By Counterforce Health Team in Brineura (cerliponase alfa) —

The Requirements Checklist to Get Brineura (Cerliponase Alfa) Covered by Blue Cross Blue Shield in Texas

Answer Box: Getting Brineura Covered in Texas

Blue Cross Blue Shield of Texas requires prior authorization for Brineura (cerliponase alfa) with strict clinical criteria including confirmed CLN2 diagnosis, specialized neurologist prescription, and documented ambulatory function. Submit via their medical PA portal with genetic/enzyme testing, baseline CLN2 scores, and medical necessity letter. If denied, Texas law provides expedited appeals and external review through independent organizations. First step today: Contact your neurologist to gather TPP1 enzyme results and baseline Hamburg CLN2 scores.

Table of Contents

  1. Who Should Use This Checklist
  2. Member & Plan Basics
  3. Clinical Criteria Requirements
  4. Coding & Billing Requirements
  5. Documentation Packet Checklist
  6. Submission Process
  7. Specialty Pharmacy Requirements
  8. After Submission: Tracking Your Request
  9. Common Denial Prevention Tips
  10. Appeals Process in Texas
  11. Printable One-Page Checklist

Who Should Use This Checklist

This comprehensive guide helps families and clinicians navigate Blue Cross Blue Shield of Texas (BCBSTX) coverage for Brineura (cerliponase alfa), the only FDA-approved enzyme replacement therapy for CLN2 disease (late infantile neuronal ceroid lipofuscinosis type 2).

Use this checklist if:

  • Your child has confirmed CLN2 disease and needs Brineura coverage
  • You received a BCBSTX denial and want to appeal
  • Your neurologist is preparing a prior authorization request
  • You're switching insurance plans and need to establish coverage

Expected outcome: With proper documentation following this checklist, most families see approval within 30 days. Counterforce Health has helped numerous families successfully appeal specialty drug denials by turning insurance rejections into targeted, evidence-backed appeals that align with payer policies.

Member & Plan Basics

Verify Active Coverage

Before starting, confirm these basics:

  • Member ID and plan type (HMO, PPO, ASO employer plan)
  • Medical benefit coverage (Brineura is not a pharmacy benefit)
  • Deductible status and specialty drug copay structure
  • Network status of your treating neurologist and infusion center
Tip: Call the number on your BCBSTX member ID card to verify Brineura requires prior authorization and confirm your plan's medical drug benefit coverage.

Plan Types and Requirements

Plan Type PA Required Special Notes
Commercial fully insured Yes Standard BCBSTX criteria apply
ASO employer plans Varies Check specific group benefits
Teacher Retirement System Yes Effective March 1, 2024

Clinical Criteria Requirements

Diagnosis Confirmation

BCBSTX requires documented CLN2 diagnosis through:

  1. TPP1 enzyme deficiency in leukocytes, fibroblasts, or validated tissue
  2. Genetic confirmation of biallelic pathogenic variants in TPP1/CLN2 gene
  3. Clinical presentation consistent with late infantile neuronal ceroid lipofuscinosis

Prescriber Requirements

  • Must be prescribed by or in consultation with a pediatric neurologist or specialist experienced in neuronal ceroid lipofuscinosis
  • Provider must be in BCBSTX network or approved for out-of-network care

Functional Status Documentation

Required baseline assessments:

  • Hamburg CLN2 Clinical Rating Scale scores (motor and language domains)
  • Documentation that patient has ambulatory function that can be preserved
  • Age verification (typically ≥3 years, though FDA label includes younger children)

Medical Necessity Criteria

According to BCBSTX medical drug benefit policies, coverage requires:

  • Confirmed CLN2 diagnosis via enzyme/genetic testing
  • Appropriate age and developmental stage
  • Absence of contraindications (active CNS infection, acute device complications)
  • Specialized infusion center capability

Coding & Billing Requirements

Primary Codes

Code Type Code Description
ICD-10 E75.4 Neuronal ceroid lipofuscinosis
HCPCS J0567 Injection, cerliponase alfa, 1 mg
NDC [Verify current] 150 mg/5 mL single-dose vial

Billing Units

  • Standard dose: 300 mg every 14 days = 300 billable units
  • Administration: Requires intraventricular infusion via implanted reservoir
  • Site requirements: Specialized facility with emergency support capabilities

Documentation Packet Checklist

Core Medical Records

☐ Neurologist consultation note confirming CLN2 diagnosis and treatment recommendation

☐ Genetic testing results showing biallelic TPP1 gene variants

☐ TPP1 enzyme activity results demonstrating deficiency

☐ Baseline Hamburg CLN2 scores with date of assessment

☐ Recent cardiac evaluation (12-lead ECG within 6 months)

Medical Necessity Letter Components

Your neurologist's letter should include:

  1. Patient identification (name, DOB, insurance ID, current age/weight)
  2. Confirmed diagnosis with specific test results and dates
  3. Baseline functional status using Hamburg CLN2 scale
  4. Treatment rationale explaining why Brineura is medically necessary
  5. Dosing plan following FDA-approved prescribing information
  6. Monitoring plan for safety and efficacy assessment
From our advocates: "We've seen the strongest approvals when families include both genetic and enzyme testing results, even if only one is required. This removes any question about diagnosis confirmation and demonstrates thorough medical workup."

Supporting Documentation

☐ Prior therapy records (if applicable - supportive care, seizure management)

☐ Surgical consultation for intraventricular device placement

☐ Infusion center verification of capability to administer Brineura safely

☐ Insurance denial letter (if appealing a previous decision)

Submission Process

BCBSTX Prior Authorization Methods

  1. Blue Approver portal (preferred method for providers)
  2. Availity.com Medical/Pharmacy Drug PA tile
  3. Phone submission: Number on member ID card
  4. Carelon Medical Benefits Management (if applicable): 1-866-455-8415

Required Forms

  • Use BCBSTX-specific prior authorization form for medical drugs
  • Complete all sections; incomplete forms cause automatic delays
  • Attach all supporting documentation as single PDF when possible

Timeline Expectations

Request Type Timeline Notes
Standard PA 5-15 business days Most common pathway
Expedited PA 72 hours For urgent medical need
Appeal review 30 days Internal appeal process

Specialty Pharmacy Requirements

Network Requirements

Brineura is classified as a provider-administered specialty infusion drug under BCBSTX medical benefits, not retail pharmacy.

Key requirements:

  • Drug must be obtained through plan-contracted specialty distributor
  • Cannot be filled at retail pharmacies
  • Requires "buy-and-bill" or white-bagging to infusion facility

Infusion Site Standards

BCBSTX requires infusion at facilities with:

  • Staff trained in managing hypersensitivity reactions
  • Cardiopulmonary resuscitation equipment on-site
  • Access to neurosurgical consultation
  • Experience with intraventricular infusions

Device Requirements

  • Surgically implanted intraventricular access device (e.g., Codman HOLTER RICKHAM reservoir)
  • First infusion ≥5-7 days after device placement
  • Appropriate syringe infusion pump meeting manufacturer specifications

After Submission: Tracking Your Request

Confirmation Steps

  1. Record confirmation number from submission
  2. Save all submission documentation including timestamps
  3. Schedule follow-up call for 5-7 business days post-submission

Status Monitoring

  • Check portal daily for updates
  • Document all phone conversations with reference numbers
  • Request written confirmation of verbal approvals

What to Record

  • Date and time of all communications
  • Names of representatives spoken with
  • Reference or case numbers
  • Any additional documentation requested

Common Denial Prevention Tips

Five Critical Pitfalls to Avoid

  1. Incomplete genetic/enzyme documentation
    • Fix: Include both genetic and enzyme testing results with lab letterhead
  2. Missing baseline CLN2 scores
    • Fix: Ensure Hamburg CLN2 motor and language scores are documented with assessment date
  3. Unqualified prescriber
    • Fix: Ensure prescription comes from or includes consultation with pediatric neurologist
  4. Inadequate medical necessity justification
    • Fix: Clearly explain why Brineura is the only appropriate treatment option for CLN2
  5. Wrong benefit category
    • Fix: Submit under medical benefit, not pharmacy benefit; use correct J-code J0567
Note: Counterforce Health specializes in identifying these common denial patterns and crafting targeted appeals that address each payer's specific requirements.

Appeals Process in Texas

Internal Appeal Rights

If BCBSTX denies your initial request:

Timeline: File within 180 days of denial notice

Process:

  1. Submit written appeal with denial letter
  2. Include additional supporting documentation
  3. Request expedited review if medically urgent

External Review Options

Texas Independent Review Organization (IRO):

  • Available after internal appeal denial
  • File within 4 months of final internal denial
  • Decision is binding on BCBSTX
  • No cost to member (insurer pays IRO fees)

Expedited External Review:

  • Available for urgent cases
  • Can file concurrently with expedited internal appeal
  • Decision within 5 days for emergencies

Texas Resources for Help

  • Texas Department of Insurance: 1-800-252-3439
  • Office of Public Insurance Counsel: 1-877-611-6742
  • IRO Information Line: 1-866-554-4926
  • Disability Rights Texas: Assistance with appeals process

Printable One-Page Checklist

Before Submission

  • ☐ Verify active BCBSTX medical benefit coverage
  • ☐ Confirm neurologist is network provider
  • ☐ Gather genetic and enzyme testing results
  • ☐ Document baseline Hamburg CLN2 scores
  • ☐ Obtain recent cardiac evaluation (ECG)
  • ☐ Verify infusion center capabilities

Documentation Required

  • ☐ Completed BCBSTX prior authorization form
  • ☐ Medical necessity letter from neurologist
  • ☐ Genetic testing confirming CLN2 diagnosis
  • ☐ TPP1 enzyme deficiency results
  • ☐ Baseline functional assessments
  • ☐ Surgical consultation for device placement

Submission Steps

  • ☐ Submit via Blue Approver portal or Availity
  • ☐ Record confirmation number
  • ☐ Schedule follow-up for 5-7 business days
  • ☐ Monitor status daily

If Denied

  • ☐ File internal appeal within 180 days
  • ☐ Request expedited review if urgent
  • ☐ Consider external IRO review
  • ☐ Contact Texas Department of Insurance for help

When navigating insurance coverage for rare disease treatments like Brineura, families often feel overwhelmed by complex requirements and potential denials. Counterforce Health understands these challenges and helps patients, clinicians, and specialty pharmacies turn insurance denials into successful appeals by identifying specific denial reasons and crafting evidence-backed responses that align with each payer's own policies.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage policies may vary by specific plan and change without notice. Always verify current requirements with your insurance plan and healthcare providers. For personalized assistance with appeals and coverage issues, consult with qualified professionals or advocacy organizations.

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