Description
Sepiapterin is used to lower blood levels of phenylalanine (Phe) in patients with sepiapterin-responsive phenylketonuria (PKU). This medicine is used along with a Phe-restricted diet.
This medicine is available only with your doctor's prescription.
This product is available in the following dosage forms:
- Powder
Before Using
In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:
Allergies
Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.
Pediatric
Appropriate studies have not been performed on the relationship of age to the effects of sepiapterin in children younger than 1 month of age. Safety and efficacy have not been established.
Geriatric
Appropriate studies performed to date have not demonstrated geriatric-specific problems that would limit the usefulness of sepiapterin in the elderly.
Breastfeeding
There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.
Drug Interactions
Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. When you are taking this medicine, it is especially important that your healthcare professional know if you are taking any of the medicines listed below. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.
Using this medicine with any of the following medicines is usually not recommended, but may be required in some cases. If both medicines are prescribed together, your doctor may change the dose or how often you use one or both of the medicines.
- Avanafil
- Levodopa
- Methotrexate
- Pemetrexed
- Piritrexim
- Pralatrexate
- Raltitrexed
- Sildenafil
- Sulfamethoxazole
- Sulfasalazine
- Tadalafil
- Trimethoprim
- Trimetrexate
- Vardenafil
Other Interactions
Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.
Other Medical Problems
The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:
- Bleeding problems—Use with caution. May make these conditions worse.
- Brain or nerve problems or
- Seizures—Use with caution in patients taking levodopa. May make these conditions worse.
Proper Use
Take this medicine exactly as directed by your doctor. Do not take more of it, do not take it more often, and do not take it for a longer time than your doctor ordered. To do so may increase the chance of side effects.
This medicine comes with a patient information leaflet and patient instructions. Read and follow these instructions carefully. Ask your doctor or pharmacist if you have any questions.
Take this medicine with food.
Carefully follow your doctor's instructions about any special diet. It is important that you maintain a diet with restricted amounts of phenylalanine.
For 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.
For 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 milliliter (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.
Dosing
The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.
The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.
- For oral dosage form (powder):
- To lower blood levels of phenylalanine (Phe):
- Adults and children 2 years of age and older—Dose is based on body weight and must be determined by your doctor. At first, 60 milligrams per kilogram (mg/kg) of body weight per day. Your doctor may adjust your dose as needed.
- Children 1 to younger than 2 years of age—Dose is based on body weight and must be determined by your doctor. At first, 30 mg/kg of body weight per day. Your doctor may adjust your dose as needed.
- Children 6 months to younger than 1 year of age—Dose is based on body weight and must be determined by your doctor. At first, 15 mg/kg of body weight per day. Your doctor may adjust your dose as needed.
- Children younger than 6 months of age—Dose is based on body weight and must be determined by your doctor. At first, 7.5 mg/kg of body weight once a day. Your doctor may adjust your dose as needed.
- Children younger than 1 month of age—Use and dose must be determined by your doctor.
- To lower blood levels of phenylalanine (Phe):
Missed Dose
If you miss a dose of this medicine, take it as soon as possible. However, if it is almost time for your next dose, skip the missed dose and go back to your regular dosing schedule. Do not double doses.
Storage
Keep out of the reach of children.
Do not keep outdated medicine or medicine no longer needed.
Ask your healthcare professional how you should dispose of any medicine you do not use.
Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light. Keep from freezing.
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.
Precautions
It is very important that your doctor check your or your child's progress at regular visits to make sure this medicine is working properly. Blood tests may be needed to check for unwanted effects.
This medicine may increased your risk of bleeding. Check with your doctor right away if you have bleeding longer than usual or that does not stop, collection of blood under the skin, deep, dark purple bruise, heavy menstrual bleeding, or itching, pain, redness, or swelling.
This medicine may cause hypophenylalaninemia (low blood levels of phenylalanine) which is common during treatment and can be severe. Talk to your doctor if you have concerns about this.
This medicine may cause your stool to have a yellow or orange color. Talk to your doctor if you have concern about this.
Do not take other medicines unless they have been discussed with your doctor. This includes prescription or nonprescription (over-the-counter [OTC]) medicines and herbal or vitamin supplements.
Side Effects
Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.
Check with your doctor immediately if any of the following side effects occur:
Incidence not known
- Collection of blood under the skin
- deep, dark purple bruise
- heavy menstrual bleeding
- itching, pain, redness, or swelling
Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:
More common
- Diarrhea
- headache
- stomach pain
Less common
- Mouth or throat pain
Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.
Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.
DRG-80008486
