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    Frequently asked questions

    FIND ANSWERS TO FREQUENTLY ASKED QUESTIONS ABOUT DOJOLVI

    No, DOJOLVI is not limited to patients with severe symptoms of LC-FAOD. DOJOLVI (triheptanoin) is the first and only FDA-approved treatment for all 6 LC-FAOD enzyme deficiency types and for patients of all ages diagnosed with LC-FAOD.1

    Explore use of DOJOLVI in all 6 types of LC-FAOD

    An illustrative case based on real patients describes a scenario where a neonate was started on DOJOLVI after acylcarnitine testing and a genetic test confirmed LC-FAOD.

    The safety and effectiveness of DOJOLVI have been established in pediatric patients. Review DOJOLVI Patient Case Studies for more details.1

    DOJOLVI Patient Case Studies

    If a patient has GI upset or difficulty tolerating 1/4 of the total daily dosage at one time, consider more frequent, smaller doses.1

    Get more details about adjusting DOJOLVI dosing

    Ultragenyx is committed to patients with rare diseases, which is why we created UltraCare® Patient Services for DOJOLVI—your patients’ guide throughout their treatment journeys.

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

    • Gain access to DOJOLVI (triheptanoin)

    • Determine eligibility for financial and patient assistance programs
    • Utilize patient support program resources

    Get more details about UltraCare Patient Services

    Check in with your patients prescribed DOJOLVI on a regular basis, especially during periods of change that can cause metabolic fluctuations that require dose adjustments. Examples include cold and allergy seasons, moving, going to college, or taking a trip.1-5

    Find out more about adjusting dosing to suit your patients’ needs.

    Before restarting a patient on DOJOLVI, have a conversation around why the patient stopped taking it. Consider if a dosage change or adjustment to their daily administration routine could relieve their concerns. Otherwise, the patient should take DOJOLVI as prescribed according to the Prescribing Information and Instructions for Use.1

    Patients taking another medium-chain triglyceride (MCT) product must discontinue it prior to the first dose of DOJOLVI. 1

    Learn more about starting patients on DOJOLVI

    References:

    1. DOJOLVI (triheptanoin) US Prescribing Information; October 2023.
    2. Merritt JL 2nd, MacLeod E, Jurecka A, Hainline B. Rev Endocr Metab Disord. 2020;21(4):479-493.
    3. Baker JJ, Burton BK. touchREV Endocrinol. 2021;17(2):108-111.
    4. Wensveen FM, Šestan M, Polić B. Cell Mol Immunol. 2024;21(9):1051-1065.
    5. Fujihira K, Takahashi M, Wang C, Hayashi N. Front Nutr. 2023;10:1192223.

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    FOR US HEALTHCARE PROFESSIONALS

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    INDICATION

    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).

    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 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 distension, abdominal pain, 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. 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.
    • 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 maternal condition.

    PATIENT COUNSELING INFORMATION

    • Instruct 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.

    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 for a complete discussion of the risks associated with DOJOLVI.

    LESS -

    INDICATION

    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).

    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 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 distension, abdominal pain, 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. 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.
    • 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 maternal condition.

    PATIENT COUNSELING INFORMATION

    • Instruct 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.

    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 for a complete discussion of the risks associated with DOJOLVI.

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