Formula for people with PKU
Because of the restricted diet, people with PKU need to get essential nutrients through a special nutritional supplement. The phenylalanine-free formula provides protein and other essential nutrients in a form that's safe for people with PKU.
Your doctor and dietitian can help you find the right type of formula.
- Formula for babies and toddlers: Because regular infant formula and breast milk contain phenylalanine, babies with PKU instead need to consume a phenylalanine-free infant formula. A dietitian can carefully calculate the amount of breast milk or regular formula to be added to the phenylalanine-free formula. Parents introduce solids foods with low levels of phenylalanine to children with PKU on the same schedule used for other infants.
- Formula for older children and adults: Older children and adults continue to drink or eat a protein substitute formula daily, as directed by a doctor or dietitian. Your daily dose of formula is divided between your meals and snacks, instead of consumed all at once. The formula for older children and adults is not the same as the one used for infants, but it works on the same principle. It provides essential protein (amino acids) without phenylalanine and is continued for life.
The need for a nutritional supplement, especially if your child does not find it appealing, and the limited food choices can make the PKU diet challenging. But families need to make a firm commitment to this lifestyle change because it's the only way to prevent the serious health problems that people with PKU can develop.
Neutral amino acid therapy
Another possible addition to the PKU diet is a supplement called neutral amino acid therapy in powder or tablet form. This supplement may block some absorption of phenylalanine. This may be treatment options for adults with PKU. Ask your doctor or dietitian if this supplement is appropriate for your diet.
Pku medication
- The Food and Drug Administration (FDA) has approved the drug sapropterin (Kuran) for the treatment of PKU. It works by increasing your tolerance to phenylalanine. The drug is for use in combination with a PKU diet. But it does not work for everyone with PKU.
- In approving sapropterin, the FDA directed that studies continue because there are no long-term studies on the drugs efficacy and long term safety.
Comments
Post a Comment