Sapropterin is used along with a restricted diet to control blood phenylalanine levels in people who have phenylketonuria (PKU; an inborn condition in which phenylalanine may build up in the blood and causes decreased intelligence and a decreased ability to focus, remember, and organize information). Sapropterin will only work for some people who have PKU, and the only way to tell if sapropterin will help a particular patient is to give the medication for a period of time and see whether his or her phenylalanine level decreases. Sapropterin is in a class of medications called cofactors. It works by helping the body to break down phenylalanine so it will not build up in the blood.
Sapropterin comes as a tablet to dissolve in water or apple juice and take by mouth. It is usually taken once a day with food. Take sapropterin at around the same time every day. Follow the directions on your prescription label carefully, and ask your doctor or pharmacist to explain any part you do not understand. Take sapropterin exactly as directed. Do not take more or less of it or take it more often than prescribed by your doctor.
Place the number of sapropterin tablets you were told to take in a cup that contains 4 to 8 ounces (1/2 to 1 cup or 120 to 240 milliliters) of water or apple juice. Stir the mixture or crush the tablets with a spoon to dissolve the tablets. The tablets may not dissolve completely; there may still be small pieces of tablet floating at the top of the liquid. When the tablets are mostly dissolved, drink the entire mixture. If pieces of tablets remain in the cup after you drink the mixture, pour more water or apple juice into the cup and drink it to be sure you swallow all the medication. Be sure to drink the entire mixture within 15 minutes after you prepare it.
Even if sapropterin tablets are stored properly, the color of the tablets may change to light yellow over time. This is normal and it is safe to take tablets that have changed color.
Your doctor will start you on an average dose of sapropterin and will check your blood phenylalanine level regularly. If your phenylalanine level does not decrease, your doctor will increase your dose of sapropterin. If your phenylalanine level does not decrease after one month of treatment with the higher dose of sapropterin, you and your doctor will know that your condition does not respond to sapropterin. Your doctor will tell you to stop taking the medication.
Sapropterin may help to control blood phenylalanine levels, but it will not cure PKU. Continue to take sapropterin even if you feel well. Do not stop taking sapropterin without talking to your doctor.
Ask your pharmacist or doctor for a copy of the manufacturer's information for the patient.
This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.
- Kuvan®
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