Medium-chain Triglyceride

Dojolvi

Approved 30 Jun 2020OralLiquid

Indications

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

Contraindications

None. None.

Warnings and precautions

Feeding Tube Dysfunction : Regularly monitor the feeding tube to ensure proper functioning and integrity. ( 5.1 ) Intestinal Malabsorption in Patients with Pancreatic Insufficiency : Low or absent pancreatic enzymes may reduce absorption of DOJOLVI. Avoid administration of DOJOLVI in patients with pancreatic insufficiency. ( 5.2 )

5.1Feeding Tube Dysfunction

Feeding tube performance and functionality can degrade over time depending on usage and environmental conditions. In clinical trials, feeding tube dysfunction was reported in patients receiving triheptanoin. The contribution of DOJOLVI cannot be ruled out. Do not administer DOJOLVI in feeding tubes manufactured of polyvinyl chloride (PVC) [see Dosage and Administration (2.4 ] . Regularly monitor the feeding tube to ensure proper functioning and integrity.

5.2Intestinal Malabsorption in Patients with Pancreatic Insufficiency

Pancreatic enzymes hydrolyze triheptanoin and release heptanoate as medium-chain fatty acids in the small intestine. Low or absent pancreatic enzymes may result in reduced absorption of heptanoate subsequently leading to insufficient supplementation of medium-chain fatty acids. Avoid administration of DOJOLVI in patients with pancreatic insufficiency.

Interactions

Pancreatic Lipase Inhibitors : Avoid co-administration due to potential for reduced clinical effect of DOJOLVI. ( 7.1 )

7.1Pancreatic Lipase Inhibitors Co-administration

At a glance

Brand name: Dojolvi
Active substance: triheptanoin
Pharmacologic class: Medium-chain Triglyceride
Route: Oral
Dosage form: Liquid
Applicant: Ultragenyx Pharm Inc
Status: Prescription
FDA approval: 30 Jun 2020
Application: NDA213687-001

Indications

Warnings and precautions

5.1Feeding Tube Dysfunction

5.2Intestinal Malabsorption in Patients with Pancreatic Insufficiency

Pregnancy and breastfeeding

8.1Pregnancy Risk Summary

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. In animal reproduction studies conducted in pregnant rats and rabbits administered triheptanoin during the period of organogenesis, the primary toxicological effect (reduced body weight gain) was considered to be specific to decreased food consumption related to taste aversion in animals, and therefore is not relevant to clinical use in the intended populations.

There is a pregnancy safety study for DOJOLVI. If a patient becomes pregnant while receiving DOJOLVI, healthcare providers should report DOJOLVI exposure by calling Ultragenyx Pharmaceutical Inc. at 1-888-756-8657. The background risk of major birth defects and miscarriage for the indicated population is unknown. All pregnancies have a background risk of birth defect, loss or other adverse outcomes. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2 to 4% and 15 to 20%, respectively.

Data Animal Data Embryofetal developmental studies have been conducted with triheptanoin in rats and rabbits following oral administration of 10% (3.2 g/kg), 30% (9.7 g/kg) and 50% (16 g/kg) DCI in rats and 10% (1.2 g/kg), 20% (2.3 g/kg) and 30% (3.5 g/kg) DCI in rabbits during the period of organogenesis. Reduced body weight gain, associated with decreased food consumption, was observed in pregnant rats and rabbits following administration of triheptanoin food mixture and was attributed to taste aversion.

The NOAEL for this maternal toxicity (lack of body weight gain) was 10% DCI for both rats and rabbits. Administration of dietary triheptanoin to pregnant rats at doses approximately 2 times above, and pregnant rabbits approximately equal to the targeted clinical dose of 35% DCI resulted in increased incidence of skeletal malformations and decreased litter weights in both species and reduced number of viable litters in rabbits.

The adverse effects on rat and rabbit embryofetal development were associated with the reduced body weight gain observed in pregnant animals. The NOAEL for embryofetal development toxicity was 30% and 20% DCI for rats and rabbits, respectively. In a pre- and postnatal developmental study in rats, reduced birthweights and delayed sexual maturation in pups were observed at 50% DCI and were considered secondary to the reductions in body weight gain in pregnant rats.

Dojolvi

Indications

Contraindications

Warnings and precautions

5.1Feeding Tube Dysfunction

5.2Intestinal Malabsorption in Patients with Pancreatic Insufficiency

Interactions

7.1Pancreatic Lipase Inhibitors Co-administration

At a glance

Two medicines in one question?

Dojolvi

Indications

Contraindications

Warnings and precautions

5.1Feeding Tube Dysfunction

5.2Intestinal Malabsorption in Patients with Pancreatic Insufficiency

Interactions

7.1Pancreatic Lipase Inhibitors Co-administration

At a glance

Two medicines in one question?

Pregnancy and breastfeeding

8.1Pregnancy Risk Summary

Adverse reactions

6.1Clinical Trial Experience

Composition

Official product information