Dojolvi - NC Standard

• Dojolvi™ (triheptanoin) oral liquid 

• For the treatment of pediatric and adult patients with molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD).

Initial Coverage:

1. The patient has a confirmed diagnosis of long-chain fatty acid oxidation disorders (LC-FAOD) [medical record documentation required]; AND
2. The patient has at least two of the following diagnostic criteria:
   1. Disease specific elevation of acylcarnitines on a newborn blood spot or in plasma; OR
   2. Low enzyme activity in cultured fibroblasts; OR
   3. One or more known pathogenic mutations in CPT2, ACADVL, HADHA, or HADHB; AND 
3. Dojolvi will NOT be used in combination with any other medium-chain triglyceride product and other medium-chain triglyceride products will be discontinued before the start of therapy; AND 
4. Dojolvi must be administered with a syringe or measuring cup of compatible materials; AND
5. The prescribed dose and quantity are appropriate based on intended use and FDA labeling; AND
6. The patient is being managed by or in consultation with a clinical specialist knowledgeable in appropriate disease-related dietary management based upon current nutritional recommendations; AND 
7. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies.

Duration of Approval: 180 days (6 months)

Continuation Coverage

1. The patient has been previously approved for Dojolvi with Blue Cross and Blue Shield of North Carolina (Blue Cross NC) or would have met initial criteria for approval upon the start of therapy; AND
2. The patient has had continued clinical benefit while on Dojolvi therapy (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) [medical record documentation required]; AND 
3. The prescribed dose and quantity are appropriate based on intended use and FDA labeling; AND 
4. The patient continues to be managed by or in consultation with a clinical specialist knowledgeable in appropriate disease-related dietary management based upon current nutritional recommendations.

Duration of Approval: 365 days (1 year)

All information referenced is from FDA package insert unless otherwise noted below.

Criteria and treatment protocols are reviewed annually by the Blue Cross NC P&T Committee, regardless of change. This policy is reviewed in Q1 annually.

October 2023: Original utilization management criteria issued.