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 1–415–723–7474.
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.
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
2. DIVIDE
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
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
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:
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:
Discontinue use of MCT products before starting DOJOLVI.
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
Administer DOJOLVI at least 4 times per day orally or enterally via a silicone or polyurethane feeding tube
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
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
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.
Store DOJOLVI upright at room temperature between 68° and 77°F (20° and 25°C)
Do not freeze DOJOLVI
Opened bottles of DOJOLVI can be used for up to 9 months after opening, but not beyond the expiration date on the bottle
Do not store DOJOLVI in containers made of polystyrene or polyvinyl chloride (PVC)
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