Dojolvi (triheptanoin) (Triheptanoin LIQUID) .96 g/mL

Dojolvi (triheptanoin) is an FDA-approved oral medication developed by Ultragenyx for treating long-chain fatty acid oxidation disorders (LC-FAOD). Approved as an orphan drug, it represents a novel therapeutic approach for this rare genetic metabolic disorder affecting neurological function and energy metabolism.

Strength

.96 g/mL

Pack Size

1 BOTTLE, GLASS in 1 CARTON (69794-050-50) / 500 mL in 1 BOTTLE, GLASS

Manufacturer / TM Owner

Ultragenyx

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Indications & Clinical Uses

Dojolvi is indicated for the treatment of long-chain fatty acid oxidation disorders (LC-FAOD), a group of rare inherited metabolic disorders affecting the body's ability to break down long-chain fatty acids for energy. These conditions include carnitine palmitoyltransferase (CPT) II deficiency, very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, and trifunctional protein (TFP) deficiency. Patients with LC-FAOD experience progressive muscle weakness, cardiac complications, hypoglycemia, and neurological deterioration. Triheptanoin works by providing an alternative seven-carbon fatty acid substrate that bypasses the enzymatic defect, enabling patients to generate energy more efficiently. This mechanism reduces the accumulation of toxic long-chain fatty acid metabolites while providing propionyl-CoA, which enters the citric acid cycle to support cellular energy production. Dojolvi is administered orally and is suitable for pediatric and adult patients with confirmed LC-FAOD diagnoses.

Side Effects

Common side effects of Dojolvi include gastrointestinal disturbances such as diarrhea, nausea, vomiting, and abdominal discomfort, particularly during initial treatment phases. Patients may experience elevated liver enzymes and ammonia levels requiring periodic monitoring. Serious adverse events include hyperammonemia, which can lead to encephalopathy, and potential cardiac complications in susceptible patients. Regular laboratory monitoring is essential, including liver function tests, ammonia levels, carnitine status, and cardiac assessment. Patients should be monitored for signs of metabolic decompensation, particularly during illness or increased metabolic stress. Dosage adjustments may be necessary based on tolerability and laboratory findings.

Precautions & Warnings

Dojolvi is contraindicated in patients with hypersensitivity to triheptanoin or any formulation components. Use with caution in patients with hepatic impairment, as the drug undergoes hepatic metabolism. Renal function should be assessed prior to initiation. Pregnancy category data is limited; use only if benefits outweigh risks. Pediatric patients require careful dose titration and monitoring. Patients with secondary carnitine deficiency require supplementation. Concurrent use with other fatty acid oxidation modulators should be evaluated. Dojolvi should not replace established dietary management protocols. Patients must maintain appropriate carbohydrate intake and avoid prolonged fasting to prevent metabolic crises.

Avoid Interactions

Limited drug-drug interactions have been documented with triheptanoin. However, concurrent use with other metabolic modulators or medications affecting liver function should be monitored. Patients taking anticoagulants may require monitoring due to potential effects on vitamin K metabolism. Avoid concurrent use with other odd-chain fatty acid supplements. Grapefruit and grapefruit juice may inhibit hepatic metabolism and should be avoided. Patients on multiple medications should inform healthcare providers of Dojolvi use to assess potential interactions. Regular medication reviews are recommended during treatment.

Frequently Asked Questions

Dojolvi (triheptanoin) is used to treat long-chain fatty acid oxidation disorders (LC-FAOD), a group of rare genetic metabolic conditions that impair the body's ability to break down long-chain fatty acids for energy. These disorders can cause muscle weakness, heart problems, low blood sugar, and neurological complications. Dojolvi provides an alternative energy source by supplying a seven-carbon fatty acid that bypasses the enzymatic defect.

Dojolvi is administered orally as a liquid formulation. The dose is individualized based on patient weight, age, and tolerance, typically divided into multiple daily doses with meals. Initial dosing is usually conservative and gradually increased over several weeks to minimize gastrointestinal side effects. Patients must follow their healthcare provider's specific dosing instructions carefully.

Common side effects include diarrhea, nausea, vomiting, and abdominal discomfort, especially when starting treatment. Some patients may experience elevated liver enzymes or ammonia levels. Most gastrointestinal side effects diminish with continued use and dose adjustment. Serious side effects are rare but may include hyperammonemia; patients should report persistent symptoms to their healthcare provider.

Dojolvi is manufactured by Ultragenyx Pharmaceutical Inc., a biopharmaceutical company specializing in rare and ultra-rare genetic diseases. Ultragenyx developed triheptanoin specifically for treating long-chain fatty acid oxidation disorders.

Yes, Dojolvi received FDA orphan drug designation for treating long-chain fatty acid oxidation disorders. This designation recognizes its therapeutic benefit for a rare disease affecting fewer than 200,000 people in the United States. Orphan drug status provides regulatory incentives and market exclusivity to encourage development of treatments for rare diseases.

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GNH India Pharmaceuticals Limited

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Last updated: 17 Jun 2026

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