Version May 2024
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. Current nutritional recommendations should be considered; neonates may need increased dosage due to higher fat intake.
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. Consider more frequent, smaller doses in patients unable to tolerate 1/4 of the total daily dosage at one time. 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. Consider more frequent, smaller doses in patients unable to tolerate 1/4 of the total daily dosage at one time. 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 1/4 of the daily dosage at one time.
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. Consider more frequent, smaller doses in patients unable to tolerate 1/4 of the total daily dosage at one time. 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. Consider more frequent, smaller doses in patients unable to tolerate 1/4 of the total daily dosage at one time. 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 1/4 of the daily dosage at one time.
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
(For additional information see "Triheptanoin: Drug information")
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. Consider more frequent, smaller doses in patients unable to tolerate one-fourth of the total daily dosage at one time. 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. Consider more frequent, smaller doses in patients unable to tolerate one-fourth of the total daily dosage at one time. 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.
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
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%)
There are no contraindications listed in the manufacturer's US labeling.
Canadian labeling: Hypersensitivity to triheptanoin or any component of the formulation.
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.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Liquid, Oral:
Dojolvi: 100% (500 mL)
No
Liquid (Dojolvi Oral)
100% (per mL): $13.95
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Liquid, Oral:
Dojolvi: 100% (500 mL)
Caloric content: 8.3 kcal/mL.
Enteral: Do not prepare or administer using products made of polystyrene or polyvinyl chloride (PVC) plastics. Use containers, dosing syringes, or measuring cups made of compatible materials (eg, stainless steel, glass, high density polyethylene [HDPE], 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 semi-solid food, liquid, or formula 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 semi-solid 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 age, 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/medical food; ensure that the amount of formula/medical food used is appropriate for the patient's age, size, and typical consumption. Administer mixed with formula/medical food via feeding tube as enteral bolus. 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. 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 in the trash; do not pour down the sink; do not save for later use.
Missed dose: If a dose is missed, administer the next dose as soon as possible, with subsequent doses administered at 3- to 4-hour intervals; if all 4 doses cannot be administered in a day, skip the missed dose.
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 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. Dispose of any amount not used within 24 hours in the trash; do not pour down the sink or save for later. Administer at mealtime or with snacks; avoid administering alone due to GI upset.
Feeding tube: Prior to administration, mix with medical food; ensure that the amount of medical food used is appropriate for the patient's size and typical consumption. Administer mixed with medical food as a bolus via feeding tube; do not add directly to the feeding bag. 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. 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 in the trash; do not pour down the sink or save for later use.
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 9 months 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. Once prepared for administration, discard any part not consumed within 24 hours in trash; do not pour down the sink.
Treatment of molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD) as a calorie and fatty acid source (FDA approved in all ages).
None known.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the Lexicomp drug interactions program by clicking on the “Launch drug interactions program” link above.
Orlistat: May decrease serum concentrations of the active metabolite(s) of Triheptanoin. Risk X: Avoid combination
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).
Total caloric intake during dosage titration (especially in patients with GI adverse reactions); routinely monitor containers, dosing components, or utensils for degradation.
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.
Protein binding: Heptanoate: ~80%.
Metabolism: Triheptanoin: Extensively hydrolyzed to heptanoate and glycerol via pancreatic lipases in intestines; Heptanoate: Metabolized to beta-hydroxypentanoate and beta-hydroxybutyrate in the liver.
Time to peak: Heptanoate: Multiple peak concentrations are observed following oral administration.
Excretion: Urine (minimally as unchanged drug and metabolites).
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