Triheptanoin

Medically reviewed by Drugs.com. Last updated on Sep 6, 2023.

Pronunciation

(trye HEP ta noyn)

Index Terms

Dojolvi
UX007

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Liquid, Oral:

Dojolvi: 100% (500 mL)

Brand Names: U.S.

Dojolvi

Pharmacologic Category

Anaplerotic Agent
Nutritional Supplement

Pharmacology

Triheptanoin is a medium-chain triglyceride consisting of 3 odd-chain 7-carbon length fatty acids (heptanoate) that provide a source of calories and fatty acids to bypass the long-chain fatty acid oxidation disorder enzyme deficiencies for energy production and replacement.

Metabolism

Triheptanoin: Extensively hydrolyzed to heptanoate and glycerol via pancreatic lipases in intestines; Heptanoate: Metabolized to beta-hydroxypentanoate and beta-hydroxybutyrate in the liver.

Excretion

Urine (minimally as unchanged drug and metabolites).

Time to Peak

Heptanoate: Multiple peak concentrations are observed following oral administration.

Protein Binding

Heptanoate: ~80%.

Use: Labeled Indications

Long-chain fatty acid oxidation disorders: As a source of calories and fatty acids for the treatment of molecularly confirmed long-chain fatty acid oxidation disorders in adults and pediatric patients.

Contraindications

There are no contraindications listed in the manufacturer's labeling.

Dosing: Adult

Long-chain fatty acid oxidation disorders:

Note: Assess patient's metabolic requirements by determining the patient's daily caloric intake (DCI) prior to calculating triheptanoin dose. May need to increase total fat intake to reach target daily dosage.

Patients not currently receiving a medium-chain triglyceride product: Oral: Initial: ~10% of the patient's total prescribed DCI divided into at least 4 times daily; increase dosage by ~5% of the patient's total prescribed DCI every 2 to 3 days until target dose of up to 35% of the patient's prescribed DCI is achieved. If GI intolerance occurs (eg, abdominal pain, diarrhea, nausea, vomiting), reduce dose until symptoms resolve; titrate as tolerated.

Patients switching from another medium-chain triglyceride product: Oral: Prior to initiation, discontinue any other medium-chain triglyceride products. Initiate at the last tolerated daily dosage of medium-chain triglyceride divided into at least 4 times daily; increase dosage by ~5% of the patient's total prescribed DCI every 2 to 3 days until target dose of up to 35% of the patient's prescribed DCI is achieved. If GI intolerance occurs (eg, abdominal pain, diarrhea, nausea, vomiting), reduce dose until symptoms resolve; titrate as tolerated.

Formula for triheptanoin dose:

Total daily dose (mL) = (Patient's DCI [kcal] x desired % of DCI) divided by 8.3 kcal/mL

Note: Round the total daily dosage to the nearest whole number and divide into at least 4 doses; smaller, more frequent doses may be considered in patients unable to tolerate one-fourth of the daily dosage at one time.

Missed dose: If a dose is missed, take the next dose as soon as possible with subsequent doses taken at 3- to 4-hour intervals; if all 4 doses cannot be completed in a day, skip the missed dose.

Dosing: Geriatric

Refer to adult dosing.

Dosing: Pediatric

Long-chain fatty acid oxidation disorders: Note: Assess patient's metabolic requirements by determining the patient's daily caloric intake (DCI) prior to calculating triheptanoin dose. May need to increase total fat intake to reach target daily dosage.

Patients not currently receiving a medium-chain triglyceride product: Oral: Initial: ~10% of the patient's total prescribed DCI divided into at least 4 times daily doses; increase dosage by ~5% of the patient's total prescribed DCI every 2 to 3 days until target dose of up to 35% of the patient's prescribed DCI is achieved. If GI intolerance occurs (eg, abdominal pain, diarrhea, nausea, vomiting), reduce dose until symptoms resolve; titrate as tolerated.

Patients switching from another medium-chain triglyceride product: Oral: Prior to initiation, discontinue any other medium-chain triglyceride products. Initiate at the last tolerated daily dosage of medium-chain triglyceride divided into at least 4 times daily doses; increase dosage by ~5% of the patient's total prescribed DCI every 2 to 3 days until target dose of up to 35% of the patient's prescribed DCI is achieved. If GI intolerance occurs (eg, abdominal pain, diarrhea, nausea, vomiting), reduce dose until symptoms resolve; titrate as tolerated.

Formula for triheptanoin dose:

Total daily dose (mL) = (Patient's DCI [kcal] x desired % of DCI) divided by 8.3 kcal/mL

Note: Round the total daily dosage to the nearest whole number and divide into at least 4 doses; smaller, more frequent doses may be considered in patients unable to tolerate ¼ of the daily dosage at one time.

Administration

Enteral: Do not prepare or administer using products made of polystyrene or PVC plastics. Use containers, dosing syringes, or measuring cups made of compatible materials (eg, stainless steel, glass, high-density polyethylene, polypropylene, low-density polyethylene, polyurethane, silicone). Using an oral syringe or measuring cup made of compatible materials, withdraw prescribed volume of triheptanoin from the bottle; add to a clean bowl, cup, or container containing semisolid food or liquid as below; mix thoroughly. Regularly inspect the containers, dosing components, or utensils that are in contact with triheptanoin to ensure proper functioning and integrity.

Oral: Prior to administration, mix with semisolid foods or liquids (eg, 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). Ensure that amount of food or liquid is appropriate for patient's size and typical consumption. Once mixed, may be stored refrigerated for up to 24 hours. Administer at mealtime or with snacks; avoid administering alone due to GI upset.

Feeding tube: Prior to administration, mix with formula; ensure that the amount of formula used is appropriate for the patient's size and typical consumption. Administer mixed with formula via feeding tube. Draw up entire mixture into a slip tip syringe and remove residual air from the syringe. Administer via an oral or enteral silicone or polyurethane feeding tube by connecting the syringe directly into feeding tube feeding port; push the mixture through the syringe using steady pressure until the syringe is empty. For patients receiving bolus delivery of enteral feeds, administer over 15 to 20 minutes. For patients receiving continuous feeds, administer over 30 to 60 minutes alternating with formula alone. After administration, flush feeding tube with 5 to 30 mL of water; may modify amount based on patient's specific needs (eg, fluid restriction). Discard any unused portion of the mixture; do not save for later use.

Storage

Store at 20°C to 25°C (68°F to 77°F); excursions permitted to 15°C to 30°C (59°F to 86°F). Do not freeze. Opened bottles can be used for up to 90 days after opening, but not beyond the expiration date on the bottle. Store only in glass or high-density polyethylene containers; do not store in polystyrene or PVC containers.

Drug Interactions

Orlistat: May decrease serum concentrations of the active metabolite(s) of Triheptanoin. Avoid combination

Adverse Reactions

The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.

>10%: Gastrointestinal: Abdominal pain (60%; including abdominal distention, abdominal distress, gastrointestinal pain, upper abdominal pain), diarrhea (44%), nausea (14%), vomiting (44%)

Warnings/Precautions

Disease-related concerns:

• Pancreatic insufficiency: Avoid use in patients with pancreatic insufficiency; reduced absorption leading to insufficient supplementation of medium-chain fatty acids may occur.

Other warnings/precautions:

• Feeding tubes: Do not use PVC feeding tubes; the performance and functionality of feeding tubes may degrade over time depending on usage and environmental conditions. Monitor feeding tube regularly to ensure proper functioning and integrity.

Monitoring Parameters

Total caloric intake during dosage titration (especially in patients with GI adverse reactions).

Pregnancy Considerations

Information from animal reproduction studies was not considered relevant to maternal use.

Data collection to monitor pregnancy and infant outcomes following exposure to triheptanoin is ongoing. Females exposed to triheptanoin during pregnancy are encouraged to contact the manufacturer (1-888-756-8657).

Patient Education

What is this drug used for?

• It is used to treat long-chain fatty acid oxidation disorders (LC-FAOD).

All drugs may cause side effects. However, many people have no side effects or only have minor side effects. Call your doctor or get medical help if any of these side effects or any other side effects bother you or do not go away:

• Stomach pain

• Diarrhea

• Upset stomach

• Throwing up

WARNING/CAUTION: Even though it may be rare, some people may have very bad and sometimes deadly side effects when taking a drug. Tell your doctor or get medical help right away if you have any of the following signs or symptoms that may be related to a very bad side effect:

• Signs of an allergic reaction, like rash; hives; itching; red, swollen, blistered, or peeling skin with or without fever; wheezing; tightness in the chest or throat; trouble breathing, swallowing, or talking; unusual hoarseness; or swelling of the mouth, face, lips, tongue, or throat.

Note: This is not a comprehensive list of all side effects. Talk to your doctor if you have questions.

Consumer Information Use and Disclaimer: This information should not be used to decide whether or not to take this medicine or any other medicine. Only the healthcare provider has the knowledge and training to decide which medicines are right for a specific patient. This information does not endorse any medicine as safe, effective, or approved for treating any patient or health condition. This is only a limited summary of general information about the medicine's uses from the patient education leaflet and is not intended to be comprehensive. This limited summary does NOT include all information available about the possible uses, directions, warnings, precautions, interactions, adverse effects, or risks that may apply to this medicine. This information is not intended to provide medical advice, diagnosis or treatment and does not replace information you receive from the healthcare provider. For a more detailed summary of information about the risks and benefits of using this medicine, please speak with your healthcare provider and review the entire patient education leaflet.