Dojolvi (Triheptanoin): Uses, Side Effects and Dosage

What is DOJOLVI?

DOJOLVI is a prescription medicine used to treat long-chain fatty acid oxidation disorders (LC-FAOD) in children and adults.

Before taking DOJOLVI, 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 DOJOLVI will harm your unborn baby.

  Pregnancy Safety Study. There is a pregnancy safety study for women who take DOJOLVI during pregnancy. The purpose of this study is to collect information about your health and your baby's health. You can talk to your healthcare provider or contact 1-888-756-8657 to enroll in this study or get more information.

• are breastfeeding or plan to breastfeed. It is not known if DOJOLVI passes into breast milk. Talk to your healthcare provider about the best way to feed your baby if you take DOJOLVI.
• are taking a pancreatic lipase inhibitor, such as orlistat, as it may affect how well DOJOLVI works.

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

How should I take DOJOLVI?

• See the detailed "Instructions for Use" at the end of this Patient Information Leaflet for instructions about how to mix and take DOJOLVI by mouth in soft food or drink or how to mix and give DOJOLVI in medical food or formula through feeding tubes.
• Take DOJOLVI exactly as your healthcare provider tells you.
• Your healthcare provider may start you on a low dose of DOJOLVI and slowly increase your dose to help avoid side effects. If you are taking another medium chain triglyceride (MCT) product, stop taking the MCT before starting DOJOLVI.
• Do not mix or give DOJOLVI using containers, oral syringes, or measuring cups made of polystyrene (a type of plastic that can be solid or foam) or polyvinyl chloride (PVC), a solid plastic material.
• Take DOJOLVI at least 4 times a day with meals or snacks, and always mix well with soft food or drink or medical food or formula before taking.

What are the possible side effects of DOJOLVI?

• Feeding tube problems. Feeding tubes may not work as well or stop working over time when taking DOJOLVI. Do not use DOJOLVI in feeding tubes made of polyvinyl chloride (PVC). Check the feeding tube to make sure it is working properly and not breaking down.
• Intestinal absorption problems in patients with pancreatic insufficiency. If you have pancreatic insufficiency, consult with your healthcare provider as it may affect how well DOJOLVI works.
• The most common side effects of DOJOLVI include:

• stomach (abdominal) pain
• diarrhea

• vomiting
• nausea

These are not all the possible side effects of DOJOLVI. Call your healthcare provider for medical advice about side effects. You may report side effects to Ultragenyx at 1-888-756-8657 or FDA at 1-800-FDA-1088.

How should I store DOJOLVI?

• Store DOJOLVI at room temperature between 68°F to 77°F.
• Do not freeze DOJOLVI.
• When the bottle of DOJOLVI has been opened, use within 9 months or by the expiration date on the bottle, whichever comes first.
• Do not store DOJOLVI in containers made of polystyrene or polyvinyl chloride (PVC).

General information about the safe and effective use of DOJOLVI.

Medicines are sometimes prescribed for purposes other than those listed in a Patient Information Leaflet. Do not use DOJOLVI for a condition for which it was not prescribed. Do not give DOJOLVI to other people, even if they have the same symptoms that you have. It may harm them. You can ask your pharmacist or healthcare provider for information about DOJOLVI that is written for health professionals.

What are the ingredients in DOJOLVI?

DOJOLVI is made of 100% triheptanoin and contains no other ingredients.

Manufactured for:

Ultragenyx Pharmaceutical Inc.

60 Leveroni Court

Novato, CA 94949

For more information, go to www.dojolvi.com or call 1-888-756-8657.

INSTRUCTIONS FOR USE

DOJOLVI (doh-johl-vee)

(triheptanoin)

oral liquid

This Instructions for Use contains information on how to take DOJOLVI. Read this Instructions for Use before you start taking DOJOLVI and each time you get a refill. There may be new information. This Instructions for Use does not take the place of talking to your healthcare provider about your medical condition or treatment.

Important Information You Need to Know Before Taking or Giving DOJOLVI:

• Use an oral syringe or measuring cup to measure your prescribed dose. Ask your healthcare provider or pharmacist to show you how to measure your prescribed dose.
• Mix or give DOJOLVI using containers, oral syringes, or measuring cups made of materials such as stainless steel, glass, or high density polyethylene (HDPE), polypropylene, low density polyethylene, polyurethane, and silicone (types of plastic materials).
• After use, rinse materials and containers well with cold water and wipe dry.
• Do not mix or give DOJOLVI using containers, oral syringes, or measuring cups made of polystyrene (a type of plastic that can be solid or foam) or polyvinyl chloride (PVC), a solid plastic material.
• Take DOJOLVI at least 4 times a day with meals or snacks, and always mix well with soft food or drink or medical food or formula before taking.

• If you take DOJOLVI by mouth, mix well with soft food or drink such as:

  • plain or artificially sweetened fat free yogurt
  • fat free milk, formula, or cottage cheese
  • whole grain hot cereal
  • fat free low carbohydrate pudding, smoothies, applesauce, or baby food
• If you give DOJOLVI by feeding tube, mix well with medical food or formula.
• Your healthcare provider should advise you on how to maintain a proper diet when taking DOJOLVI.
• If you miss a dose, take the next dose as soon as possible. Take the following doses 3 to 4 hours apart. If it is not possible to take all the doses for the day, skip the missed dose.

Taking DOJOLVI by mouth:

1. 1. Use an oral syringe or measuring cup made of the materials listed above to measure the prescribed amount of DOJOLVI from the bottle.
2. 2. Add the prescribed amount of DOJOLVI to a clean bowl, cup, or container, made of the materials listed above, containing the appropriate amount of soft food or drink as instructed by your healthcare provider.
3. 3. Mix DOJOLVI well into the soft food or liquid and swallow the mixture.
4. 4. Do not take DOJOLVI alone to avoid stomach upset.
5. 5. You can store any unused DOJOLVI mixture for up to 24 hours in the refrigerator.
6. 6. If not used within 24 hours, throw away (dispose of) the DOJOLVI mixture in the trash. Do not pour down the sink. Do not save for later.
7. 7. Check the items used to take DOJOLVI often to make sure they are working properly and are not breaking down.

Giving DOJOLVI by feeding tube:

1. 1. You can give DOJOLVI by a feeding tube.
2. 2. Mix DOJOLVI with medical food or formula before giving by a feeding tube, y-connector, or feeding tube extension set.
3. 3. Only give DOJOLVI in a feeding tube made of silicone or polyurethane.
4. 4. Do not give DOJOLVI in a feeding tube made of polyvinyl chloride (PVC), a type of plastic.
5. 5. Do not give DOJOLVI alone to avoid stomach upset and to avoid breakdown of the feeding tube.
6. 6. Do not add DOJOLVI to the feeding bag because the feeding equipment may break down over time.
7. 7. Use an oral syringe or measuring cup made of the materials listed above to measure the prescribed amount of DOJOLVI from the bottle.
8. 8. Add the prescribed amount of DOJOLVI to a clean bowl, cup, or container, made of the materials listed above, containing the appropriate amount of medical food or formula as instructed by your healthcare provider.
9. 9. Mix DOJOLVI well into the medical food or formula and draw up the entire amount of the mixture into a slip tip syringe.
10. 10. Remove the air from the syringe and connect the syringe directly into the feeding tube port.
11. 11. Push the contents of the syringe (DOJOLVI mixture) into the feeding tube port using steady pressure until empty.
12. 12. Draw up about 5 mL to 30 mL of water with the slip tip syringe and flush the feeding tube feeding port with the water.
13. 13. Throw away (dispose of) any unused DOJOLVI mixture in the trash. Do not pour down the sink. Do not save for later use.
14. 14. Check the feeding tube and other items used to give DOJOLVI often to make sure they are working properly and are not breaking down.

How should I store DOJOLVI?

• Store DOJOLVI at room temperature between 68°F to 77°F (20°C to 25°C).
• Do not freeze DOJOLVI.
• When the bottle of DOJOLVI has been opened, use within 9 months or by the expiration date on the bottle, whichever comes first.
• Do not store DOJOLVI in containers made of polystyrene or polyvinyl chloride (PVC).

Keep DOJOLVI and all medicines out of the reach of children.

This Instructions for Use has been approved by the U.S. Food and Drug Administration.

Revised: 10/2023