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

How to Get Strimvelis Gene Therapy Covered by Blue Cross Blue Shield in Illinois: Complete Guide to International Treatment Authorization and Appeals

Answer Box: Getting Strimvelis Covered in Illinois

Strimvelis gene therapy for ADA-SCID requires special authorization from Blue Cross Blue Shield of Illinois (BCBSIL) as an experimental/international treatment. Start by calling BCBSIL member services at 1-800-892-2803 to verify international coverage benefits and request experimental treatment exception forms. Submit comprehensive medical documentation including ADA enzyme deficiency confirmation, genetic testing, and failed standard therapies. If denied, Illinois provides strong appeal rights through the Health Carrier External Review Act with independent physician review. Begin the process immediately—treatment is only available at SR-TIGET in Milan, Italy.

First step today: Call BCBSIL to confirm your plan includes international benefits and request prior authorization forms.

Table of Contents

  1. Understanding Strimvelis Coverage Challenges
  2. Pre-Authorization Requirements
  3. Step-by-Step Approval Process
  4. Common Denial Reasons & Solutions
  5. Illinois Appeals Process
  6. Medical Necessity Documentation
  7. Timeline and Deadlines
  8. Cost and Financial Assistance
  9. FAQ

Understanding Strimvelis Coverage Challenges

Strimvelis (autologous CD34+ ADA-SCID gene therapy) presents unique coverage challenges for Blue Cross Blue Shield of Illinois members. This one-time gene therapy, with a list price of approximately €594,000 (~$650,000), is only available at SR-TIGET (San Raffaele Telethon Institute for Gene Therapy) in Milan, Italy.

The primary obstacles include:

  • Experimental classification: Most BCBS plans classify gene therapies as investigational unless specifically covered
  • International treatment requirements: Standard plans typically cover only emergency international care
  • Site-specific administration: Unlike other therapies, Strimvelis cannot be administered in the U.S.
  • Limited shelf life: The personalized nature requires coordination between U.S. physicians and Milan specialists

Blue Cross Blue Shield of Illinois, operated by Health Care Service Corporation (HCSC), requires prior authorization for high-cost specialty treatments, making advance planning essential.

Pre-Authorization Requirements

Essential Documentation Checklist

Before submitting your request, gather these critical documents:

Document Type Specific Requirements Where to Obtain
ADA enzyme testing <1% normal activity in red blood cells Immunology lab
Genetic confirmation Bi-allelic ADA gene mutations Genetic testing lab
Metabolite levels Elevated deoxyadenosine (dAdo) Specialized biochemistry lab
Prior treatment records Documentation of failed therapies (ERT, HSCT evaluation) Medical records
Physician letter Medical necessity with clinical rationale Treating immunologist
SR-TIGET eligibility Confirmation of candidacy for treatment Milan facility

Diagnosis Confirmation

ADA-SCID diagnosis requires absent or very low ADA enzyme activity (<1% of normal) confirmed through:

  • Baseline blood enzyme assay
  • Elevated adenosine or deoxyadenosine levels
  • Genetic testing showing pathogenic ADA variants
  • Clinical presentation consistent with severe combined immunodeficiency
Note: Many cases are now identified through newborn screening, but delayed-onset forms may be missed by standard TREC testing.

Step-by-Step Approval Process

Step 1: Verify Coverage (Days 1-2)

Call BCBSIL member services at 1-800-892-2803 to:

  • Confirm international treatment benefits
  • Verify out-of-network coverage limits
  • Request experimental treatment exception forms
  • Check if your plan includes GeoBlue or Global Core benefits

Step 2: Gather Medical Documentation (Weeks 1-4)

Work with your immunologist to compile:

  • Comprehensive medical history
  • Laboratory confirmation of ADA-SCID
  • Documentation of treatment failures or contraindications
  • Letter of medical necessity (see template below)

Step 3: Submit Prior Authorization (Day 30)

Submit through the Availity provider portal or by phone. Include:

  • Completed prior authorization forms
  • All medical documentation
  • SR-TIGET treatment plan
  • Cost estimates and timeline

Step 4: Await Initial Decision (Days 31-60)

BCBSIL typically responds within 30 days for standard requests. For urgent cases, request expedited review (24-48 hour decision).

Common Denial Reasons & Solutions

Denial Reason Solution Strategy Required Documentation
"Experimental/Investigational" File medical exception request EMA approval data, peer-reviewed studies
"Not available in U.S." Demonstrate medical necessity for international care SR-TIGET exclusivity documentation
"No international benefits" Appeal with medical necessity Physician letter stating no U.S. alternatives
"Insufficient prior therapies" Document failed treatments ERT trial records, HSCT contraindications

When facing denials, Counterforce Health specializes in turning insurance denials into targeted, evidence-backed appeals by identifying the specific denial basis and crafting point-by-point rebuttals aligned to the plan's own rules.

Illinois Appeals Process

Illinois provides robust patient protection through the Health Carrier External Review Act. The process includes:

Internal Appeal (Required First Step)

  • Timeline: Must be filed within 180 days of denial
  • Review period: 15 business days for pre-service requests
  • Expedited: 24 hours for urgent cases

External Review (Independent Physician Review)

  • Filing deadline: Within 4 months of final adverse determination
  • Process: Independent Review Organization (IRO) assigns physician reviewers
  • Decision timeline: 5 business days after receiving all information (maximum 45 days total)
  • Expedited external review: 72 hours for life-threatening situations
Critical: Illinois has a shorter external review filing window (4 months) compared to many states. Don't delay if your internal appeal is denied.

For assistance, contact:

  • Illinois Department of Insurance: 866-445-5364
  • Illinois Attorney General Health Care Helpline: 1-877-305-5145

Medical Necessity Documentation

Physician Letter Template Elements

Your treating immunologist should include:

Patient Information:

  • Confirmed ADA-SCID diagnosis with supporting lab values
  • Clinical presentation and severity
  • Current immune status and infection history

Treatment History:

  • Prior therapies attempted (enzyme replacement therapy, prophylactic treatments)
  • Reasons for HSCT ineligibility (no suitable donor, contraindications)
  • Failed or inadequate responses to standard care

Medical Necessity Rationale:

  • Why Strimvelis is the most appropriate treatment option
  • Expected clinical benefits and outcomes
  • Risks of delaying treatment
  • Confirmation that treatment is only available at SR-TIGET

Supporting Evidence:

  • Reference to EMA approval and clinical trial data
  • Relevant medical literature
  • Professional society guidelines supporting gene therapy for ADA-SCID

Timeline and Deadlines

Phase Standard Timeline Expedited Timeline Key Actions
Documentation gathering 2-4 weeks 1-2 weeks Obtain all required tests and records
Prior authorization submission Day 1 Day 1 Submit complete packet
Initial review 30 days 24-48 hours BCBSIL decision
Internal appeal (if needed) 15 business days 24 hours Submit appeal with additional evidence
External review (if needed) 45 days maximum 72 hours IRO physician review

Total potential timeline: 3-6 months for complex cases requiring appeals.

Cost and Financial Assistance

Insurance Coverage Considerations

  • List price: Approximately €594,000 (~$650,000)
  • Out-of-network maximums: Verify your plan's annual limits
  • Travel and accommodation: Often not covered; explore separate assistance

Financial Assistance Options

  • Orchard Therapeutics patient support: Contact manufacturer directly
  • Patient advocacy foundations: Research rare disease organizations
  • State programs: Illinois may have additional resources for rare diseases

The specialized nature of Strimvelis coverage makes working with experienced advocates crucial. Counterforce Health helps patients navigate these complex international treatment scenarios by providing evidence-backed appeals that address payers' specific concerns about experimental treatments and out-of-network care.

FAQ

How long does BCBS Illinois prior authorization take? Standard requests: 30 days. Expedited requests (for urgent medical situations): 24-48 hours. Always request expedited review if delays could harm the patient's health.

What if Strimvelis is classified as experimental? File a medical exception request emphasizing that it's EMA-approved and the only available treatment for ADA-SCID. Include peer-reviewed evidence and physician certification of medical necessity.

Can I appeal if my plan doesn't cover international treatment? Yes. Illinois law allows appeals for medical necessity. Document that Strimvelis is only available at SR-TIGET and no equivalent treatment exists in the U.S.

How do I request an expedited appeal in Illinois? Contact BCBSIL immediately and request expedited review based on the urgent nature of ADA-SCID. Submit documentation showing that delays could worsen the patient's condition.

What happens if the external review is approved? The decision is binding. BCBSIL must provide coverage according to the IRO's determination.

Do I need a lawyer for appeals? Not required, but complex cases benefit from professional advocacy. Patient advocates and healthcare attorneys can help navigate the process.

What documentation do I need for the external review? Include the denial letter, all medical records, physician letters, and any additional evidence supporting medical necessity. The IRO will review everything submitted.

How much does the external review cost? Nothing for patients. Illinois law requires insurers to pay for the external review process.

This guide is for informational purposes only and does not constitute medical or legal advice. Always consult with your healthcare provider and insurance company for specific guidance. For additional assistance with Illinois insurance appeals, contact the Illinois Department of Insurance at 866-445-5364 or visit insurance.illinois.gov.

Sources & Further Reading

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