Total daily dose of triheptanoin (mL) = (Patient's daily caloric intake [kcal] × desired % of daily caloric intake) divided by 8.3 kcal/mL.
PO (Adults ): Not currently receiving a medium-chain triglyceride product: ~10% of the patient's total prescribed daily caloric intake divided into at least 4 times daily; ↑ dose by ~5% of the patient's total prescribed daily caloric intake every 2–3 days until target dose of up to 35% of the patient's total prescribed daily caloric intake is achieved. Switching from another medium-chain triglyceride product: Initiate at the last tolerated daily dosage of medium-chain triglyceride divided into at least 4 times daily; ↑ dose by ~5% of the patient's total prescribed daily caloric intake every 2–3 days until target dose of up to 35% of the patient's total prescribed daily caloric intake is achieved.
US Brand Names⬆⬇
Dojolvi
Action⬆⬇
Acts as 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 fatty acid oxidation disorder enzyme deficiencies for energy production and replacement.
Therapeutic effects:
Reduction in major clinical events associated with long-chain fatty acid oxidation disorders.
Classifications⬆⬇
Therapeutic Classification: temporary class
Pharmacokinetics⬆⬇
Absorption: Extent of absorption following oral administration unknown.
Distribution: Unknown.
Metabolism/Excretion: Hydrolyzed to heptanoate and glycerol via pancreatic lipases in intestines; heptanoate is metabolized in the liver. Minimally excreted in the urine.
Half-Life: Unknown.
Time/Action Profile⬆⬇
(plasma concentrations)
ROUTE
ONSET
PEAK
DURATION
PO
unknown
0.5–1.2 hr
unknown
Patient/Family Teaching⬆⬇
Explain purpose and side effects of triheptanoin. Teach patient or caregiver to mix thoroughly into semisolid foods, liquids, or formula. Advise patient or caregiver to avoid using containers or utensils made of polystyrene or polyvinyl chloride plastics when preparing or administering drug. Counsel patient that if a dose is missed, take next dose as soon as possible, with subsequent doses taken at 3- to 4-hr intervals; if this is not possible, take all four doses in a day, skipping the missed dose. Advise patient or caregiver to read Patient Information before starting therapy.
Recommend patient or caregiver regularly inspect the feeding tube for proper functioning and report to the health care provider if any issues are identified.
Advise patient to report and seek treatment for severe abdominal pain, diarrhea, vomiting, and nausea.
Advise patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other medications.
Rep: Advise women of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding. Health care providers are encouraged to register patients in the pregnancy safety study for triheptanoin by calling Ultragenyx Pharmaceuticals at 1-888-756-8657.
