Dosing That Fits Into Your Day, Every Day
Take SEPHIENCE once a day with food.
Take SEPHIENCE once a day with food.
It’s recommended to take it at the same time each day. Additional food intake is required after dosing.
During treatment with SEPHIENCE, you or your child will have regular check-ups with your healthcare provider.
- Your healthcare provider will do blood tests before and during treatment to check blood Phe levels.
- They may change your dose, temporarily stop, or permanently stop treatment if certain side effects occur.
- They may also change your diet. Follow their instructions and don’t make dietary changes without talking to them.
Follow these steps to take SEPHIENCE
Prepare
Mix the correct number of packets with water, apple juice, or soft food (only for doses ≥1000 mg), such as applesauce or strawberry jam.
These mixing options were studied in clinical trials. For additional mixing options, please check with your healthcare provider.
Mix until combined
- Stir liquids for at least 30 seconds
- Stir soft foods for at least 60 seconds
- Oral powder is not expected to dissolve completely. This is normal
Use as directed
- Drink or swallow the full amount
- For doses <1000 mg: Use an oral syringe to draw up the prescribed dose and administer
Rinse & finish
- Rinse the cup or syringe with water or juice
- Swallow the rinse until clean to make sure you get the full dose
If you need to store it
- If not taken right away, cover and store the mix:
- In the refrigerator (36°F to 46°F / 2°C to 8°C) for up to 24 hours
- At room temperature (68°F to 77°F / 20°C to 25°C) for up to 6 hours
- Stir again before using (see step 2) for at least 30 to 60 seconds
How to Mix SEPHIENCE
Comes in 250 mg and 1000 mg powder packets
Mix with water or apple juice
For doses 1000 mg or higher, you can also mix with soft foods, like applesauce or strawberry jam
May cause staining when in contact with certain surfaces. To minimize the risk, use as directed.
SEPHIENCE Dosing Calculator
Patient Age:
Months
Patient Weight:
kg
Total Dose Per Day (mg)
mg
Administered Dose Volume Per Day:
ml
Total Daily SEPHIENCE:
mg
packet x
This dosing calculator is designed to help you or someone you care for understand how a dose is calculated.
It’s for informational purposes only and doesn’t replace medical advice. Your healthcare provider will determine the correct dose based on individual needs.
Your Step-by-Step Guide on Taking SEPHIENCE
Watch this video or read the Instructions for Use on page 18 of the Full Prescribing Information.
View Video Transcript
Welcome to the Dosing and Administration video for SEPHIENCE.
This video will walk you through the steps on how to prepare and administer your prescribed SEPHIENCE daily dose.
Please watch this video in its entirety, and see Important Safety Information at the end of this video. For the full Prescribing Information, visit www.SEPHIENCE.com/PI.
SEPHIENCE is a prescription medicine used to lower blood levels of phenylalanine, or Phe, in adults and children 1 month of age and older with a certain type of Phenylketonuria, or PKU. SEPHIENCE is used along with a Phe-restricted diet.
It is not known if SEPHIENCE is safe and effective in children younger than 1 month of age.
Now, let’s get started by gathering everything you will need.
SEPHIENCE is supplied as a yellow-to-orange oral powder in 250 milligrams or 1000 milligrams unit-dose packets. It is taken once daily with food. A high-fat meal increases exposure to the active ingredient.
Your doctor will determine the appropriate dose based on you or your loved one’s age and weight. Because SEPHIENCE dosing is based on body weight, dose modifications are essential to maintain effectiveness as you grow.
Before you start, ensure you have your SEPHIENCE packets, a cup, a container for mixing, and either water, apple juice, or soft food—depending on your prescribed dose, and something to mix with.
For doses less than 1000 milligrams, you’ll need water or apple juice and an oral syringe. You’ll also need a small spoon.
For doses of 1000 milligrams or more, SEPHIENCE can be mixed with water, apple juice, or a soft food like applesauce or strawberry jam. You’ll also need a tablespoon to measure the correct amount.
Now, let’s go over how to prepare SEPHIENCE.
First, remove the SEPHIENCE packets needed for the prescribed dose and set them aside for opening.
Next, open the packets of oral powder by carefully tearing or cutting the top.
SEPHIENCE may cause staining if it comes into contact with certain surfaces. To minimize the risk, use as directed and prepare with care.
Empty all the powder in the packets into a cup.
For doses less than 1000 milligrams, add 9 milliliters of mixing liquid for each 250 milligram packet. For 1000 milligram packets, mix with 36 milliliters.
For doses 1000 milligrams or greater, you may mix each 1000 milligram packet with 30 milliliters of water or apple juice and then add an additional quantity of 30 milliliters for up to three 250 milligram packets.
Or for soft foods, you may mix each 1000 milligram packet with 2 tablespoons of soft food, like applesauce or strawberry jam, then add an additional quantity of 2 tablespoons for up to three 250 milligram packets. Please refer to the Instructions for Use for more information on the exact volume required to mix.
Slowly add the liquid or soft food to the container for mixing that contains the SEPHIENCE powder.
Mix for at least 30 seconds if using a liquid.
If you’re using soft food, mix together well for at least 60 seconds. In both cases, make sure the mixture is smooth and free of lumps.
SEPHIENCE is a suspension and is not expected to dissolve completely. This is normal.
Once SEPHIENCE has been mixed, the dose should be administered immediately. However, SEPHIENCE can be stored if needed—we’ll go over the proper storage instructions later in the video.
Now, let’s go over how to administer SEPHIENCE.
For doses less than 1000 milligrams, draw up the prescribed dose volume into an oral syringe and give by mouth.
For doses 1000 milligrams or greater, give the entire dose from the cup.
To make sure the full dose was given, rinse the syringe or cup with water or apple juice and administer one more time. No additional SEPHIENCE medication should be added.
Rinsing may also help prevent possible staining inside the mouth or on the teeth, making it an important part of the process.
If a dose is missed, take SEPHIENCE as soon as possible. But only one dose should be taken each day.
Next, we’ll go over how to store a prepared mixture properly.
If you can’t administer it right away, the mixture can be administered within 6 hours if stored at room temperature—between 68 to 77 degrees Fahrenheit or 20 to 25 degrees Celsius.
SEPHIENCE can also be administered within 24 hours if kept in a refrigerator between 36 to 46 degrees Fahrenheit or 2 to 8 degrees Celsius.
That’s it! If you have any questions about how to prepare or administer the SEPHIENCE dose, please reach out to your healthcare provider.
INDICATION & IMPORTANT SAFETY INFORMATION
What is SEPHIENCE?
SEPHIENCE™ (sepiapterin) is a prescription medicine used to lower blood levels of phenylalanine or Phe in adults and children 1 month of age and older with a certain type of Phenylketonuria or PKU. SEPHIENCE is used along with a Phe‑restricted diet. It is not known if SEPHIENCE is safe and effective in children younger than 1 month of age.
What is the most important information I should know about SEPHIENCE?
SEPHIENCE may cause serious side effects, including:
- increased bleeding problems. SEPHIENCE may increase your risk of bleeding. Tell your healthcare provider if you develop any symptoms of bleeding including:
- bruising or red or purple skin marks
- menstrual bleeding that is heavier than normal
- bleeding longer than usual or that does not stop
- low blood Phe or hypophenylalaninemia. Low blood Phe levels are common during treatment with SEPHIENCE and can be severe.
The most common side effects of SEPHIENCE include: diarrhea, headache, stomach or abdominal pain, yellow or orange stool or feces, and throat pain.
Tell your healthcare provider if you have any side effect that bothers you or that does not go away. These are not all the possible side effects of SEPHIENCE. Call your healthcare provider for medical advice about side effects.
Before taking or giving SEPHIENCE, tell your healthcare provider about all of your medical conditions, including if you:
- are pregnant or plan to become pregnant. It is not known if SEPHIENCE will harm your unborn baby.
- are breastfeeding or plan to breastfeed. It is not known if SEPHIENCE passes into your breast milk. Talk to your healthcare provider about the best way to feed your baby if you take SEPHIENCE.
Tell your healthcare provider about all medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements. SEPHIENCE and other medicines may affect each other causing side effects. SEPHIENCE may affect the way other medicines work, and other medicines may affect how SEPHIENCE works. Especially tell your healthcare provider if you take:
- a medicine that contains levodopa
- an antifolate medicine
- another medicine also used to lower Phe
- medicines used to treat erectile dysfunction (phosphodiesterase 5 inhibitors or PDE5)
Know the medicines you take. Keep a list of them with you to show your healthcare provider and pharmacist when you get a new medicine.
How should I take or give SEPHIENCE?
- Take or give SEPHIENCE exactly as your healthcare provider tells you. Your healthcare provider will tell you how much SEPHIENCE to take or give.
- Your healthcare provider may change your or your child’s dose of SEPHIENCE depending on your weight and age.
- Take or give SEPHIENCE 1 time each day with food. You must take or give more food after the SEPHIENCE dose.
- SEPHIENCE comes as a packet containing oral powder.
- For SEPHIENCE doses less than 1,000 mg: mix SEPHIENCE oral powder with water or apple juice before giving or taking.
- For SEPHIENCE doses 1,000 mg or greater: SEPHIENCE oral powder can be mixed with water, apple juice, strawberry jam, or applesauce before taking or giving.
- See the detailed Instructions for Use that comes with SEPHIENCE for information about the correct way to prepare, take, or give a dose of SEPHIENCE oral powder.
- If you or your child miss a dose of SEPHIENCE, take or give it as soon as possible. Do not take 2 doses in a day. Continue with your normal dosing schedule the following day.
- During treatment with SEPHIENCE, you or your child will have regular check-ups with your healthcare provider.
- Your healthcare provider will do blood tests before and during your treatment with SEPHIENCE to check your blood Phe levels.
- Your healthcare provider may change your dose, temporarily stop, or permanently stop treatment with SEPHIENCE if you have certain side effects.
- Your healthcare provider may change your diet during treatment. Follow your healthcare provider’s instructions and do not make any changes to your diet without first talking to your healthcare provider.
To report an adverse event, please call 1‑866‑562‑4620 or email at usmedinfo@ptcbio.com. You may also report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.
Visit www.SEPHIENCE.com/PI for the full Prescribing Information.
If you need support through treatment, PTC Cares is here to help. Case Managers guide families through insurance, medication access, and more.
Think of your Case Manager as your personal guide. Join PTC Cares— treatment support for people and families living with PKU. To get started, visit PTCCares.com/econsent-pku.
Need extra support with SEPHIENCE?
Our dedicated PTC Cares™ support team are here to help. Connect with one today for personalized guidance and answers to your questions.
Always take SEPHIENCE as your healthcare provider prescribes. For more information, contact your PTC Cares™ Case Managers.
