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

Getting Strimvelis Gene Therapy Covered by Cigna in Virginia: Complete Decision Tree and Appeals Guide

Answer Box: Your Path to Strimvelis Coverage

Strimvelis eligibility requires confirmed ADA-SCID diagnosis via genetic testing and enzyme levels, plus documentation that no suitable matched donor is available. Since Strimvelis is only administered at SR-TIGET in Milan, you'll need Cigna's out-of-network international coverage approval. First step today: Call Cigna member services to verify your plan's gene therapy and international treatment benefits, then request prior authorization forms. If denied, Virginia's external review process provides a 120-day appeal window through the State Corporation Commission.

Table of Contents

  1. How to Use This Guide
  2. Eligibility Triage: Do You Qualify?
  3. If You're Likely Eligible: Document Checklist
  4. If You're Possibly Eligible: Tests to Request
  5. If Not Yet Eligible: Alternatives and Exceptions
  6. If Denied: Virginia Appeals Path
  7. Common Denial Reasons and Solutions
  8. Costs and Financial Assistance
  9. FAQ
  10. Sources and Further Reading

How to Use This Guide

This decision tree helps Virginia patients and their families navigate Cigna's coverage process for Strimvelis, a one-time gene therapy for ADA-SCID that's only available at SR-TIGET in Milan, Italy. Work through each section in order to determine your eligibility and next steps.

Note: Strimvelis is not FDA-approved in the U.S., making this an out-of-network, international treatment requiring special authorization.

Eligibility Triage: Do You Qualify?

Likely Eligible ✅

You probably qualify if you have:

  • Confirmed ADA-SCID diagnosis with both genetic testing (bi-allelic ADA gene mutations) and low/absent ADA enzyme levels
  • No suitable HLA-matched related donor available for stem cell transplant
  • Failed or contraindicated for enzyme replacement therapy (ERT)
  • Cigna plan that covers out-of-network gene therapy (verify this first)

Possibly Eligible ⚠️

You might qualify with additional documentation if you have:

  • Suspected ADA-SCID but incomplete genetic testing
  • Partial enzyme testing results
  • Ongoing donor search that may not yield suitable matches
  • Plan coverage unclear for international treatments

Not Yet Eligible ❌

You'll need more preparation if you have:

  • No confirmed ADA-SCID diagnosis
  • Available matched sibling donor
  • Plan that excludes gene therapy or international treatment
  • Stable on current ERT without clear medical necessity for gene therapy

If You're Likely Eligible: Document Checklist

Required Medical Documentation

Genetic Testing Results:

  • Full ADA gene sequencing report showing bi-allelic pathogenic variants
  • Laboratory accreditation details and patient identifiers
  • Genetic counselor summary if available

ADA Enzyme Activity:

  • Quantitative ADA enzyme levels in erythrocytes
  • Reference ranges and testing methodology
  • Date of testing (recent preferred)

Clinical History:

  • Complete immunological workup showing SCID pattern
  • Infection history and hospitalizations
  • Current treatment response and complications
  • Growth and development records

Donor Search Documentation:

  • HLA typing results for patient and family members
  • National Marrow Donor Program (NMDP) search results
  • Transplant center evaluation stating no suitable donor

Submission Path

  1. Contact Cigna Member Services at the number on your insurance card
  2. Request gene therapy benefits verification and international coverage details
  3. Obtain prior authorization forms (typically through Express Scripts for specialty drugs)
  4. Submit complete package including all medical documentation
  5. Follow up within 5-7 business days to confirm receipt

If You're Possibly Eligible: Tests to Request

Diagnostic Testing Needed

From your immunologist or geneticist:

Timeline to Re-apply

  • Genetic testing results: 2-4 weeks
  • Enzyme testing: 1-2 weeks
  • HLA typing: 1-3 weeks
  • Donor search completion: 3-6 months

Plan to resubmit your prior authorization once all testing is complete and documented.

If Not Yet Eligible: Alternatives and Exceptions

Standard Treatment Options

Enzyme Replacement Therapy (ERT):

  • Pegademase bovine (PEG-ADA) for ongoing management
  • May be required as "fail-first" therapy before gene therapy consideration

Hematopoietic Stem Cell Transplant:

  • Gold standard when matched donor available
  • Lower risk than gene therapy if suitable donor found

Exception Request Strategy

If your plan excludes gene therapy, you can request a formulary exception based on:

  • Lack of FDA-approved alternatives in the U.S.
  • Medical necessity due to ADA-SCID severity
  • Failure or contraindication to standard treatments
  • Single-center availability requiring international travel

If Denied: Virginia Appeals Path

Virginia provides robust appeal rights through the State Corporation Commission Bureau of Insurance.

Internal Appeals with Cigna

First-Level Appeal:

  • Deadline: 180 days from denial notice
  • Method: Submit via Cigna member portal or written request
  • Timeline: 30 days for standard review, 72 hours for urgent
  • Documents: All original submission materials plus any new clinical evidence

Peer-to-Peer Review:

  • Request direct discussion between your physician and Cigna medical director
  • Often more successful than written appeals alone
  • Can be requested simultaneously with formal appeal

Virginia External Review

If internal appeals fail, Virginia law provides independent review:

Eligibility Requirements:

  • Completed Cigna's internal appeal process
  • Denial based on medical necessity or experimental treatment designation
  • Virginia-issued plan or participating self-funded plan

Timeline:

Submission Details:

  • Email, fax (804-371-9915), or mail to Virginia Bureau of Insurance
  • Include final denial letter, insurance information, and medical records release
  • For experimental treatment denials, also complete Form 216-D
From Our Advocates: We've seen families succeed with Virginia external review by focusing their appeal on the lack of U.S. alternatives and the single-center nature of Strimvelis administration. The independent medical reviewers often understand rare disease complexity better than initial payer reviewers, especially when provided with comprehensive genetic and clinical documentation.

Common Denial Reasons and Solutions

Denial Reason How to Overturn
"Experimental/investigational" Provide EU approval documentation, published efficacy data, and FDA orphan drug designation materials
"Not medically necessary" Submit comprehensive clinical history showing SCID severity, infection patterns, and treatment failures
"Out-of-network exclusion" Request exception based on no U.S. provider availability; cite single authorized treatment center
"No coverage for international treatment" Appeal based on medical necessity and lack of domestic alternatives
"Step therapy not completed" Document ERT trial results, side effects, or contraindications

Costs and Financial Assistance

Strimvelis List Price: €594,000 (~$650,000 USD) for single treatment

Financial Support Options

Manufacturer Programs:

  • Contact Orchard Therapeutics patient support services
  • May offer assistance with travel and logistics for Milan treatment

Nonprofit Support:

  • Fondazione Telethon sometimes provides travel assistance for international families
  • Primary immunodeficiency foundations may offer emergency grants
  • Hospital social work departments can identify additional resources

Travel Considerations:

  • Plan for 4-6 months in Milan for pre-treatment, therapy, and monitoring
  • Consider temporary housing, family support, and medical travel insurance

When dealing with insurance coverage for rare disease treatments like Strimvelis, having expert support can make the difference between approval and denial. Counterforce Health specializes in turning insurance denials into targeted, evidence-backed appeals by analyzing denial letters, plan policies, and clinical notes to create compelling rebuttals aligned with payer requirements. Their platform helps patients and clinicians navigate complex prior authorization processes for specialty therapies, ensuring all required documentation and medical necessity criteria are properly addressed.

FAQ

How long does Cigna prior authorization take for Strimvelis in Virginia? Standard review takes 15 business days, but complex cases like gene therapy often require 30+ days. Request expedited review if clinically urgent.

What if Strimvelis is not on Cigna's formulary? Most gene therapies aren't on standard formularies. Request a formulary exception with medical necessity documentation and evidence of no suitable alternatives.

Can I request expedited appeals in Virginia? Yes, if your physician certifies that delay would seriously jeopardize your health. Virginia provides 72-hour expedited external review for urgent cases.

Does step therapy apply if I'm already on ERT? Cigna may require documented ERT trial, but failure, intolerance, or inadequate response can justify moving to gene therapy.

What happens if Cigna approves but won't cover travel costs? Approval typically covers the therapy itself but not travel. Explore manufacturer support programs and nonprofit assistance for logistics.

Can I appeal if I have a self-funded employer plan? Many Virginia-based self-funded plans participate in the state's external review process. If not, federal external review through HHS may be available.

How do I prove there's no suitable donor available? Provide HLA typing results, NMDP search documentation, and transplant center evaluation confirming no matched related or unrelated donors.

What if my appeal is denied at every level? Consider consulting with healthcare attorneys specializing in insurance coverage, especially for rare disease treatments. Counterforce Health can also help identify additional appeal strategies or procedural issues that might warrant further review.

Sources and Further Reading

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 directly for specific coverage decisions. Coverage policies and appeal procedures may change; verify current requirements with official sources.

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