Dojolvi — Medical Mutual

Initial criteria

• The diagnosis of LC-FAOD has been molecularly confirmed by at least TWO of the following diagnostics [documentation required]: a) Disease specific elevation of acylcarnitines on a newborn blood spot or in plasma; AND/OR b) Low enzyme activity in cultured fibroblasts; AND/OR c) Genetic testing showing one or more known pathogenic mutations in CPT2, ACADVL, HADHA, or HADHB; AND
• The patient has a history of clinical manifestations of LC-FAOD, including one episode of at least ONE of the following, unless diagnosed by newborn screen (NBS) [documentation required]: a) Hypoglycemia b) Hepatopathy c) Skeletal myopathy d) Rhabdomyolysis e) Cardiomyopathy; AND
• Dojolvi is prescribed by or in consultation with a specialist in genetic metabolic disorders; AND
• The patient will not use any other medium-chain triglyceride products concomitantly with Dojolvi; AND
• The patient does not have pancreatic insufficiency; AND
• The dosage of Dojolvi will not exceed 35% of the patient’s total daily caloric intake (DCI); AND
• The patient meets one of the following: a) Currently managed on a stable treatment regiment, which may include a low-fat, high-carbohydrate diet, avoidance of fasting, and/or medium-chain triglyceride (MCT) oil; OR b) Newly diagnosed with LC-FAOD

Reauthorization criteria

• Dojolvi is prescribed by or in consultation with a specialist in genetic metabolic disorders; AND
• The patient has documented clinical improvement, per the prescribing physician (e.g. gross motor development/motor function for infants and young children, exercise tolerance and endurance for older children and adults, and a decrease in the frequency of major medical episodes of hypoglycemia, rhabdomyolysis, and exacerbation of cardiomyopathy).

Approval duration

6 months initial, 6 months reauth