplemented diet has been the primary medical therapy for people living with PKU.
This App is designed to support families and children with PKU on phenylalanine restricted diets.
What is PKU?
Phenylketonuria (PKU), is a genetic disorder caused by a deficiency in phenylalanine hydroxylase (PAH) enzyme resulting in high phenylalanine (Phe) concentrations in the blood and brain. Read more on PKU here.
Phe is one of the important essential amino acids that we need to eat from protein in food. Protein is important for growth in children and health maintenance in adults.
PAH is an enzyme that helps break down Phe to tyrosine (Tyr).
Too much Phe in the blood may result in intellectual disability, behavioral disorders and seizures.
Why a diet?
Since the 1960’s a Phe-restricted and Tyr-supplemented diet has been the primary medical therapy for people living with PKU.
The goal is to lower blood Phe levels to treatment ranges and provide enough dietary Tyr.
People living with PKU are prescribed their daily Phe (protein) restriction by their doctor or dietitian. These daily goals often need to be changed based on each person’s nutritional needs and age requirements.
Since nutrition labels on foods do not list Phe content of foods, it is very difficult for families to track daily dietary Phe intake.
We hope this App makes daily Phe counting and meal planning easier for people following low Phe diets.
For whom?
This App is designed to support families and children with PKU on phenylalanine restricted diets.
This App is not a substitute for the health care provided by your medical team!