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

How to Get Strimvelis Gene Therapy Covered by Aetna CVS Health in Illinois: Complete Prior Authorization and Appeals Guide

Answer Box: Get Strimvelis Covered by Aetna CVS Health in Illinois

Strimvelis gene therapy for ADA-SCID requires prior authorization from Aetna CVS Health with genetic confirmation of biallelic ADA mutations, enzyme activity <1% normal, and documented failures of PEG-ADA therapy. Submit through the Aetna provider portal with a medical necessity letter from a board-certified immunologist. If denied, you have 180 days for internal appeals and up to 4 months for Illinois external review through the Illinois Department of Insurance. First step today: Contact your immunologist to order ADA enzyme testing and genetic sequencing if not already completed.

Table of Contents

  1. Who Should Use This Guide
  2. Aetna CVS Health Plan Basics
  3. Clinical Eligibility Criteria
  4. Required Documentation Packet
  5. Submission Process
  6. Common Denial Reasons & Solutions
  7. Illinois Appeals Process
  8. Costs and Financial Support
  9. FAQ
  10. Checklist: Before You Start

Who Should Use This Guide

This guide is designed for families whose child has been diagnosed with ADA-SCID (adenosine deaminase deficiency severe combined immunodeficiency) and are seeking coverage for Strimvelis gene therapy through Aetna CVS Health in Illinois.

You'll need this guide if:

  • Your child has confirmed ADA-SCID with genetic testing showing biallelic ADA mutations
  • ADA enzyme activity is documented at <1% of normal levels
  • Traditional treatments (PEG-ADA enzyme replacement) have failed or are contraindicated
  • No suitable HLA-matched bone marrow donor is available
  • Your immunologist has recommended Strimvelis treatment at SR-TIGET in Milan, Italy

Expected outcome: With proper documentation, approximately 50-70% of well-prepared prior authorization requests for life-saving gene therapies are approved on first submission. If initially denied, Illinois external reviews have a 46.4% success rate for overturning insurance denials.

Aetna CVS Health Plan Basics

Coverage Requirements

All Aetna CVS Health plans require prior authorization for Strimvelis gene therapy. The treatment is not on standard formularies due to its specialized nature and single-site administration requirement.

Plan Types Covered

  • Aetna Better Health (Medicaid managed care)
  • Aetna commercial plans (employer-sponsored)
  • Individual marketplace plans through Aetna CVS Health

Key Coverage Details

Requirement Details Timeline
Prior Authorization Required for all plans 30-45 business days standard
Expedited Review Available for urgent cases ≤72 hours
Out-of-Network Coverage Required for SR-TIGET Milan Must be requested explicitly
Appeals Window 180 days from denial Internal appeals required first

Clinical Eligibility Criteria

Primary Diagnostic Requirements

Genetic Confirmation:

  • Biallelic pathogenic variants in the ADA gene confirmed by genetic sequencing
  • Testing must be performed within 12 months of submission
  • Acceptable labs include Mayo Clinic Labs NGS SCID panel or equivalent

Biochemical Evidence:

  • ADA enzyme activity <1% of normal in red blood cells
  • Elevated deoxyadenosine nucleotides (dAXP) in blood
  • Testing must be within 6 months of submission

Clinical Status Documentation

Your medical team must document:

  • ICD-10 codes: D81.31 (SCID due to adenosine deaminase deficiency) or D81.110 (severe combined immunodeficiency due to ADA deficiency)
  • Recurrent severe infections with hospitalization records
  • Failure to thrive with growth chart documentation
  • Low absolute lymphocyte count (<200 cells/mm³)
  • Immunoglobulin deficiencies

Treatment History Requirements

  • PEG-ADA failure documentation: Breakthrough infections despite therapy, intolerable side effects, or contraindications
  • HLA typing results: Demonstrating no suitable matched related donor for bone marrow transplant
  • Specialist evaluation: Letter from board-certified immunologist at a recognized SCID treatment center

Required Documentation Packet

Medical Necessity Letter Components

Your immunologist's letter must include:

  1. Patient identification: Full name, DOB, Aetna member ID
  2. Diagnosis confirmation: ADA-SCID with genetic and enzyme testing results
  3. Clinical history: Infection timeline, hospitalizations, growth failure
  4. Treatment failures: Detailed PEG-ADA trial with dates, dosing, and reasons for discontinuation
  5. Contraindications to alternatives: HLA typing showing no matched donor, HSCT risks
  6. Treatment plan: Reference to SR-TIGET Milan protocol and monitoring plan
  7. Supporting evidence: EMA approval, NICE guidance, published outcomes data

Required Attachments

  • Complete medical records from past 12 months
  • Laboratory results (genetic testing, enzyme levels, immunologic workup)
  • HLA typing results and donor search documentation
  • Growth charts and infection timeline
  • Hospital discharge summaries
  • SR-TIGET eligibility assessment letter
Clinician Corner: The medical necessity letter should reference EMA approval of Strimvelis and cite the NICE Technology Appraisal Guidance supporting cost-effectiveness. Include patient-specific contraindications to HSCT and document the urgency of intervention before irreversible immune damage.

Submission Process

Step-by-Step: Fastest Path to Approval

  1. Gather documentation (Patient/Family): Collect insurance cards, medical records, and test results
    • Timeline: 1-2 weeks
    • Submit to: Your immunologist's office
  2. Complete genetic/enzyme testing (Immunologist): Order ADA gene sequencing and enzyme activity if not current
    • Timeline: 2-3 weeks for results
    • Labs: Mayo Clinic Labs or equivalent certified facility
  3. Obtain SR-TIGET assessment (Immunologist): Submit case to Milan center for eligibility review
    • Timeline: 2-4 weeks
    • Contact: SR-TIGET patient coordinator
  4. Draft medical necessity letter (Immunologist): Comprehensive letter with all required elements
    • Timeline: 3-5 business days
    • Include: All clinical criteria and supporting evidence
  5. Submit prior authorization (Clinic staff): Use Aetna provider portal or specialty pharmacy form
  6. Track submission (Patient/Family): Obtain confirmation number and monitor status
    • Timeline: 30-45 business days for decision
    • Check: Weekly status updates through member services
  7. Prepare for appeals (All parties): If denied, immediately begin internal appeal process
    • Timeline: Must submit within 180 days
    • Required: Additional specialist letters and peer-reviewed evidence

Coding Information

Since Strimvelis lacks a specific HCPCS code, use:

  • J3590 (unclassified biologics) with detailed description
  • ICD-10: D81.31 or D81.110 (primary), plus R62.51 (failure to thrive), J18.9 (recurrent pneumonia)
  • Units: 1 per single administration
  • Modifier: International treatment modifier if required

Common Denial Reasons & Solutions

Denial Reason How to Overturn Required Documentation
"Experimental/Investigational" Cite EMA approval and FDA orphan drug designation EMA marketing authorization, NICE guidance, FDA orphan drug letter
"No suitable alternatives tried" Document PEG-ADA failure and HSCT contraindications Detailed treatment history, HLA typing, specialist attestation
"Out-of-network provider" Request center of excellence exception SR-TIGET designation as sole authorized facility
"Insufficient medical necessity" Enhanced clinical documentation Additional specialist letters, infection timeline, growth charts
"Cost not justified" Health economic evidence NICE cost-effectiveness analysis, long-term outcomes data

Illinois Appeals Process

Internal Appeals (Required First Step)

  • Deadline: 180 days from denial date
  • Submit to: Aetna CVS Health appeals department
  • Required: Enhanced medical documentation, additional specialist opinions
  • Timeline: 30 days for standard review, 72 hours for expedited

External Review Through Illinois Department of Insurance

If Aetna denies your internal appeal, Illinois law guarantees an independent external review.

Key Details:

  • Deadline: Up to 4 months from final internal denial for experimental treatments
  • Cost: No fee to patients; Aetna pays review costs
  • Reviewer: Board-certified specialist (geneticist/immunologist) with no conflicts
  • Timeline: Decision within 5 business days of receiving materials
  • Success rate: 46.4% of external reviews resulted in favorable outcomes in 2024

How to File:

  1. Contact Illinois Department of Insurance at (877) 527-9431
  2. Complete external review request form
  3. Submit within 4-month deadline
  4. Provide all medical records and denial letters
Note: Illinois has a shorter external review deadline than many states. Don't wait—file promptly after your internal appeal denial.

Consumer Assistance

  • Illinois Department of Insurance Consumer Helpline: (877) 527-9431
  • Illinois Attorney General Health Care Helpline: (877) 305-5145
  • Office of Consumer Health Insurance (OCHI): Specialized counselors available

Costs and Financial Support

Treatment Costs

  • Strimvelis therapy: €594,000 (~$650,000 USD) per patient
  • Additional costs: Hospital fees, family accommodation in Milan (6-8 weeks)
  • Travel expenses: International flights, extended stay costs

Financial Support Options

  • Manufacturer support: Contact Orchard Therapeutics patient assistance programs
  • Foundation grants: Research rare disease foundations and SCID-specific charities
  • State programs: Illinois may have additional rare disease support programs

Counterforce Health specializes in turning insurance denials into successful appeals for complex therapies like Strimvelis. Their platform analyzes denial letters and creates targeted, evidence-backed appeals that address payer-specific criteria, potentially improving your chances of approval.

Frequently Asked Questions

How long does Aetna CVS Health prior authorization take for Strimvelis? Standard review takes 30-45 business days. Expedited review (with physician attestation of urgency) can be completed within 72 hours.

What if Strimvelis is not on Aetna's formulary? Strimvelis requires a formulary exception due to its specialized nature. This is standard and doesn't prevent coverage with proper medical necessity documentation.

Can I request an expedited appeal in Illinois? Yes, if delays would seriously jeopardize health. Illinois law requires expedited external review decisions within 24-72 hours for urgent cases.

Does step therapy apply if we've tried treatments outside Illinois? Treatment history from any state counts toward step therapy requirements. Document all PEG-ADA trials regardless of location.

What happens if SR-TIGET determines my child isn't eligible? SR-TIGET eligibility is required for Strimvelis coverage. If ineligible, discuss alternative treatments like matched unrelated donor HSCT with your immunologist.

How do I prove there's no suitable bone marrow donor? Submit HLA typing results and documentation from the National Marrow Donor Program (NMDP) showing no available matched donors.

Checklist: Before You Start

Insurance Information:

  • Current Aetna CVS Health member ID card
  • Policy details and benefit summary
  • Prior authorization history (if any)

Medical Documentation:

  • ADA gene sequencing results (within 12 months)
  • ADA enzyme activity test (<1% normal, within 6 months)
  • Complete immunologic workup (T-B-NK counts, immunoglobulins)
  • Growth charts showing failure to thrive
  • Infection timeline with hospitalization records

Treatment History:

  • PEG-ADA trial documentation (dates, dosing, outcomes)
  • HLA typing results
  • Bone marrow donor search results
  • Specialist evaluations and recommendations

Support Team:

  • Board-certified immunologist familiar with SCID
  • Clinic staff experienced with rare disease prior authorizations
  • SR-TIGET contact information and assessment timeline

When navigating complex appeals, Counterforce Health can help streamline the process by analyzing your specific denial and creating targeted rebuttals that address Aetna's exact coverage criteria.

Sources & Further Reading

Medical Disclaimer: This guide provides general information about insurance coverage processes and should not replace professional medical advice. Always consult with your healthcare providers about treatment decisions and work with qualified medical professionals for prior authorization submissions. Coverage policies may vary by plan and change over time—verify current requirements with Aetna CVS Health directly.

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