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

How to Get Orkambi (lumacaftor/ivacaftor) Covered by Aetna CVS Health in Ohio: Coding, Appeals, and Prior Authorization Guide

Answer Box: Getting Orkambi Covered by Aetna CVS Health in Ohio

Orkambi (lumacaftor/ivacaftor) requires prior authorization from Aetna CVS Health for cystic fibrosis patients who are homozygous for the F508del mutation. The fastest path to approval: (1) Confirm genetic testing shows F508del homozygous status, (2) Have a pulmonologist submit the prior authorization with ICD-10 code E84.0 and NDC 51167-0809-01, and (3) Include complete medical records showing baseline lung function. If denied, Ohio residents have 180 days to request external review through the Ohio Department of Insurance.

Table of Contents

Coding Basics: Medical vs. Pharmacy Benefit

Orkambi is typically covered under the pharmacy benefit as a self-administered oral medication dispensed through CVS Specialty Pharmacy. However, understanding both pathways helps ensure proper coding:

Pharmacy Benefit (Most Common):

  • Uses NDC numbers for billing
  • Processed through CVS Caremark
  • Requires specialty pharmacy enrollment
  • Standard copay/coinsurance applies

Medical Benefit (Rare Cases):

  • May require HCPCS codes alongside NDC
  • Used for inpatient administration or unique plan designs
  • Billed through medical claims system
  • Different prior authorization process
Note: Always verify which benefit covers Orkambi for your specific Aetna plan before submitting claims.

ICD-10 Mapping for Cystic Fibrosis

Primary Diagnosis Codes:

ICD-10 Code Description When to Use
E84.0 Cystic fibrosis with pulmonary manifestations Most common; includes lung symptoms
E84.8 Cystic fibrosis with other manifestations GI, pancreatic, or other organ involvement
E84.9 Cystic fibrosis, unspecified When specific manifestations aren't documented

Documentation Requirements:

  • Medical records must include genetic test results confirming F508del homozygous mutation
  • Laboratory report showing "homozygous F508del" or "F508del/F508del"
  • Test must be from FDA-cleared CF mutation panel or DNA sequencing
  • Include lab name, test date, and methodology

According to Aetna's 2024 clinical policy, genetic confirmation is mandatory for Orkambi approval.

Product Coding: NDC, HCPCS, and Billing Units

NDC Numbers (11-digit format):

  • Tablets: 51167-0809-01 (200mg lumacaftor/125mg ivacaftor)
  • Granules: 51167-0900-01 (for pediatric patients)

Billing Units:

  • Both forms billed as "Each" (EA)
  • Standard dosing: 2 tablets every 12 hours = 4 tablets daily
  • Monthly supply: typically 112 tablets (28-day supply)

HCPCS Considerations: Orkambi does not have a permanent J-code as of 2025. For medical benefit claims (rare), verify with Aetna whether a miscellaneous code like J3490 is required alongside the NDC.

Tip: The annual list price is approximately $302,107 according to Vertex Pharmaceuticals, making accurate coding essential for claim processing.

Clean Prior Authorization Request

Required Elements for Aetna CVS Health:

  1. Patient Information:
    • Complete demographics
    • Aetna member ID
    • ICD-10 diagnosis code (E84.0 most common)
  2. Prescriber Requirements:
    • Must be pulmonologist or documented CF specialist consultation
    • NPI and contact information
    • DEA number if required
  3. Clinical Documentation:
    • Genetic test report showing F508del homozygous mutation
    • Baseline lung function (ppFEV1 if available)
    • Medical history and current symptoms
    • Prior therapy trials and outcomes
  4. Medication Details:
    • NDC: 51167-0809-01
    • Strength: 200mg/125mg tablets
    • Quantity: 112 tablets per 28 days
    • Directions: 2 tablets every 12 hours

Submission Methods:

Common Coding Pitfalls

Unit Conversion Errors:

  • Billing 28 units instead of 112 for monthly supply
  • Using wrong NDC for age-appropriate formulation
  • Incorrect "each" vs. "package" unit designation

Missing Documentation:

  • Genetic test results not attached
  • Specialist consultation not documented
  • ICD-10 code doesn't match clinical notes

Timing Issues:

  • Submitting PA too close to desired start date
  • Missing renewal deadlines (typically 12 months)
  • Not accounting for Aetna's 30-45 day review period

Verification with Aetna CVS Health

Before Submitting:

  1. Check Formulary Status: Verify Orkambi's tier placement and any step therapy requirements on your patient's specific plan
  2. Confirm PA Requirements: Review current Aetna precertification list
  3. Validate Codes: Cross-reference NDC with Aetna's approved drug list

Contact Numbers:

  • Commercial plans: 1-888-632-3862 (TTY: 711)
  • Medicare plans: 1-800-624-0756 (TTY: 711)

Online Resources:

  • Provider portal for real-time PA status
  • Formulary lookup tools
  • Clinical policy bulletins

Appeals Process in Ohio

If Aetna denies your Orkambi prior authorization, Ohio residents have specific rights:

Internal Appeals (First Level):

  • Submit within 180 days of denial notice
  • Include additional clinical documentation
  • Decision within 30 days (72 hours for urgent)

External Review (Ohio Department of Insurance):

  • Available after internal appeals or if Aetna doesn't respond timely
  • Request within 180 days of final internal denial
  • Submit through Ohio External Review System
  • Independent Review Organization (IRO) renders binding decision
  • Standard review: 30 days; expedited: 72 hours

Key Ohio Advantages:

  • State regulators can override insurer eligibility determinations
  • No cost to patient for external review
  • Consumer hotline: 1-800-686-1526

For complex cases, Counterforce Health specializes in turning insurance denials into targeted, evidence-backed appeals by analyzing denial letters and crafting point-by-point rebuttals aligned to each plan's specific policies.

Pre-Submission Checklist

Documentation Review:

  • ICD-10 code matches clinical presentation
  • NDC number is correct (51167-0809-01 for tablets)
  • Genetic test results clearly show F508del homozygous
  • Prescriber is pulmonologist or CF specialist
  • Quantity calculation is accurate (112 tablets/28 days)
  • Prior therapy failures documented if step therapy applies

Submission Verification:

  • All required fields completed
  • Supporting documents attached
  • Submission method confirmed (Availity portal preferred)
  • Timeline allows for 30-45 day review
  • Contact information current for follow-up

Follow-Up Plan:

  • PA tracking number recorded
  • Calendar reminder for decision deadline
  • Appeal strategy prepared if denied
  • Patient informed of timeline and next steps

FAQ

How long does Aetna CVS Health prior authorization take in Ohio? Standard decisions take 30-45 days. Expedited reviews for urgent medical needs are completed within 72 hours. Submit requests at least two weeks before the desired start date.

What if Orkambi is non-formulary on my plan? Request a formulary exception with medical necessity documentation. Include evidence that preferred alternatives are inappropriate due to the patient's specific F508del genotype.

Can I request an expedited appeal in Ohio? Yes, if delay would seriously jeopardize health. Both Aetna's internal process and Ohio's external review offer expedited timelines (typically 72 hours).

Does step therapy apply if I've tried other CFTR modulators outside Ohio? Previous therapy trials from any location count toward step therapy requirements. Ensure documentation includes dates, dosages, and reasons for discontinuation.

What happens if my external review is denied in Ohio? The IRO decision is binding on Aetna, but you retain rights to legal remedies or regulatory complaints. Contact the Ohio Department of Insurance at 1-800-686-1526 for guidance.

How much will Orkambi cost with Aetna coverage? Costs depend on your plan's specialty tier structure. Typical copays range from $50-$150 monthly, though some plans may require coinsurance. Check with CVS Specialty Pharmacy for exact amounts.

From our advocates: We've seen cases where genetic testing was completed years ago, but the specific mutation results weren't clearly documented in recent medical records. One patient's appeal was initially denied until we helped locate the original lab report showing F508del homozygous status. Always ensure genetic results are easily accessible in current clinical notes.

When Professional Help Makes Sense

For patients facing repeated denials or complex appeals, specialized support can make a significant difference. Counterforce Health helps patients, clinicians, and specialty pharmacies navigate insurance denials by creating evidence-backed appeals tailored to each payer's specific requirements. Their platform analyzes denial letters, identifies the specific basis for denial, and crafts targeted rebuttals using the right clinical evidence and procedural requirements.

Disclaimer: This information is for educational purposes and does not constitute medical or legal advice. Coverage policies and procedures may change. Always verify current requirements with Aetna CVS Health and consult healthcare providers for medical decisions. For official appeals assistance in Ohio, contact the Ohio Department of Insurance at 1-800-686-1526.

Sources & Further Reading

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