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

Do You Qualify for Xenpozyme (olipudase alfa-rpcp) Coverage by Cigna in Ohio? Decision Tree & Next Steps

Answer Box: Getting Xenpozyme (olipudase alfa-rpcp) Covered by Cigna in Ohio

Eligibility: You likely qualify if you have confirmed ASMD (Niemann-Pick disease type B or A/B) with genetic testing showing biallelic SMPD1 variants and enzyme activity <10% of normal. Fastest path: Have your metabolic specialist complete Cigna's Xenpozyme prior authorization form with genetic test results and organ involvement documentation. First step today: Call Cigna at the number on your card to confirm your plan's specialty pharmacy network and get the Ohio-specific PA form.

Table of Contents

  1. How to Use This Decision Tree
  2. Eligibility Triage: Do You Qualify?
  3. If "Likely Eligible": Document Checklist
  4. If "Possibly Eligible": Tests to Request
  5. If "Not Yet": Alternatives and Exception Requests
  6. If Denied: Ohio Appeal Path
  7. Coverage Requirements at a Glance
  8. Common Denial Reasons & How to Fix Them
  9. Frequently Asked Questions
  10. Sources & Further Reading

How to Use This Decision Tree

This guide helps patients with acid sphingomyelinase deficiency (ASMD) and their clinicians navigate Cigna's coverage process for Xenpozyme (olipudase alfa-rpcp) in Ohio. Start with the eligibility triage below, then follow the path that matches your situation.

Note: This covers pharmacy benefit prior authorization. If your doctor plans to bill Xenpozyme under your medical benefit (Part B or hospital outpatient), different forms and processes may apply.

Eligibility Triage: Do You Qualify?

Work through these questions with your healthcare team:

✅ Likely Eligible if you answer YES to all:

  • Confirmed ASMD diagnosis: You have genetic testing showing biallelic pathogenic variants in SMPD1 AND enzyme testing showing acid sphingomyelinase activity <10% of normal
  • Non-CNS disease: You have type B or type A/B ASMD (not pure type A with severe neurological involvement)
  • Organ involvement: You have documented hepatosplenomegaly, lung disease (decreased DLCO), or other non-CNS manifestations
  • Specialist care: A metabolic disease specialist or geneticist is prescribing or consulting on your treatment

⚠️ Possibly Eligible if you answer YES to some but not all:

  • You have clinical signs of ASMD (enlarged spleen/liver, lung problems, blood abnormalities) but incomplete genetic testing
  • You have one confirmed SMPD1 variant but need deletion/duplication analysis
  • You're seeing a general practitioner but haven't been evaluated by a metabolic specialist

❌ Not Yet Eligible if:

  • You don't have a confirmed ASMD diagnosis
  • You have type A ASMD with primarily CNS manifestations
  • You're requesting Xenpozyme for off-label use

If "Likely Eligible": Document Checklist

Gather these documents before submitting your prior authorization:

Required Clinical Documentation

  • Genetic test results: SMPD1 sequencing showing biallelic pathogenic variants
  • Enzyme testing: Acid sphingomyelinase activity results (<10% of normal controls)
  • Specialist consultation: Notes from metabolic disease specialist or geneticist
  • Organ assessment: Recent imaging (spleen/liver volumes), pulmonary function tests (DLCO), or relevant lab values

Submission Process

  1. Get the right form: Use Cigna's Xenpozyme-specific PA form
  2. Complete thoroughly: Your specialist must document disease type (B or A/B), organ involvement, and treatment rationale
  3. Submit via preferred method: CoverMyMeds, electronic PA through your EHR, or fax as directed on the form
  4. Track timing: Cigna must respond within 72 hours for standard requests, 24 hours for urgent requests
Counterforce Health Tip: If you're struggling with the prior authorization process, Counterforce Health specializes in turning insurance denials into targeted, evidence-backed appeals for specialty medications like Xenpozyme.

If "Possibly Eligible": Tests to Request

Missing Genetic Confirmation

  • Request: Complete SMPD1 sequencing if you only have partial results
  • If one variant found: Ask for deletion/duplication analysis of SMPD1
  • Timeline: Genetic testing typically takes 2-3 weeks
  • Where to get it: Most major reference labs (Mayo, ARUP, Quest, Labcorp) serve Ohio

Missing Enzyme Testing

  • Request: Acid sphingomyelinase activity in leukocytes or fibroblasts
  • Specimen: Blood sample (EDTA tube) sent to biochemical genetics lab
  • Timeline: Usually 1-3 weeks
  • Important: This test should be done first, before genetic testing

Missing Specialist Evaluation

  • Find a specialist: Look for metabolic disease specialists at Ohio children's hospitals or academic medical centers
  • What they'll assess: Disease severity, organ involvement, treatment goals, and monitoring plan
  • Timeline: Initial consultation may take 4-8 weeks to schedule

If "Not Yet": Alternatives and Exception Requests

For Unconfirmed Diagnosis

  • Focus on diagnostic workup: Complete genetic and enzyme testing before pursuing Xenpozyme coverage
  • Consider supportive care: Manage symptoms with lipid therapy, organ-specific treatments while pursuing diagnosis

For Off-Label Requests

  • Prepare robust documentation: Literature review, case series, expert opinion letters
  • Consider formulary exception: Use Cigna's formulary exception process for non-formulary requests
  • Timeline: Exception decisions typically within 72 hours of complete submission

If Denied: Ohio Appeal Path

Level 1: Internal Appeal with Cigna

  • Timeline: File within 180 days of denial
  • Process: Submit written appeal with additional clinical evidence
  • Decision timeframe: Generally 15-30 days for standard review, faster for urgent

Level 2: Peer-to-Peer Review

  • Request: Ask your specialist to request a peer-to-peer call with Cigna's medical director
  • Preparation: Have your doctor prepare key clinical points and guideline references
  • Often effective: Many denials are overturned when specialists explain rare disease complexities

Level 3: Ohio External Review

  • Eligibility: Available after completing Cigna's internal appeals for medical necessity denials
  • Timeline: Request within 180 days of final internal denial through Ohio Department of Insurance
  • Process: Independent Review Organization (IRO) with relevant clinical expertise reviews your case
  • Decision timeframe: 30 days standard, 72 hours expedited
  • Binding: If IRO reverses denial, Cigna must cover the treatment
Get Help: Contact Ohio Department of Insurance Consumer Services at 1-800-686-1526 for assistance with the external review process.

Coverage Requirements at a Glance

Requirement What It Means Where to Find It
Prior Authorization Required for all Xenpozyme prescriptions Cigna PA form
Diagnosis Confirmation Biallelic SMPD1 variants + enzyme activity <10% Cigna coverage policy
Specialist Requirement Metabolic disease specialist or geneticist Cigna coverage policy
Site of Care Clinical setting for escalation, home possible for maintenance FDA prescribing information
Initial Approval Up to 12 months Cigna coverage policy
Reauthorization Requires documented beneficial response Cigna coverage policy

Common Denial Reasons & How to Fix Them

Denial Reason How to Overturn
"Diagnosis not confirmed" Submit genetic testing (SMPD1) and enzyme assay results
"Experimental/investigational" Cite FDA approval for non-CNS ASMD (August 2022)
"Not medically necessary" Document organ involvement, functional impairment, disease progression
"Site of care not appropriate" Provide medical necessity rationale for requested infusion setting
"Quantity over limit" Submit weight-based dosing calculation per FDA label

Frequently Asked Questions

How long does Cigna prior authorization take in Ohio? Standard requests: 72 hours after complete submission. Urgent requests: 24 hours. If Cigna doesn't respond within these timeframes, the request is considered approved by default.

What if Xenpozyme is non-formulary on my plan? Request a formulary exception using Cigna's exception process. Your specialist must document why formulary alternatives are inappropriate for ASMD.

Can I request an expedited appeal? Yes, if delay would seriously jeopardize your health or interrupt ongoing treatment. Both Cigna internal appeals and Ohio external review offer expedited options.

Does step therapy apply to Xenpozyme? There are no FDA-approved alternatives for ASMD, so traditional step therapy shouldn't apply. If encountered, request a step therapy exception citing lack of therapeutic alternatives.

What if I need home infusion? Cigna may require clinical justification. Document if you cannot safely travel to an infusion center or if home administration is medically preferable after completing dose escalation.

How much does Xenpozyme cost? Wholesale acquisition cost ranges from approximately $1,515 for a 4mg vial to $7,576 for a 20mg vial. Annual costs can exceed $200,000. Check for manufacturer copay assistance and patient support programs.

Counterforce Health helps patients navigate these complex coverage challenges by creating targeted appeals that address each payer's specific requirements and deadlines.

Sources & Further Reading

This guide is for informational purposes only and does not constitute medical or legal advice. Coverage decisions depend on your specific plan terms and clinical circumstances. Always consult with your healthcare provider and insurance plan for the most current requirements and procedures.

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