By Counterforce Health Team in Xenpozyme (olipudase alfa-rpcp) —

How to Get Xenpozyme (olipudase alfa-rpcp) Covered by Cigna in Washington: Complete Prior Authorization and Appeals Guide

Answer Box: Getting Xenpozyme Covered by Cigna in Washington

Xenpozyme (olipudase alfa-rpcp) requires prior authorization from Cigna for ASMD (Niemann-Pick types A/B). Your fastest path: (1) Confirm genetic diagnosis with SMPD1 variants and enzyme deficiency <10% normal, (2) Have your specialist submit PA with weight-based dosing plan via Cigna's provider portal or Express Scripts/Accredo, (3) If denied, file internal appeal within 180 days, then external IRO review through Washington's insurance commissioner within 60 days of final denial.

Start today: Call Cigna member services (number on your ID card) to confirm PA requirements and get current forms.

Table of Contents

Who Should Use This Guide

This guide is for Washington residents with Cigna coverage who need Xenpozyme (olipudase alfa-rpcp) for acid sphingomyelinase deficiency (ASMD), also known as Niemann-Pick disease types A and B. You'll need:

  • Confirmed ASMD diagnosis through genetic testing and enzyme activity
  • A specialist (geneticist, metabolic disease expert, or pulmonologist) managing your care
  • Active Cigna coverage (commercial, Medicare Advantage, or Medicaid managed care)

Expected outcome: With proper documentation, most medically necessary Xenpozyme requests get approved. Cigna's Medicare Advantage members appeal PA denials at ~18% rates, suggesting many initial denials are overturned when complete evidence is provided.

Member & Plan Basics

Coverage Requirements

Requirement Details Where to Verify
Active Coverage Must be enrolled with current premium payments Cigna member portal or ID card
Plan Type Commercial, Medicare Advantage, or Medicaid managed care Benefits summary or member services
Specialty Pharmacy Usually requires Express Scripts/Accredo fulfillment Cigna specialty pharmacy network
Prior Authorization Required for all Xenpozyme prescriptions Cigna formulary or provider portal
Note: Self-funded employer plans may have different appeal timelines (45 days for external review vs. 20 days for fully-insured plans).

Clinical Criteria Requirements

Cigna requires strict medical necessity documentation for Xenpozyme approval:

Primary Criteria

  1. Confirmed ASMD Diagnosis
    • Genetic testing showing biallelic SMPD1 pathogenic variants
    • Enzyme activity <10% of normal controls in leukocytes or fibroblasts
    • Clinical features consistent with non-CNS manifestations
  2. Specialist Involvement
    • Prescribed by or in consultation with geneticist, metabolic specialist, or pulmonologist
    • Documentation of specialist qualifications and ASMD experience
  3. Non-CNS Disease
    • Must document non-central nervous system manifestations only
    • Xenpozyme is not approved for CNS symptoms

Dosing & Monitoring Plan

  • Weight-based dosing: Starting at 0.1 mg/kg IV every 14 days, escalating to maintenance dose
  • Maximum dose: 3 mg/kg (up to 344 mg total) every 14 days
  • Monitoring requirements: Baseline and periodic assessments of spleen size, lung function, and lipid levels
  • Infusion safety plan: Documentation of premedication protocol and reaction management

Coding & Billing Information

Required Codes

Code Type Code Description Units
ICD-10 E75.241 Niemann-Pick disease type B/ASMD -
HCPCS J-Code J0218 Injection, olipudase alfa-rpcp, 1 mg Per mg administered
NDC 58468-0051-xx 4 mg single-dose vial Verify current NDC
Administration 96365/96366 IV infusion codes Per hour
Tip: Use modifier JW for any drug waste that needs to be reported to Medicare or other payers requiring waste documentation.

Documentation Packet Essentials

Medical Necessity Letter Components

Your specialist's letter should include:

  1. Patient Demographics & Insurance Info
    • Full name, DOB, Cigna member ID
    • Policy/group numbers from insurance card
  2. Diagnosis Documentation
    • ASMD confirmation with genetic test results
    • Enzyme activity levels showing <10% normal
    • Clinical manifestations (hepatosplenomegaly, lung disease, etc.)
  3. Treatment Rationale
    • Why Xenpozyme is medically necessary
    • Expected clinical benefits
    • Absence of alternative FDA-approved treatments for ASMD
  4. Dosing & Safety Plan
    • Weight-based calculation showing exact dose
    • Escalation schedule from 0.1 mg/kg to maintenance
    • Infusion site and safety monitoring plan

Required Attachments

  • Genetic testing report showing SMPD1 variants
  • Enzyme activity lab results
  • Baseline imaging (spleen/liver size, chest CT/X-ray)
  • Pulmonary function tests
  • Recent clinic notes documenting current symptoms
  • Specialist consultation notes

Submission Process

Step-by-Step Submission

  1. Get Current Forms
    • Contact Cigna provider services at the number on your member ID card
    • Download PA forms from Cigna provider portal
    • Verify Express Scripts/Accredo requirements
  2. Complete Documentation
    • Specialist completes PA request form
    • Attach all required clinical documents
    • Include weight-based dose calculations
  3. Submit Request
    • Preferred: Cigna provider portal (fastest processing)
    • Alternative: Fax to number on PA form (verify current fax)
    • Timeline: Submit at least 2-3 weeks before needed start date
  4. Confirm Receipt
    • Get confirmation number or receipt
    • Document submission date and method
    • Set calendar reminder to check status in 5-7 business days

Specialty Pharmacy Routing

Cigna typically requires Xenpozyme fulfillment through Express Scripts/Accredo specialty pharmacy:

Transfer Process

  1. Prescription Routing
    • Your doctor sends prescription to Accredo (not local pharmacy)
    • Accredo coordinates with Cigna for PA status
    • Patient enrollment in Accredo's support program
  2. Shipment & Administration
    • Medication ships to infusion center or home
    • Coordinate with infusion nurse or clinic
    • Verify cold storage and handling requirements
Important: Don't let your local pharmacy order Xenpozyme before PA approval—this can cause billing complications and delays.

After Submission: What to Expect

Timeline & Status Checks

Milestone Timeframe Action Required
Initial Review 5-7 business days Check status via provider portal
Additional Info Request 3-5 days to respond Submit requested documents promptly
Final Decision 14 days maximum Review approval letter or denial notice
Appeal Deadline 180 days from denial File internal appeal if denied

What to Track

  • Confirmation numbers for all submissions
  • Names and dates of phone calls with Cigna
  • Case reference numbers
  • Copies of all faxed or mailed documents

Common Denial Reasons & How to Fix Them

Denial Reason How to Overturn Required Documentation
Insufficient diagnosis proof Submit complete genetic testing SMPD1 variants + enzyme activity <10%
Non-specialist prescriber Get specialist consultation Geneticist or metabolic specialist letter
Missing monitoring plan Provide detailed safety protocol Baseline labs, imaging, infusion plan
Dosing concerns Justify weight-based calculation FDA labeling + patient weight documentation
Site of care issues Confirm approved infusion location Network provider verification

Appeals Process in Washington

If Cigna denies your Xenpozyme request, Washington state provides strong consumer protections:

Internal Appeals (Required First Step)

  • Timeline: File within 180 days of denial
  • Process: Submit written appeal to Cigna with additional clinical evidence
  • Decision: Standard review within 30 days, expedited within 72 hours for urgent cases

External Review (Independent Review Organization)

Washington residents can request external review after exhausting internal appeals:

  • Eligibility: After final internal denial from Cigna
  • Timeline: Request within 60 days of final denial
  • Process: Washington Office of Insurance Commissioner assigns certified IRO
  • Decision: 20 days for fully-insured plans, 45 days for self-funded plans
  • Expedited: 72 hours if health urgency documented
  • Contact: Washington OIC at 1-800-562-6900 for guidance
Key Advantage: IRO decisions are binding on Cigna—if they overturn the denial, Cigna must provide coverage.

Counterforce Health specializes in turning insurance denials into successful appeals by analyzing denial letters, plan policies, and clinical notes to draft targeted, evidence-backed rebuttals. Their platform identifies the specific denial basis and creates point-by-point responses aligned with the plan's own rules, significantly improving approval rates for complex medications like Xenpozyme.

Quick Reference Checklist

Before You Start

  • Confirm active Cigna coverage and benefits
  • Verify specialist is in-network or get referral
  • Gather genetic testing and enzyme activity results
  • Document current symptoms and functional status

For PA Submission

  • Complete Cigna PA form with specialist
  • Include weight-based dose calculation
  • Attach genetic testing report
  • Include enzyme activity lab results (<10% normal)
  • Provide baseline imaging and PFTs
  • Submit via Cigna provider portal
  • Get confirmation number

If Denied

  • Review denial letter for specific reasons
  • Gather additional supporting evidence
  • File internal appeal within 180 days
  • Consider expedited review if urgent
  • Prepare for external IRO review if needed
  • Contact Counterforce Health for appeal assistance

FAQ

Q: How long does Cigna prior authorization take for Xenpozyme in Washington? A: Standard review is typically 5-14 business days. Expedited review (if health urgency is documented) must be completed within 72 hours per Washington state requirements.

Q: What if Xenpozyme is not on Cigna's formulary? A: You can request a formulary exception through Cigna's standard process. Include clinical justification for why no formulary alternatives are appropriate for ASMD.

Q: Can I request an expedited appeal if denied? A: Yes, if the delay would seriously jeopardize your health. Washington allows expedited internal appeals (72 hours) and expedited external IRO review (72 hours) with proper medical documentation.

Q: Does step therapy apply to Xenpozyme? A: Typically no, since Xenpozyme is the only FDA-approved treatment for ASMD. However, some plans may require documentation that supportive care measures have been tried.

Q: What if my employer plan is self-funded? A: Self-funded plans may have different appeal timelines (45 days for external review vs. 20 days for fully-insured). Contact the Washington OIC to confirm which process applies to your specific plan.

Q: How much does Xenpozyme cost without insurance? A: Wholesale acquisition cost ranges from approximately $1,515 per 4mg vial to $7,576 per 20mg vial as of January 2025. Annual costs can exceed $400,000 depending on patient weight and dosing.

Sources & Further Reading

This guide is for educational purposes only and does not constitute medical advice. Always consult with your healthcare provider and insurance company for personalized guidance. For additional help with insurance appeals, contact the Washington Office of Insurance Commissioner at 1-800-562-6900.

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