By Counterforce Health Team in Evkeeza (evinacumab-dgnb) —

How to Get Evkeeza (evinacumab-dgnb) Covered by Cigna in California: Prior Authorization, Appeals, and Success Strategies

Quick Answer: Getting Evkeeza Covered by Cigna in California

To get Evkeeza (evinacumab-dgnb) covered by Cigna in California for homozygous familial hypercholesterolemia (HoFH), you'll need prior authorization with genetic testing confirmation, documented failure of high-intensity statin plus ezetimibe, and (for ages 10+) a PCSK9 inhibitor trial. If denied, California's Independent Medical Review (IMR) through DMHC provides strong external appeal rights. Start today: Gather your genetic test results, recent LDL-C levels, and medication history, then work with your specialist to submit a comprehensive prior authorization request through Cigna's provider portal.

Table of Contents

  1. Patient Profile: Who Qualifies for Evkeeza
  2. Pre-Authorization Preparation
  3. Submitting Your Request
  4. Initial Outcome: Approval or Denial
  5. The Appeal Process
  6. Resolution and Next Steps
  7. What We'd Do Differently
  8. Templates and Checklists
  9. Cost Assistance Options
  10. When to Escalate to State Regulators
  11. FAQ

Patient Profile: Who Qualifies for Evkeeza

Evkeeza (evinacumab-dgnb) is an ANGPTL3 monoclonal antibody approved for patients with homozygous familial hypercholesterolemia (HoFH) who are at least 5 years old. This ultra-rare condition affects roughly 1 in 300,000 people and causes severely elevated LDL cholesterol levels that don't respond adequately to standard treatments.

Cigna's coverage criteria require:

  • Age: 5 years or older
  • Confirmed HoFH diagnosis through genetic testing or clinical criteria
  • Failed prior therapies: High-intensity statin, ezetimibe, and (for ages 10+) PCSK9 inhibitor
  • Recent LDL-C levels showing inadequate response to maximum tolerated therapy

The medication costs approximately $450,000 annually, making prior authorization essential for coverage.

Pre-Authorization Preparation

Success starts with thorough documentation. Here's what you'll need:

Essential Documentation Checklist

Medical Records:

  • Genetic test results confirming HoFH mutations (LDLR, APOB, PCSK9, or LDLRAP1)
  • Recent lipid panel (within 90 days) showing elevated LDL-C
  • Complete medication history with dates, doses, and outcomes
  • Documentation of any statin intolerance or contraindications

Prior Therapy Requirements:

  • High-intensity statin trial with ezetimibe (unless contraindicated)
  • PCSK9 inhibitor trial for 8+ weeks (ages 10+; not required for ages 5-9)
  • Evidence of inadequate LDL-C response despite maximum tolerated therapy
Tip: If your patient can't tolerate statins, document intolerance to at least two different statins, including attempts at lower doses or alternate-day dosing.

Medical Necessity Letter Components

Your specialist should include:

  • HoFH diagnosis confirmation and genetic testing results
  • Current LDL-C levels and cardiovascular risk assessment
  • Detailed prior therapy history and reasons for inadequacy
  • Clinical rationale for Evkeeza based on FDA labeling
  • Weight-based dosing calculation (15 mg/kg IV every 4 weeks)

Download the manufacturer's sample letter template and customize it for your specific case.

Submitting Your Request

Cigna processes Evkeeza prior authorizations through their standard channels:

Submission Methods

Electronic Prior Authorization (ePA):

  • Submit through Cigna's provider portal
  • Fastest processing time (typically 2-5 business days)
  • Real-time status updates

Phone/Fax:

  • Call 1-800-882-4462 for provider assistance
  • Fax completed forms to the number provided in your denial letter

Required Forms

Submit Cigna's Evkeeza Prior Authorization Form along with:

  • Medical necessity letter
  • Recent lab results
  • Genetic testing report
  • Prior medication trial documentation
  • Current treatment plan

Initial Outcome: Approval or Denial

Cigna typically responds within 5-15 business days for standard requests (72 hours for urgent cases).

If Approved

Your approval will specify:

  • Coverage duration (typically 12 months)
  • Specialty pharmacy requirements (often Express Scripts/Accredo)
  • Any quantity limits or administration site restrictions

Common Denial Reasons

Denial Reason How to Address
Missing HoFH confirmation Submit genetic testing results or detailed clinical criteria documentation
Inadequate prior therapy Provide complete medication trial history with dates and outcomes
Missing recent LDL-C levels Submit lipid panel within 90 days showing elevated levels
Age restrictions Confirm patient is 5+ years old; adjust PCSK9 requirements for ages 5-9

The Appeal Process

If your initial request is denied, don't give up. California offers robust appeal rights, especially for rare disease treatments.

Step 1: Internal Appeal with Cigna

Timeline: File within 180 days of denial Process:

  1. Call Cigna at 1-800-882-4462 to request peer-to-peer review
  2. Submit additional documentation addressing denial reasons
  3. Request expedited review if delay could harm patient health

Peer-to-Peer Script: "I'm requesting a peer-to-peer review for my patient with genetically confirmed HoFH who has failed maximum tolerated statin therapy, ezetimibe, and PCSK9 inhibitor treatment. The patient's LDL-C remains dangerously elevated at [X] mg/dL despite optimal conventional therapy. Evkeeza is FDA-approved and medically necessary for this ultra-rare condition."

Step 2: California Independent Medical Review (IMR)

If Cigna upholds the denial, California residents have powerful external appeal rights through the Department of Managed Health Care (DMHC).

Who's Eligible: Cigna HMO and most PPO members Process:

  1. File grievance with Cigna first (or wait 30 days)
  2. Contact DMHC Help Center: 1-888-466-2219
  3. Submit IMR application with supporting documentation

Timeline:

  • Standard IMR: 30 days for decision
  • Expedited IMR: 3-7 days for urgent cases
  • Implementation: Cigna must authorize within 5 days if IMR rules in your favor
Note: IMR decisions are binding on Cigna. California has high success rates for specialty drug appeals, especially when supported by strong clinical evidence.

Resolution and Next Steps

If Approved After Appeal

Once approved, ensure:

  • Specialty pharmacy enrollment (Express Scripts/Accredo typically)
  • Infusion site coordination (hospital outpatient or infusion center)
  • Renewal timeline tracking (start 60-90 days before expiration)

Ongoing Management

Annual Renewals: Submit updated documentation including:

  • Current LDL-C levels showing continued need
  • Treatment response data
  • Any changes in clinical status
  • Confirmation of continued HoFH diagnosis

What We'd Do Differently: Lessons Learned

From our advocates: We've seen the strongest Evkeeza approvals when families work closely with lipid specialists who understand payer requirements. One case that stood out involved a pediatric patient whose initial denial was overturned after the specialist provided detailed genetic counseling records and growth charts showing the medication's weight-based dosing was appropriate. The key was comprehensive documentation from the start.

Key Success Factors:

  1. Work with a lipid specialist familiar with HoFH and payer requirements
  2. Gather all documentation upfront rather than submitting piecemeal
  3. Use manufacturer resources including patient support programs and appeal templates
  4. Don't accept initial denials - California's appeal system strongly favors patients with rare diseases

Templates and Checklists

Pre-Submission Checklist

  • Genetic testing confirming HoFH
  • Recent lipid panel (within 90 days)
  • Complete prior therapy documentation
  • Medical necessity letter from specialist
  • Patient weight for dosing calculation
  • Insurance card and policy information

Appeal Documentation

  • Original denial letter
  • Additional clinical evidence
  • Updated lab results
  • Specialist attestation of medical necessity
  • Relevant clinical guidelines or studies

Cost Assistance Options

Even with insurance coverage, out-of-pocket costs can be significant. Explore these options:

Manufacturer Support:

Foundation Grants:

  • Patient Advocate Foundation
  • National Organization for Rare Disorders (NORD)
  • HealthWell Foundation

State Programs:

  • California's Medi-Cal may provide additional coverage options
  • Covered California plans with enhanced rare disease benefits

When to Escalate to State Regulators

Contact California regulators if:

  • Cigna fails to meet appeal timelines
  • You suspect improper denial practices
  • IMR decisions aren't implemented promptly

DMHC Help Center: 1-888-466-2219 or www.dmhc.ca.gov California Department of Insurance: 1-800-927-4357 (for CDI-regulated plans)

FAQ

How long does Cigna prior authorization take in California? Standard requests: 5-15 business days. Urgent requests: 72 hours. Use expedited processing if delay could harm patient health.

What if Evkeeza is non-formulary on my Cigna plan? Request a formulary exception with clinical justification. California law supports access to medically necessary non-formulary drugs.

Can I request an expedited appeal? Yes, if delay in treatment could seriously jeopardize your health. Both Cigna and California IMR offer expedited timelines.

Does step therapy apply if I failed treatments outside California? Yes, prior therapy trials from any provider count toward meeting step therapy requirements. Ensure complete documentation.

What's the success rate for Evkeeza appeals in California? While specific data isn't published, California's IMR system has high overturn rates for specialty drug denials, especially with strong clinical evidence.

About Counterforce Health

Counterforce Health specializes in turning insurance denials into successful appeals for prescription drugs. Our platform analyzes denial letters, identifies the specific denial basis, and creates targeted, evidence-backed appeals that align with each payer's requirements. We help patients, clinicians, and specialty pharmacies navigate complex prior authorization processes and improve approval rates for essential medications.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Always consult with your healthcare provider and insurance plan for specific coverage decisions. Coverage criteria and processes may change; verify current requirements with your insurer.

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