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

Get Strimvelis Gene Therapy Covered by UnitedHealthcare in Ohio: Complete Prior Authorization and Appeals Guide 2025

Quick Answer: Getting Strimvelis Covered in Ohio

Strimvelis requires comprehensive prior authorization from UnitedHealthcare due to its status as a non-FDA approved gene therapy only available in Milan, Italy. In Ohio, you'll need: (1) confirmed ADA-SCID diagnosis with genetic testing, (2) documentation that standard treatments (HSCT, enzyme replacement) are unavailable or contraindicated, and (3) prior authorization for both the therapy and international treatment. If denied, Ohio provides up to 180 days for external review through an Independent Review Organization. Start today by calling UnitedHealthcare at the number on your member ID card to request a complex case manager.

Table of Contents

Coverage Requirements at a Glance

Requirement What It Means Documentation Needed Source
Prior Authorization Required for all gene therapies Complete clinical packet + international treatment request UHC Gene Therapy Policy
Diagnosis Confirmation ADA-SCID with genetic proof Enzyme testing (<1% normal), genetic variants, immune phenotype UHC Clinical Guidelines
Failed Alternatives Standard treatments unavailable HSCT donor search results, contraindications to enzyme therapy UHC Prior Auth Requirements
Site Restriction Only at SR-TIGET Milan Letter from treating center confirming availability SR-TIGET Authorization
Appeals Deadline 180 days from denial Complete internal appeals first Ohio External Review

Step-by-Step: Fastest Path to Approval

1. Secure Complex Case Management

Who: Patient or caregiver
Action: Call UnitedHealthcare member services and request assignment of a rare disease or complex case manager
Timeline: Same day
Documents: Member ID, diagnosis summary

2. Gather Diagnostic Documentation

Who: Treating immunologist/geneticist
Action: Compile complete ADA-SCID diagnostic packet
Timeline: 1-2 weeks
Documents: Enzyme levels, genetic testing, immune phenotype, growth charts

3. Document Treatment History

Who: Medical team
Action: Obtain records of all prior treatments and outcomes
Timeline: 3-5 days
Documents: HSCT evaluation, enzyme replacement trials, infection history

4. Coordinate with SR-TIGET Milan

Who: Treating physician
Action: Obtain acceptance letter and cost estimate from Milan center
Timeline: 2-4 weeks
Documents: Clinical acceptance, treatment timeline, cost breakdown

5. Submit Dual Prior Authorization

Who: Provider office
Action: Submit both drug PA and international treatment authorization
Timeline: Same day
Portal: UnitedHealthcare Provider Portal (verify current link)

6. Track and Follow Up

Who: Patient/provider
Action: Monitor PA status and respond to information requests immediately
Timeline: 14-30 days for decision
Method: Provider portal, case manager contact

7. Prepare Appeal Documentation

Who: Medical team
Action: Begin gathering additional evidence while awaiting decision
Timeline: Concurrent with step 6
Documents: Literature review, specialist letters, urgency documentation

Medical Necessity Documentation

Core Requirements for ADA-SCID

Your medical team must provide comprehensive documentation proving both the diagnosis and treatment urgency:

Diagnostic Confirmation:

  • ADA enzyme activity <1% of normal (CLIA-certified lab)
  • Genetic testing showing biallelic pathogenic ADA variants
  • Immune phenotype: severe T-cell lymphopenia, B-cell dysfunction
  • Growth parameters and infection history

Treatment History:

  • HLA typing and donor search results
  • Contraindications to matched sibling donor HSCT
  • Prior enzyme replacement therapy outcomes
  • Current supportive care requirements

Clinical Urgency:

  • Recurrent serious infections requiring hospitalization
  • Failure to thrive or developmental delays
  • Progressive immune deterioration
  • Time-sensitive nature of gene therapy window
From Our Advocates: We've seen families succeed by having their immunologist create a timeline showing disease progression alongside failed treatment attempts. One composite case involved documenting how a child's infection frequency increased from monthly to weekly episodes despite optimal supportive care, making the urgency for definitive therapy undeniable to reviewers.

Coding and Billing Essentials

ICD-10 Coding for ADA-SCID

Primary Diagnosis: Use the most specific severe combined immunodeficiency code available:

  • D81.31 - Severe combined immunodeficiency due to adenosine deaminase deficiency (if available in your coding system)
  • D81.9 - Combined immunodeficiency, unspecified (alternative)

Supporting Codes:

  • R62.51 - Failure to thrive
  • J18.9 - Pneumonia, unspecified organism (for recurrent infections)
  • Z87.891 - Personal history of nicotine dependence (if applicable)

HCPCS and Billing Considerations

Since Strimvelis lacks a specific J-code, billing typically uses:

  • J3590 - Unclassified biologics (most common)
  • J9999 - Not otherwise classified, antineoplastic drugs (alternative)

Critical Billing Notes:

  • Include detailed description: "Strimvelis autologous CD34+ gene therapy for ADA-SCID"
  • Document units based on single administration
  • Attach prior authorization approval letter
  • Use international billing modifier if required by plan

Clean Request Checklist

Before submitting your prior authorization:

✓ Diagnosis codes match medical records and PA request
✓ HCPCS code includes detailed drug description
✓ Units and dosing calculated per patient weight/BSA
✓ Site of care clearly identified as SR-TIGET Milan
✓ Start date allows time for travel and preparation
✓ Supporting documentation attached in correct format

Common Denial Reasons & Solutions

Denial Reason How to Overturn Key Documents
"Experimental/Investigational" Cite EMA approval and published outcomes data EMA approval letter, peer-reviewed studies
"Not available in US" Document that Milan is only authorized site worldwide SR-TIGET official letter, manufacturer confirmation
"Alternative treatments available" Prove HSCT unavailable and enzyme therapy insufficient Donor search results, treatment failure documentation
"Out-of-network exclusion" Request center of excellence exception Life-threatening condition attestation, no US equivalent
"Insufficient documentation" Submit comprehensive clinical packet Complete diagnostic workup, specialist consultations

Ohio Appeals Process

Internal Appeals with UnitedHealthcare

Timeline: Up to 180 days from denial to file
Levels: Typically 1-2 internal review levels
Expedited: Available for urgent medical situations (72-hour review)
Submission: Via provider portal, fax, or mail as specified in denial letter

Required Documents:

  • Original denial letter
  • Additional medical records
  • Specialist letter addressing denial reasons
  • Updated treatment plan

Ohio External Review

If UnitedHealthcare upholds their denial after internal appeals, Ohio residents can request an independent external review:

Eligibility: Fully-insured plans regulated by Ohio (not self-funded ERISA plans)
Timeline: 180 days from final internal denial
Cost: No fee to patient
Decision Time: 30 days standard, 72 hours expedited

How to Request:

  1. Contact Ohio Department of Insurance at 1-800-686-1526
  2. Complete external review request form
  3. Submit all medical records and denial letters
  4. Independent Review Organization (IRO) assigned
  5. Binding decision issued
Note: The IRO decision is binding on UnitedHealthcare for eligible Ohio plans. If approved, UHC must cover the treatment.

Cost and Financial Support

Expected Costs

  • Strimvelis therapy: Approximately €594,000 (~$650,000 USD)
  • Travel and accommodation: $10,000-15,000 for family
  • Follow-up care: Ongoing monitoring costs

Financial Support Options

Manufacturer Programs: Contact Orchard Therapeutics patient support
Foundation Grants: National Organization for Rare Disorders (NORD), HealthWell Foundation
Hospital Financial Aid: SR-TIGET Milan may offer payment plans
Crowdfunding: GoFundMe, CaringBridge for family campaigns

Counterforce Health helps families navigate the complex prior authorization and appeals process for gene therapies like Strimvelis. Our platform analyzes denial letters and creates targeted, evidence-backed appeals that address payer-specific requirements and improve approval rates for life-saving treatments.

When to Escalate

Contact the Ohio Department of Insurance if:

  • UnitedHealthcare refuses to process your appeal
  • Deadlines are not being met
  • You suspect bad faith denial practices
  • External review eligibility is disputed

Ohio DOI Contact: Phone: 1-800-686-1526
Website: insurance.ohio.gov
Email: Via online complaint form

Frequently Asked Questions

Q: How long does UnitedHealthcare prior authorization take for gene therapy?
A: Standard review takes 14-30 days. Expedited review for urgent cases takes up to 72 hours. Request expedited review if delays would seriously jeopardize health.

Q: Does step therapy apply to Strimvelis?
A: UnitedHealthcare typically waives step therapy for orphan drugs when alternatives are contraindicated or unavailable. Document why HSCT and enzyme replacement are not viable options.

Q: Can I appeal if I have a self-funded employer plan?
A: Self-funded ERISA plans use federal external review, not Ohio's process. Check your denial letter for specific appeal instructions and timelines.

Q: What if UnitedHealthcare says the treatment is cosmetic or not medically necessary?
A: ADA-SCID is universally recognized as life-threatening. Submit additional specialist letters and published outcome data showing the medical necessity of gene therapy.

Q: How do I know if my plan covers international treatment?
A: Review your Summary of Benefits or call member services. Most plans have provisions for life-saving care unavailable in the US, but require prior authorization.

Q: What happens after Strimvelis treatment in Milan?
A: Long-term follow-up care in the US should be covered as standard medical benefits. Ensure your treating US immunologist coordinates with the Milan team for seamless care transitions.

Q: Can Counterforce Health help with my Strimvelis appeal?
A: Yes, our platform specializes in gene therapy appeals and can help create evidence-backed appeals that address UnitedHealthcare's specific denial reasons and policy requirements.

Sources & Further Reading

Disclaimer: This guide provides general information about insurance coverage and appeals processes. It is not medical advice, legal advice, or a guarantee of coverage. Always consult with your healthcare providers and insurance company for guidance specific to your situation. Coverage policies and procedures may change; verify current requirements with UnitedHealthcare and Ohio regulators.

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