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Dojolvi (triheptanoin) — CareFirst (Caremark)

Long-chain fatty acid oxidation disorders (LC-FAOD) including carnitine palmitoyltransferase type 1 (CPT1) deficiency, carnitine palmitoyltransferase type 2 (CPT2) deficiency, carnitine-acylcarnitine translocase (CACT) deficiency, very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, long-chain L-3 hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, or trifunctional protein (TFP) deficiency

Initial criteria

  • Member has a diagnosis of CPT1 deficiency, CPT2 deficiency, CACT deficiency, VLCAD deficiency, LCHAD deficiency, or TFP deficiency.
  • Prescribed by or in consultation with a physician who specializes in the treatment of enzyme or metabolic disorders.
  • Member has been receiving a low-fat/high-carbohydrate diet and medium-chain triglyceride (MCT) supplementation (e.g., MCT oil supplements, specialized formulas such as Lipistart, Monogen, Portagen, Enfaport, MCT Procal, MCT Oil, or Liquigen).
  • Member has experienced at least one hospitalization or ER visit within the past year due to rhabdomyolysis, cardiomyopathy, or hypoglycemic episodes.
  • At least two of the following diagnostic criteria are met: elevated acylcarnitine profile characteristic of disease; low enzyme activity in cultured fibroblasts; one or more known pathogenic variant(s) in CPT1A, SLC25A20, CPT2, ACADVL, HADHA, or HADHB genes.

Reauthorization criteria

  • Member continues to have an indication listed in the coverage criteria section.
  • Member is currently receiving Dojolvi through a paid pharmacy or medical benefit.
  • Member is experiencing benefit from therapy as evidenced by disease stability or improvement (e.g., improvement in cardiomyopathy, glycemic control, or exercise tolerance, or a reduction in episodes of cardiomyopathy, rhabdomyolysis, hypoglycemia, or hospitalizations).

Approval duration

Initial: 6 months; Reauthorization: 12 months