CurrentCignaPolicy N/A

Metabolic Disorders - Dojolvi Prior Authorization Policy

Defines prior authorization requirements and medical necessity criteria for coverage of Dojolvi (triheptanoin oral liquid) for treatment of molecularly confirmed long-chain fatty acid oxidation disorders for Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyMetabolic Disorders - Dojolvi Prior Authorization Policy

Policy CodePolicy N/A

Change TypeNo material change

Effective DateN/A

Next Review DateJul 16, 2025

Key ActionObtain prior authorization and document molecular confirmation of LC-FAOD plus specialist involvement; approvals are for 1 year when criteria are met.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

1 yearlength of approval when criteria met

molecular confirmationdiagnostic requirement

specialistprescriber requirement

prohibitedconcomitant MCTs

Coverage Criteria for Dojolvi (triheptanoin)

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Main criteria

A) Molecular confirmation (require at least TWO of the following): i. Disease-specific elevations of acylcarnitines on a newborn blood spot or in plasma; OR ii. Enzyme activity assay in cultured fibroblasts or lymphocytes below the lower limit of the normal reference range for the reporting laboratory (examples: CPT-2, VLCAD, LCHAD/TFP); OR iii. Genetic testing demonstrating a pathogenic variant in a gene associated with long-chain fatty acid oxidation disorders (examples: CPT2, ACADVL, HADHA, HADHB).2
Examples of genes and enzyme assays are provided in the source document.
B) Concomitant therapy: Patient will not use any other medium-chain triglyceride products concomitantly with Dojolvi.
C) Clinical justification (at least ONE): i. According to the prescriber, the patient has had inadequate efficacy or significant intolerance to an over-the-counter medium-chain triglyceride product (e.g., nutraceutical supplements) [other than Dojolvi]; OR ii. According to the prescriber, the patient has a history of at least one severe or recurrent manifestation of LC-FAOD (cardiomyopathy, rhabdomyolysis, or hypoglycemia); OR iii. Patient is currently receiving Dojolvi.1
D) Prescriber specialty: Medication is prescribed by or in consultation with a metabolic disease specialist or a physician who specializes in the management of long-chain fatty acid oxidation disorders.

Approval note: Prior authorization is required; approvals are provided for 1 year when all criteria are met.

Refer to policy statement for prior authorization requirement and approval duration.

Coverage of Dojolvi (triheptanoin oral liquid) is limited to the treatment of molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAODs) when the medical necessity criteria in this policy are met. Prior authorization is required and, when all criteria are satisfied, approvals are issued for 1 year. The policy and its criteria will be updated as new published data become available.

Prior Authorization and Provider Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required. Approvals are provided for 1 year when criteria are met. Because of the specialized evaluation and monitoring required, Dojolvi must be prescribed by or in consultation with a metabolic disease specialist or a physician who specializes in long-chain fatty acid oxidation disorders.

Approval duration: 1 year when all criteria are met
Prescriber requirement: metabolic disease specialist or physician specializing in LC-FAOD

Step Therapy

Step Through OTC MCTs

Step therapy requires documentation of inadequate efficacy or intolerance to over-the-counter (OTC) medium-chain triglyceride (MCT) products prior to approval, unless the patient has a history of severe or recurrent manifestations of LC-FAOD or is already receiving Dojolvi.

Provider must document inadequate efficacy or significant intolerance to OTC MCT product (e.g., nutraceutical supplements) OR
Provider may document history of ≥1 severe or recurrent LC-FAOD manifestation (cardiomyopathy, rhabdomyolysis, or hypoglycemia) OR
Patient currently receiving Dojolvi

Documentation Required

Required Documentation

Required documentation includes molecular confirmation of a long-chain fatty acid oxidation disorder by supported methods and evidence that no other MCT products will be used concomitantly with Dojolvi.

Molecular confirmation by at least TWO of the following: disease-specific elevations of acylcarnitines on newborn blood spot or plasma; enzyme activity assay below laboratory reference range (e.g., CPT-2, VLCAD, LCHAD, TFP); OR genetic testing demonstrating a pathogenic variant in a gene associated with LC-FAOD (e.g., CPT2, ACADVL, HADHA, HADHB).
Statement that patient will not use any other medium-chain triglyceride products concomitantly with Dojolvi.
Prescriber attestation that Dojolvi is prescribed by or in consultation with an appropriate specialist.

Denial Risk

Triggers for Denial

Denial may be issued if diagnostic or documentation requirements are not met, or if the patient will use other MCT products concomitantly with Dojolvi.

Failure to provide molecular confirmation of LC-FAOD by supported methods (as detailed above)
No documentation of inadequate efficacy or intolerance to OTC MCTs when required and no qualifying severe/recurrent manifestations or prior Dojolvi use
Concomitant use of other medium-chain triglyceride products with Dojolvi

Background

Long-chain fatty acid oxidation disorders (LC-FAODs) are inherited, autosomal recessive metabolic conditions in which the body cannot properly oxidize long-chain fatty acids, often due to defects in enzymes such as CPT2, ACADVL (VLCAD), HADHA (LCHAD/TFP), or HADHB. Clinical presentation is heterogeneous, ranging from neonatal onset to manifestations in later life, and management options may include dietary modification and specialized medium-chain triglyceride supplementation such as triheptanoin (Dojolvi).

Definitions

inv-11: LC-FAODs definition

DefinitionLong‑chain fatty acid oxidation disorders (LC‑FAODs) are autosomal recessive inherited metabolic disorders in which the body cannot properly oxidize long‑chain fatty acids in mitochondria.

Common enzyme defects (examples)Carnitine palmitoyl transferase 2 (CPT2); ACADVL (very‑long‑chain acyl‑CoA dehydrogenase, VLCAD); HADHA (long‑chain 3‑hydroxyacyl‑CoA dehydrogenase, LCHAD) as part of mitochondrial trifunctional protein (TFP); HADHB.

Typical diagnostic methodsNewborn screening acylcarnitines, plasma acylcarnitine measurement, enzyme activity assays in fibroblasts or lymphocytes, and genetic testing.

Clinical noteOnset can occur from the neonatal period to adulthood with heterogeneous manifestations; genetic findings may require functional enzyme assays for confirmation.