By Counterforce Health Team in Elfabrio (pegunigalsidase alfa-iwxj) —

The Requirements Checklist to Get Elfabrio (pegunigalsidase alfa-iwxj) Covered by Cigna in Virginia

Answer Box: Getting Elfabrio Covered by Cigna in Virginia

Eligibility: Adults with confirmed Fabry disease (genetic testing or enzyme deficiency) can get Elfabrio covered by Cigna without step therapy as of March 2024. Fastest path: Submit complete prior authorization with ICD-10 code E75.21, HCPCS J0219, specialist prescription, and genetic/enzyme test results. First step today: Contact your specialist to initiate the PA process through Cigna's provider portal or CoverMyMeds. If denied, Virginia's external review process via Form 216-A gives you a second chance with independent medical experts.

Table of Contents

  1. Who Should Use This Checklist
  2. Member & Plan Basics
  3. Clinical Criteria Requirements
  4. Coding & Billing Essentials
  5. Documentation Packet
  6. Submission Process
  7. Specialty Pharmacy Coordination
  8. After Submission: What to Expect
  9. Common Denial Prevention Tips
  10. Appeals Process in Virginia
  11. Quick Reference Checklist

Who Should Use This Checklist

This guide is for Virginia residents with Cigna coverage who need Elfabrio (pegunigalsidase alfa-iwxj) for Fabry disease. You'll benefit from this checklist if:

  • Your doctor has recommended Elfabrio for confirmed Fabry disease
  • You're facing a prior authorization requirement from Cigna
  • You've received a denial and need to understand your appeal options
  • You're a clinician helping patients navigate Cigna's coverage process

Expected outcome: With complete documentation, most medically appropriate Elfabrio requests get approved. If initially denied, Virginia's robust external review process provides a fair second opinion from independent specialists.

Member & Plan Basics

Coverage Verification Checklist

✓ Active Cigna coverage in Virginia (commercial, Medicare Advantage, or Medicaid managed care)
✓ Specialty pharmacy benefits included (most Cigna plans route high-cost biologics through Express Scripts/Accredo)
✓ Prior authorization requirement confirmed (call member services or check online portal)
✓ Deductible status verified (some plans require meeting deductible before coverage kicks in)

Tip: Cigna typically manages specialty drugs like Elfabrio through their specialty pharmacy network. Verify your plan's specific routing requirements before starting the PA process.

Clinical Criteria Requirements

Cigna's Coverage Criteria for Elfabrio

Requirement Details Documentation Needed
Diagnosis Confirmed Fabry disease Genetic testing OR enzyme assay
Age Adults 18+ years Date of birth verification
Prescriber Relevant specialist Geneticist, cardiologist, nephrologist, or neurologist
Concurrent therapy Not on other Fabry ERTs Attestation of no Fabrazyme/Galafold use
Step therapy None required (as of March 2024) N/A - major policy change

Diagnostic Evidence Required

Option 1: Genetic Testing

  • Pathogenic or likely pathogenic GLA gene mutation
  • Include laboratory report with variant classification
  • Must be from a CLIA-certified lab

Option 2: Enzyme Activity

  • Alpha-galactosidase A deficiency (<5% of normal activity)
  • Dried blood spot or leukocyte enzyme assay
  • Include reference ranges and testing methodology
Important Policy Update: Cigna removed step therapy requirements for Elfabrio in March 2024, meaning patients no longer need to try and fail other enzyme replacement therapies first.

Coding & Billing Essentials

Required Codes

Code Type Code Description
ICD-10-CM E75.21 Fabry (-Anderson) disease
HCPCS J0219 Injection, pegunigalsidase alfa, 1 mg
Dosing 1 mg/kg Every 2 weeks IV infusion

Additional Coding Considerations

  • NDC codes: Must match the specific Elfabrio formulation being billed
  • Units: Calculate based on patient weight (1 mg/kg dosing)
  • Site of care: Outpatient infusion center or hospital outpatient department
  • Administration codes: Use appropriate CPT codes for IV infusion services

Documentation Packet

Provider Note Elements

Your specialist's clinical note should include:

  1. Patient demographics and insurance information
  2. Confirmed Fabry disease diagnosis with supporting test results
  3. Clinical manifestations (renal, cardiac, neurological symptoms)
  4. Treatment history and rationale for Elfabrio
  5. Dosing calculation based on current weight
  6. Monitoring plan for infusion reactions and effectiveness

Medical Necessity Letter Components

A strong letter of medical necessity should address:

  • Clinical presentation and symptom progression
  • Diagnostic confirmation (attach genetic or enzyme test results)
  • Treatment goals and expected outcomes
  • Safety considerations and monitoring plan
  • Alternative therapies considered and why Elfabrio is preferred
Clinician Corner: Counterforce Health offers evidence-backed appeal templates that align with payer-specific requirements, helping clinicians craft more effective medical necessity letters for complex specialty drugs like Elfabrio.

Required Attachments

✓ Genetic testing report OR enzyme activity assay
✓ Recent clinical notes (within 90 days)
✓ Prior therapy documentation (if applicable)
✓ Current weight and vital signs
✓ Completed Cigna prior authorization form

Submission Process

Step-by-Step Submission

  1. Obtain current PA form from Cigna provider portal or CoverMyMeds
  2. Complete all required fields (incomplete forms cause automatic delays)
  3. Attach supporting documentation in PDF format
  4. Submit via preferred method:
    • CoverMyMeds portal (fastest processing)
    • Cigna provider portal
    • Fax to specialty pharmacy PA line (verify current number)
  5. Document submission with confirmation number and date
  6. Set follow-up reminder for 72 hours to check status

Common Submission Errors to Avoid

  • Missing patient weight (required for dosing calculations)
  • Incomplete diagnostic documentation
  • Using outdated PA forms
  • Submitting to wrong department (medical vs. pharmacy)

Specialty Pharmacy Coordination

Cigna's Preferred Specialty Pharmacies

Cigna typically routes Elfabrio through:

  • Express Scripts Specialty Pharmacy
  • Accredo Specialty Pharmacy

Coordination Steps

  1. Verify network status of your preferred specialty pharmacy
  2. Transfer prescription once PA is approved
  3. Coordinate delivery with infusion center
  4. Confirm insurance billing before first shipment
Note: Some patients prefer using their infusion center's buy-and-bill program. Verify with Cigna whether this is covered under your specific plan.

After Submission: What to Expect

Timeline Expectations

Stage Timeframe Action Required
Initial review 72 hours Monitor for additional information requests
Clinical review 5-7 business days Respond promptly to any questions
Final determination 10-14 business days Prepare appeal if denied

Status Monitoring

  • Check online portal daily for updates
  • Respond immediately to requests for additional information
  • Document all communications with dates and reference numbers
  • Prepare appeal materials while waiting (don't wait for denial)

Common Denial Prevention Tips

Five Critical Pitfalls and Solutions

  1. Incomplete diagnostic proof
    • Fix: Include both genetic testing AND enzyme activity if available
  2. Wrong prescriber type
    • Fix: Ensure prescription comes from appropriate specialist
  3. Missing weight documentation
    • Fix: Include current weight within 30 days for dosing calculations
  4. Outdated clinical notes
    • Fix: Use notes dated within 90 days of submission
  5. Incorrect coding
    • Fix: Double-check ICD-10 (E75.21) and HCPCS (J0219) codes
From our advocates: We've seen cases where patients were initially denied due to "insufficient documentation," but when the same clinical evidence was resubmitted with a more detailed medical necessity letter citing FDA labeling and Fabry disease guidelines, approval came within days. The key is presenting the medical story clearly and comprehensively from the start.

Appeals Process in Virginia

Internal Appeals with Cigna

Timeline: File within 180 days of denial notice Process:

  1. Submit written appeal with additional clinical evidence
  2. Request peer-to-peer review with Cigna medical director
  3. Include updated clinical notes and specialist support

External Review Through Virginia SCC

If Cigna's internal appeal fails, Virginia offers robust external review:

Form 216-A External Review Request

  • Deadline: 120 days from final internal denial
  • Timeline: Decision within 45 days (72 hours if expedited)
  • Cost: Free to patients
  • Submit to: Virginia State Corporation Commission Bureau of Insurance

Contact Information:

When to Request Expedited Review

Use Form 216-C for expedited external review if:

  • Delay could seriously jeopardize your health
  • You need immediate access to Elfabrio
  • Your doctor certifies urgent medical necessity

Virginia's external review process has helped many patients access specialty medications initially denied by their insurers. The independent medical experts reviewing your case specialize in rare diseases and understand Fabry disease treatment needs.

Quick Reference Checklist

Before You Start

□ Active Cigna coverage verified
□ Specialist appointment scheduled
□ Fabry disease diagnosis confirmed
□ Current weight documented

Required Documentation

□ Genetic testing OR enzyme assay results
□ Specialist prescription and clinical notes
□ Completed Cigna PA form
□ ICD-10: E75.21, HCPCS: J0219

Submission

□ Form submitted via preferred portal
□ Confirmation number documented
□ Follow-up scheduled for 72 hours
□ Appeal materials prepared (just in case)

If Denied

□ Internal appeal filed within 180 days
□ Peer-to-peer review requested
□ External review Form 216-A ready
□ Virginia SCC contact information saved

Patient Support Resources:

For additional help navigating insurance coverage challenges, Counterforce Health specializes in turning insurance denials into successful appeals by providing evidence-backed documentation that aligns with payer-specific requirements. Their platform helps patients and clinicians build stronger cases for specialty medications like Elfabrio.

Manufacturer Support:

  • Chiesi Total Care: Patient assistance programs and copay support
  • Elfabrio Access Resource Guide: Clinical and billing resources for providers

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage policies and requirements may change. Always verify current requirements with your insurer and consult with your healthcare provider for medical decisions. For official guidance on Virginia insurance appeals, contact the State Corporation Commission Bureau of Insurance at (877) 310-6560.

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