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

The Requirements Checklist to Get Elfabrio (Pegunigalsidase Alfa-iwxj) Covered by UnitedHealthcare in North Carolina

Answer Box: Getting Elfabrio Covered by UnitedHealthcare in North Carolina

Elfabrio (pegunigalsidase alfa-iwxj) requires prior authorization from UnitedHealthcare and is often excluded from standard formularies. The fastest path to approval: (1) Submit complete diagnostic documentation proving confirmed Fabry disease through enzyme deficiency or GLA gene testing, (2) Document step therapy failures with preferred alternatives like Fabrazyme if required, and (3) Use UnitedHealthcare's provider portal with HCPCS code J2508. If denied, North Carolina residents can appeal internally within 180 days, then request Smart NC external review within 120 days of final denial.

Table of Contents

  1. Who Should Use This Checklist
  2. Member & Plan Basics
  3. Clinical Criteria Requirements
  4. Coding and Billing Information
  5. Documentation Packet
  6. Submission Process
  7. Specialty Pharmacy Routing
  8. After Submission: Tracking Your Request
  9. Common Pitfalls and How to Avoid Them
  10. Appeals Process in North Carolina
  11. Quick Reference Checklist

Who Should Use This Checklist

This guide is designed for patients with confirmed Fabry disease and their healthcare providers seeking UnitedHealthcare coverage for Elfabrio in North Carolina. You'll need this checklist if:

  • Your doctor has prescribed Elfabrio for confirmed Fabry disease
  • You have UnitedHealthcare coverage (commercial, Medicare Advantage, or Medicaid managed care)
  • You've received a prior authorization denial or want to prevent one
  • You're preparing for a step therapy requirement or formulary exclusion

Expected outcome: Following this checklist significantly improves your chances of approval by ensuring all UnitedHealthcare requirements are met upfront, potentially avoiding the 6-8 week appeals process.

Member & Plan Basics

Coverage Verification

Before starting your prior authorization:

  • Confirm active coverage through UnitedHealthcare member portal or call the member services number on your insurance card
  • Identify your plan type: Commercial, Medicare Advantage, or Medicaid managed care (different policies may apply)
  • Check deductible status: High-deductible plans may require meeting your deductible before coverage begins
  • Verify specialty drug benefits: Elfabrio typically falls under specialty pharmacy benefits with separate authorization requirements
Note: Self-funded employer plans may have different policies than standard UnitedHealthcare commercial plans. Check with your HR department if unsure.

Clinical Criteria Requirements

Primary Diagnosis Requirements

UnitedHealthcare requires confirmed Fabry disease documented by:

For Males:

  • Alpha-galactosidase A enzyme activity less than 5% of mean normal activity in leukocytes, dried blood spot, or serum analysis, OR
  • Molecular genetic testing confirming pathogenic variant in the GLA gene

For Females:

  • GLA gene sequencing is required (enzyme testing alone is insufficient due to X-linked inheritance patterns)
  • Genetic testing must confirm pathogenic or likely pathogenic variant

Clinical Documentation Needed

  • Presenting symptoms: Document acroparesthesias, angiokeratomas, anhidrosis, renal involvement, cardiac manifestations, or neurological symptoms
  • Disease severity assessment: Include impact on quality of life and functional status
  • Prior treatments: Document any previous enzyme replacement therapies, outcomes, and reasons for discontinuation

Step Therapy Considerations

UnitedHealthcare may require trial and failure of preferred alternatives:

  • Fabrazyme (agalsidase beta) is typically the preferred ERT
  • Document medical reasons if step therapy cannot be completed (contraindications, allergies, access issues)
  • Galafold (migalastat) may be preferred for patients with amenable mutations

Coding and Billing Information

Essential Codes for Elfabrio

Code Type Code Description Notes
HCPCS J-Code J2508 Injection, pegunigalsidase alfa-iwxj, 1 mg Effective January 1, 2024
NDC Numbers 10122-160-01, 10122-160-02, 10122-160-05, 10122-160-10, 10122-165-02 National Drug Codes Use 11-digit format
ICD-10 E75.21 Fabry disease Primary diagnosis
Billing Units Per 1 mg Each unit = 1 mg of drug Standard dose: 1 mg/kg every 2 weeks

Required Modifiers

  • UD modifier: Required when billing for drugs purchased under 340B agreement
  • Verify payer-specific modifier requirements with UnitedHealthcare

Dosing and Administration

  • Standard dose: 1 mg/kg IV infusion every two weeks
  • Package strength: 20 mg/10 mL (2 mg/mL) single-dose vial
  • Administration: Intravenous infusion only, typically in outpatient infusion center

Documentation Packet

Provider Note Requirements

Your healthcare provider's documentation should include:

Clinical Assessment:

  • Confirmed Fabry disease diagnosis with supporting lab/genetic results
  • Current symptoms and disease progression
  • Previous treatments attempted and outcomes
  • Contraindications to alternative therapies (if applicable)

Treatment Plan:

  • Specific rationale for choosing Elfabrio
  • Dosing plan based on patient weight
  • Monitoring plan for efficacy and safety
  • Expected treatment duration

Letter of Medical Necessity Components

A strong letter of medical necessity should address:

  1. Patient identification and confirmed Fabry disease diagnosis
  2. Clinical presentation and disease severity
  3. Previous treatments and reasons for discontinuation or failure
  4. Medical rationale for Elfabrio specifically
  5. Supporting literature and clinical guidelines
  6. Monitoring plan and treatment goals
Clinician Corner: Reference UnitedHealthcare's enzyme replacement therapy policy (effective 10/01/2025) which states Elfabrio is medically necessary when diagnostic criteria are met and clinical symptoms are present.

Required Attachments

  • Laboratory results showing enzyme deficiency or genetic test results
  • Clinical notes documenting Fabry disease symptoms
  • Previous treatment records (if applicable)
  • Signed physician order for Elfabrio
  • Any relevant imaging or cardiac/renal function tests

Submission Process

UnitedHealthcare Submission Methods

Provider Portal (Recommended):

Alternative Methods:

  • Fax: Use the prior authorization fax number provided in your provider manual (verify current number)
  • Phone: Call OptumRx at 1-800-711-4555 for urgent cases

Critical Submission Details

  • Complete all required fields: Incomplete forms are the #1 cause of delays
  • Include patient weight: Essential for dosing calculations
  • Specify infusion site: Outpatient infusion center details
  • Attach all supporting documents: Don't rely on "available upon request"
Tip: Save confirmation numbers and take screenshots of successful portal submissions.

Specialty Pharmacy Routing

UnitedHealthcare Preferred Specialty Pharmacies

UnitedHealthcare typically routes specialty medications through OptumRx specialty pharmacy network. Key steps:

  1. Verify network participation of your preferred specialty pharmacy
  2. Coordinate with infusion center for drug delivery and administration
  3. Confirm cold-chain handling requirements for Elfabrio
  4. Verify insurance benefits transfer to specialty pharmacy

Transfer Process

If switching from another specialty pharmacy:

  • Request prescription transfer through new pharmacy
  • Ensure continuity of care during transition
  • Confirm delivery schedule aligns with dosing intervals

After Submission: Tracking Your Request

Timeline Expectations

  • Standard review: 5-10 business days
  • Expedited review: 24-72 hours (for urgent medical situations)
  • Complex cases: May require additional clinical review or peer-to-peer discussion

Status Monitoring

  • Check status through UnitedHealthcare provider portal
  • Document all communications and reference numbers
  • Set calendar reminders for follow-up if no response within expected timeframe

What to Record

  • Submission date and confirmation number
  • Case reference number (if provided)
  • Names of any representatives spoken with
  • Dates of status checks and outcomes

Common Pitfalls and How to Avoid Them

Common Pitfall How to Avoid Prevention Strategy
Incomplete diagnostic documentation Submit both enzyme and genetic testing results Include comprehensive lab reports with reference ranges
Missing step therapy documentation Document medical reasons why alternatives cannot be used Provide detailed history of prior ERT trials and outcomes
Incorrect dosing information Use patient's actual body weight for calculations Include current weight and dosing rationale in submission
Wrong billing codes Use HCPCS J2508 and correct NDC numbers Double-check codes against current CMS listings
Incomplete provider information Include NPI, specialty, and contact information Use provider portal auto-fill features when available

Appeals Process in North Carolina

Internal Appeals with UnitedHealthcare

If your initial request is denied:

  1. File internal appeal within 180 days of denial notice
  2. Submit via UnitedHealthcare member portal or use their appeals form
  3. Include point-by-point response to denial reasons
  4. Request expedited review if medically urgent (72-hour response)

Smart NC External Review

North Carolina residents have additional appeal rights through Smart NC:

Eligibility:

  • Must complete UnitedHealthcare's internal appeal process first
  • Plan must be state-regulated (not self-funded ERISA plans unless opted-in)
  • Denial must be based on medical necessity or experimental treatment determination

Process:

  1. Submit external review request within 120 days of final internal denial
  2. Call Smart NC at 1-855-408-1212 for free assistance
  3. Complete external review form available on NC Department of Insurance website

Timeline:

  • Standard review: 45 days maximum
  • Expedited review: 24-72 hours for urgent cases
Important: Smart NC's decision is binding on UnitedHealthcare. This independent review has overturned many denials for expensive specialty medications.

At Counterforce Health, we help patients and providers navigate complex prior authorization requirements by turning insurance denials into targeted, evidence-backed appeals. Our platform analyzes denial letters and drafts point-by-point rebuttals aligned to each payer's specific requirements, including UnitedHealthcare's enzyme replacement therapy policies.

Quick Reference Checklist

Before Submission:

  • Confirm active UnitedHealthcare coverage
  • Obtain confirmed Fabry disease diagnosis (enzyme or genetic testing)
  • Document current symptoms and disease severity
  • Gather records of previous treatments (if applicable)
  • Calculate correct dosing based on patient weight
  • Identify preferred infusion center

Required Documentation:

  • Completed prior authorization form
  • Letter of medical necessity
  • Lab results showing enzyme deficiency or genetic testing
  • Clinical notes documenting Fabry symptoms
  • Signed physician order with HCPCS J2508
  • Previous treatment records (if step therapy applies)

Submission Details:

  • Use UnitedHealthcare provider portal (preferred method)
  • Include all required billing codes and modifiers
  • Specify infusion site and administration plan
  • Save confirmation numbers and reference codes
  • Set follow-up reminders for status checks

If Denied:

  • File internal appeal within 180 days
  • Address each denial reason specifically
  • Consider Smart NC external review if internal appeal fails
  • Contact Smart NC at 1-855-408-1212 for assistance

Sources & Further Reading

For additional support navigating UnitedHealthcare prior authorizations and appeals, Counterforce Health provides specialized assistance in turning insurance denials into successful approvals through evidence-based appeal strategies.

Disclaimer: This guide provides general information about insurance coverage requirements and should not be considered medical or legal advice. Coverage decisions depend on individual plan benefits, medical circumstances, and current policies. Always consult with your healthcare provider and insurance company for specific guidance regarding your situation. For official appeals assistance in North Carolina, contact Smart NC at 1-855-408-1212.

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