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All about PKU
Phenylketonuria (PKU) is a rare, inherited condition that prevents your body from metabolizing phenylalanine (Phe), which is one of the amino acids that are joined together to form proteins. A person with PKU does not have the enzyme needed to change Phe into another amino acid, tyrosine.
As a result, when the infant or adult is untreated or poorly treated, Phe rises to high levels in the blood and can damage the brain by causing mental retardation, seizures, learning disabilities and emotional problems. According to the March of Dimes, about one in every 14,000 babies born in the United States has PKU.
PKU is passed through families and people who have PKU are born with it. Both parents need to have a PKU gene to inherit the condition. If you inherit only one gene of PKU that means you are a carrier, you have no symptoms and you may not even know that you are a carrier.
Controlling PKU through diet by avoiding most forms of protein keeps Phe levels low and allows you to be healthier. Phe is found in all protein-containing foods, such as meat, eggs, dairy, and nuts. It is in many other foods, which are not generally thought of as containing protein like pasta and bread, and some fruit, such as oranges and cherries.
However, a diet without any protein would not allow you to grow and develop normally. So to control PKU and receive proper nutrition, you must also take a special formula that contains all the necessary amino acids found in proteins, except Phe. This diet must be followed your entire life in order for you to be as healthy, both mentally and physically, as possible.
It is important to understand that an individual cannot cause or prevent having PKU. We have no control over the genes that are in our body, or that are inherited from our parents, so be proud of who you are … including your genes!