By Counterforce Health Team in Exondys 51 (eteplirsen) —

Cigna's Coverage Criteria for Exondys 51 (Eteplirsen) in Michigan: What Counts as "Medically Necessary"?

Answer Box: Getting Exondys 51 Covered by Cigna in Michigan

To get Exondys 51 (eteplirsen) covered by Cigna in Michigan, you need genetic confirmation of a DMD mutation amenable to exon 51 skipping, baseline cardiac and pulmonary function tests, and prior authorization through Accredo Specialty Pharmacy. Your neurologist must submit comprehensive clinical documentation including 6-minute walk test results (≥180 meters) and proof of stable corticosteroid therapy. If denied, you have 180 days to file an internal appeal and 127 days for external review through Michigan DIFS.

First step today: Contact your neurologist to request genetic testing results and schedule baseline assessments if not already completed.

Table of Contents

Policy Overview

Cigna manages Exondys 51 (eteplirsen) as a specialty medication requiring prior authorization across all plan types in Michigan. The drug is typically classified as a non-formulary or high-tier specialty medication, meaning standard formulary coverage doesn't apply without specific clinical justification.

Plan Types Affected:

  • Commercial HMO/PPO plans
  • Medicare Advantage plans
  • Employer-sponsored plans managed by Cigna

All Exondys 51 requests must be processed through Accredo Specialty Pharmacy, Cigna's contracted specialty pharmacy provider. Requests submitted through other channels will not be processed, even from qualified specialists.

Note: Self-insured employer plans may have different appeal procedures and may not be subject to Michigan state external review requirements.

Indication Requirements

Exondys 51 received FDA accelerated approval specifically for Duchenne muscular dystrophy (DMD) patients with confirmed genetic mutations amenable to exon 51 skipping. Cigna's coverage criteria align closely with this narrow FDA indication.

FDA-Approved Indication:

  • Treatment of DMD in patients with a confirmed mutation of the DMD gene amenable to exon 51 skipping
  • Based on increase in dystrophin production in skeletal muscle observed in some patients

Off-Label Considerations: Cigna does not typically cover Exondys 51 for off-label uses. The genetic requirement is strictly enforced, making broader DMD populations ineligible without the specific exon 51-amenable mutation.

Step Therapy & Exceptions

Unlike many medications, Exondys 51 doesn't follow traditional step therapy protocols due to its targeted genetic indication. However, Cigna requires documentation of current or prior corticosteroid therapy, which serves as the standard first-line treatment for DMD.

Required Prior Therapy Documentation:

  • Stable corticosteroid therapy (deflazacort/prednisone) for at least 6 months
  • If corticosteroids were discontinued, medical justification for discontinuation must be provided
  • Documentation of treatment response or intolerance to corticosteroids

Medical Exception Pathways:

  • Contraindication to corticosteroids (documented adverse reactions)
  • Medical reasons preventing corticosteroid use
  • Patient/family preference with physician support (less commonly approved)

Required Diagnostics

Cigna requires comprehensive baseline testing to establish medical necessity and monitor safety. These assessments serve dual purposes: confirming appropriate candidacy and establishing baseline values for ongoing monitoring.

Genetic Testing (Mandatory):

  • Laboratory confirmation from CLIA-certified facility
  • Specific identification of exon 51-amenable DMD mutation
  • Generic "DMD positive" results are insufficient

Functional Assessments:

  • 6-Minute Walk Test: Minimum 180 meters for ambulatory patients
  • Brooke Upper Extremity Scale: For non-ambulatory patients
  • Current weight and date measured

Cardiac Function:

  • Baseline cardiac assessment (echocardiogram or MUGA scan)
  • Left ventricular ejection fraction (LVEF) documentation
  • No active congestive heart failure

Pulmonary Function:

  • Forced vital capacity (FVC) ≥50% predicted (if testable)
  • Not ventilator-dependent
  • Stable respiratory status

Laboratory Studies:

  • Urinalysis showing absence of proteinuria
  • Blood urea nitrogen (BUN) and creatinine (baseline renal function)
  • Recent comprehensive metabolic panel
Tip: Schedule all baseline assessments within 90 days of your prior authorization submission for best results.

Site of Care Requirements

Cigna, through its specialty pharmacy network, has specific site of care preferences for Exondys 51 infusions. These requirements aim to ensure appropriate clinical oversight while managing costs.

Preferred Sites:

  • Home infusion (with appropriate nursing support)
  • Physician's office
  • Non-hospital ambulatory infusion centers

Hospital Outpatient Exceptions: Hospital-based infusions require additional justification and are approved only when:

  • Patient has history of severe adverse reactions requiring immediate medical intervention
  • Clinical instability necessitating hospital-level monitoring
  • Inability to safely manage infusion in preferred settings due to medical complexity

Documentation for Site Exceptions: Detailed clinical rationale explaining why lower-acuity settings are inappropriate for the specific patient.

Evidence to Support Medical Necessity

Building a compelling case for medical necessity requires combining clinical documentation with evidence-based support. Counterforce Health specializes in helping patients and clinicians navigate this complex process by identifying the specific evidence that resonates with each payer's criteria.

Clinical Guidelines to Reference:

  • FDA prescribing information and approval basis
  • Duchenne muscular dystrophy care considerations from neuromuscular societies
  • Published studies on dystrophin expression and functional outcomes

Key Clinical Rationale Points:

  • Progressive nature of DMD without treatment
  • Specific genetic mutation making patient appropriate candidate
  • Functional status supporting potential benefit
  • Safety profile in similar patients

Documentation Best Practices:

  • Include specific functional measurements with dates
  • Reference peer-reviewed literature supporting use in similar patients
  • Clearly state treatment goals and monitoring plan
  • Address any potential contraindications or concerns

Coverage at a Glance

Requirement What It Means Where to Find It Source
Prior Authorization Required before dispensing Accredo Specialty Pharmacy Cigna Policy
Genetic Testing Exon 51-amenable DMD mutation CLIA-certified lab report FDA Label
Functional Status 6MWT ≥180m or Brooke scale Clinical assessment Cigna Criteria
Baseline Cardiac LVEF documentation Echocardiogram/MUGA Clinical guidelines
Specialist Prescriber Neurologist/neuromuscular specialist Provider credentials Payer requirements
Appeal Deadline (Internal) 180 days from denial Denial notice Michigan DIFS
External Review Deadline 127 days from final denial DIFS process Michigan Law

Fastest Path to Approval

Step 1: Confirm Genetic Eligibility (Patient & Physician)

Contact your neurologist to obtain genetic testing results confirming exon 51-amenable DMD mutation. If testing hasn't been completed, request referral to genetic counselor or neuromuscular specialist. Timeline: 2-4 weeks for testing if needed Documents: CLIA-certified genetic laboratory report

Step 2: Complete Baseline Assessments (Physician)

Schedule comprehensive baseline testing including cardiac function (echocardiogram), pulmonary function (if applicable), 6-minute walk test, and laboratory studies (urinalysis, BUN, creatinine). Timeline: 1-2 weeks Documents: Test results dated within 90 days

Step 3: Gather Treatment History (Physician)

Document current corticosteroid therapy or provide medical justification for discontinuation. Include dosing, duration, and clinical response. Timeline: Same day with medical records review Documents: Treatment timeline and clinical notes

Step 4: Submit Prior Authorization (Physician)

Submit comprehensive prior authorization request through Accredo Specialty Pharmacy with all required clinical documentation and genetic confirmation. Timeline: 30 days for standard review; 72 hours for expedited Contact: Accredo provider portal (verify current link with Cigna)

Step 5: Follow Up on Decision (Patient & Physician)

Monitor prior authorization status and respond promptly to any requests for additional information. Timeline: Within decision timeframe Action: Check member portal or call Cigna member services

Step 6: Initiate Appeals if Denied (Patient & Physician)

If denied, immediately begin internal appeal process with additional clinical justification and peer-reviewed evidence. Timeline: Must file within 180 days of denial Contact: Cigna appeals department at 800-244-6224

Step 7: Pursue External Review if Needed (Patient)

After exhausting internal appeals, file external review with Michigan DIFS within 127 days of final denial. Timeline: 60 days for standard; 72 hours for expedited Contact: Michigan DIFS at 877-999-6442

Common Denial Reasons & Fixes

Denial Reason How to Overturn Required Documentation
No genetic confirmation Submit CLIA-certified genetic report Laboratory results identifying specific exon 51-amenable mutation
Insufficient functional status Provide current 6MWT or Brooke scale Recent functional assessment by qualified clinician
Non-specialist prescriber Transfer care or obtain consultation Neurologist or neuromuscular specialist involvement
Missing baseline assessments Complete required testing Cardiac, pulmonary, and renal function studies
Inadequate prior therapy documentation Document corticosteroid history Treatment timeline with dosing and clinical response
Site of care not approved Provide medical justification Clinical rationale for requested infusion setting
From Our Advocates: We've seen cases where initial denials were overturned simply by providing more detailed genetic testing results that clearly identified the specific mutation type. Don't assume the first submission included enough detail—payers often need very specific genetic nomenclature to approve these targeted therapies.

Appeals Process in Michigan

Internal Appeals with Cigna:

  • First Level: Submit within 180 days of denial notice
  • Review Timeline: 30 days for standard; 72 hours for expedited
  • Contact: 800-244-6224 or Cigna member portal
  • Required: Written appeal with additional clinical documentation

Expedited Internal Appeals: Available when standard timeline would seriously jeopardize health. Requires physician certification of urgency.

Michigan DIFS External Review:

  • Deadline: 127 days after final internal denial
  • Standard Review: 60 days maximum
  • Expedited Review: 72 hours with physician certification
  • Cost: No fee to patients
  • Contact: 877-999-6442 or online portal

Required for External Review:

  • Copy of final denial letter from Cigna
  • All relevant medical records
  • Physician statement of medical necessity
  • External review request form

The external review decision is binding on Cigna. If approved, Cigna must provide coverage as directed by the Independent Review Organization.

FAQ

How long does Cigna prior authorization take for Exondys 51 in Michigan? Standard prior authorization decisions are completed within 30 days. Expedited reviews for urgent situations are completed within 72 hours when physician certifies medical urgency.

What if Exondys 51 is non-formulary on my Cigna plan? You can request a formulary exception through your physician. The process follows the same timeline as prior authorization (72 hours for standard exception requests) and requires clinical justification for why formulary alternatives are inappropriate.

Can I request an expedited appeal if my child needs immediate treatment? Yes, both internal appeals with Cigna and external reviews with Michigan DIFS offer expedited pathways when your physician certifies that delays would seriously jeopardize health or ability to regain maximum function.

Does step therapy apply if my child tried corticosteroids outside Michigan? Treatment history from other states is generally accepted. Provide comprehensive documentation of prior therapies, including dates, dosing, duration, and clinical outcomes or reasons for discontinuation.

What happens if Cigna denies coverage after Michigan DIFS approves it? DIFS external review decisions are binding. If Cigna fails to comply with an external review approval, you can file a complaint with DIFS at 877-999-6442 or pursue legal remedies.

How much does Exondys 51 cost without insurance coverage? The typical U.S. cash price is approximately $7,822 for a 10 mL vial (50 mg/mL), though actual costs vary by pharmacy and patient weight-based dosing requirements.

Are there financial assistance programs available? Sarepta Therapeutics offers patient assistance programs. Additionally, organizations like Counterforce Health help families navigate insurance coverage to avoid out-of-pocket costs.

What if my neurologist isn't familiar with the prior authorization process? Many specialty pharmacies and patient advocacy organizations provide support to physicians unfamiliar with rare disease prior authorization requirements. Consider connecting your physician with resources that specialize in DMD treatment access.

Counterforce Health helps patients, clinicians, and specialty pharmacies get prescription drugs approved by turning insurance denials into targeted, evidence-backed appeals. The platform ingests denial letters, plan policies, and clinical notes, then identifies the denial basis and drafts point-by-point rebuttals aligned to the plan's own rules. For evidence, Counterforce Health pulls the right citations for medications and weaves them into appeals with required clinical facts, helping families access life-changing treatments like Exondys 51.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage decisions depend on your specific plan terms and clinical circumstances. Always consult with your healthcare provider and insurance plan for personalized guidance. For additional help with insurance appeals in Michigan, contact the Michigan Department of Insurance and Financial Services at 877-999-6442.

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