How is phenylketonuria managed?

How is phenylketonuria managed?

PKU is treated by cutting back on protein to limit the intake of phenylalanine. If you or your child is diagnosed with moderate to severe PKU, you will need to adjust the diet to include special formulas and meals. These changes include a diet that is low in phenylalanine, while ensuring adequate protein intake.

What are the products that can trigger phenylketonuria?

A dangerous buildup of phenylalanine can develop when a person with PKU eats protein-rich foods, such as milk, cheese, nuts or meat, and even grains such as bread and pasta, or eats aspartame, an artificial sweetener.

How do you treat PKU?

A: The main treatment for PKU is following a strict diet that eliminates high protein food such as, meat and dairy products and introduces a special formula. Additionally, your child needs to come to Children’s regularly for PKU checkups and to test his blood phenylalanine.

What causes phenylketonuria?

PKU is caused by a defect in the gene that helps create the enzyme needed to break down phenylalanine. Without the enzyme necessary to process phenylalanine, a dangerous buildup can develop when a person with PKU eats foods that contain protein or eats aspartame, an artificial sweetener.

What is the main treatment for patients with phenylketonuria Why?

Diet. The main treatment for PKU is a low-protein diet that completely avoids high-protein foods (such as meat, eggs and dairy products) and controls the intake of many other foods, such as potatoes and cereals.

How phenylketonuria is caused?

What is PKU diet?

The diet for PKU consists of a phenylalanine-free medical formula and carefully measured amounts of fruits, vegetables, bread, pasta, and cereals. Many people who follow a low phenylalanine (phe) food pattern eat special low protein breads and pastas.

What is phenylketonuria diet?

What triggers phenylketonuria?

What should you do if you have phenylketonuria?

Therefore, a person with PKU needs to work with a health care professional to develop an individualized diet. The goal is to eat only the amount of phenylalanine necessary for healthy growth and body processes but not any extra.

What foods should you avoid if you have phenylketonuria?

For the rest of their lives, people with PKU — babies, children and adults — need to follow a diet that limits phenylalanine, which is found mostly in foods that contain protein.

When to stop taking phenylalanine for PKU?

The PKU Diet. People with PKU need to follow a diet that limits foods with phenylalanine. The diet should be followed carefully and be started as soon after birth as possible. In the past, experts believed that it was safe for people to stop following the diet as they got older.

Which is the best treatment for PKU in adults?

This may be a treatment option for adults with PKU. Ask your doctor or dietitian if this supplement is appropriate for your diet. The Food and Drug Administration (FDA) has approved the drug sapropterin (Kuvan) for the treatment of PKU.