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Now approved.

Introducing DOJOLVI (triheptanoin).

DOJOLVI (triheptanoin) is the first and only FDA-approved treatment for LC-FAOD.

CPT I (carnitine palmitoyltransferase I) deficiency
CPT I(carnitine palmitoyltransferase I) deficiency

Select a type to learn more.

LC-FAOD=long-chain fatty acid oxidation disorders.

Prescribe DOJOLVI

Complete the Start Form with your patient and fax it to 14157237474.

For additional support, you may also call our UltraCare Guides at 1-888-756-8657.


Access DOJOLVI

UltraCare® Patient Services provides a suite of services to help patients and caregivers:

  • Gain access to DOJOLVI
  • Determine eligibility for financial and patient assistance programs
  • Utilize patient support program resources
  • Gain access to DOJOLVI
  • Determine eligibility for financial and patient assistance programs
  • Utilize patient support program resources

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LC-FAOD Defined

LC-FAOD are a group of rare, often severe, and life-threatening autosomal recessive disorders that result from defective enzymes involved in the mitochondrial transport and catabolism of long-chain fatty acids (LCFAs).1-4

Each type of LC-FAOD is named for the specific enzyme that is affected.4

Select a type to learn more.

CPT I(carnitine palmitoyltransferase I) deficiency

References: 1. Knottnerus SJG, Bleeker JC, Wüst RCI, et al. Rev Endocr Metab Disord. 2018;19(1):93-106. 2. Wajner M, Amaral AU. Biosci Rep. 2015;36(1):e00281. 3. Lindner M, Hoffmann GF, Matern D. J Inherit Metab Dis. 2010;33(5):521-526. 4. Wanders RJ, Ruiter JP, IJLst L, Waterham HR, Houten SM. J Inherit Metab Dis. 2010;33(5):479-494.

DOJOLVI IS A UNIQUE, ODD-CHAIN MEDIUM-LENGTH FATTY ACID

DOJOLVI is a synthetic medium odd-chain (C7) triglyceride consisting of three odd-chain, 7-carbon-length fatty acids.

It is the first and only FDA-approved treatment for patients of all ages diagnosed with LC-FAOD.

DOJOLVI is a synthetic medium odd-chain (C7) triglyceride consisting of three odd-chain, 7-carbon-length fatty acids.

It is the first and only FDA-approved treatment for patients of all ages diagnosed with LC-FAOD.


dojolvi molecule

Each 7-carbon fatty acid (heptanoate) in DOJOLVI provides a source of calories and fatty acids to bypass the enzyme deficiencies in LC-FAOD for energy production and replacement.

DOJOLVI dosing and administration
Calculating the DOJOLVI dose

The recommended target daily dosage of DOJOLVI is up to 35% of the patient’s total prescribed daily caloric intake (DCI), divided into at least 4 doses and administered at mealtimes or with snacks.*

To find the DOJOLVI dose for your patient:

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1. MULTIPLY

MULTIPLY the total DCI (in kcal) by the target percentage of the DCI that will be provided by DOJOLVI

Total DCI (kcal) x target % of DCI

2. DIVIDE

DIVIDE by 8.3 kcal/mL, the caloric value of DOJOLVI

DIVIDE by 8.3 kcal/mL, the caloric value of DOJOLVI

3. ROUND

ROUND the calculated total daily dosage of DOJOLVI (in mL) to the nearest whole number

total daily dosage (in mL)

4. DIVIDE

DIVIDE the total daily dosage into at least 4 approximately equal doses to be given at each interval mixed thoroughly with a meal or snack

mL per dose

In order to reach a target daily dosage, patients may require an increase in their total fat intake. All patients treated with DOJOLVI should be under the care of a clinical specialist knowledgeable in appropriate disease-related dietary management based upon current nutritional recommendations.

The neonatal population may require higher fat intake and therefore an increased amount of DOJOLVI. Current nutritional recommendations should be considered when dosing the neonatal population.

Initiating and titrating DOJOLVI

For patients not currently taking a medium-chain triglyceride (MCT) product:

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Initiate DOJOLVI at a total daily dosage of approximately 10% DCI, divided into at least 4 times per day, and increase to the recommended total daily dosage of up to 35% DCI over a period of 2 to 3 weeks.


For patients switching from another MCT product:

mct icon

Discontinue use of MCT products before starting DOJOLVI.

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Initiate DOJOLVI at the last tolerated daily dosage of MCT divided into at least 4 times per day. Increase the total daily dosage by approximately 5% DCI every 2 to 3 days until the target dosage of up to 35% DCI is achieved.

Tolerability:

If a patient has difficulty tolerating 1/4 of the total daily dosage at one time, more frequent, smaller doses may be considered.

Monitor patients’ total caloric intake during dosage titration, especially in patients with gastrointestinal adverse reactions, and adjust all components of the diet as needed.

If a patient experiences gastrointestinal adverse reaction(s), consider dosage reduction until the gastrointestinal symptoms resolve. If a patient is unable to achieve the target daily dosage of up to 35% DCI during dosage titration, maintain the patient at the maximum tolerated dosage.

Administering DOJOLVI

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Administer DOJOLVI at least 4 times per day orally or enterally via a silicone or polyurethane feeding tube


syringe icon

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

  • Compatible materials include stainless steel, glass, high-density polyethylene (HDPE), polypropylene, low-density polyethylene (LDPE), polyurethane, and silicone
  • Do not prepare or administer DOJOLVI using containers, dosing syringes, or measuring cups made of polystyrene or polyvinyl chloride (PVC) plastics
  • Regularly monitor the containers, dosing components, or utensils that are in contact with DOJOLVI to ensure proper functioning and integrity

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Add the prescribed amount of DOJOLVI to a clean bowl, cup, or container made of compatible materials, which contains an appropriate amount of semisolid food or liquid


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To avoid gastrointestinal upset, always mix DOJOLVI thoroughly with semisolid food or liquids; DOJOLVI can be mixed into:

  • 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, or baby food, etc.

The mixture may be stored for up to 24 hours in the refrigerator

If a dose is missed, take the next dose as soon as possible, with subsequent doses taken at 3- to 4-hour intervals. Skip the missed dose if it will not be possible to take all 4 doses in a day.

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.

Avoid administration of DOJOLVI in patients with pancreatic insufficiency.

For complete information on the preparation and administration of DOJOLVI, please refer to Section 2.3 of the full Prescribing Information.

storing dojolvi

DOJOLVI is a clear, colorless to light yellow liquid supplied in 500 mL bottles containing 100% w/w of triheptanoin.

temperature icon

Store DOJOLVI upright at room temperature between 68° and 77°F (20° and 25°C)

freeze icon

Do not freeze DOJOLVI

expire icon

Opened bottles of DOJOLVI can be used for up to 9 months after opening, but not beyond the expiration date on the bottle

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Do not store DOJOLVI in containers made of polystyrene or polyvinyl chloride (PVC)

DOJOLVI™ (triheptanoin) product image

dosing guide

Provide your patient and their caregivers with a step-by-step guide for storing, administering, and keeping track of DOJOLVI doses.

Download the Guide

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FOR US HEALTHCARE PROFESSIONALS

INDICATION

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

IMPORTANT SAFETY INFORMATION

WARNINGS AND PRECAUTIONS

Feeding Tube Dysfunction

  • Feeding tube performance and functionality can degrade over time depending on usage and environmental conditions. In clinical trials, feeding tube feeding 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). Regularly monitor the feeding tube to ensure proper functioning and integrity.

Intestinal 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.

ADVERSE REACTIONS

Gastrointestinal (GI)

  • The most common GI-related adverse reactions reported in the pooled safety population of Studies 1 and 2 were abdominal pain (abdominal discomfort, abdominal pain, abdominal distension, abdominal pain upper, GI pain) [60%], diarrhea [44%], vomiting [44%], and nausea [14%].

DRUG INTERACTIONS

Pancreatic Lipase Inhibitors

  • Co-administration of triheptanoin with a pancreatic lipase inhibitor (e.g., orlistat) may reduce exposure to the triheptanoin metabolite, heptanoate, and reduce the clinical effect of triheptanoin. Avoid co-administration of DOJOLVI with pancreatic lipase inhibitors.

USE IN SPECIFIC POPULATIONS

Pregnancy and Lactation

  • 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. Advise women to report pregnancies to Ultragenyx Pharmaceutical Inc. at 1-888-756-8657.
  • There are no data on the presence of triheptanoin or its metabolites in human or animal milk, the effects on the breastfed infant, or the effects on milk production. The developmental and health benefits of breastfeeding should be considered along with the clinical need for DOJOLVI and any potential adverse effect on the breastfed infant from DOJOLVI or from the underlying condition.

PATIENT COUNSELING INFORMATION

  • Advise the patient or caregiver to read the FDA-approved patient labeling, which includes information on the appropriate oral or feeding tube preparation, administration, and storage.
  • Advise the patient or caregiver to regularly inspect the feeding tube for proper functioning and integrity and report to the healthcare provider if any issues are identified.
  • Inform the patient or caregiver that pancreatic insufficiency may reduce clinical effect of DOJOLVI. Any known pancreatic insufficiency should be reported to the healthcare provider.

You may report side effects to Ultragenyx Pharmaceutical Inc. at 1-888-756-8657 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.


Please see full Prescribing Information, including the Patient Information leaflet, for a complete discussion of the risks associated with DOJOLVI.

INDICATION

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

IMPORTANT SAFETY INFORMATION

WARNINGS AND PRECAUTIONS

Feeding Tube Dysfunction

  • Feeding tube performance and functionality can degrade over time depending on usage and environmental conditions. In clinical trials, feeding tube feeding 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). Regularly monitor the feeding tube to ensure proper functioning and integrity.

Intestinal 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.

ADVERSE REACTIONS

Gastrointestinal (GI)

  • The most common GI-related adverse reactions reported in the pooled safety population of Studies 1 and 2 were abdominal pain (abdominal discomfort, abdominal pain, abdominal distension, abdominal pain upper, GI pain) [60%], diarrhea [44%], vomiting [44%], and nausea [14%].

DRUG INTERACTIONS

Pancreatic Lipase Inhibitors

  • Co-administration of triheptanoin with a pancreatic lipase inhibitor (e.g., orlistat) may reduce exposure to the triheptanoin metabolite, heptanoate, and reduce the clinical effect of triheptanoin. Avoid co-administration of DOJOLVI with pancreatic lipase inhibitors.

USE IN SPECIFIC POPULATIONS

Pregnancy and Lactation

  • 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. Advise women to report pregnancies to Ultragenyx Pharmaceutical Inc. at 1-888-756-8657.
  • There are no data on the presence of triheptanoin or its metabolites in human or animal milk, the effects on the breastfed infant, or the effects on milk production. The developmental and health benefits of breastfeeding should be considered along with the clinical need for DOJOLVI and any potential adverse effect on the breastfed infant from DOJOLVI or from the underlying condition.

PATIENT COUNSELING INFORMATION

  • Advise the patient or caregiver to read the FDA-approved patient labeling, which includes information on the appropriate oral or feeding tube preparation, administration, and storage.
  • Advise the patient or caregiver to regularly inspect the feeding tube for proper functioning and integrity and report to the healthcare provider if any issues are identified.
  • Inform the patient or caregiver that pancreatic insufficiency may reduce clinical effect of DOJOLVI. Any known pancreatic insufficiency should be reported to the healthcare provider.

You may report side effects to Ultragenyx Pharmaceutical Inc. at 1-888-756-8657 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.


Please see full Prescribing Information, including the Patient Information leaflet, for a complete discussion of the risks associated with DOJOLVI.