Sepiapterin

Sepiapterin is used along with diet to reduce blood levels of phenylalanine in adults and children who are at least one month old and have a specific type of phenylketonuria (PKU).

Sepiapterin may also be used for reasons that are not mentioned in this medication guide.

Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose. Use the medicine exactly as directed.

Sepiapterin can cause phenylalanine levels to get too low in people who use this medicine.

Sepiapterin is usually taken once per day with food. Be sure to eat more food after your dose.

Doses are based on age and weight. Your dose may change if you gain or lose weight.

Read and carefully follow the Instructions for Use provided with your medicine. Ask your doctor or pharmacist if you don't understand these instructions.

• For doses less than 1,000 mg: mix the powder with water or apple juice before taking.
• For doses of 1,000 mg or more: mix the powder with water, apple juice, strawberry jam, or applesauce before taking.
• You may save the mixture for later use at room temperature or refrigerated. Stir for 30 seconds before use (if prepared with water or apple juice) or 60 seconds (if prepared with strawberry jam or applesauce).
• Throw away the mixture if not used within 6 hours at room temperature or within 24 hours if refrigerated.

You may need to follow a special diet. Follow all instructions of your doctor or dietitian. Learn about the foods you should eat or avoid.

Your doctor will need to check your progress on a regular basis. Your blood will need to be tested before and during your treatment with sepiapterin.

You may have increased bleeding while taking sepiapterin. Tell your doctor if you notice any unusual bleeding, bruising, or if your bleeding continues or is very heavy.

Store unopened packets at room temperature away from moisture and heat.

Use only as directed. Inform your doctor if you are taking other medicines or have other medical conditions or allergies.

Tell your doctor if you have or have ever had:

• active bleeding or bleeding problems.

It is not known if sepiapterin will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

Ask a doctor if it is safe to breastfeed while using sepiapterin.

Get emergency medical help if you have signs of an allergic reaction: hives, difficulty breathing, swelling of your face, lips, tongue, or throat.

Sepiapterin may cause serious side effects. Call your doctor right away if you have:

• low blood levels of phenylalanine;
• bruising, purple or red skin spots;
• any bleeding that does not stop; or
• heavy menstrual bleeding.

Common side effects of sepiapterin may include:

• diarrhea;
• headache;
• stomach pain;
• yellow or orange stools; or
• pain in your throat.

This is not a complete list of side effects and others may happen. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Usual Adult Dose for Hyperphenylalaninemia:

Starting dosage: 60 mg/kg orally once a dayMaximum dose: 60 mg/kg/dayEvaluation Period:-Discontinuation for lack of biochemical response:---Discontinue this drug in patients whose blood phenylalanine (Phe) does not decrease after 2 weeks of therapy at the maximum dosage of 60 mg/kg/day.Use: In conjunction with a Phe-restricted diet, for the treatment of hyperphenylalaninemia (HPA) in patients with sepiapterin-responsive phenylketonuria (PKU)

Usual Pediatric Dose for Hyperphenylalaninemia:

Starting dosage:-Age less than 6 months: 7.5 mg/kg orally once a day-Age 6 months to less than 1 year: 15 mg/kg orally once a day-Age 1 to less than 2 years: 30 mg/kg orally once a day-Age 2 years and older: 60 mg/kg orally once a dayMaximum dose: 60 mg/kg/dayEvaluation Period:-Dosage titration in patients less than 2 years of age:---After initiating therapy at the starting dosage by age, blood Phe levels should be checked within 2 weeks to verify response to therapy.---If blood Phe does not decrease, this drug may be titrated incrementally based on blood Phe levels to a maximum of 60 mg/kg/day.---Existing dietary protein and Phe intake should not be modified during the evaluation period.-Discontinuation for lack of biochemical response:---Discontinue this drug in patients whose blood Phe does not decrease after 2 weeks of therapy at the maximum dosage of 60 mg/kg/day.Comments:-For calculated doses less than 1000 mg/day, the final concentration of the prepared liquid mixture is 25 mg/mL.Use: In conjunction with a Phe-restricted diet, for the treatment of HPA in patients with sepiapterin-responsive PKU

Take the medicine as soon as you remember, but skip the missed dose if it is nearly time for your next dose. Do not take two doses at once.

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

• Follow your doctor's instructions about any restrictions on food, beverages, or activity.

Tell your doctor about all your other medicines, especially:

• levodopa;
• turmeric (curcumin);
• an antifolate medicine such as methotrexate, trimethoprim, trimetrexate, pemetrexed, pralatrexate, raltitrexed, or piritrexim;
• sulfasalazine, sulfamethoxazole;
• other medicine used to lower blood levels of phenylalanine--sapropterin; or
• erectile dysfunction medicine--sildenafil, vardenafil, tadalafil, Viagra, Cialis, and others.

This list is not complete. Other drugs may affect sepiapterin, including prescription and over-the-counter medicines, vitamins, and herbal products. Not all possible drug interactions are listed here.