Sepiapterin (se-pi-AP-ter-in)

Lowers phenylalanine (Phe) levels in the blood.

Brand Name(s):

Sephience

When This Medicine Should Not Be Used:

How to Use This Medicine:

Packet, Powder

• Take your medicine as directed. Your dose may need to be changed several times to find what works best for you.
• It is best to take this medicine with food or milk.
• Sephience™ doses less than 1000 milligrams (mg):
  • Mix this medicine with water or apple juice before taking or giving.
  • Wash your hands before and after using this medicine.
  • Open the packet by folding and tearing at the tear notch, or use scissors to cut at the dotted line. Empty the entire contents of the packet into a container.
  • Place the tip of the oral syringe into the container with water or apple juice. Pull back on the plunger and draw up the needed amount of water or apple juice.
  • Use 9 milliliters (mL) of water or apple juice for each 250 mg packet. Use 36 mL of water or apple juice for one 1000 mg packet. Slowly add the water or apple juice into the container. Stir the oral powder mixed with water or apple juice for 30 seconds or more with a spoon until the mixture is free of lumps.
  • Place the tip of the oral syringe into the mixture. Pull back on the plunger until the edge of the plunger lines up with the prescribed dose. Give the mixture right away.
  • Place the tip of the oral syringe into your child's mouth. Point the tip toward either cheek. Push on the plunger slowly until all the mixture is given. If there is any mixture left, draw up more water or apple juice into the syringe and give until no mixture is left.
  • Throw away any mixture left in the container.
• Sephience™ doses 1000 milligrams (mg) or more:
  • Mix this medicine with water, apple juice, strawberry jam, or applesauce before taking or giving.
  • Wash your hands before and after using this medicine.
  • Open the packet by folding and tearing at the tear notch, or use scissors to cut at the dotted line. Empty the entire contents of the packet into a container.
  • Slowly add the water, apple juice, strawberry jam, or applesauce into the container. Stir the oral powder mixed with water or apple juice for 30 seconds or more with a tablespoon until the mixture is free of lumps. If you used strawberry jam or applesauce, stir the mixture for 60 seconds or more with a tablespoon.
  • Give or take all the mixture right away. If there is any mixture left, rinse the container with at least 15 milliliters (mL) of water or apple juice. Swallow the rinse right away. Repeat until no mixture remains in the container.
  • Wash the container with warm water and air dry. Store the container for the next use.
• Read and follow the patient instructions that come with this medicine. Talk to your doctor or pharmacist if you have any questions.
• Missed dose: Take a dose as soon as you remember. If it is almost time for your next dose, wait until then and take a regular dose. Do not take extra medicine to make up for a missed dose.
• Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light. You may store the mixture for up to 6 hours at room temperature or for up to 24 hours in the refrigerator. Throw away unused mixture after 6 hours at room temperature or after 24 hours if refrigerated.
• Sephience™ mixed with water or apple juice: Stir for 30 seconds or more before taking or giving the dose.
• Sephience™ mixed with strawberry jam or applesauce: Stir for 60 seconds or more before taking or giving the dose.

Drugs and Foods to Avoid:

Ask your doctor or pharmacist before using any other medicine, including over-the-counter medicines, vitamins, and herbal products.

• Some foods and medicines can affect how sepiapterin works. Tell your doctor if you are using any of the following:
  • Curcumin, levodopa, tyrosine
  • Antifolate medicine (including methotrexate, pemetrexed, piritrexim, pralatrexate, raltitrexed, trimethoprim, trimetrexate)
  • Medicine to treat erectile dysfunction (including sildenafil, tadalafil, vardenafil)
  • Other medicine to lower phenylalanine

Warnings While Using This Medicine:

• Tell your doctor if you are pregnant or breastfeeding, or if you have bleeding problems, brain or nerve problems, or seizures.
• This medicine may cause the following problems:
  • Increased risk of bleeding
  • Hypophenylalaninemia (low blood levels of phenylalanine)
• This medicine may cause your stool to have a yellow or orange color. Talk to your doctor if you have concern about this.
• Your doctor will do lab tests at regular visits to check on the effects of this medicine. Keep all appointments.
• Keep all medicine out of the reach of children. Never share your medicine with anyone.

Possible Side Effects While Using This Medicine:

Call your doctor right away if you notice any of these side effects:

• Allergic reaction: Itching or hives, swelling in your face or hands, swelling or tingling in your mouth or throat, chest tightness, trouble breathing
• Unusual bleeding or bruising

If you notice these less serious side effects, talk with your doctor:

• Diarrhea, stomach pain, yellow or orange stool
• Headache
• Throat pain

If you notice other side effects that you think are caused by this medicine, tell your doctor

Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088