Triheptanoin

What is Triheptanoin?

• Triheptanoin (DOJOLVI) is a medium-chain triglyceride indicated as a source of calories and fatty acids used to treat long-chain fatty acid oxidation disorders (LC-FAOD) in children and adults.

What are the uses of this medicine?

• This medicine is indicated for as a source of calories and fatty acids for the treatment of pediatric and adult patients with molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD).

How does this medicine work?

Triheptanoin is a medium-chain triglyceride consisting of three odd-chain 7-carbon length fatty acids (heptanoate) that provide a source of calories and fatty acids to bypass the long-chain FAOD enzyme deficiencies for energy production and replacement.

Who Should Not Use this medicine ?

• This medicne have no usage limitations.

What drug interactions can this medicine cause?

• Avoid co-administration of DOJOLVI with pancreatic lipase inhibitors (e.g., orlistat).

Is this medicine FDA approved?

• It was approved for use in the United States in 2020.

How should this medicine be used?

• Assess metabolic requirements by determining daily caloric intake (DCI) prior to calculating the dosage of DOJOLVI.
• For patients receiving another medium-chain triglyceride product, discontinue prior to the first dose of DOJOLVI.
• The recommended target daily dosage of DOJOLVI is up to 35% of the patient’s total prescribed DCI divided into at least four doses and administered orally diluted with foods, liquids, or formula via a silicone or polyurethane feeding tube.

For patients not currently taking a medium-chain triglyceride product

• Initiate DOJOLVI at a total daily dosage of approximately 10% DCI divided into at least four times per day and increase to the recommended total daily dosage of up to 35% DCI over a period of 2 to 3 weeks.

For patients switching from another medium-chain triglyceride product

• Discontinue use of MCT products before starting DOJOLVI.
• Initiate DOJOLVI at the last tolerated daily dosage of MCT divided into at least four times per day. Increase the total daily dosage by approximately 5% DCI every 2 to 3 days until the target dosage of up to 35% DCI is achieved.

Administration

• 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, dosing 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.
• DOJOLVI should be taken at least 4 times a day with meals or snacks, and always mixed well with soft food or drink.

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form: As oral Liquidː 100% w/w of triheptanoin

This medicine is available in fallowing brand namesː

• DOJOLVI

What side effects can this medication cause?

Common possible side effects of this medicine include:

• abdominal pain
• diarrhea
• vomiting
• nausea
• Feeding tube problems
• Intestinal absorption problems in patients with pancreatic insufficiency

What special precautions should I follow?

• This medicine may cause feeding tube dysfunction regularly monitor the tube to ensure proper functioning and integrity.
• Low or absent pancreatic enzymes may reduce absorption of DOJOLVI. Avoid administration of DOJOLVI in patients with pancreatic insufficiency.

What to do in case of emergency/overdose?

• In case of overdose, call the poison control helpline of your country. In the United States, call 1-800-222-1222.

• Overdose related information is also available online at poisonhelp.org/help.
• In the event that the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services. In the United States, call 911.

Can this medicine be used in pregnancy?

• There are no available data on triheptanoin use in pregnant women to evaluate for a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes.
• Advise women to report pregnancies to Ultragenyx Pharmaceutical Inc. at 1-888-756-8657.

Can this medicine be used in children?

• The safety and effectiveness of DOJOLVI have been established in pediatric patients aged birth and older.

What should I know about storage and disposal of this medication?

• Store DOJOLVI at room temperature between 68°F to 77°F (20°C - 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).

• Dailymed label info on Triheptanoin
• FDA Triheptanoin

Triheptanoin Resources
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