By Counterforce Health Team in Exondys 51 (eteplirsen) —

How to Get Exondys 51 (Eteplirsen) Covered by Cigna in California: Complete Appeals Guide with Forms and Timelines

Answer Box: Getting Exondys 51 Covered by Cigna in California

Quick Path to Approval: Cigna requires genetic testing proving exon 51 amenability, specialist prescriber, and prior authorization through Express Scripts/Accredo. If denied, file internal appeal within 180 days, then request California Independent Medical Review (IMR) through DMHC. Success rates for rare disease appeals in California are significantly higher than national averages.

First Step Today: Ensure genetic test results clearly state "mutation amenable to exon 51 skipping" and submit PA electronically via CoverMyMeds or ExpressPAth with complete clinical documentation.

Table of Contents

  1. Why California State Rules Matter
  2. Prior Authorization Requirements
  3. Turnaround Standards and Timelines
  4. Step Therapy Protections in California
  5. Appeals Playbook for Cigna
  6. California's Independent Medical Review
  7. Common Denial Reasons & Solutions
  8. Costs and Patient Support
  9. When to Escalate
  10. FAQ

Why California State Rules Matter

California's robust insurance regulations provide stronger patient protections than most states, especially for specialty drugs like Exondys 51 (eteplirsen). The state has two regulatory agencies: the Department of Managed Health Care (DMHC) oversees most HMOs and managed care plans, while the California Department of Insurance (CDI) regulates other health policies.

For Duchenne muscular dystrophy (DMD) patients, these protections are crucial because Exondys 51 costs approximately $7,822 per vial and requires weekly IV infusions. California's step therapy override laws (AB 347) and Independent Medical Review system give patients powerful tools to challenge denials.

Note: Most Cigna plans in California fall under DMHC regulation, which provides the strongest appeal rights and fastest external review timelines in the nation.

Prior Authorization Requirements

Coverage at a Glance

Requirement What It Means Where to Find It Source
PA Required Yes, through Express Scripts/Accredo Cigna formulary Cigna Specialty Pharmacy
Genetic Testing Must confirm exon 51 amenability CLIA-certified lab report FDA Label Requirements
Prescriber Specialist (neurologist/geneticist) preferred PA form requirements Accredo PA Process
Age Limits FDA-approved for pediatric/adult DMD Clinical documentation FDA Approval
Site of Care Infusion center or home health Administration plan required PA Documentation

Step-by-Step: Fastest Path to Approval

  1. Obtain Genetic Testing (Patient/Clinic)
    • Ensure test specifically states "mutation amenable to exon 51 skipping"
    • Use CLIA-certified laboratory
    • Timeline: 1-2 weeks for results
  2. Gather Clinical Documentation (Clinic)
    • DMD diagnosis with ICD-10 code G71.01
    • Baseline cardiac and renal function tests
    • 6-minute walk test (if applicable)
    • Prior therapy history
  3. Submit Prior Authorization (Prescriber)
    • Use CoverMyMeds, ExpressPAth, or MyAccredoPatients
    • Include genetic report and clinical rationale
    • Timeline: Electronic submission preferred for speed
  4. Track Response (Patient/Clinic)
    • Standard review: 72 hours
    • Expedited review: 24 hours (if urgent)
    • Follow up if no response within timeframe

Turnaround Standards and Timelines

California law requires specific response times for prior authorization requests:

  • Urgent requests requiring PA: 96 hours (4 calendar days) under DMHC rules
  • Cigna expedited review: 72 hours for urgent cases
  • Standard requests: Typically 72 hours for specialty drugs
  • Emergency situations: No PA required - seek immediate care

For DMD patients experiencing disease progression or complications, providers should mark requests as urgent and provide clinical justification for expedited review.

Tip: If Cigna doesn't respond within the required timeframe, the request may be deemed automatically approved under California's step therapy law (AB 347).

Step Therapy Protections in California

California's step therapy override law (AB 347 of 2021) provides strong protections for patients who need Exondys 51. Exception criteria include:

Medical Exception Requirements

  • Prior failure of step-therapy medications
  • Contraindications to required drugs
  • Likelihood of adverse events from step therapy
  • Provider attestation that step therapy would cause harm or delay effective treatment

Documentation That Helps

When requesting a step therapy exception, include:

  • Detailed history of prior corticosteroid use (deflazacort/Emflaza)
  • Any adverse reactions or contraindications
  • Clinical rationale for immediate Exondys 51 therapy
  • Reference to DMD treatment guidelines supporting genetic-specific therapy

The standard DMHC prior authorization form includes sections specifically for step therapy overrides and medical exceptions.

Appeals Playbook for Cigna

Internal Appeal Process

Timeline: File within 180 days of denial notice

Required Documents:

  • Written appeal letter addressing specific denial reason
  • Genetic testing report confirming exon 51 amenability
  • Clinical notes supporting medical necessity
  • Prescriber attestation letter

How to Submit:

  • Use Cigna Provider Appeal Form
  • Submit via address provided in denial letter
  • Track submission with certified mail or electronic confirmation

Expedited Appeals

For urgent situations where delay could jeopardize health:

  • Request expedited review in writing
  • Provide clinical justification for urgency
  • Cigna must respond within 72 hours
  • Available for both PA and appeal requests

California's Independent Medical Review

If Cigna upholds the denial after internal appeal, California's Independent Medical Review (IMR) provides a powerful second opinion. Recent data shows dramatically different success rates compared to other states:

Success Rates

  • Blue Shield of California: 55.3% of medical necessity denials overturned by DMHC in 2023
  • General health plan IMRs: 45-70% overturn rates for medical necessity denials
  • Workers' compensation: Only 11-13% success rate (different system)

How to Request IMR

  1. Eligibility: Available after internal appeal is denied or if plan fails to respond within 30 days
  2. Timeline: File within specified timeframe (usually 6 months)
  3. Process: DMHC assigns independent physician experts in relevant specialty
  4. Decision: Standard IMR decided within 45 days; expedited within 7 days
  5. Cost: No fee to patients

Contact: DMHC Help Center at 888-466-2219

Common Denial Reasons & Solutions

Denial Reason How to Overturn Documentation Needed
Genetic testing unclear Resubmit with specific exon 51 language CLIA lab report stating "amenable to exon 51 skipping"
Non-specialist prescriber Transfer care or obtain consultation Neurologist or geneticist evaluation
Missing baseline tests Complete required assessments Cardiac echo, renal function, pulmonary tests
Step therapy not tried Request medical exception Document contraindications or prior failures
Quantity/frequency limits Provide weight-based dosing rationale BSA calculation and FDA dosing guidelines

Costs and Patient Support

Financial Assistance Options

  • Sarepta Therapeutics Patient Support: SareptAssist Program provides copay assistance and prior authorization support
  • Foundation Grants: Organizations like the Muscular Dystrophy Association offer financial assistance
  • California State Programs: Low-income patients may qualify for additional support through state programs

Typical Costs

  • Cash price: ~$7,822 per 10 mL vial
  • With insurance: Varies by plan; copay assistance available
  • Annual cost: Can exceed $300,000 without insurance coverage

Counterforce Health helps patients navigate complex prior authorization requirements by analyzing denial letters and crafting evidence-backed appeals specifically tailored to each payer's criteria. Their platform ingests clinical notes, genetic testing results, and payer policies to create targeted rebuttals that address denial reasons point-by-point.

When to Escalate

Contact California Regulators

DMHC Help Center: 888-466-2219

  • File complaints about delayed responses
  • Request assistance with IMR applications
  • Report violations of timely access standards

What to Include:

  • Copy of denial letter
  • Timeline of communications
  • Clinical documentation
  • Impact on patient health

Consumer Assistance Organizations

  • Health Consumer Alliance: Provides free assistance with appeals
  • Legal aid organizations: May help with complex cases
  • Patient advocacy groups: DMD-specific organizations offer guidance

FAQ

How long does Cigna prior authorization take in California? Standard PA requests must be processed within 72 hours. Urgent requests requiring expedited review must be completed within 24-72 hours depending on the specific circumstances.

What if Exondys 51 is not on Cigna's formulary? You can request a formulary exception through Cigna's medical exception process. California law requires plans to have a clear exception process with specific timelines.

Can I request an expedited appeal if my child's condition is worsening? Yes. Mark the appeal as urgent and provide clinical documentation showing that delay would jeopardize health. Cigna must respond within 72 hours for expedited appeals.

Does step therapy apply if we've tried other DMD treatments outside California? Yes, prior therapy history from other states counts toward step therapy requirements. Provide complete documentation of previous treatments and outcomes.

What genetic testing is required for Exondys 51 coverage? The genetic test must specifically confirm a dystrophin gene mutation that is "amenable to exon 51 skipping." Generic DMD genetic testing may not be sufficient.

How much does an Independent Medical Review cost? IMR is free for patients in California. The health plan pays the cost of the independent review.

Can my doctor request a peer-to-peer review with Cigna? Yes, physicians can request peer-to-peer reviews to discuss medical necessity directly with Cigna's medical director. This often helps resolve denials before formal appeals.

What happens if I miss the appeal deadline? California provides some flexibility for late appeals if there were extenuating circumstances. Contact the DMHC Help Center for guidance on late filing options.

From our advocates: We've seen families successfully overturn initial denials by ensuring their genetic testing report uses the specific FDA language about "exon 51 amenability" rather than general DMD mutation descriptions. This targeted documentation, combined with a neurologist's letter explaining why immediate treatment is medically necessary, significantly improves approval odds. While outcomes vary, thorough preparation of clinical documentation makes a substantial difference in the review process.

Sources & Further Reading

For additional support with complex prior authorization cases, Counterforce Health specializes in turning insurance denials into successful appeals by analyzing payer-specific requirements and generating evidence-backed documentation that meets each plan's criteria.

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Insurance coverage decisions depend on individual policy terms and clinical circumstances. Always consult with your healthcare provider and insurance plan for specific guidance regarding your situation. For assistance with California insurance regulations, contact the DMHC Help Center at 888-466-2219.

Powered by Counterforce Health—AI that turns drug denials into evidence-based appeals patients and clinicians can submit today.