By Counterforce Health Team in Strimvelis (autologous CD34+ ADA-SCID gene therapy) —

The Requirements Checklist to Get Strimvelis Gene Therapy Covered by Cigna in Michigan: Forms, Appeals, and Milan Treatment Guide

Answer Box: Getting Strimvelis Covered by Cigna in Michigan

Strimvelis requires prior authorization through Cigna's Express Scripts specialty pharmacy division using their Non-Specific Gene Therapy form. Key requirements: confirmed ADA-SCID diagnosis with bi-allelic ADA gene mutations, documented absence of HLA-matched related donor, and failed/contraindicated PEG-ADA enzyme replacement therapy. Since Strimvelis is only available at SR-TIGET in Milan, Italy, you'll need out-of-network international coverage approval. If denied, Michigan residents have 127 days to file external review with DIFS after completing Cigna's internal appeals.

First step today: Call Cigna member services (number on your insurance card) to verify gene therapy benefits and request the Non-Specific Gene Therapy prior authorization form.

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. Milan Treatment Logistics
  8. After Submission: What to Expect
  9. Common Denial Reasons & Solutions
  10. Michigan Appeals Process
  11. Quick Reference Checklist

Who Should Use This Checklist

This guide is for patients diagnosed with adenosine deaminase deficiency-severe combined immunodeficiency (ADA-SCID) who:

  • Have confirmed bi-allelic ADA gene mutations
  • Lack a suitable HLA-matched related donor for stem cell transplant
  • Have failed or cannot tolerate PEG-ADA enzyme replacement therapy
  • Are considering Strimvelis gene therapy at San Raffaele Hospital in Milan, Italy

Expected outcome: With proper documentation, approximately 60-70% of well-prepared prior authorization requests for rare disease gene therapies are approved on initial submission. For denials, Michigan's external review process has a favorable overturn rate for rare diseases when strong clinical evidence is provided.

From our advocates: We've seen families successfully navigate this process by starting early—ideally 3-6 months before planned treatment. The key is coordinating between your immunologist, Cigna's case management team, and the Milan center simultaneously. One family told us their breakthrough came when they framed the request as a "single-case agreement" for lifesaving treatment not available in the U.S.

Member & Plan Basics

Verify Your Coverage

Call Cigna member services (number on insurance card) and confirm:

  • Gene therapy benefits: Ask specifically about coverage for "experimental" or "investigational" therapies
  • Out-of-network benefits: International treatment coverage
  • Specialty pharmacy: Whether Express Scripts manages gene therapy prior authorizations
  • Case management: Request assignment to a case manager for complex/rare disease treatments

Plan Type Verification

Important: Self-funded employer plans (ERISA) may have different appeal rights than fully-insured plans. Ask Cigna: "Is this a fully-insured plan regulated by Michigan, or a self-funded employer plan?"

This affects your external review rights with Michigan DIFS.

Clinical Criteria Requirements

Primary Diagnosis Requirements

Requirement Documentation Needed Where to Obtain
ADA-SCID Diagnosis Genetic testing showing bi-allelic ADA gene mutations Clinical genetics lab
Enzyme Deficiency ADA enzyme activity <1% of normal in erythrocytes Specialized immunology lab
SCID Confirmation Lymphocyte subset analysis, immunological workup Immunology specialist
No Matched Donor HLA typing results, donor search documentation Transplant center/NMDP

Treatment History Requirements

PEG-ADA Failure Documentation:

  • Clinical notes showing inadequate immune reconstitution
  • Infection patterns despite treatment
  • Intolerance or adverse reactions
  • Growth/development concerns

HSCT Evaluation:

  • HLA typing of family members
  • National Marrow Donor Program search results
  • Transplant center letter stating no suitable donor available

Coding and Billing Information

ICD-10 Diagnosis Codes

  • D81.0: SCID with reticular dysgenesis
  • D81.1: SCID with low T- and B-cell numbers
  • D81.2: SCID with low or normal B-cell numbers
  • D81.9: Combined immunodeficiency, unspecified

HCPCS/Billing Codes

  • C9399: Unclassified drugs or biologicals (for Strimvelis)
  • J3590: Unclassified biologicals
  • Note: Strimvelis lacks a specific J-code; use unclassified codes with detailed description

Site of Care

  • International hospital-based administration at SR-TIGET, Milan, Italy
  • Not available through U.S. specialty pharmacies or infusion centers

Documentation Packet

Medical Necessity Letter Components

Your immunologist or pediatric hematologist/oncologist should include:

Patient History:

  • Age at diagnosis, presenting symptoms
  • Infection history and hospitalizations
  • Growth and developmental milestones
  • Current clinical status

Treatment Rationale:

  • Why HSCT is not feasible (no matched donor)
  • PEG-ADA trial results and limitations
  • Expected outcomes with Strimvelis based on published data
  • Urgency of treatment (progressive immune dysfunction)

Supporting Evidence:

  • EMA approval documentation
  • Published efficacy data (100% overall survival in clinical trials)
  • NICE guidance supporting cost-effectiveness
  • FDA orphan drug designation

Required Attachments

✓ Genetic testing report (full ADA gene sequencing)
✓ Enzyme activity results (quantitative ADA levels)
✓ HLA typing and donor search documentation
✓ PEG-ADA treatment records and response
✓ Immunological workup (lymphocyte subsets, immunoglobulin levels)
✓ Bone marrow biopsy confirming adequate CD34+ cells for harvest
✓ Specialist consultation notes from immunology/hematology

Submission Process

Cigna Prior Authorization Forms

Primary form: Non-Specific Gene Therapy Prior Authorization

Submission methods:

  • Online: Cigna provider portal (preferred)
  • Fax: Check current fax number on form
  • Express Scripts: For specialty pharmacy routing

Required Form Fields

Patient Information:

  • Full name, DOB, Cigna member ID
  • Primary care physician and specialist information
  • Diagnosis codes (ICD-10)

Treatment Details:

  • Drug name: Strimvelis (autologous CD34+ gene therapy)
  • Indication: ADA-SCID
  • Proposed treatment site: SR-TIGET, Milan, Italy
  • Expected treatment date

Clinical Justification:

  • Medical necessity narrative
  • Prior therapy failures
  • Supporting literature references

Milan Treatment Logistics

Understanding the Strimvelis Model

Strimvelis is not a traditional drug—it's a personalized gene therapy manufactured on-site in Milan using the patient's own stem cells. This unique model affects insurance coverage:

  • Single treatment site: Only available at San Raffaele Hospital, Milan
  • Extended stay required: Families typically spend 6-8 weeks in Milan
  • Complex procedure: Involves cell harvest, genetic modification, conditioning chemotherapy, and reinfusion

Coverage Considerations

Hospital costs: Professional fees, facility charges, conditioning chemotherapy Travel expenses: May be covered under transplant/complex care benefits Accommodation: Some plans provide lodging allowances for out-of-area treatment

Counterforce Health can help navigate the unique billing and coverage challenges of international gene therapy administration.

After Submission: What to Expect

Timeline Expectations

Review Type Timeline Next Steps
Standard Review 7-30 days Check status via Cigna portal
Expedited Review 24-72 hours For urgent cases with physician attestation
Peer-to-Peer Additional 5-7 days Specialist discusses case with Cigna medical director

Confirmation and Tracking

Record these details:

  • Prior authorization reference number
  • Submission date and method
  • Cigna representative name (if submitted by phone)
  • Expected decision date

Status checks:

  • Call Cigna provider services every 5-7 days
  • Monitor online portal for updates
  • Document all communications

Common Denial Reasons & Solutions

Denial Reason How to Address Supporting Evidence
Experimental/Investigational Cite EMA approval, published outcomes EMA product information, clinical trial data
Not Medically Necessary Document disease progression, failed alternatives PEG-ADA failure records, infection history
Out-of-Network Emphasize sole treatment site globally Letter from Milan center, no U.S. alternatives
Insufficient Documentation Submit complete genetic/clinical package All items from documentation checklist

Five Critical Pitfalls to Avoid

  1. Incomplete genetic testing: Ensure full ADA gene sequencing, not just targeted variants
  2. Missing donor search: Document comprehensive HLA typing and registry searches
  3. Inadequate PEG-ADA trial: Show meaningful trial duration and objective failure criteria
  4. Generic medical necessity letter: Use ADA-SCID specialist, not general pediatrician
  5. Late submission: Start process 3-6 months before planned treatment date

Michigan Appeals Process

Internal Appeals with Cigna

Timeline: 180 days from denial to file internal appeal Process: Submit via Cigna appeals portal or mail Documentation: Include all original materials plus additional evidence addressing denial reason

Michigan DIFS External Review

If Cigna upholds the denial, Michigan residents can request external review through the Department of Insurance and Financial Services.

Key deadlines:

  • 127 days from final Cigna denial to file DIFS external review
  • 72 hours for expedited review (with physician letter documenting urgency)
  • 60 days maximum for standard external review decision

Filing options:

  • Online: DIFS External Review portal
  • Paper: Form FIS 0018 via email, fax, or mail

Required documentation:

  • All Cigna denial letters
  • Insurance card and policy information
  • Complete medical records
  • Specialist letter of medical necessity
  • Supporting literature and guidelines

When Treatment Delay Could Cause Harm

For expedited external review, your physician must document that delay would:

  • Seriously jeopardize life or health
  • Impair ability to regain maximum function
  • Cause irreversible disease progression

This is particularly relevant for ADA-SCID, where delayed treatment can lead to permanent immune system damage and increased infection risk.

Quick Reference Checklist

Before Starting

☐ Verify Cigna gene therapy benefits
☐ Confirm out-of-network/international coverage
☐ Request case manager assignment
☐ Contact Milan center for eligibility assessment

Documentation Gathering

☐ ADA gene sequencing results
☐ Enzyme activity testing
☐ HLA typing and donor search
☐ PEG-ADA treatment records
☐ Immunological workup
☐ Bone marrow biopsy results
☐ Specialist consultation notes

Submission Package

☐ Completed Non-Specific Gene Therapy PA form
☐ Medical necessity letter from specialist
☐ All supporting clinical documentation
☐ EMA approval and efficacy literature
☐ Cost-benefit analysis vs. lifetime care

After Submission

☐ Record PA reference number
☐ Schedule status check calls
☐ Prepare appeal materials if needed
☐ Coordinate with Milan center timeline

If Denied

☐ File Cigna internal appeal within 180 days
☐ Address specific denial reasons
☐ Prepare DIFS external review within 127 days
☐ Consider expedited review if urgent

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Always consult with your healthcare provider and insurance plan for specific coverage decisions. Treatment decisions should be made in consultation with qualified medical specialists.

For complex prior authorization and appeals support, Counterforce Health specializes in turning insurance denials into evidence-backed approvals for rare disease treatments and gene therapies.

Sources & Further Reading

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