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

Complete Guide: Getting Strimvelis Gene Therapy Covered by Cigna in Pennsylvania (Requirements, Forms & Appeals)

Answer Box: Quick Start Guide

Getting Strimvelis covered by Cigna in Pennsylvania requires careful preparation since this gene therapy is only available at SR-TIGET in Milan, Italy. First step: Confirm your diagnosis with genetic testing showing bi-allelic ADA mutations and enzyme activity <1% of normal. Fastest path: Submit a comprehensive prior authorization with medical necessity letter, genetic test results, and documentation that no suitable stem cell donor exists. Key advantage in Pennsylvania: If denied, you can use the state's Independent External Review program, which overturns roughly 50% of denials. Start by gathering all diagnostic documentation and contacting Cigna's Gene Therapy Program for their specific ADA-SCID worksheet.

Table of Contents

Who Should Use This Guide

This guide is for families and clinicians seeking Cigna coverage for Strimvelis (autologous CD34+ gene therapy) for ADA-SCID patients in Pennsylvania. You'll need this if:

  • Your child has confirmed ADA-SCID with no suitable matched stem cell donor
  • You're considering treatment at SR-TIGET in Milan, Italy (the only administration site)
  • You've received an initial Cigna denial or want to prevent one
  • You need to navigate Pennsylvania's external review system

Expected outcome: With proper documentation, many rare disease appeals succeed, especially when clinical criteria are clearly met and international travel is medically necessary.

Member & Plan Basics

Coverage Requirements

  • Active Cigna coverage: Verify your plan includes out-of-network and international benefits
  • Plan type: Most commercial plans require prior authorization; Medicare Advantage and Medicaid managed care have additional layers
  • Deductible status: High-deductible plans may require meeting your deductible first
  • Gene therapy program: Cigna routes all gene therapies through their specialized program
Note: Express Scripts manages Cigna's specialty pharmacy benefits and applies additional formulary restrictions for gene therapies.

Clinical Criteria for Approval

Cigna requires strict documentation for ADA-SCID gene therapy coverage:

Primary Requirements

  1. Confirmed ADA-SCID diagnosis with:
    • Clinical presentation consistent with severe combined immunodeficiency
    • Bi-allelic pathogenic variants in the ADA gene (genetic testing required)
    • Absent or very low ADA catalytic activity (<1% of normal baseline)
  2. No suitable stem cell donor available through registry searches
  3. Prescriber qualifications: Board-certified immunologist, hematologist/oncologist, or ADA-SCID specialist
  4. Treatment setting: SR-TIGET Milan is the only approved site due to Strimvelis's 6-hour shelf life

Medical Necessity Standards

  • Patient must be medically stable for international travel
  • No history of malignancy or myelodysplastic syndrome
  • Negative for HIV and certain other infections per SR-TIGET protocols
  • Age and weight within treatment parameters

Medical Coding Requirements

Since Strimvelis lacks a permanent HCPCS J-code, billing uses miscellaneous codes:

Code Type Code Description Usage
HCPCS J3590 Unclassified biologics Most common for gene therapy
HCPCS J3490 Unclassified drug Alternative option
ICD-10 D81.3 Adenosine deaminase deficiency Primary diagnosis
NDC Variable 11-digit code from product packaging Required on claims
Tip: Always verify the current NDC from the actual product packaging, as batch numbers change.

Documentation Packet

Core Documents Required

  1. Genetic testing report showing bi-allelic ADA mutations
  2. ADA enzyme activity results (must show <1% of normal)
  3. Stem cell donor search results (confirming no suitable match)
  4. SR-TIGET acceptance letter with treatment timeline
  5. Medical necessity letter from prescribing physician

Medical Necessity Letter Components

Your physician's letter must include:

  • Patient identification: Full name, DOB, Cigna member ID
  • Diagnosis confirmation: ADA-SCID with supporting lab values
  • Clinical rationale: Why gene therapy is appropriate vs. alternatives
  • Treatment plan: Specific details about SR-TIGET protocol
  • Prognosis: Expected outcomes and monitoring plan
  • Provider credentials: Specialty certification and experience with ADA-SCID

Supporting Attachments

  • Recent immunology consultation notes
  • Hospital discharge summaries if applicable
  • Infectious disease clearance for international travel
  • Insurance authorization request form (Cigna-specific)

Submission Process

Step-by-Step Submission

  1. Contact Cigna Gene Therapy Program at the number on your member card
  2. Request ADA-SCID specific worksheet (if available) or general gene therapy form
  3. Complete prior authorization form with all required fields
  4. Attach documentation packet - incomplete submissions cause delays
  5. Submit via preferred method: Online provider portal or designated fax
  6. Request confirmation number and expected review timeline
Important: Cigna typically requires 15 business days for gene therapy reviews, but complex cases may take longer.

Submission Timeline

  • Preparation: 2-4 weeks to gather all documentation
  • Review period: 15-30 business days from complete submission
  • Decision notification: Via phone, fax, or provider portal

After Submission: What to Expect

Tracking Your Request

  • Confirmation number: Keep this for all follow-up calls
  • Status checks: Call every 5-7 business days if no update
  • Additional information requests: Respond within 48 hours to avoid delays

Possible Outcomes

  • Approval: Coverage determination letter with specific terms
  • Partial approval: May cover treatment but not travel/lodging
  • Denial: Detailed letter explaining rejection reasons

Common Denial Reasons & Solutions

Denial Reason Solution Strategy
"Not FDA approved in US" Emphasize no US alternative exists; cite medical necessity for international treatment
"Experimental/investigational" Provide EU approval documentation; cite published efficacy data
"No contracted provider" Request single-case agreement with SR-TIGET
"Out-of-network exclusion" Appeal based on no in-network alternative available
"Insufficient documentation" Resubmit with complete genetic testing and enzyme activity results

Pennsylvania Appeals Process

Pennsylvania offers robust appeal rights for denied coverage:

Internal Appeals (Required First)

  1. Timeline: Must file within 180 days of denial
  2. Process: Submit appeal letter with additional documentation
  3. Review period: 30 days for standard, 72 hours for expedited
  4. Outcome: Final Adverse Benefit Determination letter if denied

Pennsylvania External Review

If internal appeals fail, Pennsylvania's Independent External Review program provides a powerful option:

  • Success rate: Approximately 50% of appeals are overturned
  • Timeline: Must request within 4 months of final internal denial
  • Process: Independent medical reviewers evaluate the case
  • Decision timeline: 45 days standard, 72 hours expedited
  • Cost: Free to patients
From our advocates: "We've seen families successfully overturn initial denials for international gene therapy by providing comprehensive genetic testing documentation and emphasizing the lack of US alternatives. The key is demonstrating that travel to Milan isn't a preference but a medical necessity - it's the only place this life-saving treatment exists."

Escalation Resources

  • Pennsylvania Insurance Department: Consumer hotline for guidance
  • Pennsylvania Health Law Project: Free legal assistance for complex cases
  • Counterforce Health: Specialized platform that helps turn insurance denials into evidence-backed appeals by analyzing denial letters and drafting targeted rebuttals aligned to each plan's specific policies

Quick Reference Checklist

Before You Start

  • Active Cigna coverage confirmed
  • ADA-SCID diagnosis with genetic testing
  • ADA enzyme activity <1% documented
  • Stem cell donor search completed (no match)
  • SR-TIGET acceptance obtained
  • Prescriber identified (immunologist/hematologist)

Documentation Packet

  • Genetic test report (bi-allelic ADA mutations)
  • ADA enzyme activity results
  • Donor search documentation
  • Medical necessity letter
  • SR-TIGET treatment plan
  • Insurance authorization form
  • Recent consultation notes

Submission

  • Prior authorization form complete
  • All attachments included
  • Submitted via correct method
  • Confirmation number received
  • Follow-up schedule established

If Denied

  • Internal appeal filed within 180 days
  • Additional evidence gathered
  • Final denial letter received
  • External review requested (within 4 months)
  • Pennsylvania Insurance Department contacted if needed

FAQ

How long does Cigna prior authorization take for Strimvelis? Typically 15-30 business days from complete submission. Gene therapy reviews often require additional clinical consultation, which can extend timelines.

What if Strimvelis isn't on Cigna's formulary? Most gene therapies aren't on standard formularies. You'll need to request a formulary exception with medical necessity documentation.

Can I request an expedited review? Yes, if the patient's condition is rapidly deteriorating or life-threatening. Provide clinical documentation supporting urgency.

Does Cigna cover travel expenses for international treatment? Coverage varies by plan. Some policies include travel benefits for medically necessary care unavailable domestically. Check your specific benefits or request a single-case agreement.

What happens if I pay out-of-pocket and win an appeal later? If Pennsylvania's external review overturns the denial, Cigna must provide retroactive coverage, including reimbursing amounts you paid during the appeal process.

How do I find an ADA-SCID specialist for my medical necessity letter? Contact major children's hospitals with immunology departments, or ask your current physician for a referral to a specialist familiar with primary immunodeficiencies.

Sources & Further Reading

Disclaimer: This guide provides educational information only and is not medical or legal advice. Coverage decisions depend on your specific plan terms and clinical circumstances. Always consult with your healthcare team and insurance representatives for personalized guidance. For assistance with appeals and coverage issues, contact the Pennsylvania Insurance Department or qualified patient advocacy services.

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