CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Dojolvi (triheptanoin) — Coverage Criteria for Long‑Chain Fatty Acid Oxidation Disorders

Covers clinical criteria and authorization requirements for Dojolvi (triheptanoin) for treatment of molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD) in pediatric and adult members; applies to members of Neighborhood Health Plan of Rhode Island.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyDojolvi (triheptanoin) — Coverage Criteria for LC-FAOD

Policy CodePolicy N/A

Change TypeMinor / no material changes

Effective DateN/A

Next Review DateN/A

Key ActionObtain prior authorization with documentation of molecularly confirmed LC-FAOD and required diagnostic evidence.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 moinitial auth

12 mocontinuation auth

≥1prior hospitalization/ER

≥2diagnostic criteria

Coverage Criteria for Dojolvi (triheptanoin)

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyDojolvi (triheptanoin) — Coverage Criteria for LC-FAOD

Policy CodePolicy N/A

Change TypeMinor / no material changes

Effective DateN/A

Next Review DateN/A

Key ActionObtain prior authorization with documentation of molecularly confirmed LC-FAOD and required diagnostic evidence.

SourceLink

On This Page

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Continuation Therapy

Authorization of 12 months may be granted when ALL of the following are met:

Continuation Authorization - all criteria: Documented diagnosis of CPT2, VLCAD, LCHAD, or TFP deficiency; member is currently receiving Dojolvi and is experiencing benefit from therapy evidenced by disease stability or improvement (examples: improvement in cardiomyopathy, glycemic control, exercise tolerance, or reduction in episodes of cardiomyopathy, rhabdomyolysis, hypoglycemia, or hospitalizations).

Provider Requirements and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required. Initial authorizations may be granted for up to 6 months when all required initial criteria are met. Continuation/renewal authorizations may be granted for up to 12 months for members who are currently receiving the medication and demonstrate clinical benefit (disease stability or improvement such as improved cardiomyopathy, improved glycemic control, improved exercise tolerance, or reduced episodes/hospitalizations for cardiomyopathy, rhabdomyolysis, or hypoglycemia).

Prior authorization required for initial therapy: up to 6 months
Continuation authorization: up to 12 months

Denial Risk

Indication Restricted to LC-FAOD

Therapy is indicated only for patients with molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD) — specifically: carnitine palmitoyltransferase type 2 (CPT2) deficiency, very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, long-chain L-3 hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, or trifunctional protein (TFP) deficiency. All other indications are considered experimental/investigational and not medically necessary.

Documentation Required

Diagnostic Documentation Required

Authorization requests must include diagnostic documentation confirming LC-FAOD. Acceptable documentation includes molecular/ genetic confirmation (report of one or more pathogenic variants in CPT2, ACADVL, HADHA or HADHB) and supporting biochemical or functional evidence such as elevated disease‑specific acylcarnitines on newborn screen or plasma (e.g., CPT2: elevated C16 and/or C18:1; VLCAD: elevated C14:1 and related acylcarnitines; LCHAD/TFP: elevated C16‑OH and/or C18‑OH) or low enzyme activity in cultured fibroblasts. Failure to provide required diagnostic documentation may result in denial of the request.

Molecular/genetic test results showing pathogenic variant(s) in CPT2, ACADVL, HADHA or HADHB
Supporting biochemical evidence: disease-specific elevated acylcarnitines (newborn blood spot or plasma)
Optional/confirmatory testing: low enzyme activity in cultured fibroblasts

Definitions

LC-FAOD — definition and examples

DefinitionGenetic disorders impairing metabolism of long‑chain fatty acids resulting in energy deficiency; includes carnitine palmitoyltransferase type 2 (CPT2) deficiency, very‑long‑chain acyl‑CoA dehydrogenase (VLCAD) deficiency, long‑chain 3‑hydroxyacyl‑CoA dehydrogenase (LCHAD) deficiency, and trifunctional protein (TFP) deficiency.

Examples (specific diagnoses)CPT2, VLCAD (ACADVL), LCHAD (HADHA), TFP (HADHB)

Typical clinical presentationsRhabdomyolysis, cardiomyopathy, and hypoglycemia may occur in affected patients.

Diagnostic evidenceDiagnosis is established using elevated disease‑specific acylcarnitine species, low enzyme activity in cultured fibroblasts, or identification of pathogenic mutations in CPT2, ACADVL, HADHA, or HADHB (at least two criteria documented for authorization).

Dietary management contextManagement includes a low‑fat/high‑carbohydrate diet with medium‑chain triglyceride (MCT) supplementation prior to therapy initiation.

OpenPayer

Dojolvi (triheptanoin) — Coverage Criteria for Long‑Chain Fatty Acid Oxidation Disorders

Coverage Criteria for Dojolvi (triheptanoin)

Provider Requirements and Documentation

Definitions

Background