triheptanoin (Rx)

Brand and Other Names:Dojolvi
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Dosing & Uses

AdultPediatric

oral liquid

  • 500mL/bottle
  • Contains 100% w/w of triheptanoin

Long-chain Fatty Acid Oxidation Disorders

Indicated as a source of calories and fatty acids for molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAODs)

Assess metabolic requirements by determining patient’s daily caloric intake (DCI) before calculating dose

Triheptanoin provides 8.3 kcal/mL

Total daily dosage (TDD) (mL) = Patient DCI (kcal) x target % dose of DCI divided by 8.3 kcal/mL

Not currently taking a medium-chain triglyceride (MCT) product

  • Initiate at a TDD of ~10% DCI divided into at least 4x/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 prior to initiation
  • Initiate at the last tolerated daily dosage of MCT divided into at least 4x/day
  • Increase TDD by ~5% DCI every 2-3 days until the target dosage of up to 35% DCI is achieved

Tolerability

  • Consider more frequent smaller doses if 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 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 at the maximum tolerated dosage

Dosage Considerations

Patients may require an increase in total fat intake to reach target daily dose

All patients should be under the care of a clinical specialist knowledgeable in appropriate disease-related dietary management based on current nutritional recommendations

oral liquid

  • 500mL/bottle
  • Contains 100% w/w of triheptanoin

Long-chain Fatty Acid Oxidation Disorders

Indicated as a source of calories and fatty acids for molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAODs)

Assess metabolic requirements by determining patient’s daily caloric intake (DCI) before calculating dose

Triheptanoin provides 8.3 kcal/mL

Total daily dosage (TDD) (mL) = Patient DCI (kcal) x target % dose of DCI divided by 8.3 kcal/mL

Not currently taking a medium-chain triglyceride (MCT) product

  • Initiate at a TDD of ~10% DCI divided into at least 4x/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 prior to initiation
  • Initiate at the last tolerated daily dosage of MCT divided into at least 4x/day
  • Increase TDD by ~5% DCI every 2-3 days until the target dosage of up to 35% DCI is achieved

Tolerability

  • Consider more frequent smaller doses if 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 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 at the maximum tolerated dosage

Dosage Considerations

Patients may require an increase in total fat intake to reach target daily dose

All patients should be under the care of a clinical specialist knowledgeable in appropriate disease-related dietary management based on current nutritional recommendations

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Interactions

Interaction Checker

and triheptanoin

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    Contraindicated

      Serious - Use Alternative

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            Adverse Effects

            >10%

            Abdominal pain (60%)

            Diarrhea (44%)

            Vomiting (44%)

            Nausea (14%)

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            Warnings

            Contraindications

            None

            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)
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            Pregnancy & Lactation

            Pregnancy

            No data available on use in pregnant females

            Advise females to report pregnancies to Ultragenyx 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, effects on breastfed infants, or 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

            Pregnancy Categories

            A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

            B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

            C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

            D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

            X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

            NA: Information not available.

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            Pharmacology

            Mechanism of Action

            MCT consisting of three odd-chain 7-carbon length fatty acids (heptanoate)

            Provides a source of calories and fatty acids to bypass the LC-FAOD enzyme deficiencies for energy production and replacement

            Absorption

            Peak plasma concentration

            • Single dose: 178.9 micromol/L (0.3 g/kg-dose); 259.1 micromol/L (0.4 g/kg-dose)
            • Multiple doses: 319.9 micromol/L (TDD: 1.3 g/kg/day)

            Peak plasma time

            • Single dose: 0.5 hr (0.3 g/kg-dose); 0.8 hr (0.4 g/kg-dose)
            • Multiple doses: 1.2 hr (TDD: 1.3 g/kg/day)

            AUC

            • Single dose: 336.5 micromol⋅hr/L (0.3 g/kg-dose); 569.1 micromol⋅hr/L (0.4 g/kg-dose)
            • Multiple doses: 789.8 micromo⋅hr/L (TDD: 1.3 g/kg/day)

            Distribution

            Protein bound: ~80% (heptanoate)

            Metabolism

            Heptanoate, formed by hydrolysis of triheptanoin, can be metabolized to beta-hydroxypentanoate (BHP) and beta-hydroxybutyrate (BHB) in the liver

            Elimination

            Half-life: Not determined owing to multiple peak concentrations observed

            Excretion: Minimally excreted in urine (triheptanoin and metabolites)

            Clearance

            0.3 g/kg-dose: 6.05 L/hr/kg

            0.4 g/kg-dose: 4.31 L/hr/kg

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            Administration

            Compatibility

            Oral compatibility

            • Use containers, dosing syringes, or measuring cups made of compatible materials suchas stainless steel, glass, high-density polyethylene (HDPE), polypropylene, low-densitypolyethylene, polyurethane, and silicone
            • Not compatible with certain plastics; do not use containers, dosing syringes, or measuring cups made of polystyrene or polyvinyl chloride (PVC) plastics
            • Monitor containers, dosing components, or utensils that are in contact with triheptanoin to ensure proper functioning and integrity

            Feeding tube compatibility

            • Administer via oral or enteral feeding tubes manufactured of silicone or polyurethane
            • Do not use PVC feeding tubes
            • Feeding device performance and functionality can degrade over time depending on usage and environmental conditions
            • Monitor feeding tube to ensure proper functioning and integrity

            Oral Preparation

            Use an oral syringe or measuring cup made of compatible materials as listed above to withdraw prescribed volume from the bottle

            Mix into semisolid foods and liquids (eg, plain or artificially sweetened fat-free yogurt, fat-free milk, formula, cottage cheese, whole grain hot cereal, fat-free low-carbohydrate pudding, smoothies, applesauce, baby food)

            Add prescribed amount to a clean bowl, cup, or container, which contains an appropriate amount of semisolid food or liquid that takes into consideration the age, size, and average consumption of the patient in order to ensure administration of the full dose

            Mix thoroughly into the food or liquid

            Oral Administration

            Administer orally or enterally via a silicone or polyurethane feeding tube

            Administer with food to avoid GI upset

            Feeding tube

            • Draw up entire amount of formula mixture into a slip-tip syringe
            • Remove residual air from the syringe and connect syringe directly into the feeding tube feeding port
            • Push contents into the feeding tube feeding port using steady pressure until empty
            • Flush feeding tubes with 5-30 mL of water
            • Modify flush volume based on specific patient needs and in cases of fluid restriction
            • Discard any unused portion; do not save for later use
            • Patients receiving bolus delivery of enteral feeds: Administer over 15-20 minutes
            • Patients receiving continuous feeds: Administer over 30-60 minutes alternating with formula alone

            Missed dose

            • Take the next dose as soon as possible with subsequent doses taken at 3- to 4­hr intervals
            • Skip missed dose if unable to take all 4 doses in a day

            Storage

            Unopened bottle

            • Store at 20-25ºC (68-77ºF); excursions permitted to 15-30ºC (59-86ºF)
            • Dispense only in glass or HDPE bottles
            • Do not freeze

            Opened bottles

            • Discard after 90 days of opening, but not beyond the expiration date on the bottle
            • Do not dose or store in polystyrene or PVC containers

            Prepared doses mixed with food or liquid

            • Refrigerate for up to 24 hr
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            Formulary

            FormularyPatient Discounts

            Adding plans allows you to compare formulary status to other drugs in the same class.

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            The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

            Tier Description
            1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
            2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
            3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
            4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            NC NOT COVERED – Drugs that are not covered by the plan.
            Code Definition
            PA Prior Authorization
            Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
            QL Quantity Limits
            Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
            ST Step Therapy
            Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
            OR Other Restrictions
            Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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            Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.