Triheptanoin (Dojolvi) Form

Triheptanoin (Dojolvi™) is medium-chain triglyceride.
FDA Approved Indication(s) Dojolvi is 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). Policy/Criteria Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria.
It is the policy of health plans affiliated with Centene Corporation® that Dojolvi is medically necessary when the following criteria are met:
I. Initial Approval Criteria
A. Long-Chain Fatty Acid Oxidation Disorders (must meet all):

1. Diagnosis of a LC-FAOD (see Appendix D);
   2. Prescribed by or in consultation with an endocrinologist, geneticist, or metabolic disease specialist;
2. Member has gene mutation(s) associated with LC-FAOD (see Appendix D for examples);
3. Medical justification supports the inability to use medium-chain triglyceride (MCT) oil (e.g., contraindications to excipients in MCT oil brands);
4. Documentation of member’s daily caloric intake (DCI);
   
   6. Dose does not exceed 35% of the member’s DCI (see Appendix D).
      Approval duration: 6 months B. Other diagnoses/indications (must meet 1 or 2):

5. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: Page 1 of 6

   CLINICAL POLICY Triheptanoin CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

6. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid.
   II. Continued Therapy A. Long-Chain Fatty Acid Oxidation Disorders (must meet all):
7. Member meets one of the following (a or b): a. Currently receiving medication via Centene benefit or member has previously met initial approval criteria; b. Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations (refer to state specific addendums for CC.PHARM.03A and CC.PHARM.03B);
8. Member is responding positively to therapy (see Appendix E);
   3. If request is for a dose increase, new dose does not exceed 35% of the member’s DCI (see Appendix D). Approval duration: 12 months
      B. Other diagnoses/indications (must meet 1 or 2):
9. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

10. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid.
    III. Diagnoses/Indications for which coverage is NOT authorized:
    A. Non-FDA approved indications, which are not addressed in this policy, unless there is sufficient documentation of efficacy and safety according to the off label use policies – CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid, or evidence of coverage documents.
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    CLINICAL POLICY Triheptanoin IV. Appendices/General Information Appendix A: Abbreviation/Acronym Key DCI: daily caloric intake FDA: Food and Drug Administration LC-FAOD: long-chain fatty acid oxidation disorders MCT: medium-chain triglycerides Dose Limit/ Maximum Dose Varies Appendix B: Therapeutic Alternatives
    This table provides a listing of preferred alternative therapy recommended in the approval criteria. The drugs listed here may not be a formulary agent for all relevant lines of business and may require prior authorization.
    Dosing Regimen Drug Name MCT oil 0.5 g/kg/day in three divided doses, which can be gradually increased to 1.0 to 1.5 g/kg per day Therapeutic alternatives are listed as Brand name® (generic) when the drug is available by brand name only and generic (Brand name®) when the drug is available by both brand and generic. Appendix C: Contraindications/Boxed Warnings None reported
    Appendix D: General Information
    • LC-FAOD examples and associated genetic mutation locations: LC-FAOD examples trifunctional protein deficiency (TFPD) long-chain 3-hydroxyacyl CoA dehydrogenase deficiency (LCHADD) very long-chain acyl CoA dehydrogenase deficiency (VLCADD) carnitine palmitoyltransferase-1 or 2 deficiency (CPT1/2D) carnitine-acylcarnitine translocase deficiency (CACTD) Associated Genes HADHA, HADHB HADHA ACADVL CPT1A, CPT2 SLC25A20 • Dosage calculation: o Caloric value of Dojolvi = 8.3 kcal/mL o Round the total daily dosage to the nearest whole number o Total Daily Dose (in mLs) = Patient’s DCI ( # kcal) x Target # % dose of DCI 8.3 kcal/mL of Dojolvi Appendix E: Response to Therapy
    • In adults, examples may include but are not limited to a reduced incidence of muscle myalgias, rhabdomyolysis, exercise intolerance, cardiac symptoms, hypoglycemia, hepatomegaly symptoms, or vomiting and dehydration precipitated by infections. In pediatrics, early treatment may not prevent symptomatology. Positive response can be demonstrated by continued tolerance to Dojolvi administration. • Page 3 of 6

    CLINICAL POLICY Triheptanoin V. Dosage and Administration
    Indication Dosing Regimen LC-FAOD Target daily dosage of Dojolvi is up to 35% of the patient’s total prescribed DCI divided into at least four doses and administered at mealtimes or with snacks Maximum Dose 35% of patient’s DCI VI. Product Availability
    Oral liquid: 500 mL (100% w/w of triheptanoin) VII.