By Counterforce Health Team in Upstaza (eladocagene exuparvovec) —

How to Get Upstaza (Eladocagene Exuparvovec) Covered by Cigna in Washington: Complete Appeals Guide with Forms and Timelines

Answer Box: Getting Upstaza Covered by Cigna in Washington

Cigna requires prior authorization for Upstaza (eladocagene exuparvovec) under Coverage Policy IP0725 for patients ≥18 months with confirmed AADC deficiency, managed through Express Scripts/Accredo specialty pharmacy. Submit PA via CoverMyMeds portal or call Accredo at 800-753-2851. If denied, Washington residents have strong appeal rights including external review through certified Independent Review Organizations (IROs) with up to 180 days to file and binding decisions on insurers.

First step today: Verify your Cigna plan covers specialty drugs and confirm you have AADC deficiency genetic testing results, then have your prescriber initiate the PA request.

Table of Contents

  1. Coverage Requirements at a Glance
  2. Step-by-Step: Fastest Path to Approval
  3. Understanding Your Denial Letter
  4. Common Denial Reasons & How to Fix Them
  5. First-Level Appeal Process
  6. Peer-to-Peer Review Strategy
  7. Washington State External Review Rights
  8. Appeal Templates and Scripts
  9. When Treatment Centers Aren't in Washington
  10. Cost Assistance Options
  11. FAQ

Coverage Requirements at a Glance

Requirement What It Means Where to Find It Source
Prior Authorization All gene therapies require PA through Express Scripts/Accredo Cigna PA Forms Cigna Policy
Age Eligibility ≥18 months with skull maturity confirmed by imaging Medical records, neuroimaging FDA Label
Genetic Confirmation Biallelic DDC gene mutations via genetic testing Lab reports Clinical Guidelines
Biochemical Testing Decreased plasma AADC enzyme activity, abnormal CSF neurotransmitters Lab results (5-HIAA, HVA, 3-MT levels) AADC Diagnosis Guidelines
Failed Standard Care Documentation of inadequate response to dopamine agonists, MAO inhibitors Medical necessity letter, chart notes Coverage Policy IP0725
Certified Center Treatment at PTC-authorized neurosurgical center Provider verification PTC Therapeutics

Step-by-Step: Fastest Path to Approval

1. Verify Your Coverage (Patient/Family)

  • Log into myCigna portal or call customer service
  • Confirm specialty drug benefits and Express Scripts/Accredo management
  • Timeline: Same day

2. Gather Required Documentation (Clinic)

  • Genetic testing results showing biallelic DDC mutations
  • Plasma AADC enzyme activity levels
  • CSF neurotransmitter analysis (low 5-HIAA, HVA; elevated 3-OMD)
  • Documentation of failed standard therapies
  • Timeline: 1-2 weeks if tests needed

3. Submit Prior Authorization (Prescriber)

  • Use CoverMyMeds portal or call Accredo 800-753-2851
  • Include comprehensive medical necessity letter
  • Timeline: Same day submission

4. Track Your Request

  • Standard review: 72 hours
  • Expedited review: 24 hours if neurological harm risk
  • Monitor via Express Scripts portal
  • Timeline: 1-3 business days

5. If Approved, Coordinate Treatment

  • Locate nearest certified treatment center (may require travel)
  • Arrange insurance verification for out-of-network facilities
  • Timeline: 2-4 weeks coordination

6. If Denied, File Appeal Immediately

  • Internal appeal within 180 days of denial
  • Gather additional evidence and clinical support
  • Timeline: Start within 30 days for best outcomes

Understanding Your Denial Letter

When Cigna denies Upstaza coverage, your denial letter will include specific reason codes. Here's how to decode them:

Medical Necessity Denials: Look for phrases like "not medically necessary" or "investigational." These often mean the reviewer didn't find sufficient evidence of AADC deficiency severity or failed standard care.

Administrative Denials: "Missing information" or "incomplete documentation" typically means genetic testing results, enzyme levels, or prior therapy records weren't included.

Policy Exclusions: "Non-covered benefit" may indicate the reviewer incorrectly classified Upstaza as experimental despite FDA approval in August 2024.

Tip: The denial letter must include your appeal rights and deadlines. In Washington, you have 180 days from the denial date to file an internal appeal.

Common Denial Reasons & How to Fix Them

Denial Reason Solution Strategy Key Evidence to Include
"Experimental/Investigational" Cite FDA approval (August 2024) and Coverage Policy IP0725 FDA approval letter, Cigna policy reference
"Not medically necessary" Emphasize no disease-modifying alternatives exist Medical necessity letter, literature review, failed therapy documentation
"Step therapy not met" Request prescriber override attestation Clinical rationale for urgency, contraindications to other treatments
"Facility not certified" Verify treatment center authorization PTC Therapeutics center verification, neurosurgeon credentials
"Age/genetic criteria not met" Provide complete diagnostic workup Genetic testing, enzyme assays, neuroimaging

First-Level Appeal Process

Required Components for Your Appeal

Medical Necessity Letter Checklist:

  • Detailed AADC deficiency diagnosis with genetic confirmation
  • Symptom severity and developmental impact
  • Prior treatments attempted and reasons for failure/intolerance
  • Clinical rationale for gene therapy
  • Post-treatment monitoring plan
  • Guideline citations supporting treatment

Supporting Documentation:

  • Complete genetic testing report
  • Plasma AADC enzyme activity results
  • CSF neurotransmitter metabolite analysis
  • Prior therapy trial records
  • Neuroimaging confirming skull maturity
  • Treatment center certification verification

Submission Process

Submit appeals through the Cigna provider portal or mail to the address on your denial letter. Include the "Request for Health Care Provider Payment Review" form with all supporting documentation.

Timeline: Cigna has 30 days to review pre-service appeals and 60 days for post-service appeals. Expedited reviews are available if delay could seriously jeopardize health.

Peer-to-Peer Review Strategy

If your initial appeal is denied, request a peer-to-peer review with Cigna's medical director. This conversation can be pivotal for rare disease cases.

Preparation Checklist:

  • Review Cigna's Coverage Policy IP0725 thoroughly
  • Prepare concise talking points on AADC deficiency severity
  • Have genetic testing and enzyme results readily available
  • Document all failed standard therapies with dates and outcomes

Key Discussion Points:

  • Emphasize the one-time nature of gene therapy vs. lifelong supportive care
  • Highlight lack of disease-modifying alternatives
  • Reference FDA approval and clinical trial efficacy data
  • Address any safety concerns with published literature
From our advocates: We've seen peer-to-peer reviews succeed when prescribers focus on the irreversible nature of AADC deficiency progression and the unique opportunity for disease modification that Upstaza provides. Having specific enzyme levels and genetic variants ready during the call demonstrates thorough diagnostic workup.

Washington State External Review Rights

Washington residents have exceptionally strong external review protections under RCW 48.43.535. After exhausting internal appeals, you can request an independent review that's binding on Cigna.

How External Review Works

Eligibility: Available after final internal denial for any coverage dispute involving medical necessity or treatment appropriateness.

Timeline:

  • 180 days to request external review from final internal denial
  • 30 days for standard external review decision
  • 72 hours for expedited reviews when health is in serious jeopardy

Process:

  1. Submit request to Washington Office of the Insurance Commissioner (OIC) or directly to Cigna
  2. OIC assigns certified Independent Review Organization (IRO)
  3. Cigna sends all records to IRO within 3 business days
  4. You have 5+ business days to submit additional information
  5. IRO panel reviews case and issues binding decision

Contact: Washington Insurance Commissioner Consumer Advocacy: 1-800-562-6900

Why External Review Works for Rare Diseases

External reviewers are medical specialists who can override Cigna's internal policies if they find them unreasonable or not aligned with standard practice. For gene therapies like Upstaza, independent reviewers often have more expertise in rare diseases than insurance company medical directors.

When Counterforce Health helps families navigate these appeals, we've seen external review success rates significantly higher than internal appeals, particularly when the clinical evidence clearly supports medical necessity.

Appeal Templates and Scripts

Patient Phone Script for Cigna

"Hi, I'm calling about a prior authorization denial for Upstaza for my child with AADC deficiency. The denial reference number is [X]. I'd like to understand the specific reason for denial and start the appeals process. Can you transfer me to someone who can help with specialty drug appeals?"

Medical Necessity Letter Template

"[Patient] is an [age]-month-old with genetically confirmed AADC deficiency (biallelic DDC mutations: [specify variants]) presenting with [specific symptoms]. Plasma AADC enzyme activity is severely decreased at [value], and CSF analysis shows characteristic neurotransmitter abnormalities (low 5-HIAA: [value], low HVA: [value], elevated 3-OMD: [value]).

Standard therapies including [list specific medications and doses] have failed to provide meaningful clinical improvement over [timeframe]. AADC deficiency is a progressive neurodegenerative disorder with no other disease-modifying treatments available. Upstaza represents the only therapy that can restore AADC enzymatic activity and halt disease progression.

Based on FDA approval for this exact indication and published clinical trial data showing [cite specific outcomes], Upstaza is medically necessary for this patient."

When Treatment Centers Aren't in Washington

Currently, no Upstaza-certified treatment centers operate in Washington state. The nearest facilities are likely in California or Oregon. Here's how to handle out-of-network situations:

Insurance Coordination

  • Request single-case agreement from Cigna for out-of-network coverage
  • Emphasize no in-network alternatives exist for this specialized procedure
  • Document travel necessity in medical records

Travel Support

  • PTC Therapeutics may provide travel assistance for eligible families
  • Contact patient support: 1-833-4PTCBUS
  • Some foundations offer travel grants for rare disease treatments

Local Follow-up

Coordinate with Seattle Children's Hospital or UW Medicine for post-treatment monitoring, even though they may not perform the initial gene therapy infusion.

Cost Assistance Options

Manufacturer Support: PTC Therapeutics offers patient assistance programs. Contact 1-833-4PTCBUS to verify eligibility.

Foundation Grants: Organizations like the National Organization for Rare Disorders (NORD) and HealthWell Foundation may provide assistance for travel and treatment costs.

State Programs: Washington Apple Health (Medicaid) may cover Upstaza for eligible patients. Contact DSHS at 1-800-562-3022.

Cigna Coverage: If approved, Cigna typically covers 80-90% of costs after deductible for specialty drugs, though out-of-pocket maximums apply.

FAQ

How long does Cigna prior authorization take for Upstaza in Washington? Standard review is 72 hours; expedited review is 24 hours if delay could cause neurological harm. Submit through CoverMyMeds or call Accredo at 800-753-2851.

What if Upstaza is non-formulary on my Cigna plan? Request a formulary exception with medical necessity documentation. Cigna must review non-formulary requests when no therapeutic alternatives exist.

Can I request an expedited appeal in Washington? Yes, if delay could seriously jeopardize health. Expedited appeals receive decisions within 72 hours instead of 30 days.

Does step therapy apply if my child failed treatments outside Washington? Prior therapy failures from any location count toward step therapy requirements. Include complete medical records from previous providers.

What happens if external review denies coverage? External review decisions are binding, but you can still contact the Washington Insurance Commissioner for additional guidance or file complaints about insurer conduct.

How much does Upstaza cost with Cigna coverage? Gene therapies typically cost millions, but with Cigna coverage, your out-of-pocket costs are limited by annual maximums (usually $2,000-8,000 for individual plans).

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Insurance coverage decisions depend on individual plan terms and medical circumstances. Always consult with your healthcare provider and insurance company for guidance specific to your situation. For personalized appeals assistance, Counterforce Health specializes in turning insurance denials into targeted, evidence-backed appeals for complex medications like gene therapies.

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