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Dojolvi Prior Authorization Program Summary
Policy Number: PH-1138
This prior authorization applies to Blue Partner, Commercial, GenPlus, NetResults A series, SourceRx and Health Insurance Marketplace formularies.
POLICY REVIEW CYCLE
Effective Date |
Date of Origin |
1/1/2024 |
|
FDA APPROVED INDICATIONS AND DOSAGE
Agent(s) |
FDA Indication(s) |
Notes |
Ref# |
Dojolvi® (triheptanoin) Oral liquid |
A source of calories and fatty acids for the treatment of pediatric and adult patients with molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD) |
|
1 |
See package insert for FDA prescribing information: https://dailymed.nlm.nih.gov/dailymed/index.cfm
CLINICAL RATIONALE
Long Chain Fatty Acid Oxidation Disorders |
Long-chain fatty acid oxidation disorders (LCFAOD) is one of the most severe categories of fatty acid oxidation disorders (FAOD) and often present within a few days of life, though milder disease can have an onset in adolescents or adulthood. LCFAOD consists of a family of rare genetic disorders caused by impaired fatty acid metabolism pathways. LCFAOD can manifest with severe symptoms including cardiomyopathy, arrhythmia, skeletal myopathy, rhabdomyolysis, transaminitis, liver failure, and retinal degeneration.(2) FAOD are often captured as part of newborn screenings (NBS). A plasma acylcarnitine profile is necessary for diagnosis following an abnormal NBS. DNA testing is considered standard for confirmation and can be helpful in genotype/phenotype correlations. DNA sequencing may reveal variants of uncertain significance, so further investigation of enzyme activity through fibroblast or lymphocyte testing may provide additional information of functional significance.(2,4,5) Management of FAOD involve prevention of metabolic decompensation which includes avoidance of prolonged fasting and maintenance of a constant energy supply via carbohydrates during catabolism.(2) The diet of individuals with LCFAOD should be high in carbohydrates and low in long-chain fats. Medium chain fatty acid supplements are provided as a source for beta-oxidation.(3) |
Safety(1) |
Triheptanoin carries no contraindications nor black box warnings. Dojolvi is not compatible with certain plastics and should not be prepared or administered using containers, dosing syringes or measuring cups made of polystyrene or polyvinyl chloride (PVC) plastics. |
REFERENCES
Number |
Reference |
1 |
Dojolvi prescribing information. Ultragenyx Pharmaceutical Inc. November 2021. |
2 |
Vockley J, et al. Overview of Fatty Acid Oxidation Disorders. UpToDate. Literature review current through June 2023. Last updated April 2022. |
3 |
Vockley J, et al. Specific Fatty Acid Oxidation Disorders. UpToDate. Literature review current through June 2023. Last updated January 2022. |
4 |
Knottnerus SJG, Bleeker JC, Wust RCI, et al. Disorders of Mitochondrial Long-Chain Fatty Acid Oxidation and the Carnitine Shuttle. Rev Endocr Metab Disord. 2018;19(1):93-106. |
5 |
Merritt JL, Norris M, Kanungo S. Fatty Acid Oxidation Disorders. Ann Transl Med. 2018;6(24):473. |
POLICY AGENT SUMMARY PRIOR AUTHORIZATION
Target Brand Agent(s) |
Target Generic Agent(s) |
Strength |
Targeted MSC |
Available MSC |
Final Age Limit |
Preferred Status |
|
||||||
Dojolvi |
triheptanoin oral liquid |
100 % |
M ; N ; O ; Y |
N |
|
|
CLIENT SUMMARY – PRIOR AUTHORIZATION
Target Brand Agent Name(s) |
Target Generic Agent Name(s) |
Strength |
Client Formulary |
Dojolvi |
triheptanoin oral liquid |
100 % |
Blue Partner ; Commercial ; GenPlus ; Health Insurance Marketplace ; NetResults A Series ; SourceRx |
PRIOR AUTHORIZATION CLINICAL CRITERIA FOR APPROVAL
Module |
Clinical Criteria for Approval |
|
Initial Evaluation Target Agent will be approved when ALL of the following are met:
Length of Approval: 12 months Compendia Allowed: AHFS, or DrugDex 1 or 2a level of evidence
Renewal Evaluation Target Agent will be approved when ALL of the following are met:
Length of Approval: 12 months |
This pharmacy policy is not an authorization, certification, explanation of benefits or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All pharmacy policies are based on (i) information in FDA approved package inserts (and black box warning, alerts, or other information disseminated by the FDA as applicable); (ii) research of current medical and pharmacy literature; and/or (iii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.
The purpose of Blue Cross and Blue Shield of Alabama’s pharmacy policies are to provide a guide to coverage. Pharmacy policies are not intended to dictate to physicians how to practice medicine. Physicians should exercise their medical judgment in providing the care they feel is most appropriate for their patients.
Neither this policy, nor the successful adjudication of a pharmacy claim, is guarantee of payment.
Commercial _ PS _ Dojolvi _PA _ProgSum_ 1/1/2024