By Counterforce Health Team in Brineura (cerliponase alfa) —

How to Get Brineura (Cerliponase Alfa) Covered by Cigna in North Carolina: Appeals, Prior Authorization, and Coverage Guide

Answer Box: Getting Brineura Covered by Cigna in North Carolina

Brineura (cerliponase alfa) requires prior authorization from Cigna for CLN2 disease patients ≥3 years old. First step: Have your pediatric neurologist submit a PA request through Express Scripts/Accredo with CLN2 diagnosis confirmation, TPP1 enzyme testing, and medical necessity documentation. If denied, you have 180 days for internal appeals, then 120 days for external review through North Carolina's Smart NC program. Timeline: PA decisions within 5-10 business days; appeals take 45 days (or 72 hours for urgent cases).

Table of Contents

  1. Coverage Requirements at a Glance
  2. Step-by-Step: Fastest Path to Approval
  3. Common Denial Reasons & Solutions
  4. Appeals Process in North Carolina
  5. Clinician Documentation Requirements
  6. Finding Approved Treatment Centers
  7. Cost Assistance Programs
  8. FAQ: Brineura Coverage Questions

Coverage Requirements at a Glance

Requirement Details Source
Prior Authorization Required for all Cigna plans Cigna Specialty Pharmacy
Age Requirement ≥3 years old FDA Label
Diagnosis Confirmed CLN2 disease with TPP1 deficiency FDA Access Data
Specialty Pharmacy Accredo or Express Scripts network Cigna RX Benefits
Site of Care Approved infusion center with neurosurgical support Brineura.com
Appeal Deadline 180 days internal, 120 days external review North Carolina Insurance Law

Step-by-Step: Fastest Path to Approval

1. Confirm CLN2 Diagnosis (Specialist Required)

Who: Pediatric neurologist
Documents: TPP1 enzyme test results, genetic testing showing CLN2 mutations
Timeline: 1-2 weeks for lab results
Source: CLN2 Connection Diagnostic Guidelines

2. Surgical Device Placement

Who: Pediatric neurosurgeon
Procedure: Intraventricular reservoir implantation (Ommaya reservoir)
Timeline: Outpatient procedure, 1-2 week healing period
Coverage: Typically covered under medical benefit with separate PA

3. Submit Prior Authorization

Who: Prescribing neurologist or clinic staff
Method: Express Scripts ePrescribing or Accredo PA portal
Timeline: 5-10 business days for decision
Required: Medical necessity letter, diagnostic results, treatment plan

4. Schedule at Approved Center

Who: Patient/family with clinic coordination
Frequency: Every 2 weeks, 300mg dose
Duration: Several hours per infusion
Monitoring: Pre-infusion CSF testing, vital signs

Common Denial Reasons & Solutions

Denial Reason Solution Strategy Documentation Needed
Age < 3 years Confirm birth date; appeal if close to 3rd birthday Birth certificate, current age verification
Lack of CLN2 confirmation Submit complete diagnostic workup TPP1 enzyme assay, genetic testing results
No specialist involvement Transfer care to pediatric neurologist Referral documentation, specialist evaluation
Site of care not approved Identify Cigna network infusion center Provider directory search, center credentials
"Experimental" designation Cite FDA approval and clinical evidence FDA approval letter, peer-reviewed studies

Appeals Process in North Carolina

Internal Appeals (Cigna)

  • First Level: 180 days from denial date
  • Second Level: Available if first appeal denied
  • Expedited: For urgent medical situations (72-hour decision)
  • Submission: Cigna member portal or written request

External Review (Smart NC)

When to Use: After exhausting internal appeals or for urgent cases
Deadline: 120 days from final internal denial
Process: Submit external review form to Smart NC
Timeline: 45 days standard, 72 hours expedited
Contact: Smart NC Helpline 1-855-408-1212

From our advocates: We've seen families succeed by combining the clinical evidence with personal impact statements. One composite case involved documenting specific motor decline milestones and how Brineura could preserve remaining function—the external reviewer found this compelling alongside the medical necessity documentation.

Required Appeal Documents

  • Copy of original denial letter
  • Medical records supporting CLN2 diagnosis
  • Specialist treatment recommendations
  • Evidence of failed alternative treatments (if applicable)
  • Patient/family impact statement

Clinician Documentation Requirements

Medical Necessity Letter Checklist

Clinical History:

  • Age at symptom onset and current age
  • Specific CLN2 symptoms (motor decline, seizures, language regression)
  • Prior therapies attempted and outcomes
  • Current functional status using CLN2 Clinical Rating Scale

Diagnostic Evidence:

  • TPP1 enzyme deficiency results
  • CLN2 gene mutation analysis
  • Brain MRI findings consistent with CLN2
  • ICD-10 codes: E75.4 (neuronal ceroid lipofuscinosis)

Treatment Plan:

  • Dosing: 300mg every 2 weeks intraventricularly
  • Administration site and monitoring plan
  • Expected outcomes and treatment goals
  • Long-term management strategy

Supporting Guidelines:

Finding Approved Treatment Centers

North Carolina Pediatric Centers

UNC Children's Hospital (Chapel Hill)

  • Pediatric neurology and infusion services
  • Neurosurgery for device placement
  • Cigna network participation (verify current status)
  • Contact: 984-974-1000

Brenner Children's Hospital (Winston-Salem)

  • Atrium Health Wake Forest Baptist network
  • Pediatric specialty infusions
  • Typically Cigna in-network
  • Contact: General hospital line for infusion center

Levine Children's Hospital (Charlotte)

  • Atrium Health affiliate
  • Pediatric infusion services available
  • Contact: 704-512-5370
Note: Brineura requires specialized centers with intraventricular access capability. Contact BioMarin ACCESS program at 888-4BRINEURA to locate certified sites and verify current Cigna network status.

Cost Assistance Programs

Manufacturer Support

BioMarin ACCESS Program

  • Copay assistance for eligible patients
  • Prior authorization support
  • Reimbursement navigation
  • Contact: 888-4BRINEURA

Additional Resources

  • Patient Access Network Foundation (rare disease grants)
  • HealthWell Foundation (CLN2-specific assistance)
  • North Carolina Medicaid (if eligible after expansion)

Annual Cost Context: Brineura's list price is approximately $702,000 annually, with additional costs for administration and monitoring. Most insurance plans cover the medication under medical benefits rather than pharmacy benefits.

Smart NC: North Carolina's Insurance Advocate

Counterforce Health helps patients navigate complex prior authorization and appeals processes by analyzing denial letters, identifying specific coverage criteria, and drafting evidence-based appeals. For families facing Brineura denials, this type of specialized support can be invaluable in preparing comprehensive documentation that addresses payer-specific requirements.

North Carolina residents have access to Smart NC, a state-sponsored insurance advocacy program that provides free assistance with external reviews and appeals. This program has successfully overturned many denials for expensive specialty medications, particularly when strong clinical evidence supports medical necessity.

FAQ: Brineura Coverage Questions

Q: How long does Cigna prior authorization take in North Carolina? A: Typically 5-10 business days for standard reviews. Expedited reviews (for urgent medical situations) are processed within 72 hours.

Q: What if Brineura is non-formulary on my plan? A: Request a formulary exception through your prescriber. Provide evidence that no formulary alternatives exist for CLN2 disease, as Brineura is the only FDA-approved treatment.

Q: Can I request an expedited appeal? A: Yes, if delay would seriously jeopardize health or ability to regain function. Both Cigna internal appeals and Smart NC external reviews offer expedited pathways.

Q: Does step therapy apply to Brineura? A: Unlikely, as no alternative treatments exist for CLN2 disease. However, payers may require documentation of supportive care measures tried previously.

Q: What happens if my appeal is denied? A: After exhausting Cigna's internal appeals, you can request external review through Smart NC within 120 days. The external reviewer's decision is binding on Cigna.

Q: Are there age restrictions beyond the FDA approval? A: FDA approval is for patients ≥3 years old. Some payers may have additional restrictions, but these can often be appealed with appropriate clinical justification.

Q: How do I find a Cigna network infusion center? A: Use Cigna's provider directory or contact member services at 800-244-6224. Verify the center can handle intraventricular infusions and has pediatric neurosurgical support.

Q: What if I need treatment while appealing? A: Request continuation of benefits during the appeal process. For urgent situations, seek expedited review through both internal and external channels.

Sources & Further Reading

Disclaimer: This information is for educational purposes and does not constitute medical or legal advice. Always consult with your healthcare provider and insurance company for personalized guidance. Coverage policies and requirements may change; verify current information with official sources.

For complex appeals and prior authorization challenges, Counterforce Health specializes in turning insurance denials into successful approvals by analyzing payer policies and crafting targeted, evidence-based appeals that address specific coverage criteria.

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