By Counterforce Health Team in Brineura (cerliponase alfa) —

How to Get Brineura (Cerliponase Alfa) Covered by Cigna in Illinois: Appeals Guide with Forms and Timelines

Answer Box: Getting Brineura (Cerliponase Alfa) Covered by Cigna in Illinois

Brineura requires prior authorization from Cigna for confirmed CLN2 disease patients ≥3 years old. The fastest path: Submit PA through Express Scripts/Accredo with TPP1 enzyme deficiency results, genetic testing, and a pediatric neurologist's letter documenting motor decline. If denied, you have 180 days for internal appeals and 4 months for Illinois external review. Start today by requesting prior authorization forms from your clinic or Cigna's provider portal.

Table of Contents

Coverage Requirements at a Glance

Requirement What It Means Where to Find It Source
Prior Authorization Required before first dose Express Scripts/Accredo portal Cigna PA Forms
Age Requirement Patient must be ≥3 years old FDA label indication FDA Access Data
Diagnosis Confirmed CLN2 disease with TPP1 deficiency Genetic testing + enzyme assay Cigna Policy
Prescriber Pediatric neurologist or metabolic specialist Medical necessity letter Cigna Policy
Site of Care Approved infusion center with neurosurgical support Hospital or specialized clinic BioMarin Guide
ICD-10 Code E75.4 (Other sphingolipidoses) Diagnosis coding Cigna Policy

Step-by-Step: Fastest Path to Approval

1. Confirm CLN2 Diagnosis (Clinic) Your pediatric neurologist orders TPP1 enzyme testing and genetic analysis. Results showing enzyme deficiency plus two pathogenic TPP1 variants confirm CLN2 disease. Timeline: 2-4 weeks for results.

2. Gather Required Documentation (Clinic Staff) Collect medical records showing symptom progression, CLN2 Clinical Rating Scale scores, brain MRI findings, and treatment history. Include ICD-10 code E75.4 in all documentation.

3. Submit Prior Authorization (Clinic) Submit PA request through Express Scripts ePrescribing or Accredo portal with complete documentation package. Timeline: 5-10 business days for standard review.

4. Schedule Intraventricular Device Placement (If Approved) Neurosurgical placement of reservoir and catheter must occur 5-7 days before first infusion. Coordinate with approved infusion center.

5. Begin Treatment (Patient + Clinic) First Brineura infusion administered at approved facility with cardiac monitoring and emergency preparedness. Each infusion takes approximately 4.5 hours.

Tip: Request expedited review (72-hour timeline) if motor decline is rapid or seizures are worsening.

Common Denial Reasons & How to Fix Them

Denial Reason How to Overturn Required Documentation
Age/diagnosis mismatch Submit updated genetic testing results TPP1 enzyme assay + genetic report showing biallelic pathogenic variants
Lack of specialist care Obtain referral to pediatric neurologist Letter from board-certified pediatric neurologist or metabolic specialist
Insufficient documentation Complete CLN2 functional assessment CLN2 Clinical Rating Scale scores showing motor decline
Out-of-network facility Request network exception Letter documenting no in-network facilities capable of ICV administration
Not medically necessary Submit comprehensive appeal with guidelines FDA labeling, peer-reviewed studies, CLN2 disease progression data

Appeals Playbook for Cigna in Illinois

Internal Appeals Process

Level 1 Internal Appeal

  • Deadline: 180 days from denial date
  • Timeline: 15 business days for standard review, 24 hours for expedited
  • How to file: Submit written appeal to Cigna with denial letter and supporting documentation
  • Required: Medical necessity letter from prescriber, updated clinical notes, CLN2 progression data

Level 2 Internal Appeal (if Level 1 denied)

  • Deadline: 60 days from Level 1 denial
  • Timeline: 15 business days for standard review
  • Review: Independent medical reviewer not involved in original decision

Illinois External Review

If internal appeals fail, Illinois residents can request binding external review under the Health Carrier External Review Act.

Eligibility:

  • Exhausted internal appeals OR carrier delayed response beyond legal timeframes
  • Adverse determination based on medical necessity, experimental/investigational status, or emergency care

Timeline:

  • File within: 4 months of final internal denial
  • Decision: 5 business days after Independent Review Organization receives complete file
  • Expedited: 72 hours for urgent cases

How to File: Submit external review request to Illinois Department of Insurance, 320 W. Washington St., Springfield, IL 62767. Include denial notices, medical records, and completed external review form.

Medical Necessity Letter Checklist

Your pediatric neurologist's letter should include:

Patient History & Diagnosis

  • CLN2 disease confirmation date and testing method
  • TPP1 enzyme deficiency results
  • Genetic testing showing biallelic pathogenic variants
  • Age at symptom onset and current age (≥3 years)

Clinical Progression

  • Serial CLN2 Clinical Rating Scale scores showing motor decline
  • Loss of ambulation milestones (walking unassisted, stairs, wheelchair dependence)
  • Seizure history and current management
  • Language regression documentation

Treatment Rationale

  • No alternative disease-modifying therapies available for CLN2
  • FDA approval for slowing motor function loss in symptomatic patients
  • Clinical trial data showing 67% reduction in motor decline vs. natural history
  • Prognosis without treatment (progressive decline, death by age 10-12)

Monitoring Plan

  • CLN2 assessments every 6 months
  • Regular brain MRI monitoring
  • Safety laboratory monitoring
  • Infection surveillance for ICV device

Counterforce Health helps clinicians draft evidence-backed appeals by analyzing denial letters and payer policies, then generating targeted rebuttals with the right medical citations and clinical facts. Their platform streamlines the prior authorization process for complex specialty medications like Brineura.

Scripts & Templates

Patient Phone Script for Cigna

"Hi, I'm calling about prior authorization for Brineura for my child with CLN2 disease. Our member ID is [number]. Can you tell me the status of our PA request submitted on [date]? If it was denied, I'd like to request the denial letter and information about filing an appeal."

Clinic Staff Peer-to-Peer Request

"I'm requesting a peer-to-peer review for [patient name], member ID [number], for Brineura coverage. This is for confirmed CLN2 disease with documented motor decline. The prescribing physician is Dr. [name], a board-certified pediatric neurologist. When is the earliest available appointment?"

Email Template for Medical Records

"Subject: Urgent - Medical Records Needed for Brineura Appeal

Dear [Provider],

We need the following records for [patient name]'s insurance appeal for Brineura:

  • CLN2 diagnostic testing results (enzyme + genetic)
  • CLN2 Clinical Rating Scale scores from all visits
  • Brain MRI reports
  • Seizure logs and medication trials

Please send within 48 hours to [fax/email]. This medication is time-sensitive for preventing irreversible neurologic decline.

Thank you, [Name and contact]"

Illinois External Review Process

Illinois provides strong patient protections through the Health Carrier External Review Act. Here's what makes Illinois unique:

Key Advantages

  • Binding decisions: If the Independent Review Organization approves coverage, Cigna must provide the treatment
  • No cost to patients: Cigna pays all external review fees
  • Physician reviewers: Board-certified specialists with relevant expertise review your case
  • Fast timeline: 5 business days for standard decisions, 72 hours for expedited

When to Use External Review

  • Internal appeals denied for "not medically necessary"
  • Experimental/investigational determination you believe is incorrect
  • Cigna delays internal appeal response beyond legal timeframes

Illinois Resources for Help

Note: Illinois has a stricter 4-month deadline for external review compared to some states. Don't delay if your internal appeal is denied.

Costs & Financial Assistance

Brineura Pricing: Approximately $702,000 annually ($27,000 per biweekly infusion), plus facility and procedure costs.

Financial Support Options

  • BioMarin RareConnections: Patient assistance program and copay support. Contact (833) 274-6342 or visit biomarin-rareconnections.com
  • CLN2 Foundation: Grants for treatment-related expenses and family support
  • National Organization for Rare Disorders (NORD): Patient assistance programs for rare disease medications
  • Illinois Medicaid: Expanded coverage may include Brineura for eligible families

FAQ

How long does Cigna prior authorization take for Brineura in Illinois? Standard PA review takes 5-10 business days. Expedited review (when motor decline is rapid) provides decisions within 72 hours.

What if Brineura is non-formulary on my Cigna plan? Request a formulary exception through Express Scripts. Since no alternatives exist for CLN2, exceptions are often approved with proper documentation.

Can I request expedited appeal if my child's condition is worsening? Yes. Document rapid motor decline or seizure escalation. Expedited internal appeals receive decisions within 24 hours, external reviews within 72 hours.

Does step therapy apply to Brineura in Illinois? No. Brineura is the only FDA-approved treatment for CLN2 disease, so step therapy requirements don't apply.

What happens if Cigna denies my external review? External review decisions are binding. If approved, Cigna must cover treatment. If denied, consider seeking a second opinion or exploring clinical trials.

Can I switch to a different Cigna plan during open enrollment? Yes, but check formulary coverage and provider networks. Some Cigna plans may have better specialty drug coverage or preferred infusion centers.

Do I need to stay in Illinois for treatment? No, but coordinate with Cigna about out-of-state infusion centers. Some specialized CLN2 treatment centers may be out-of-network and require exceptions.

How do I track my appeal status? Use Cigna's member portal or call member services. Keep detailed records of all submissions, reference numbers, and representative names. Counterforce Health also provides appeal tracking tools for complex cases.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Insurance coverage varies by plan and individual circumstances. Always consult with your healthcare provider and insurance company for specific coverage decisions. For personalized assistance with complex appeals, consider consulting with patient advocacy services or legal professionals specializing in healthcare coverage.

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