Coding That Helps Get Juxtapid (lomitapide) Approved by Cigna in Michigan: ICD-10, HCPCS, and NDC Guide

Answer Box: Getting Juxtapid (lomitapide) Coded Correctly for Cigna Coverage in Michigan

Juxtapid (lomitapide) requires precise coding to avoid Cigna denials in Michigan. Use ICD-10 code E78.010 (homozygous familial hypercholesterolemia) effective 2025, HCPCS code J8499 (oral non-chemotherapeutic drug, NOS), and verify NDC numbers for specific capsule strengths through the REMS program. Submit via Cigna's prior authorization process with genetic testing documentation and step therapy failure records. First step today: Contact your prescriber to confirm HoFH diagnosis documentation and request Cigna's Juxtapid PA form.


Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit Paths
  2. ICD-10 Mapping for HoFH
  3. Product Coding: HCPCS, J-Codes, and NDC Numbers
  4. Clean Request Anatomy
  5. Frequent Coding Pitfalls
  6. Verification with Cigna Resources
  7. Quick Audit Checklist
  8. Michigan Appeals Process
  9. FAQ

Coding Basics: Medical vs. Pharmacy Benefit Paths

Juxtapid (lomitapide) typically falls under medical benefit coverage at Cigna due to its specialty drug status and REMS requirements. However, the coding path depends on your specific plan design.

Medical Benefit Path

  • When: Most commercial Cigna plans with specialty pharmacy benefits
  • Codes: HCPCS J8499, ICD-10 E78.010
  • Dispensing: Through Accredo specialty pharmacy only
  • Prior Auth: Required via Cigna's medical PA process

Pharmacy Benefit Path

  • When: Some Medicare Part D plans or specific commercial formularies
  • Codes: NDC-based billing with quantity limits
  • Dispensing: Still restricted to REMS-certified pharmacies
  • Prior Auth: Through Express Scripts pharmacy benefit management
Note: Even under pharmacy benefit, Juxtapid requires specialty pharmacy dispensing due to FDA REMS requirements.

ICD-10 Mapping for HoFH

Primary Code: E78.010 (Effective 2025)

E78.010 specifically identifies homozygous familial hypercholesterolemia, replacing the deleted E78.01. This precision is crucial for Cigna coverage.

Documentation Required Supporting Evidence
Genetic confirmation Biallelic mutations in LDLR, APOB, or PCSK9 genes
Clinical criteria Untreated LDL-C >400-500 mg/dL, treated ≥300 mg/dL
Family history Both parents with heterozygous FH (code Z83.42 if applicable)
Physical findings Cutaneous xanthomas, corneal arcus

Documentation Words That Support E78.010

Your medical records should include specific terminology:

  • "Homozygous familial hypercholesterolemia" or "HoFH"
  • "Biallelic mutations" or "compound heterozygous"
  • "Genetic testing confirms..." with specific gene variants
  • "Severe phenotype consistent with HoFH"
Warning: Using generic codes like E78.5 (unspecified hyperlipidemia) will trigger automatic denials. Cigna requires HoFH-specific documentation.

Product Coding: HCPCS, J-Codes, and NDC Numbers

HCPCS/J-Code: J8499

Juxtapid lacks a permanent HCPCS code. Use J8499 (Prescription drug, oral, non-chemotherapeutic, NOS) for medical benefit billing.

Alternative codes for specific situations:

  • J3490: Unclassified drugs (some payers prefer this)
  • C9399: Unclassified drugs or biologicals
  • J9999: Compounded drug, not otherwise classified

NDC Numbers by Strength

Strength NDC Format Typical Use
5 mg Verify with pharmacy Initial dosing
10 mg Verify with pharmacy Titration phase
20 mg Verify with pharmacy Maintenance dosing
40 mg Verify with pharmacy Maximum for dialysis patients
60 mg Verify with pharmacy Maximum standard dose
Important: NDC numbers must be verified through REMS-certified pharmacies as they change with manufacturing updates.

Units Calculation

For medical benefit billing:

  • 1 unit = 1 capsule for most payers
  • Quantity = days supply × daily dose
  • Example: 20 mg daily × 30 days = 30 units (30 capsules of 20 mg)

Common unit errors:

  • Billing total mg as units (600 units for 20 mg × 30 days instead of 30 units)
  • Using per-kg calculations (Juxtapid is weight-neutral)
  • Splitting capsules (not permitted under REMS)

Clean Request Anatomy

Example Prior Authorization Request

Patient Information:

  • Diagnosis: E78.010 (Homozygous familial hypercholesterolemia)
  • Requested drug: Juxtapid (lomitapide) 20 mg capsules
  • Quantity: 30 capsules (30-day supply)
  • HCPCS: J8499

Required Documentation Checklist: ✅ Genetic testing report showing biallelic mutations
✅ Baseline LDL-C levels (>300 mg/dL on current therapy)
✅ Documentation of statin trial/failure or intolerance
✅ PCSK9 inhibitor trial/failure documentation
✅ Baseline liver function tests (ALT, AST, bilirubin)
✅ REMS enrollment confirmation
✅ Low-fat diet counseling documentation

Medical Necessity Letter Template

"Patient has genetically confirmed HoFH with biallelic LDLR mutations. Despite maximally tolerated atorvastatin 80mg daily and ezetimibe 10mg daily, LDL-C remains 350 mg/dL. Previous trial of evolocumab (Repatha) for 6 months showed insufficient response (LDL-C reduction <30%). Lomitapide is indicated per FDA labeling as adjunctive therapy for HoFH when LDL-C remains elevated despite optimal conventional therapy."


Frequent Coding Pitfalls

Unit Conversion Errors

❌ Incorrect ✅ Correct Why
600 units for 20mg × 30 days 30 units Units = capsules, not total mg
Billing twice daily Once daily dosing Juxtapid is once daily only
Using mg/kg calculations Fixed mg dosing Not weight-based

Mismatched Codes

Problem: ICD-10 E78.5 (unspecified) with J8499 for lomitapide
Solution: Use E78.010 to match HoFH indication

Problem: NDC for 10mg with SIG for 20mg daily
Solution: Verify NDC matches prescribed strength

Missing Start Dates

Cigna requires specific therapy start dates for step therapy documentation. Include:

  • Statin start date and maximum tolerated dose
  • PCSK9 inhibitor trial dates and outcomes
  • Previous therapy discontinuation reasons with dates

Verification with Cigna Resources

Pre-Submission Verification

  1. Check formulary status: Cigna formulary lookup
  2. Verify PA requirements: Download current Juxtapid PA form
  3. Confirm pharmacy network: Verify Accredo is in-network for your patient's plan
  4. Review medical policy: Check Cigna's lomitapide coverage criteria

Code Cross-Check Resources

  • ICD-10: CMS ICD-10 lookup
  • HCPCS: Cigna provider manual (verify current link with Cigna)
  • NDC: REMS program database or pharmacy verification
Pro Tip: Call Cigna provider services at the number on your provider card to verify current PA requirements before submission.

Quick Audit Checklist

Before Submitting PA Request

Diagnosis Coding:

  • E78.010 used (not E78.01 or E78.5)
  • Medical records explicitly state "HoFH" or "homozygous familial hypercholesterolemia"
  • Genetic testing or clinical criteria documented

Product Coding:

  • J8499 for medical benefit OR correct NDC for pharmacy benefit
  • Units = number of capsules (not total mg)
  • Strength matches prescribed dose

Documentation:

  • Step therapy failures documented with dates
  • Baseline liver function tests within 6 months
  • REMS enrollment confirmed or planned
  • Medical necessity letter addresses Cigna's specific criteria

Submission:

  • Using current PA form (check date)
  • All required attachments included
  • Expedited request marked if clinically urgent

Michigan Appeals Process

If Cigna denies your properly coded Juxtapid request, Michigan offers robust appeal rights under the Patient's Right to Independent Review Act.

Internal Appeals (First Step)

Timeline: 180 days from denial to file
Process: Submit to Cigna directly via member portal or written request
Decision: Standard 30 days, expedited 72 hours

External Review (Final Step)

Timeline: 127 days after final internal denial
Agency: Michigan Department of Insurance and Financial Services (DIFS)
Process: Online form or paper submission
Decision: 60 days standard, 72 hours expedited
Contact: DIFS at 877-999-6442

Michigan Advantage: The 127-day external review window is longer than the federal 120-day standard, giving you extra time to gather documentation.

When navigating complex prior authorization requirements for specialty medications like Juxtapid, having expert support can make the difference between approval and denial. Counterforce Health helps patients, clinicians, and specialty pharmacies turn insurance denials into targeted, evidence-backed appeals. The platform analyzes denial letters, identifies the specific basis for rejection, and drafts point-by-point rebuttals aligned to each plan's own coverage rules.


FAQ

Q: How long does Cigna prior authorization take for Juxtapid in Michigan?
A: Standard review is 5 business days. Expedited review (for urgent cases) is completed within 24-72 hours.

Q: What if Juxtapid is non-formulary on my Cigna plan?
A: Request a formulary exception using Cigna's exception process. You'll need to demonstrate that formulary alternatives are ineffective or contraindicated.

Q: Can I use retail pharmacy for Juxtapid?
A: No. FDA REMS requirements mandate dispensing through certified specialty pharmacies only. Cigna typically uses Accredo.

Q: Does step therapy apply if I tried PCSK9 inhibitors outside Michigan?
A: Yes, but you must provide documentation of the trial, including dates, doses, duration, and reason for discontinuation.

Q: What's the difference between E78.010 and the old E78.01 code?
A: E78.010 specifically identifies homozygous FH (effective 2025), while E78.01 was deleted. The new code provides the specificity Cigna requires for coverage.

Q: Can I request expedited appeal in Michigan?
A: Yes, if delay would seriously jeopardize your health. Requires physician attestation and results in 72-hour external review decision.


For additional support with Juxtapid coverage challenges, Counterforce Health specializes in converting insurance denials into successful appeals through evidence-based advocacy and payer-specific expertise.


Sources & Further Reading


Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage decisions vary by individual plan and clinical circumstances. Always consult with your healthcare provider and insurance plan for specific coverage requirements. For questions about Michigan insurance regulations, contact the Department of Insurance and Financial Services at 877-999-6442.

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