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Overview: Sephience is used to treat phenylketonuria in certain people. Common side effects include headache, changes in your poop color, stomach pain, and diarrhea. Talk with your health care provider about your diet, and do not change your diet without talking with your health care provider. Keep all appointments to have your blood checked.

Uses

What is Sephience used for?

Sephience (sepiapterin) is commonly used to treat phenylketonuria in certain people.

Sephience may also be used for other conditions as determined by your health care provider.

How does Sephience work (mechanism of action)?

Phenylketonuria causes high levels of a protein-building block called phenylalanine. People with phenylketonuria have problems with a specific protein (enzyme) that breaks down phenylalanine. Sephience works to activate the enzyme that breaks down phenylalanine, which helps lower phenylalanine levels. 

How is Sephience supplied (dosage forms)?

Brands and other names

  • Sephience

Dosage forms and strengths

  • 250 mg powder per packet
  • 1000 mg powder per packet

How should I store Sephience?

Unopened Sephience packets should be stored at room temperature, between 68 F and 77 F (20 C and 25 C). It can be exposed to temperatures between 59 F and 86 F (15 C and 30 C) for shorter periods of time, such as when transporting it. Store in a cool, dry place.

If you have mixed Sephience with a liquid or soft food, and have not taken the mixture right away, it can be stored for up to six hours at room temperature, between 68 F and 77 F (20 C and 25 C) or for up to 24 hours in the refrigerator, between 36 F and 46 F (2 C and 8 C). Cover the mixture before storing it. If you plan to take the mixture after it has been stored, stir the mixture for at least 30 to 60 seconds before taking it.

If the mixture is not taken within six hours at room temperature or within 24 hours in the refrigerator, throw it away.

Side Effects

What are the most common side effects of Sephience?

The most common side effects of Sephience are listed below. Tell your health care provider if you have any of these side effects that bother you.

  • Diarrhea
  • Headache
  • Stomach pain
  • Low phenylalanine levels (see below)
  • Yellow or orange colored poop
  • Sore throat

There may be other side effects of Sephience that are not listed here. Contact your health care provider if you think you are having a side effect of a medicine. In the U.S., you can report side effects to the FDA at www.fda.gov/medwatch or by calling 800-FDA-1088 (800-332-1088). In Canada, you can report side effects to Health Canada at www.health.gc.ca/medeffect or by calling 866-234-2345.

What are the serious side effects of Sephience?

While less common, the most serious side effects of Sephience are described below, along with what to do if they happen.

Severe Allergic Reactions. Sephience may cause allergic reactions, which can be serious. Stop taking Sephience and get help right away if you have any of the following symptoms of a serious allergic reaction.

  • Breathing problems or wheezing
  • Racing heart
  • Fever or general ill feeling
  • Swollen lymph nodes
  • Swelling of the face, lips, mouth, tongue, or throat
  • Trouble swallowing or throat tightness
  • Itching or skin rash
  • Bumps on the skin called hives that can be red, pink, white, or brown depending on your skin tone
  • Nausea or vomiting
  • Dizziness, feeling lightheaded, or fainting
  • Stomach cramps
  • Joint pain

Bleeding. Sephience can increase your risk of bleeding. This can include bleeding under the skin, which can look like a bruise (hematoma), heavy bleeding during your period, or bleeding that takes longer time to stop. Some people with serious bleeding may need a blood transfusion or surgery. Call your health care provider right away if you have any of the following signs of severe bleeding while taking Sephience.

  • Pain, swelling, or discoloration of your skin (bruising)
  • Heavy periods or periods that last longer than normal
  • Pink, red, or brown urine (pee)
  • Vomit with blood that looks like coffee grounds
  • Red or black stools (looks like tar)
  • Coughing up blood or blood clots
  • Bleeding longer than usual or that does not stop

Low Phenylalanine Levels. Sephience may cause the amount of phenylalanine in your body to get too low. Keep all appointments to have your blood checked. Your health care provider will check your blood work to help keep your phenylalanine levels at a normal level.

Warnings & Precautions

Who should not use Sephience?

Allergies to Ingredients. People who are allergic to any of the following should not take Sephience.

  • Sepiapterin
  • Sephience
  • Any of the ingredients in the specific product dispensed

Your pharmacist can tell you all of the ingredients in Sephience.

What should I know about Sephience before using it?

Do not take Sephience unless it has been prescribed to you by a health care provider. Take it as prescribed. See the “Instructions for Use” that comes with this medicine. This document tells you how to prepare, take, or give a dose of Sephience.

Do not share Sephience with other people, even if they have the same condition as you. It may harm them.

Keep Sephience out of the reach of children. If you are storing the medicine mixture in the refrigerator, take special precautions to keep it away from children who also use the refrigerator.

If you mix Sephience in liquid, use an accurate measuring device to measure your dose. A household spoon is not an accurate measuring device and may cause you to take the wrong dose. Ask your pharmacist what measuring device to use.

Keep all appointments to have your blood checked while taking Sephience. Your health care provider will need to do blood tests to check your phenylalanine levels. This can help your health care provider determine if the medicine is working and decide if your Sephience dose needs to be changed. 

Sephience should be taken with a meal, preferably at the same time each day. Eat or give more food after taking or giving a dose. Talk with your health care provider about protein and phenylalanine in your diet. Talk with your health care provider if you want to change your diet. Phenylalanine levels that are either too high or too low can be dangerous.

What should I tell my health care provider before using Sephience?

Tell your health care provider about all of your health conditions and any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using. This will help them know if Sephience is right for you.

In particular, make sure that you discuss any of the following.

Current and Past Health Conditions. Tell your health care provider if you have any of the following.

  • Bleeding problems
  • Heavy periods

Other Medicines and Supplements. Sephience may interact with other medicines and supplements. Before taking Sephience, tell your health care provider about any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using. See the Interactions section for more details.

Pregnancy. It is not known if or how Sephience could affect pregnancy or harm an unborn baby. Tell your health care provider if you are pregnant or plan to become pregnant. Control of phenylalanine levels is needed during pregnancy. Your health care provider will let you know if you should take Sephience while you are pregnant or trying to get pregnant.

Breastfeeding. It is not known if Sephience passes into breast milk. Tell your health care provider if you are breastfeeding or plan to breastfeed. Your health care provider will let you know if you should take Sephience while breastfeeding.

How can I get more information about Sephience?

For more information about Sephience, you can visit the drugmaker’s website at www.sephience.com.

Interactions

Does Sephience interact with foods or drinks?

Sephience should be taken with food. 

It is unknown if drinking alcohol will affect Sephience, but both Sephience and alcohol can increase your risk of bleeding. It is best to limit the amount of alcohol you drink.

Does Sephience interact with other medicines (drug interactions)?

Always tell your health care provider about any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using.

In particular, make sure that you discuss if you are using any of the following before taking Sephience.

  • Trimethoprim or sulfamethoxazole/trimethoprim, which is an antibiotic used to treat certain infections
  • Methotrexate (Trexall and others), which is a medicine commonly used for rheumatoid arthritis or certain types of cancer
  • Pemetrexed (Alimta, Pemfexy) or pralatrexate (Folotyn), which is a medicine to treat certain cancers
  • Sulfasalazine (Azulfidine), which is a medicine used for certain inflammatory conditions, such as ulcerative colitis or rheumatoid arthritis
  • Levodopa or a medicine that contains levodopa, which is a medicine for Parkinson’s disease
  • A phosphodiesterase-5 (PDE-5) inhibitor, such as avanafil (Stendra), sildenafil (Viagra, Revatio, and others), tadalafil (Cialis and others), or vardenafil (Levitra, Staxyn), which is a medicine used for erectile dysfunction or pulmonary arterial hypertension (PAH)
  • Another medicine used to lower phenylalanine levels, such as sapropterin (Kuvan)

This may not be a complete list of medicines that can interact with Sephience. Always check with your health care provider.

Does interact with other drugs you are taking?

Enter your medication into the WebMD interaction checker

Overdose/Missed Dose

What should I do if I accidentally use too much Sephience?

If you or someone else has used too much Sephience, get medical help right away, call 911, or contact a Poison Control center at 800-222-1222.

What should I do if I miss a dose of Sephience?

If you miss a dose, take it as soon as you remember. If it is almost time for your next dose, skip the missed dose and only take the next dose. Do not take two doses in the same day.