Sepiapterin Powder for Solution

What is this medication?

SEPIAPTERIN (SE pi AP ter in) treats high phenylalanine levels in people with phenylketonuria (PKU). It works by helping your body break down phenylalanine. This prevents it from building up in the blood.

This medicine may be used for other purposes; ask your health care provider or pharmacist if you have questions.

COMMON BRAND NAME(S): Sephience

What should I tell my care team before I take this medication?

They need to know if you have any of these conditions:

• An unusual or allergic reaction to sepiapterin, other medications, foods, dyes, or preservatives
• Pregnant or trying to get pregnant
• Breastfeeding

How should I use this medication?

Take this medication by mouth. Take it as directed on the prescription label at the same time every day. You will mix this medication with liquid or soft foods (see below). You should eat food after you take this medication. Keep taking this medication unless your care team tells you to stop.

This medication comes in 250 mg or 1,000 mg packets. Check to make sure you have the correct number of packets and the packet strength(s) for your dose. Open the packets needed for your dose. Empty the powder into a small container.

Doses less than 1,000 mg: Talk to your care team about the amount of liquid you need to mix your dose and the amount of mixture your dose will be. Mix the powder with water or apple juice as directed by your care team. They will tell you how much liquid you need to mix your dose. It is important to follow their directions, so you take the right dose. Stir the mixture for at least 30 seconds. Use a specially marked oral syringe to measure your dose. Ask your pharmacist if you do not have one. Household spoons are not accurate. Your care team will tell you how many milliliters (mL) you need for your dose. The amount of mixture you take for your dose may be less than the amount you mixed; there may be medication left in the container used for mixing. Take your dose right away after it is drawn up into the syringe. If there are still particles in the syringe, rinse it with water or juice. Consume that mixture in the syringe to make sure you have taken your entire dose. Get rid of any mixture remaining in the container used for mixing.

Doses 1,000 mg or more (full packets): Mix the powder with water, apple juice, strawberry jam, or applesauce as directed by your care team. They will tell you how much liquid or soft food you need to mix your dose. Stir the mixture for 30 seconds (liquids) or 60 seconds (soft foods). Take your dose right away. If there are still particles in the container, rinse it with water or juice. Consume that mixture to make sure you have taken your entire dose.

If you do not consume the mixture right away, you may cover and store it at room temperature or in the refrigerator. Get rid of any unused mixture after 6 hours if stored at room temperature or 24 hours if refrigerated.

This medication comes with INSTRUCTIONS FOR USE. Ask your pharmacist for directions on how to use this medication. Read the information carefully. Talk to your pharmacist or care team if you have questions.

Talk to your care team about the use of this medication in children. While it may be prescribed for children as young as 1 month for selected conditions, precautions do apply.

Overdosage: If you think you have taken too much of this medicine contact a poison control center or emergency room at once.

NOTE: This medicine is only for you. Do not share this medicine with others.

What if I miss a dose?

If you miss a dose, take it as soon as you can. If it is almost time for your next dose, take only that dose. Do not take 2 doses on the same day. Do not take double or extra doses.

What may interact with this medication?

• Levodopa
• Methotrexate
• Pemetrexed
• Sildenafil
• Sulfamethoxazole
• Sulfasalazine
• Tadalafil
• Trimethoprim
• Vardenafil

This medication may affect how other medications work, and other medications may affect the way this medication works. Talk with your care team about all the medications you take. They may suggest changes to your treatment plan to lower the risk of side effects and to make sure your medications work as intended.

This list may not describe all possible interactions. Give your health care provider a list of all the medicines, herbs, non-prescription drugs, or dietary supplements you use. Also tell them if you smoke, drink alcohol, or use illegal drugs. Some items may interact with your medicine.

What should I watch for while using this medication?

Visit your care team for regular checks on your progress. Tell your care team if your symptoms do not start to get better or if they get worse.

You may need blood work done while you are taking this medication.

What side effects may I notice from receiving this medication?

Side effects that you should report to your care team as soon as possible:

• Allergic reactions—skin rash, itching, hives, swelling of the face, lips, tongue, or throat
• Bleeding—bloody or black, tar-like stools, vomiting blood or brown material that looks like coffee grounds, red or dark brown urine, small red or purple spots on skin, unusual bruising or bleeding

Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):

• Dark yellow or orange stools
• Diarrhea
• Headache
• Sore throat
• Stomach pain

This list may not describe all possible side effects. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Where should I keep my medication?

Keep out of the reach of children and pets.

Store at room temperature between 20 and 25 degrees C (68 and 77 degrees F). Get rid of any unused medication after the expiration date.

To get rid of medications that are no longer needed or have expired:

• Take the medication to a take-back program. Check with your pharmacy or law enforcement to find a location.
• If you cannot return the medication, check the label or package insert to see if the medication should be thrown out in the garbage or flushed down the toilet. If you are not sure, ask your care team. If it is safe to put it in the trash, empty the medication out of the container. Mix it with cat litter, dirt, coffee grounds, or another unwanted substance. Seal the mixture in a bag or container. Put it in the trash.

NOTE: This sheet is a summary. It may not cover all possible information. If you have questions about this medicine, talk to your doctor, pharmacist, or health care provider.