By Counterforce Health Team in Raxone (idebenone) —

The Requirements Checklist to Get Raxone (Idebenone) Covered by Blue Cross Blue Shield of Illinois

Answer Box

Getting Raxone (idebenone) covered by Blue Cross Blue Shield of Illinois requires a formulary exception plus prior authorization since it's not FDA-approved in the U.S. Your fastest path: (1) Have your prescriber submit a pharmacy PA request through MyPrime with genetic testing confirming LHON and detailed medical necessity documentation, (2) Request peer-to-peer review if initially denied, (3) File internal appeal within 180 days if needed. Illinois residents can escalate to independent external review within 4 months of final denial.

Table of Contents

  1. Who Should Use This Checklist
  2. Member & Plan Basics
  3. Clinical Criteria Requirements
  4. Coding and Documentation Standards
  5. Documentation Packet Components
  6. Submission Process
  7. Specialty Pharmacy Requirements
  8. After Submission: What to Expect
  9. Common Denial Prevention Tips
  10. Appeals Process in Illinois
  11. Printable Checklist

Who Should Use This Checklist

This comprehensive requirements checklist is designed for patients diagnosed with Leber Hereditary Optic Neuropathy (LHON) and their healthcare providers seeking Raxone (idebenone) coverage through Blue Cross Blue Shield of Illinois (BCBSIL).

You'll need this if:

  • Your BCBSIL plan flagged Raxone as requiring prior authorization
  • You received a denial for "not FDA-approved" or "experimental"
  • Your prescriber needs guidance on documentation requirements
  • You're preparing an appeal after initial rejection

Expected outcome: With proper documentation meeting BCBSIL's medical necessity criteria, approval rates improve significantly. However, expect initial denial due to Raxone's non-FDA-approved status in the U.S., requiring appeals with strong clinical evidence.

Member & Plan Basics

✓ Verify Active Coverage

  • Check eligibility through Availity or your provider portal
  • Confirm Raxone falls under pharmacy benefit (not medical benefit)
  • Note your specific BCBSIL plan type: commercial, ACA Marketplace, or government program

✓ Plan Requirements Check

Requirement What to Verify Where to Check
Prior Authorization Required for all non-formulary specialty drugs MyPrime formulary lookup
Formulary Status Non-formulary (requires exception) Plan drug list via member portal
Step Therapy May require trial of alternatives first PA criteria in MyPrime system
Quantity Limits 180 tablets per 30-day supply typical Plan-specific limitations
Note: BCBSIL processes most pharmacy PAs through Prime Therapeutics. Always verify current requirements through MyPrime or your member portal.

Clinical Criteria Requirements

✓ Indication and Label Status

  • Primary indication: Leber Hereditary Optic Neuropathy (LHON)
  • Regulatory status: Not FDA-approved in U.S. (approved in EU)
  • Off-label use: Must justify with clinical evidence and lack of alternatives

✓ Diagnostic Confirmation Required

Genetic Testing Documentation:

  • Confirmed pathogenic mtDNA mutation in LHON-associated genes:
    • MT-ND4 m.11778G>A (most common)
    • MT-ND1 m.3460G>A
    • MT-ND6 m.14484T>C
  • Full mitochondrial genome sequencing if primary mutations negative

Clinical Documentation:

  • Bilateral, painless, subacute central vision loss
  • Visual acuity measurements (typically ≤20/200)
  • Central or cecocentral scotomas on visual fields
  • OCT showing RNFL and ganglion cell changes
  • Fundus findings: optic disc changes consistent with LHON

✓ Treatment Timeline

  • Optimal window: Within 12 months of symptom onset
  • Duration: Typically long-term therapy
  • Dosing: 300 mg three times daily with food (900 mg total daily)

Coding and Documentation Standards

✓ ICD-10 Coding

Primary diagnosis code:

  • H47.22 - Hereditary optic atrophy (most specific for LHON)
  • Avoid H47.9 (unspecified optic neuropathy)

Secondary codes as appropriate:

  • H54.0X - Blindness, both eyes (if applicable)
  • H54.1X - Severe/moderate visual impairment codes based on acuity

✓ Drug Identification

  • Generic name: Idebenone
  • Brand name: Raxone
  • Strength: 150 mg tablets
  • NDC: Not assigned in U.S. (non-FDA approved)
  • HCPCS/J-code: None (oral medication, not injectable)

✓ Billing Units

  • Unit of measure: Tablet count, not milligrams
  • 30-day supply: 180 tablets (6 tablets daily)
  • 90-day supply: 540 tablets

Documentation Packet Components

✓ Provider Note Elements

History section must include:

  • Age at onset and timeline of visual loss
  • Painless, bilateral nature of symptoms
  • Maternal family history of early vision loss
  • Absence of other neurological symptoms

Physical exam findings:

  • Best-corrected visual acuity each eye
  • Color vision testing results
  • Visual field defects (central/cecocentral scotomas)
  • Fundoscopic findings with optic disc description
  • OCT RNFL and ganglion cell layer measurements

✓ Medical Necessity Letter Components

Your prescriber's letter should address:

  1. Patient identification and diagnosis
    • Confirmed LHON with specific genetic mutation
    • ICD-10 code H47.22
  2. Clinical rationale
    • No FDA-approved alternatives for LHON
    • European approval and clinical evidence
    • Patient within optimal treatment window
  3. Supporting evidence
    • Genetic test results
    • Ophthalmologic documentation
    • Literature supporting idebenone use in LHON
  4. Treatment plan
    • Dosing: 300 mg TID with food
    • Monitoring plan and expected outcomes
    • Duration of therapy

✓ Required Attachments

  • Genetic testing report showing LHON mutation
  • Ophthalmology consultation notes
  • Visual field testing results
  • OCT reports
  • Fundus photography (if available)
  • Any prior medication trial documentation

Submission Process

✓ Correct Form and Portal

  • Primary channel: MyPrime electronic PA system
  • Alternative: BCBSIL Commercial Pharmacy Prior Authorization Request Form
  • Avoid: Medical benefit PA forms (this is a pharmacy benefit)

✓ Required Fields Check

Patient information:

  • BCBSIL member ID and name
  • Date of birth and contact information
  • Prescriber NPI and DEA numbers

Drug details:

  • Generic name: idebenone
  • Brand name: Raxone
  • Strength: 150 mg
  • Quantity: 180 tablets per 30 days
  • Days supply: 30 (recommend starting with 30-day)

Clinical information:

  • Primary diagnosis: H47.22
  • Onset date and clinical timeline
  • Prior therapies tried (if any)
  • Medical necessity justification

Specialty Pharmacy Requirements

✓ Network Verification

BCBSIL requires specialty drugs to be dispensed through contracted specialty pharmacy networks. Common in-network options include:

  • Accredo Specialty Pharmacy
  • CVS Specialty
  • AllianceRx Walgreens Prime
  • Other BCBSIL-contracted specialty vendors

✓ Transfer Process

  1. Verify coverage approval before transferring prescription
  2. Contact specialty pharmacy to confirm they can source Raxone
  3. Coordinate with prescriber for electronic prescription transfer
  4. Confirm patient address for direct shipment
Important: Since Raxone is not FDA-approved, verify the specialty pharmacy can obtain it through their distribution channels before approval.

After Submission: What to Expect

✓ Confirmation and Tracking

  • Confirmation number: Record from MyPrime submission
  • Status checks: Every 3-5 business days via portal
  • Decision timeline: Up to 15 business days for standard PA

✓ Documentation to Maintain

  • PA submission confirmation
  • All correspondence with BCBSIL/Prime
  • Dates and names of representatives contacted
  • Peer-to-peer review scheduling (if requested)

Counterforce Health specializes in turning insurance denials into targeted, evidence-backed appeals. Our platform helps patients, clinicians, and specialty pharmacies navigate complex prior authorization requirements by identifying denial reasons and drafting point-by-point rebuttals aligned with each plan's specific policies.

Common Denial Prevention Tips

Top 5 Pitfalls and Solutions

1. Using wrong ICD-10 code

  • ❌ Avoid: H47.9 (unspecified optic neuropathy)
  • ✅ Use: H47.22 (hereditary optic atrophy)

2. Incomplete genetic documentation

  • ❌ Avoid: Mentioning "suspected LHON" without confirmation
  • ✅ Include: Complete genetic test report with specific mutation

3. Missing medical necessity justification

  • ❌ Avoid: Generic "patient needs this medication"
  • ✅ Include: Detailed explanation of no FDA alternatives and European evidence

4. Wrong submission channel

  • ❌ Avoid: Medical benefit PA forms
  • ✅ Use: MyPrime pharmacy PA system

5. Inadequate clinical timeline

  • ❌ Avoid: Vague onset descriptions
  • ✅ Include: Specific dates of symptom onset and progression

Appeals Process in Illinois

✓ Internal Appeals Timeline

Appeal Level Deadline to File Decision Timeline How to Submit
Level 1 180 days from denial 15 business days Written appeal to address on denial letter
Peer-to-Peer Within 14 days 7 days for scheduling Call provider services line
Expedited When urgent 24-72 hours Phone request with urgency justification

✓ External Review Rights

If internal appeals fail, Illinois residents have strong external review protections:

  • Deadline: 4 months from final internal denial
  • Cost: Free to consumers
  • Process: Independent physician reviewer
  • Timeline: 45 days standard, 72 hours expedited
  • Binding: BCBSIL must comply if overturned

How to request: Contact Illinois Department of Insurance at 877-850-4740 or submit online external review form.

For additional support with complex appeals, Counterforce Health provides comprehensive assistance in developing evidence-backed appeals that address specific payer criteria and procedural requirements.

Printable Checklist

Pre-Submission Checklist

Patient Information:

  • BCBSIL member ID verified
  • Active coverage confirmed
  • Plan type identified (commercial/ACA/other)

Clinical Documentation:

  • Genetic test confirming LHON mutation
  • Ophthalmology consultation with visual fields
  • OCT reports showing RNFL/GCL changes
  • ICD-10 code H47.22 documented
  • Medical necessity letter completed

Submission Requirements:

  • MyPrime PA form completed
  • All attachments included
  • Prescriber NPI/DEA verified
  • Quantity: 180 tablets/30 days
  • Confirmation number recorded

Follow-up Tasks:

  • Status check schedule set (every 3-5 days)
  • Peer-to-peer review requested if denied
  • Appeal deadlines noted (180 days internal, 4 months external)
  • Specialty pharmacy network verified

Disclaimer: This guide provides educational information about insurance coverage requirements and is not medical advice. Coverage decisions depend on individual plan terms and medical circumstances. Consult your healthcare provider for medical decisions and your insurance company for definitive coverage information.

Sources & Further Reading

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