Dojolvi (triheptanoin oral liquid – Ultragenyx) — Cigna

Initial criteria

• Patient has a molecularly confirmed diagnosis of a long-chain fatty acid oxidation disorder based on at least TWO of the following (i, ii, or iii): 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; OR iii. Genetic testing demonstrating a pathogenic variant in a gene associated with long-chain fatty acid oxidation disorders
• Patient will not use any other medium-chain triglyceride products concomitantly with Dojolvi
• Patient meets at least ONE of the following (i, ii, or iii): 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 long-chain fatty acid oxidation disorders (i.e., cardiomyopathy, rhabdomyolysis, or hypoglycemia); OR iii. Patient is currently receiving Dojolvi
• The 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 duration

1 year