Ert Batten Brineura denied as not FDA-approved for this use by Cigna?

Cigna's specific coverage criteria for ert batten brineura are defined in its own published medical/coverage policy and the FDA-approved prescribing label. A successful appeal documents that your medical records satisfy each criterion those sources list — confirmed diagnosis, any required prior treatments (with dates and outcomes), and clinical severity. If the exact criteria weren't included with your denial, request them in writing; your appeal then maps each requirement to the matching fact in your chart.

## Why Cigna Issues a "Not FDA-Approved" Denial for Brineura — and Why It Is Almost Certainly Wrong

Brineura (cerliponase alfa) received FDA approval specifically for CLN2 Batten disease via the Accelerated Approval pathway. If Cigna has issued a "not FDA-approved" denial, the most likely explanations are: (1) an administrative coding or billing error where the drug's approval was not recognized; (2) the denial is being applied to an off-label use that does not match the exact approved indication language; or (3) a formulary-system error. Before appealing, verify the exact denial reason language in your Explanation of Benefits — sometimes "not FDA-approved" is shorthand for a coverage exclusion that has a different underlying rationale.

If the prescribed use matches the FDA-approved indication, this denial is factually incorrect and should be overturned.

## Federal Appeal Rights

Under ACA Section 2719 and ERISA Section 503, all adverse benefit determinations — including coverage denials based on an assertion that a drug lacks approval — are subject to internal and external review. A factually incorrect denial (drug is FDA-approved and is being used within its approved indication) is among the strongest categories for external review. The Independent Review Organization (IRO) external-review process is available after internal exhaustion, within approximately a 4-month window from the denial. Expedited review is available when the standard timeline would seriously jeopardize health.

## Appeal Process and Timeline

1. Immediate internal appeal — attach the FDA approval letter and the current FDA-approved prescribing label for Brineura as exhibits. State clearly that the drug holds FDA approval for the exact indication being prescribed. 2. Request the specific denial basis — ask Cigna to identify the specific approval-status determination it relied upon, so you can rebut it directly. 3. External review — if the internal appeal is denied, escalate to IRO review. External reviewers can independently verify FDA approval status.

## Documentation to Gather

• FDA approval documentation: the current Brineura (cerliponase alfa) prescribing label, available from the FDA's Drugs@FDA database, showing the approved indication.
• Diagnosis confirmation: genetic and enzymatic lab reports confirming CLN2 disease matching the approved indication.
• Prescriber letter: stating that the drug is being prescribed within its FDA-approved indication and that the patient meets the criteria described in the prescribing label.
• Billing/coding review: confirm that the correct HCPCS/NDC code was submitted and matches what Cigna's system expects for Brineura.

## Criteria-Mapping Structure

The mapping is direct: (1) FDA approval status — attach the prescribing label showing approval; (2) Indication match — show the patient's confirmed diagnosis against the label's approved indication language; (3) Cigna's policy — pull the applicable Cigna coverage policy and confirm whether it recognizes the FDA approval. If Cigna's policy text itself is outdated or incorrect, flag that discrepancy explicitly in the appeal letter.