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

Getting Strimvelis Gene Therapy Covered by Cigna in New Jersey: Complete Guide to Prior Authorization and Appeals

Quick Answer: Cigna Coverage for Strimvelis in New Jersey

Eligibility: Strimvelis requires prior authorization from Cigna as it's not FDA-approved and only available at SR-TIGET in Milan, Italy. Coverage depends on demonstrating medical necessity and exhausting standard treatments.

Fastest Path: 1) Confirm international coverage benefits, 2) Submit comprehensive prior authorization with genetic testing and failed HSCT documentation, 3) If denied, file internal appeal within 180 days, then external review through New Jersey's IHCAP program.

Start Today: Call Cigna member services (number on your card) to verify out-of-network/international benefits and request gene therapy prior authorization forms.

Table of Contents

  1. Understanding Strimvelis Coverage Requirements
  2. Pre-Authorization Preparation
  3. Submission Process
  4. Initial Review Outcomes
  5. Appeals Process in New Jersey
  6. Medical Necessity Documentation
  7. Common Denial Reasons & Solutions
  8. New Jersey External Review Process
  9. Patient Resources and Support
  10. FAQ

Understanding Strimvelis Coverage Requirements

Strimvelis (autologous CD34+ ADA-SCID gene therapy) presents unique coverage challenges since it's only available at the San Raffaele Hospital in Milan, Italy, and isn't FDA-approved in the United States. This ex vivo gene therapy requires extracting the patient's stem cells, genetically modifying them, and reinfusing them—a one-time treatment for adenosine deaminase severe combined immunodeficiency (ADA-SCID).

Coverage at a Glance

Requirement Details Source
Prior Authorization Required for all non-formulary therapies Cigna Gene Therapy Program
Formulary Status Not on standard formulary; requires exception Cigna Coverage Policies
Site of Care SR-TIGET Milan only EMA Authorization
International Coverage Must verify out-of-network benefits Verify with member services
Appeal Deadline (NJ) 4 months for external review NJ IHCAP

The therapy costs approximately €594,000 per patient, making it one of the most expensive treatments available. However, for families facing ADA-SCID—where children typically don't survive beyond age two without treatment—this represents the only gene therapy option when bone marrow transplant isn't feasible.

Pre-Authorization Preparation

Before submitting your prior authorization request, gather comprehensive documentation to demonstrate medical necessity. Cigna's review process focuses heavily on whether standard treatments have been exhausted or are contraindicated.

Essential Documentation Checklist

Genetic and Laboratory Evidence:

  • Complete ADA gene sequencing showing bi-allelic pathogenic variants
  • Quantitative ADA enzyme activity levels (typically <1% of normal)
  • Immunological workup confirming SCID diagnosis
  • Pre-transfusion laboratory values

Treatment History:

  • Documentation of failed enzyme replacement therapy (PEG-ADA)
  • HLA typing results for patient and family members
  • National Marrow Donor Program search results showing no suitable donor
  • Transplant center evaluation letter stating HSCT isn't viable

Clinical Documentation:

  • Comprehensive medical history including infections and hospitalizations
  • Growth and development records
  • Current immunological status
  • Specialist letters from immunologist or pediatric hematologist/oncologist
Tip: Start gathering documentation early. The genetic testing and donor search processes can take several weeks, and incomplete submissions often delay approval by 2-3 weeks.

Submission Process

Cigna processes gene therapy requests through their specialized review team, typically coordinating with Express Scripts for specialty medications. The submission process requires careful attention to procedural requirements.

Step-by-Step Submission

  1. Contact Member Services: Call the number on your insurance card to confirm international coverage benefits and request gene therapy prior authorization forms.
  2. Complete Forms: Fill out Cigna's prior authorization request completely. If specific gene therapy forms aren't available, use their standard investigational therapy request.
  3. Compile Evidence Package: Include all documentation from your checklist, ensuring everything is clearly labeled and organized.
  4. Submit via Preferred Channel: Most Cigna submissions go through the provider portal, though fax submission may be accepted (verify current fax numbers with member services).
  5. Request Expedited Review: If the patient's condition is deteriorating, explicitly request expedited review with supporting urgency documentation.
  6. Confirm Receipt: Follow up within 5-7 business days to confirm Cigna received your complete submission.

Timeline Expectations

  • Standard Review: 7-14 days for initial determination
  • Expedited Review: 24-72 hours if urgency is documented
  • Incomplete Submissions: Additional 2-3 weeks for resubmission

Initial Review Outcomes

Based on similar rare disease gene therapy cases, initial outcomes typically fall into three categories:

Immediate Approval (~10%): Usually occurs when there's clear medical necessity, documented failure of standard treatments, and urgent clinical need.

Conditional Approval (~30%): May require additional documentation, second opinion, or confirmation from SR-TIGET in Milan. Travel costs typically aren't covered.

Denial (~60%): Most commonly due to experimental/investigational status or lack of U.S. availability.

Appeals Process in New Jersey

If Cigna denies your initial request, New Jersey offers robust appeal rights through a two-level internal process followed by external review.

Internal Appeals

Cigna provides at least two levels of internal appeal:

  • First Level: Review by different medical director
  • Second Level: Additional clinical review, often including specialist consultation
  • Timeline: 180 days from initial denial to file internal appeals

Preparing Your Appeal

When appealing, directly address Cigna's stated denial reasons:

If denied as "experimental/investigational":

  • Cite EMA approval and regulatory authorization in Europe
  • Include peer-reviewed studies on Strimvelis efficacy and safety
  • Reference successful treatment of 45+ patients from over 20 countries

If denied for "not medically necessary":

  • Document why HSCT isn't suitable (no donor, contraindications)
  • Show progression of immunodeficiency despite current treatments
  • Include specialist attestation of treatment urgency

If denied for "out-of-network" reasons:

  • Emphasize that SR-TIGET Milan is the only authorized treatment center globally
  • Request single-case agreement for out-of-network coverage
  • Provide cost-benefit analysis comparing to lifetime supportive care

Medical Necessity Documentation

A compelling medical necessity letter from your treating specialist should address several key elements:

Letter Components

Patient Background:

  • Age, diagnosis date, and clinical presentation
  • Family history and genetic counseling results
  • Current clinical status and prognosis without treatment

Treatment Rationale:

  • Why standard treatments (HSCT, PEG-ADA) aren't suitable
  • Expected outcomes from gene therapy
  • Monitoring and follow-up plan

Supporting Evidence:

  • Relevant clinical guidelines and literature
  • EMA approval documentation
  • SR-TIGET treatment protocols

Urgency Factors:

  • Timeline for treatment necessity
  • Risks of delay
  • Patient-specific factors requiring prompt intervention
From our advocates: We've seen families strengthen their appeals by including a detailed timeline showing how their child's condition has progressed despite standard treatments. One family's successful appeal included month-by-month infection rates and hospitalization data, clearly demonstrating the urgency for definitive treatment. While outcomes vary, this type of concrete clinical progression data helps reviewers understand why gene therapy represents the best remaining option.

Common Denial Reasons & Solutions

Denial Reason How to Address
"Experimental/Investigational" Cite EMA approval, peer-reviewed studies, and regulatory authorization
"Not medically necessary" Document failed standard treatments, specialist attestation, clinical urgency
"Out-of-network provider" Emphasize SR-TIGET is only authorized center; request single-case agreement
"Travel not covered" Separate travel costs from treatment costs; focus on medical necessity
"Insufficient documentation" Provide complete genetic testing, enzyme levels, donor search results

New Jersey External Review Process

If internal appeals are unsuccessful, New Jersey's Independent Health Care Appeals Program (IHCAP) provides external review through Maximus Federal Services.

IHCAP Process Overview

Eligibility: Available after completing Cigna's internal appeal process for denials based on medical necessity or experimental status.

Timeline: File within 4 months (120 days) of final internal denial.

Process: Independent physicians with relevant specialty expertise review your case, considering medical records, specialist opinions, and standard medical guidelines.

Cost: Free to patients; insurers pay all review costs.

Outcome: Decisions are binding on insurance companies.

Filing Your External Appeal

  1. Contact IHCAP: Call 1-888-393-1062 for guidance and forms
  2. Submit Documentation: Include all medical records, denial letters, and supporting evidence
  3. Request Expedited Review: Available if delay would cause serious harm
  4. Timeline: Standard reviews completed within 45 days; expedited reviews much faster

Success rates for external reviews nationwide average around 50%, though specific data for gene therapy cases in New Jersey isn't publicly available.

Patient Resources and Support

Financial Assistance

While insurance coverage is the primary goal, several resources may help with costs:

  • Orchard Therapeutics Patient Support: Contact the manufacturer for assistance programs
  • Telethon Foundation: Offers "Just like home" program for families traveling to Milan
  • National Organization for Rare Disorders (NORD): Provides grants and advocacy support
  • Patient Advocate Foundation: Assists with insurance appeals and financial hardship

Advocacy Support

Counterforce Health specializes in turning insurance denials into targeted, evidence-backed appeals. Their platform helps patients, clinicians, and specialty pharmacies navigate complex prior authorization requirements by identifying denial basis and drafting point-by-point rebuttals aligned to payer policies. For rare therapies like Strimvelis, their expertise in payer-specific workflows and procedural requirements can be invaluable in preparing compelling appeals.

State Resources

  • NJ Department of Banking and Insurance: 1-800-446-7467 for consumer assistance
  • NJ IHCAP Hotline: 1-888-393-1062 for external review questions
  • NJ Managed Care Ombudsman: Available through Department of Human Services

FAQ

How long does Cigna's prior authorization process take in New Jersey? Standard reviews typically take 7-14 days, though expedited reviews for urgent cases can be completed within 24-72 hours. Complex cases involving international treatment may take longer.

What if Strimvelis is considered non-formulary by Cigna? Non-formulary status requires a formulary exception request. Include comprehensive medical necessity documentation and evidence that formulary alternatives aren't suitable for ADA-SCID.

Can I request an expedited appeal if my child's condition is worsening? Yes, both Cigna's internal appeals and New Jersey's external review process offer expedited pathways when delays could cause serious harm. Document the urgency with clinical evidence.

Does Cigna cover travel expenses for treatment in Milan? Travel expenses are typically not covered and must be arranged separately. Focus your coverage request on the medical treatment itself rather than associated travel costs.

What happens if my employer plan is self-funded? Self-funded employer plans follow federal ERISA rules rather than New Jersey's external review process. Contact your plan administrator for specific appeal procedures.

How can I verify my Cigna plan includes international coverage? Call member services using the number on your insurance card. Ask specifically about out-of-network international coverage and any limitations or exclusions.

What documentation is most important for approval? The genetic testing confirming ADA-SCID diagnosis, ADA enzyme levels, and documentation showing why bone marrow transplant isn't viable are typically the most critical elements.

Can my doctor file the appeal on my behalf? Yes, healthcare providers can file appeals with patient consent. Many specialists experienced with rare diseases are familiar with this process.

This guide provides general information about insurance coverage and appeals processes. It is not medical advice. For specific medical questions, consult your healthcare provider. For insurance-specific guidance, contact Cigna member services or consult with Counterforce Health for specialized appeal assistance.

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Insurance coverage varies by plan and individual circumstances. Always verify current procedures and requirements with your insurance provider and consult healthcare professionals for medical decisions.

Sources & Further Reading

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