By Counterforce Health Team in Exondys 51 (eteplirsen) —

How to Get Exondys 51 (eteplirsen) Covered by Cigna in Virginia: Appeals Guide, Forms & Timelines

Quick Answer: To get Exondys 51 (eteplirsen) covered by Cigna in Virginia, you need genetic testing confirming an exon 51-amenable DMD mutation, a neuromuscular specialist's prescription, and documentation of ambulatory status with cardiac/pulmonary baselines. Submit prior authorization through Accredo Specialty Pharmacy. If denied, appeal within 180 days using Cigna's internal process, then pursue external review through Virginia's State Corporation Commission within 120 days. First step today: Contact your neurologist to order genetic testing if not already completed.

Table of Contents

Policy Overview: How Cigna Covers Exondys 51

Cigna classifies Exondys 51 (eteplirsen) as a specialty drug requiring prior authorization across all plan types—commercial PPO/HMO, Medicare Advantage, and Medicaid managed care plans. The drug is distributed exclusively through Accredo Specialty Pharmacy, Cigna's preferred specialty pharmacy network.

Plan Coverage Variations:

  • Commercial plans: Subject to formulary tier placement (typically Tier 4 or 5 specialty)
  • Medicare Advantage: Covered under Part B if administered in outpatient settings; Part D if self-administered
  • Medicaid managed care: Follows Virginia Medicaid fee-for-service guidelines with additional managed care restrictions
Note: Always verify your specific plan's formulary status through Cigna's member portal or by calling the number on your insurance card.

Medical Necessity Requirements

Cigna's coverage policy IP0135 establishes strict medical necessity criteria for Exondys 51:

Core Eligibility Criteria

Requirement Specification Documentation Needed
Diagnosis Confirmed Duchenne muscular dystrophy ICD-10 code G71.01, clinical notes
Genetic Testing DMD gene mutation amenable to exon 51 skipping Laboratory report with specific mutation identified
Age ≤13 years at therapy initiation Birth certificate or medical records
Prescriber Neurologist or neuromuscular specialist Provider credentials, NPI verification
Ambulatory Status Ability to walk independently 6-minute walk test ≥180 meters
Cardiac Function LVEF >40% Echocardiogram or MUGA scan
Pulmonary Function FVC ≥50% predicted Pulmonary function tests

Step Therapy and Exception Pathways

Unlike many specialty drugs, Exondys 51 does not require step therapy through corticosteroids or other DMD treatments. However, Cigna requires documentation that the patient hasn't previously received gene therapy for DMD unless there's been documented clinical decline post-therapy.

Exception Scenarios

  • Prior gene therapy failure: Document clinical decline with objective measures
  • Contraindications to alternatives: Provide medical rationale for avoiding corticosteroids
  • Urgent clinical need: Submit with expedited prior authorization request

Quantity Limits and Renewal Requirements

Initial Authorization: Up to 6 months of therapy Renewal Requirements:

  • Continued ambulatory status
  • Stable or improved cardiac function (LVEF maintained >40%)
  • Adherence to weekly infusion schedule (≥80% compliance)
  • Absence of serious adverse reactions

Quantity Calculations:

  • Dosing: 30 mg/kg IV weekly
  • Vial size: 50 mg/mL (10 mL vials)
  • Monthly quantity varies by patient weight
Tip: Track infusion dates and any missed doses to demonstrate adherence during renewal reviews.

Required Diagnostics and Documentation

Essential Laboratory Values

  • Genetic testing report: Must specify exact DMD mutation and confirm exon 51 amenability
  • Baseline cardiac assessment: ECHO or MUGA within 6 months of initiation
  • Pulmonary function: Spirometry within 3 months
  • Renal function: Creatinine, BUN (eteplirsen is renally excreted)
  • 6-minute walk test: Baseline and every 6 months

Documentation Timeline Requirements

  • Genetic testing: No expiration (one-time requirement)
  • Cardiac/pulmonary baselines: Within 6 months of request
  • Clinical notes: Within 30 days supporting current functional status

Specialty Pharmacy and Site of Care Rules

Mandatory Routing: All Exondys 51 prescriptions must be processed through Accredo Specialty Pharmacy. Attempts to fill at retail pharmacies will result in automatic rejection.

Site of Care Requirements:

  • Approved locations: Hospital outpatient departments, accredited infusion centers, MDA Care Centers
  • Prohibited locations: Home infusion (unless specifically approved), physician offices without infusion certification
  • Administration protocol: 35-60 minute IV infusion with 0.2 micron in-line filter

Infusion Center Coordination

Accredo coordinates directly with approved infusion centers for:

  • Drug delivery and storage
  • Infusion scheduling
  • Insurance verification and prior authorization tracking

Evidence to Support Medical Necessity

When submitting prior authorization or appeals, include these evidence sources:

Primary Guidelines:

  • FDA prescribing information for Exondys 51
  • Muscular Dystrophy Association care guidelines
  • American Academy of Neurology practice parameters

Supporting Literature:

  • Pivotal clinical trial data (Study 201/202 extension)
  • Real-world evidence studies on functional outcomes
  • Dystrophin expression data supporting mechanism of action

Clinical Narrative Template: "[Patient name] is a [age]-year-old male with genetically confirmed Duchenne muscular dystrophy due to [specific mutation] amenable to exon 51 skipping. Current ambulatory status is preserved with 6MWT of [distance] meters. Baseline cardiac function shows LVEF of [%] and pulmonary function demonstrates FVC of [%] predicted. Exondys 51 represents the only FDA-approved targeted therapy for this specific genetic subset of DMD patients."

Appeals Process in Virginia

Internal Appeals with Cigna

Level 1 - Standard Appeal:

  • Deadline: 180 days from denial notice
  • Timeline: 30 days for determination (15 days for urgent)
  • Submission: Cigna member portal, fax, or mail
  • Required documents: Denial letter, medical records, prescriber letter

Level 2 - Independent Review:

  • Automatic: If Level 1 denied
  • Timeline: 30 days for determination
  • Reviewer: Independent medical professional in same specialty

External Review Through Virginia

If Cigna's internal appeals are exhausted or denied, Virginia residents can request external review through the State Corporation Commission Bureau of Insurance.

Process Overview:

  • Deadline: 120 days from final Cigna denial
  • Form: Submit Form 216-A (External Review Request)
  • Timeline: 45 days for standard review (72 hours for expedited)
  • Cost: Free to consumers
  • Decision: Binding on Cigna if overturned
Important: Virginia's external review has strong success rates—many specialty drug denials are overturned when proper clinical evidence is provided.

At Counterforce Health, we help patients and clinicians turn insurance denials into successful appeals by creating targeted, evidence-backed letters that address each payer's specific requirements. Our platform analyzes denial reasons and drafts point-by-point rebuttals using the right clinical evidence and payer-specific workflows.

Common Denial Reasons & Solutions

Denial Reason Solution Strategy Required Documentation
Missing genetic testing Submit complete genetic report Laboratory report with specific DMD mutation
Non-specialist prescriber Transfer care or obtain consultation Neurologist consultation notes
Insufficient cardiac/pulmonary data Complete baseline assessments ECHO, PFTs within required timeframes
Age >13 years Document exceptional circumstances Medical necessity letter explaining delayed diagnosis
"Experimental" designation Emphasize FDA approval status FDA label, peer-reviewed efficacy data
Site of care denial Justify infusion center necessity Safety protocols, monitoring requirements

Peer-to-Peer Review Strategy

Request a peer-to-peer review when initial PA is denied. Prepare your neurologist with:

  • Patient's specific genetic mutation and clinical course
  • Comparison to clinical trial inclusion criteria
  • Discussion of alternative treatments considered and rejected
  • Long-term prognosis and treatment goals

Cost Assistance Options

Manufacturer Support:

Foundation Grants:

  • Muscular Dystrophy Association financial assistance programs
  • Patient Advocate Foundation copay relief funds
  • HealthWell Foundation rare disease grants

Virginia-Specific Resources:

  • Virginia Medicaid expansion covers many previously uninsured DMD patients
  • Children's Health Insurance Program (CHIP) for qualifying families

FAQ

How long does Cigna prior authorization take in Virginia? Standard PA decisions are made within 15 business days. Urgent requests (when delay could jeopardize health) are decided within 72 hours. Submit through Accredo for fastest processing.

What if Exondys 51 is non-formulary on my plan? File a formulary exception request with clinical justification. Emphasize that no therapeutic alternatives exist for exon 51-amenable DMD mutations.

Can I request expedited appeal if my child is declining? Yes. Virginia allows expedited external review when delays could seriously jeopardize health. Your neurologist must certify medical urgency.

Does step therapy apply if we tried treatments in another state? Document all prior therapies regardless of location. Cigna recognizes treatment history from any licensed provider.

What happens if Cigna denies but Virginia external review approves? Cigna must provide coverage within 24-72 hours of an overturned external review decision. This is legally binding.

How often do appeals succeed for Exondys 51? While specific data isn't published, Medicare Advantage appeals succeed over 80% of the time nationally when proper documentation is provided. Cigna has one of the highest appeal rates among major insurers.

For complex cases or repeated denials, Counterforce Health specializes in creating comprehensive appeal packages that address specific payer requirements and maximize approval chances.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage decisions depend on individual plan terms and clinical circumstances. Always consult with your healthcare provider and insurance plan for personalized guidance. For assistance with Virginia insurance regulations, contact the State Corporation Commission Bureau of Insurance at 1-877-310-6560.

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