Triheptanoin

Triheptanoin is a prescription medication used as a source of calories and fatty acids for molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAODs).

• Triheptanoin is available under the following different brand names: Dojolvi

Common side effects of Triheptanoin include:

• abdominal pain
• abdominal distension 
• diarrhea
• vomiting, and 
• nausea

Serious side effects of Triheptanoin include:

• hives
• difficult breathing 
• swelling of the face, lips, tongue, or throat

Rare side effects of Triheptanoin include:

• none 

Seek medical care or call 911 at once if you have the following serious side effects:

• Severe headache, confusion, slurred speech, arm or leg weakness, trouble walking, coordination loss, unsteady, very stiff muscles, high fever, profuse sweating, or tremors.
• Serious eye symptoms such as sudden vision loss, blurred vision, tunnel vision, eye pain or swelling, or seeing halos around lights.
• Serious heart symptoms include fast, irregular, or pounding heartbeats; fluttering in the chest; shortness of breath; sudden dizziness, lightheadedness, or passing out.

This is not a complete list of side effects and other serious side effects or health problems that may occur because of the use of this drug. Call your doctor for medical advice about serious side effects or adverse reactions. You may report side effects or health problems to FDA at 1-800-FDA-1088.

Adult and pediatric dosage

Oral liquid

• 500 mL/bottle
• Contains 100% w/w of triheptanoin
• Long-chain fatty acid oxidation disorders

Adult and pediatric dosage

• Assess metabolic requirements by determining the patient's daily caloric intake (DCI) before calculating the dose
• Triheptanoin provides 8.3 kcal/mL
• Total daily dosage (TDD) (mL) = Patient DCI (kcal) × target % dose of DCI divided by 8.3 kcal/mL
• Not currently taking a medium-chain triglyceride (MCT) product
• Initiate at a TDD of approximately 10% DCI divided into at least 4 doses per day and increase TDD to up to 35% DCI over a period of 2-3 weeks
• Switching from another MCT product
  • Discontinue use of MCT products before initiation
  • Initiate at the last tolerated daily dosage of MCT divided into at least 4 doses per day
  • Increase TDD by approximately 5% DCI every 2-3 days until the target dosage of up to 35% DCI is achieved
• Tolerability
  • Consider more frequent smaller doses if the patient has difficulty tolerating 25% of the TDD
  • Monitor total caloric intake during dosage titration, especially in patients with gastrointestinal (GI) adverse reactions, and adjust all components of the diet as needed
  • If a patient experiences GI adverse reaction(s), consider dosage reduction until the GI symptoms resolve
  • If unable to achieve the target daily dosage of up to 35% DCI during titration, maintain the maximum tolerated dosage

Dosage Considerations – Should be Given as Follows:

• See "Dosages"

If your medical doctor is using this medicine to treat your pain, your doctor or pharmacist may already be aware of any possible drug interactions and may be monitoring you for them. Do not start, stop, or change the dosage of any medicine before checking with your doctor, healthcare provider, or pharmacist first.

• Triheptanoin has severe interactions with no other drugs.
• Triheptanoin has serious interactions with the following drug:
  • orlistat
• Triheptanoin has moderate interactions with no other drugs.
• Triheptanoin has minor interactions with no other drugs.

This information does not contain all possible interactions or adverse effects. Visit the RxList Drug Interaction Checker for any drug interactions. Therefore, before using this product, tell your doctor or pharmacist about all the products you use. Keep a list of all your medications with you and share this information with your doctor and pharmacist. Check with your healthcare professional or doctor for additional medical advice, health questions, or concerns.

Contraindications

• None

Effects of drug abuse

• None

Short-Term Effects

• See “What Are Side Effects Associated with Using Triheptanoin?”

Long-Term Effects

• See “What Are Side Effects Associated with Using Triheptanoin?”

Cautions

• Feeding tube performance and functionality can degrade over time depending on usage and environmental conditions
• Avoid use with pancreatic insufficiency; low or absent pancreatic enzymes may result in reduced heptanoate absorption and subsequently lead to insufficient medium-chain fatty acid supplementation
• Drug interaction overview
  • Avoid coadministration with pancreatic lipase inhibitors (eg, orlistat) owing to reduced efficacy and reduced exposure to heptanoate (triheptanoin metabolite)

Pregnancy and Lactation

• No data available on use in pregnant women
• Advise women to report pregnancies to Ultragenyx for Pharmaceutical Inc at 1-888-756-8657
• Lactation
  • There are no data on the presence of triheptanoin or its metabolites in human or animal milk, its effects on breastfed infants, or its effects on milk production
  • MCT and other fatty acids are normal components of breastmilk, and the composition of breastmilk varies within feedings, over stages of lactation, and between mothers and populations owing to maternal factors including genetics, environment, and diet