Ert Batten Brineura denied for failing step therapy 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 Applies Step Therapy to Brineura — and Why That Is Inappropriate for CLN2 Disease

Step therapy requires that a patient try one or more other medications before the requested drug will be covered. When Cigna applies step-therapy criteria to Brineura (cerliponase alfa) for CLN2 Batten disease, the policy is almost certainly being applied in error — or being applied through an automated system that does not account for the unique nature of this condition. CLN2 Batten disease is an ultra-rare, rapidly fatal pediatric neurodegenerative disorder with no approved disease-modifying alternatives. There is no therapeutic substitute to "step through." Step-therapy logic that requires trial of another agent before Brineura has no clinical or regulatory basis for this indication.

Many states have enacted step-therapy override laws that require insurers to grant exceptions when no clinically appropriate alternative exists, when a patient has already tried the required alternative, or when requiring the step would cause serious harm. Confirm whether your state's law applies to your plan type.

## Federal Appeal Rights

Step-therapy denials are adverse benefit determinations appealable under ACA Section 2719 and ERISA Section 503. The core legal and clinical argument is: there is no adequate alternative therapy for CLN2 disease recognized by the FDA or by the relevant specialist-society guidelines, making any step-therapy requirement inapplicable. Expedited internal and external review is appropriate given the progressive nature of the disease. External review by an IRO is available after internal exhaustion.

## Appeal Process and Timeline

1. Identify the required step — obtain from Cigna's denial letter or policy the specific drug(s) Cigna claims the patient must try first. 2. Step-therapy exception request — immediately file an exception, citing: (a) no FDA-approved alternative for CLN2 disease exists; (b) requiring a step would delay or deny the only disease-modifying therapy; and (c) state step-therapy override law protections if applicable. 3. Peer-to-peer review — the treating neurologist should request a direct conversation with Cigna's medical reviewer to explain the absence of alternatives. 4. Internal appeal and external review — if the exception is denied, escalate through the full appeal chain.

## Documentation to Gather

• FDA approval and indication: the Brineura prescribing label showing the narrow, specific approved indication (CLN2 disease).
• No alternative documentation: a prescriber letter stating explicitly that no other FDA-approved disease-modifying therapy exists for CLN2 Batten disease, and that any alternative Cigna might propose treats a different condition.
• Specialist-society support: a reference (without specific statistics) to the relevant Batten disease specialist network or metabolic neurology society's guidance on CLN2 management, supporting Brineura as the appropriate treatment.
• Disease severity: current neurologist assessments documenting the patient's progression, underscoring the urgency of initiating treatment without delay.
• State step-therapy law: if your state has a step-therapy override statute, cite it by name in the appeal.

## Criteria-Mapping Structure

Brineura's step-therapy exception case rests on a single, decisive point: the absence of any adequate alternative. Frame the criteria map as: (1) Cigna's step requirement — identify the named drug; (2) FDA approval check — confirm whether that drug is approved for CLN2 disease (it will not be); (3) Clinical equivalence — cite the prescribing label to show no therapeutic substitute exists. This structure makes the exception argument undeniable on its face.