By Counterforce Health Team in Orkambi (lumacaftor/ivacaftor) —

Getting Orkambi (Lumacaftor/Ivacaftor) Covered by Blue Cross Blue Shield in California: Complete Guide to Prior Authorization and Appeals

Answer Box: Quick Path to Orkambi Coverage in California

Orkambi (lumacaftor/ivacaftor) requires prior authorization from Blue Cross Blue Shield of California for patients with cystic fibrosis who are F508del homozygous. Success requires three key elements: genetic testing documentation, specialist prescription, and proper ICD-10 coding (E84.0-E84.9). If denied, California's Independent Medical Review (IMR) has a 72% success rate for Orkambi appeals. Start today by: 1) Verify your genetic test results show F508del homozygosity, 2) Get your CF specialist to submit PA with complete clinical documentation, 3) If denied, file internal appeal within 180 days, then request IMR through DMHC.

Table of Contents

Coverage Requirements at a Glance

Requirement What It Means Where to Find It
Prior Authorization Mandatory approval before dispensing Blue Shield CA Authorization List
Genetic Testing F508del homozygous mutation documented Lab report or genetic counselor summary
Specialist Prescription CF specialist or pulmonologist required Provider credentials verification
ICD-10 Diagnosis E84.0-E84.9 (cystic fibrosis codes) Medical records and claim forms
Age Requirement 2 years and older per FDA label FDA prescribing information
NDC Codes 51167-0809-01 (tablets), 51167-0900-01 (granules) Pharmacy billing systems

Step-by-Step: Fastest Path to Approval

1. Verify Genetic Status and Gather Documentation

Who: Patient/family
What: Confirm F508del homozygous mutation status
Timeline: Before PA submission

Obtain a copy of your genetic test results showing two copies of the F508del mutation. If you don't have recent results, ask your CF care team about testing.

2. Schedule CF Specialist Visit

Who: Patient
What: Get prescription from qualified specialist
Timeline: 1-2 weeks

Blue Shield requires prescriptions from CF specialists or pulmonologists familiar with CFTR modulators.

3. Submit Prior Authorization

Who: Prescriber/clinic staff
What: Complete PA form with supporting documentation
Timeline: Submit within 5 business days of prescription

Use Blue Shield's AuthAccel system or standard PA forms. Include:

  • Genetic test results
  • Clinical summary with diagnosis (ICD-10: E84.x)
  • Treatment history
  • Specialist credentials

4. Track PA Decision

Who: Patient/clinic
What: Monitor for approval/denial
Timeline: 72 hours to 14 days

Blue Shield aims for faster PA decisions through automated systems for qualifying requests.

5. If Denied: File Internal Appeal

Who: Patient or prescriber
What: Submit appeal with additional clinical evidence
Timeline: Within 180 days of denial

Strengthen your case with peer-reviewed literature supporting Orkambi use in F508del homozygous CF patients.

6. Request Independent Medical Review (IMR)

Who: Patient
What: File with California DMHC
Timeline: After internal appeal denial or 30-day non-response

California's IMR has a 72% success rate for Orkambi appeals when proper documentation is submitted.

Coding and Billing Essentials

Medical vs. Pharmacy Benefit

Orkambi is billed under the pharmacy benefit as an oral specialty medication, not the medical benefit. This affects prior authorization pathways and coverage determination processes.

ICD-10 Diagnosis Codes

Use appropriate cystic fibrosis codes:

  • E84.0 - Cystic fibrosis with pulmonary manifestations
  • E84.11 - Meconium ileus in cystic fibrosis
  • E84.19 - Cystic fibrosis with other intestinal manifestations
  • E84.8 - Cystic fibrosis with other manifestations
  • E84.9 - Cystic fibrosis, unspecified
Note: Do not use Z14.1 (genetic carrier status) for diagnosed CF patients.

NDC Numbers and Billing Units

  • Tablets: NDC 51167-0809-01 (11-digit format)
  • Oral Granules: NDC 51167-0900-01 (11-digit format)
  • Billing Unit: "Each" blister pack or packet as dispensed
  • No J-Code: Orkambi does not have a HCPCS J-code as it's an oral medication

Common Denial Reasons & How to Fix Them

Denial Reason How to Overturn
Missing genetic testing Submit lab report confirming F508del homozygosity
Non-specialist prescriber Transfer prescription to CF specialist or pulmonologist
Incomplete clinical documentation Add detailed treatment history, current symptoms, rationale
Age restrictions Verify patient meets FDA-approved age requirements (2+ years)
Step therapy requirements Document failed prior therapies or contraindications
Quantity limits exceeded Provide clinical justification for dosing requirements

Appeals Playbook for California

Internal Appeal (Level 1)

Timeline: File within 180 days of denial
Process: Submit to Blue Shield with additional clinical evidence
Decision: 30 days (expedited: 72 hours for urgent cases)

Independent Medical Review (IMR)

Timeline: After internal appeal denial or 30-day non-response
Process: File with DMHC Help Center at (888) 466-2219
Decision: 30 days standard, 3-7 days expedited
Success Rate: 72% for Orkambi when properly documented

California Advantage: IMR decisions are binding on health plans and cannot be appealed by either party.

Required IMR Documentation

  • Complete medical records
  • Genetic test results
  • Specialist's clinical summary
  • Treatment history and failures
  • Scientific literature supporting use

When Step Therapy or Formulary Issues Arise

Formulary Exception Process

If Orkambi isn't on your plan's formulary, request an exception by documenting:

  • Failed trials of formulary alternatives
  • Contraindications to preferred drugs
  • Adverse reactions to step therapy requirements

Submit via Blue Shield's formulary exception form with clinical justification.

Step Therapy Override

For patients required to try other CFTR modulators first:

  • Document specific reasons why step therapy is inappropriate
  • Include contraindications or predicted poor outcomes
  • Provide specialist recommendation for Orkambi as first-line therapy

Costs and Patient Assistance

Manufacturer Support

Vertex Pharmaceuticals offers patient assistance through:

  • Vertex GPS (co-pay assistance and patient support)
  • Income-based patient assistance programs
  • Free drug programs for qualifying patients

Foundation Grants

  • Cystic Fibrosis Foundation Compass program
  • HealthWell Foundation specialty drug assistance
  • Patient Access Network Foundation

Annual list price for Orkambi is approximately $302,107, making patient assistance crucial for most families.

Counterforce Health specializes in turning insurance denials into successful appeals for complex specialty medications like Orkambi. Our platform analyzes denial letters, identifies the specific basis for rejection, and creates targeted, evidence-backed appeals that align with each payer's own criteria. For families facing Orkambi denials, we help navigate the complex prior authorization and appeals process with documentation that meets California's specific regulatory requirements.

Frequently Asked Questions

How long does Blue Shield's prior authorization take in California?
Standard PA decisions are typically made within 72 hours to 14 days. Blue Shield has implemented faster decision-making technology for qualifying requests that meet clear clinical criteria.

What if my child's CF center isn't in Blue Shield's network?
You may need a referral for out-of-network specialist care. Contact Blue Shield member services to understand your specific plan's referral requirements and potential coverage for out-of-network CF specialists.

Can I request expedited review for Orkambi?
Yes, if your doctor documents clinical urgency or deteriorating condition. Expedited PAs are decided within 72 hours, and expedited IMRs within 3-7 days.

What happens if both internal appeals and IMR fail?
Explore manufacturer patient assistance programs and foundation grants. Counterforce Health can also help identify alternative appeal strategies or coverage pathways.

Does step therapy apply if we've tried other CFTR modulators outside California?
Document all prior therapies regardless of location. Out-of-state treatment history should be accepted if properly documented by your current CF specialist.

How often do I need to renew Orkambi authorization?
Most plans require annual reauthorization. Some may require more frequent reviews to document ongoing clinical benefit.

From Our Advocates: We've seen families succeed with Orkambi appeals by focusing on three key elements: comprehensive genetic documentation, detailed treatment failure history, and strong specialist support. One family's appeal was initially denied for "insufficient clinical justification" but succeeded at IMR when they included peer-reviewed studies showing Orkambi's specific benefits for F508del homozygous patients and documented the child's declining lung function despite standard therapies.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Insurance policies and procedures change frequently. Always verify current requirements with your specific Blue Cross Blue Shield plan and consult your healthcare provider for medical decisions. For personalized assistance with appeals and prior authorizations, consider consulting with healthcare advocates or legal professionals specializing in insurance coverage.

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