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

Denied for Strimvelis Gene Therapy by Cigna in Texas? Complete Appeal Guide with Forms & Timelines

Answer Box: Getting Strimvelis Covered by Cigna in Texas

Strimvelis (autologous CD34+ ADA-SCID gene therapy) requires prior authorization from Cigna and is often initially denied as "experimental." To get coverage in Texas: (1) Gather genetic testing confirming ADA-SCID, enzyme deficiency results, and HLA typing showing no matched related donor, (2) Have your immunologist submit a detailed medical necessity letter citing EMA approval and NICE guidelines, (3) File internal appeals within 180 days if denied, then request Texas Independent Review Organization (IRO) external review within 4 months of final denial. Texas IRO decisions are binding and typically rendered within 20 days (5 days if expedited).

Table of Contents

  1. Understanding Your Cigna Coverage
  2. Required Documentation for Strimvelis
  3. Filing Your Initial Prior Authorization
  4. First-Level Internal Appeal Strategy
  5. Peer-to-Peer Review Preparation
  6. Texas External Review (IRO) Process
  7. Appeal Letter Templates & Scripts
  8. Tracking Your Case & Escalation
  9. When Appeals Fail: Alternative Options
  10. FAQ

Understanding Your Cigna Coverage

Coverage Framework for Gene Therapy

Cigna typically processes Strimvelis under the medical benefit (not pharmacy) due to its specialized administration requirements. The therapy requires:

  • Prior authorization through Cigna's utilization management
  • Express Scripts clinical review for pharmacy-related components
  • Center of excellence approval for overseas treatment
Note: Most Cigna plans exclude experimental/investigational treatments, making the medical necessity argument crucial.

Coverage at a Glance

Requirement What It Means Where to Find It Source
Prior Authorization Required before treatment Cigna provider portal Cigna Precertification
Medical Necessity Must meet ADA-SCID criteria EMA product information Strimvelis EPAR
Center of Excellence SR-TIGET Milan only International coverage review Cigna International Plans
Appeal Deadline 180 days from denial Plan documents Texas Insurance Code

Required Documentation for Strimvelis

Essential Clinical Evidence

Your immunologist must provide comprehensive documentation proving:

1. Confirmed ADA-SCID Diagnosis

  • Bi-allelic pathogenic variants in ADA gene (genetic testing report)
  • Markedly reduced ADA enzyme activity in lymphocytes/erythrocytes
  • Elevated toxic metabolites (deoxyadenosine nucleotides)
  • Clinical SCID presentation (severe infections, lymphopenia, failure to thrive)

2. Disease Severity Documentation

  • Complete infection history with hospitalizations
  • Current immunologic parameters (T-cell counts, immunoglobulin levels)
  • Growth charts and developmental assessments
  • ICU admissions and life-threatening episodes

3. Treatment History and Rationale

  • Current PEG-ADA enzyme replacement therapy details
  • Documentation of partial immune reconstitution only
  • HLA typing showing no suitable matched related donor
  • Explanation why matched unrelated donor HSCT is suboptimal
Tip: Request your complete medical records early—genetic testing and enzyme assays can take weeks to obtain from different laboratories.

Medical Necessity Letter Checklist

Your treating physician's letter should address:

  • Diagnosis confirmation with specific test results and dates
  • Regulatory status: EMA approval for ADA-SCID without matched related donor
  • Clinical urgency: ADA-SCID is uniformly fatal without definitive treatment
  • Alternative limitations: Why PEG-ADA alone and unrelated donor HSCT are inadequate
  • Center expertise: SR-TIGET's specialized experience and outcomes data
  • Cost-effectiveness: One-time treatment vs. lifelong PEG-ADA costs

Filing Your Initial Prior Authorization

Step-by-Step Process

1. Contact Cigna Customer Service (number on ID card)

  • Confirm whether your plan covers international treatment
  • Request specific gene therapy coverage policy
  • Ask for case manager assignment

2. Submit Prior Authorization Request

  • Use Cigna provider portal or fax to utilization management
  • Include complete clinical documentation package
  • Request expedited review if clinically urgent (72-hour decision)

3. Express Scripts Coordination

  • Call 1-800-753-2851 for pharmacy benefit components
  • Fax clinical information to 1-877-328-9660
  • Confirm receipt and tracking number

Expected Timelines

  • Standard review: 1-3 business days
  • Expedited review: 24-72 hours for urgent cases
  • Complex cases: May require additional clinical review time

First-Level Internal Appeal Strategy

Reading Your Denial Letter

Common denial reasons and responses:

Denial Reason Your Response Strategy
"Experimental/Investigational" Cite EMA approval, NICE HST7 recommendation
"Not medically necessary" Provide updated clinical data, infection history
"Alternative treatments available" Document PEG-ADA limitations, donor search results
"Out of network coverage" Request center of excellence exception

Building Your Appeal

1. Clinical Evidence Update

  • Recent infection episodes or hospitalizations
  • Current immune parameters showing inadequate reconstitution
  • Quality of life impact documentation

2. Regulatory and Guideline Support

3. Economic Argument

  • Lifetime PEG-ADA costs (€50,000+ annually) vs. one-time Strimvelis
  • Reduced hospitalization and complication costs
  • Improved quality-adjusted life years

Peer-to-Peer Review Preparation

Scheduling the Call

Contact Cigna's medical management department to request peer-to-peer review:

  • Have your immunologist call directly
  • Request review with pediatric immunology or transplant specialist
  • Prepare for 15-30 minute clinical discussion

Key Talking Points

For Your Physician:

  1. Patient-specific urgency: Current clinical status and infection risk
  2. Standard of care: Strimvelis is EMA-approved, not experimental
  3. Comparative outcomes: Lower mortality vs. unrelated donor HSCT
  4. Center expertise: SR-TIGET's unique experience and safety record
  5. Long-term perspective: Durable immune reconstitution with single treatment
Script: "This patient has confirmed ADA-SCID with no matched related donor. Strimvelis is the EMA-approved standard of care for this exact indication, with superior outcomes compared to alternative HSCT approaches."

Texas External Review (IRO) Process

When to Request External Review

After exhausting Cigna's internal appeals (typically 2 levels), Texas law provides Independent Review Organization (IRO) review for:

  • Medical necessity denials
  • Experimental/investigational determinations
  • Treatment appropriateness disputes

IRO Request Process

1. Obtain IRO Request Form

2. Submit Complete Package

  • IRO request form
  • All denial and appeal correspondence
  • Complete medical records
  • Physician support letters
  • Published evidence and guidelines

3. Timeline Expectations

  • Standard IRO: Decision within 20 days
  • Expedited IRO: Decision within 5 days for urgent cases
  • No cost to patient (Cigna pays IRO fees)
Important: IRO decisions are binding on Cigna—if approved, coverage must be provided.

Building Your IRO Case

Medical Necessity Arguments:

  • Document that ADA-SCID meets accepted medical definitions
  • Show Strimvelis is standard care per international guidelines
  • Demonstrate inadequacy of covered alternatives

Regulatory Support:

  • EMA approval validates non-experimental status
  • NICE cost-effectiveness analysis supports coverage
  • Published safety and efficacy data from clinical trials

Appeal Letter Templates & Scripts

Patient Phone Script for Cigna

"Hello, I'm calling about a prior authorization denial for my child's ADA-SCID gene therapy treatment. The denial letter states [specific reason]. I'd like to understand the exact coverage policy and start the internal appeal process. Can you connect me with a case manager who handles rare disease cases?"

Medical Necessity Letter Framework

Opening: "I am writing to appeal the denial of Strimvelis gene therapy for [patient name] who has confirmed adenosine deaminase-deficient severe combined immunodeficiency (ADA-SCID)."

Clinical Section:

  • Confirmed diagnosis with specific test results
  • Current clinical status and infection history
  • Prior treatments and their limitations

Medical Necessity Section:

  • EMA approval for this exact indication
  • NICE recommendation and cost-effectiveness
  • Comparison with alternative treatments

Urgency Section:

  • Life-threatening nature of untreated ADA-SCID
  • Limited treatment window considerations
  • Risk of delay in definitive therapy

Tracking Your Case & Escalation

Documentation Log

Keep detailed records of:

  • All phone calls (date, time, representative name, reference number)
  • Fax confirmations and delivery receipts
  • Email correspondence with timestamps
  • Appeal submission dates and tracking numbers

When to Escalate

State Insurance Department Complaint

Additional Resources

When Appeals Fail: Alternative Options

Manufacturer Support Programs

Contact Orchard Therapeutics patient access team:

  • Named patient access programs
  • Compassionate use protocols
  • International treatment coordination

Clinical Trial Options

Search ClinicalTrials.gov for:

  • ADA-SCID gene therapy trials
  • Alternative gene therapy approaches
  • Expanded access programs

Medical Tourism Coverage

Some Cigna international plans may cover:

  • Emergency medical evacuation
  • Treatment at designated centers of excellence
  • Coordinated care programs
From our advocates: One family successfully appealed after documenting their child's third hospitalization for opportunistic infections while on PEG-ADA. The key was showing that enzyme replacement provided only partial immune correction and that continued delays increased mortality risk. The IRO approved coverage within the expedited 5-day timeline.

FAQ

How long does Cigna prior authorization take for Strimvelis in Texas? Standard review takes 1-3 business days, but complex gene therapy cases often require additional clinical review. Request expedited review (24-72 hours) if delay could jeopardize health.

What if Strimvelis is considered experimental by Cigna? Appeal with EMA approval documentation and NICE cost-effectiveness analysis. The therapy is approved for your exact indication (ADA-SCID without matched related donor).

Can I request an expedited appeal? Yes, if delay could seriously jeopardize life, health, or maximum function. ADA-SCID's life-threatening nature typically qualifies for expedited review.

Does step therapy apply to ADA-SCID treatment? Most insurers recognize that PEG-ADA is temporizing therapy, not definitive treatment. Document ongoing infections and partial immune reconstitution despite enzyme replacement.

How much does the Texas IRO process cost? Nothing—Cigna pays all IRO fees. You have the right to external review at no cost after exhausting internal appeals.

What happens if IRO approves but Cigna still refuses? IRO decisions are legally binding in Texas. If Cigna doesn't comply, file a complaint with Texas Department of Insurance and consider legal action.

Can I get coverage for family travel to Milan? Standard coverage typically doesn't include family travel or accommodation. Some international medical evacuation benefits may cover patient transport costs.

How do I prove there's no matched related donor? Provide HLA typing results for patient and immediate family members, plus formal donor search documentation from your transplant center.

Counterforce Health specializes in turning complex insurance denials into successful appeals through evidence-based advocacy. Our platform helps patients and clinicians navigate the prior authorization process for specialty therapies like Strimvelis, providing targeted appeals that address specific payer criteria and regulatory requirements. When facing denials for life-saving treatments, having expert support can make the difference between coverage approval and continued delays in care.

For immediate assistance with your Strimvelis appeal, consider working with experienced advocates who understand both the clinical complexities of ADA-SCID and the specific coverage policies of major insurers like Cigna. Counterforce Health provides comprehensive support throughout the appeals process, from initial prior authorization through external review.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage decisions depend on individual plan terms and clinical circumstances. Always consult with your healthcare provider and insurance company for specific guidance regarding your situation. Appeal deadlines and requirements may vary by plan type.

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