How to Get Upstaza (Eladocagene Exuparvovec) Covered by Cigna in Pennsylvania: Complete Forms, Appeals, and Contact Guide

Answer Box: Getting Upstaza Covered by Cigna in Pennsylvania

Cigna requires prior authorization for Upstaza (eladocagene exuparvovec) through Express Scripts/Accredo, with specific criteria including genetic confirmation of AADC deficiency and failed standard therapies. Submit via CoverMyMeds portal or call 800-753-2851 with genetic testing, severity assessment, and certified neurosurgical center details. If denied, Pennsylvania's new external review program offers strong appeal rights—about 50% of appeals are overturned. Start today: Verify your plan coverage through myCigna, gather genetic testing results, and contact your prescriber to initiate the prior authorization process.

Table of Contents

  1. Start Here: Verify Your Plan and Coverage
  2. Required Forms and Documentation
  3. Submission Portals and Processes
  4. Fax Numbers and Mailing Addresses
  5. Specialty Pharmacy Setup
  6. Support Phone Lines
  7. Pennsylvania Appeals and External Review
  8. Common Denial Reasons and Solutions
  9. When to Contact Regulators

Start Here: Verify Your Plan and Coverage

Before beginning the prior authorization process, confirm your specific Cigna plan details through the myCigna portal or by calling the customer service number on your insurance card.

Key Coverage Information:

  • Upstaza (eladocagene exuparvovec) is covered under Cigna Coverage Policy IP0725 for AADC deficiency
  • All gene therapies require prior authorization across Cigna plans
  • Specialty pharmacy management through Express Scripts/Accredo is mandatory
  • Treatment must occur at a certified neurosurgical center
Note: Cigna uses the brand name Kebilidi in their policy documents, which is the same medication as Upstaza (eladocagene exuparvovec).

Required Forms and Documentation

Prior Authorization Requirements

Essential Documentation Checklist:

  • Genetic testing confirming biallelic DDC gene mutations
  • Plasma AADC enzyme activity results (decreased levels)
  • CSF neurotransmitter analysis (5-HIAA, HVA, 3-MT)
  • Documentation of failed standard therapies (dopamine agonists, MAO inhibitors, or pyridoxine)
  • Certified treatment center verification
  • Age confirmation (≥18 months with skull maturity imaging)

Medical Necessity Letter Components

Your prescriber's medical necessity letter should include:

  • Confirmed AADC deficiency diagnosis with genetic evidence
  • Severity of symptoms (developmental delays, motor dysfunction)
  • Prior treatment history and limitations of supportive care
  • Rationale for gene therapy intervention
  • Post-treatment monitoring plan

Counterforce Health helps patients navigate complex prior authorization requirements by analyzing denial letters and crafting targeted appeals with the right clinical evidence. Their platform identifies specific denial reasons and creates point-by-point rebuttals aligned to each payer's policies, saving time for both patients and providers.

Submission Portals and Processes

Primary Submission Methods

1. CoverMyMeds Portal (Recommended)

2. Accredo Direct Submission

  • Call: 800-753-2851
  • Dedicated gene therapy support team
  • 72-hour standard review timeline
  • 24-hour expedited review for urgent cases

3. Gene Therapy Program Team

Submission Timeline Expectations

Review Type Processing Time When to Use
Standard 72 hours Non-urgent cases
Expedited 24 hours Delay risks neurological harm
Peer-to-peer Upon request After initial denial

Fax Numbers and Mailing Addresses

Gene Therapy Prior Authorization

  • Fax: 833-910-1625
  • Mail: Cigna Precertification Department, 1640 Dallas Parkway, Plano, TX 75093

Internal Appeals

  • Mail: Cigna National Appeals Unit, P.O. Box 188011, Chattanooga, TN 37422
Tip: Include a cover sheet with patient Cigna ID, prescriber NPI, and urgency level. Mark "GENE THERAPY - AADC DEFICIENCY" prominently.

Specialty Pharmacy Setup

Accredo Enrollment Process

Step 1: Prescriber Initiation

  • E-prescribe to Accredo (fastest method)
  • Fax prescription with prior auth approval
  • Call 888-327-9791 for fax instructions

Step 2: Patient Enrollment

  • Call Accredo: 877-826-7657
  • Provide insurance information and prescriber details
  • Schedule delivery coordination

Step 3: Transfer from Current Pharmacy

  • Request new prescription from your prescriber (recommended)
  • Use myCigna electronic transfer for eligible medications
  • Call Express Scripts: 1-800-835-3784 for assistance
Note: Controlled substances and expired prescriptions cannot be automatically transferred and require new prescriptions.

Support Phone Lines

Patient Support Numbers

Service Phone Number Hours What They Help With
Cigna Customer Service Number on ID card 24/7 Coverage questions, claim status
Express Scripts 1-800-835-3784 24/7 Prescription transfers, pharmacy benefits
Accredo 877-826-7657 24/7 Specialty medication support
Gene Therapy Team 855-678-0051 Business hours Prior auth, case management

Questions to Ask Customer Service

When calling Cigna, have this information ready:

  • Your member ID number
  • Prescriber's name and NPI
  • Medication name (Upstaza or Kebilidi)
  • Prior authorization reference number (if applicable)

Sample Script: "I need to check the prior authorization status for Upstaza for AADC deficiency. My member ID is [number] and the prescriber is [name]. Can you tell me what documentation is still needed?"

Pennsylvania Appeals and External Review

Internal Appeal Process

Level 1 Internal Appeal

  • Deadline: 180 days from denial date
  • Processing time: 30 days for medical necessity appeals
  • Submit to: Cigna National Appeals Unit (address above)

Level 2 Internal Appeal

  • Deadline: 60 days from Level 1 decision
  • Different medical director reviews case
  • Final internal review before external options

Pennsylvania External Review

Pennsylvania's Independent External Review Program launched in January 2024 with strong patient success rates.

Key Statistics:

  • 50% of external appeals overturned in first year
  • 517 external appeals filed in 2024
  • 259 cases ruled in favor of patients

External Review Process:

  1. Complete all internal appeals first
  2. File within 4 months of final denial
  3. Submit through Pennsylvania Insurance Department portal
  4. Standard review: 45 days
  5. Expedited review: 72 hours (urgent cases)

Appeals Support Resources

Pennsylvania Insurance Department

  • Consumer Services: Available through pa.gov
  • Free assistance with complex appeals
  • External review guidance and forms

Pennsylvania Health Law Project

  • Consumer Assistance Program
  • Free help for low-income individuals
  • Specialized rare disease advocacy

Common Denial Reasons and Solutions

Denial Reason Solution Strategy Required Documentation
Experimental/Investigational Cite FDA approval (August 2024) FDA approval announcement, Cigna policy IP0725
Not medically necessary Emphasize no disease-modifying alternatives Medical necessity letter, literature review
Step therapy required Request override with prescriber attestation Clinical rationale for immediate treatment
Facility requirements Verify certified treatment center Center accreditation documentation
Insufficient genetic evidence Provide complete genetic testing Biallelic DDC mutations, enzyme activity

Medical Necessity Appeal Strategy

When appealing a medical necessity denial, Counterforce Health's platform can help identify the specific denial basis and craft targeted rebuttals using FDA labeling, peer-reviewed studies, and specialty guidelines that align with Cigna's own coverage criteria.

Key Appeal Points:

  • AADC deficiency is a rare, progressive disorder
  • No other disease-modifying treatments available
  • Gene therapy offers potential for functional improvement
  • Meets all Cigna coverage criteria per policy IP0725

When to Contact Regulators

Pennsylvania Insurance Department Complaints

Contact the Pennsylvania Insurance Department if:

  • Cigna fails to respond within required timeframes
  • Internal appeals process is not followed properly
  • You need assistance understanding your rights

Filing a Complaint:

  • Online portal available at pa.gov
  • Include all correspondence and denial letters
  • Document timeline of appeals process

Federal Options

For Medicare Advantage plans, additional appeal rights may apply through CMS. Contact 1-800-MEDICARE for guidance on federal appeal processes.

Frequently Asked Questions

Q: How long does Cigna prior authorization take for Upstaza? A: Standard review is 72 hours, with expedited 24-hour review available if delay risks neurological harm.

Q: What if Upstaza is non-formulary on my plan? A: Request a formulary exception through Cigna's non-covered medication process, emphasizing the lack of disease-modifying alternatives.

Q: Can I request an expedited appeal in Pennsylvania? A: Yes, both Cigna internal appeals and Pennsylvania external reviews offer expedited timelines for urgent medical situations.

Q: Does step therapy apply to gene therapies? A: Cigna's policy requires documentation of failed standard therapies (dopamine agonists, MAO inhibitors, or pyridoxine) before approving gene therapy.

Q: What happens if my external review is successful? A: Cigna must provide coverage immediately, including retroactive coverage if you paid out-of-pocket during the appeal process.

Sources and Further Reading


This guide is for informational purposes only and does not constitute medical or legal advice. Coverage decisions are made by your insurance plan based on your specific policy terms and medical circumstances. For personalized assistance with your coverage appeal, consult with your healthcare provider or contact the Pennsylvania Insurance Department's consumer services.

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