Coding That Helps Get Zokinvy (Lonafarnib) Approved in Illinois with Aetna (CVS Health): ICD-10, HCPCS/J-Code, NDC
Answer Box
Zokinvy (lonafarnib) requires prior authorization from Aetna (CVS Health) using ICD-10 code E34.8 (progeria), HCPCS J8499, and specific NDCs (60923-0544-53, 73079-0050-30). Submit genetic confirmation, BSA calculations, and specialist oversight through CVS Specialty Pharmacy. Illinois residents have 4 months to file external appeals through the Illinois Department of Insurance if denied. First step: Contact your geneticist to gather LMNA mutation testing and BSA documentation before submitting the PA request.
Table of Contents
- Coding Basics: Medical vs. Pharmacy Benefit Paths
- ICD-10 Mapping for HGPS and Progeroid Laminopathies
- Product Coding: HCPCS, NDC, and Unit Calculations
- Clean Request Anatomy: Sample PA Submission
- Frequent Coding Pitfalls
- Verification with Aetna (CVS Health) Resources
- Quick Audit Checklist
- Appeals Process in Illinois
- FAQ
Coding Basics: Medical vs. Pharmacy Benefit Paths
Zokinvy (lonafarnib) typically falls under specialty pharmacy benefits with Aetna (CVS Health), not medical benefits. This distinction affects coding requirements and submission pathways.
Coverage Path Overview
| Benefit Type | When Used | Coding Requirements | Submission Method |
|---|---|---|---|
| Specialty Pharmacy | Standard dispensing for HGPS/progeroid laminopathies | HCPCS J8499 + ICD-10 E34.8 + NDC | CVS Specialty Pharmacy |
| Medical Benefit | Buy-and-bill scenarios (rare) | Same codes + place of service | Provider portal/claims |
Key Point: Aetna requires CVS Specialty Pharmacy dispensing for Zokinvy, making pharmacy benefit the primary pathway. Medical benefit coding applies only in specific buy-and-bill situations.
ICD-10 Mapping for HGPS and Progeroid Laminopathies
Primary Diagnosis Code
E34.8 (Other specified endocrine disorders) is the correct ICD-10 code for Hutchinson-Gilford Progeria Syndrome. This code explicitly includes "Progeria" and "Hutchinson-Gilford syndrome" as inclusion terms.
Documentation Words That Support E34.8 Coding
When documenting for prior authorization, include these specific terms:
- Genetic confirmation: "LMNA gene mutation confirmed by CLIA-certified laboratory"
- Clinical presentation: "Failure to thrive, alopecia, scleroderma-like skin changes, lipodystrophy"
- Cardiovascular findings: "Premature atherosclerosis, cardiac stress test abnormalities"
- Skeletal manifestations: "Osteopenia, joint contractures, growth retardation"
Tip: Include the exact genetic variant (e.g., "c.1824C>T, p.Gly608Gly") in clinical notes to strengthen medical necessity documentation.
Product Coding: HCPCS, NDC, and Unit Calculations
HCPCS/J-Code Requirements
HCPCS J8499 (Prescription drug, oral, non-chemotherapeutic, Not Otherwise Specified) is the standard billing code for Zokinvy. No dedicated J-code exists as of 2026 HCPCS updates.
NDC Codes and Strengths
| Strength | NDC | Package Size | Typical Use |
|---|---|---|---|
| 50 mg | 60923-0544-53 | 30 capsules | Initial dosing, smaller patients |
| 75 mg | 60923-0545-54 | 30 capsules | Mid-range dosing |
| 50 mg | 73079-0050-30 | 30 capsules | Alternative supplier |
| 75 mg | 73079-0075-30 | 30 capsules | Alternative supplier |
Units and Dosing Math
Zokinvy dosing is based on body surface area (BSA). Here's the FDA-approved dosing calculation:
BSA-Based Dosing Table:
- BSA 0.39-0.49 m²: 50 mg twice daily
- BSA 0.50-0.59 m²: 62.5 mg twice daily
- BSA 0.60-0.69 m²: 75 mg twice daily
- BSA ≥0.70 m²: 87.5 mg twice daily
Units Calculation Example: For a patient with BSA 0.55 m² requiring 62.5 mg BID:
- Daily dose: 125 mg (62.5 mg × 2)
- 30-day supply: 3,750 mg total
- Using 50 mg capsules: 75 capsules per month
- Using 75 mg capsules: 50 capsules per month
Clean Request Anatomy: Sample PA Submission
Complete PA Request Example
Patient: [Name], DOB [Date], Member ID [Number]
Diagnosis: E34.8 - Hutchinson-Gilford Progeria Syndrome
Drug: Zokinvy (lonafarnib) 50mg capsules
NDC: 60923-0544-53
HCPCS: J8499
Quantity: 60 capsules per 30 days
Days Supply: 30
Clinical Justification:
1. GENETIC CONFIRMATION: LMNA c.1824C>T mutation confirmed by [Lab Name] on [Date]
2. BSA CALCULATION: Current BSA 0.45 m² requires 50mg BID per FDA labeling
3. SPECIALIST OVERSIGHT: Managed by Dr. [Name], pediatric geneticist at [Institution]
4. NO CONTRAINDICATIONS: No concurrent CYP3A inhibitors or prohibited medications
Supporting Documents:
- Genetic testing report
- Growth charts with BSA calculation
- Specialist consultation notes
- FDA prescribing information
Required Attachments Checklist
- CLIA-certified genetic testing results
- Current height/weight measurements
- BSA calculation documentation
- Specialist credentials/board certification
- Clinical notes documenting HGPS diagnosis
- Prior therapy documentation (if applicable)
Frequent Coding Pitfalls
Common Submission Errors
Unit Conversion Mistakes:
- ❌ Submitting monthly quantity without BSA justification
- ✅ Include BSA calculation showing FDA-aligned dosing
Mismatched Codes:
- ❌ Using experimental drug codes or wrong NDCs
- ✅ Verify current NDCs with manufacturer before submission
Missing Start Dates:
- ❌ Leaving therapy start date blank
- ✅ Include planned start date and rationale for timing
Incomplete Genetic Documentation:
- ❌ Submitting clinical suspicion without confirmed testing
- ✅ Include complete CLIA lab report with specific mutation
Documentation Red Flags to Avoid
- Vague language like "possible progeria" instead of confirmed diagnosis
- Missing BSA calculations or using non-FDA dosing
- Lack of specialist involvement or credentials
- Incomplete prior authorization forms
Verification with Aetna (CVS Health) Resources
Cross-Check Your Codes
Before submission, verify coding accuracy using these Aetna resources:
- Aetna Precertification Lists - Confirm PA requirements
- CVS Specialty Pharmacy - Verify dispensing requirements
- Aetna Provider Portal - Submit PA electronically
Contact Information for Verification
| Purpose | Phone | Fax |
|---|---|---|
| Specialty PA Support | 1-855-240-0535 | 1-888-267-3277 |
| Non-Specialty PA | 1-855-240-0535 | 1-877-269-9916 |
| Urgent/Expedited | Mark "Urgent" on form | Same as above |
Note: Call during business hours (M-F 8am-6pm CT) for fastest response on complex rare disease cases.
Quick Audit Checklist
Pre-Submission Review
Patient Information:
- Correct member ID and demographic details
- Current contact information for patient/guardian
Clinical Documentation:
- ICD-10 E34.8 with supporting clinical notes
- LMNA genetic testing results attached
- BSA calculation with current measurements
- Specialist credentials and consultation notes
Coding Accuracy:
- HCPCS J8499 selected
- Correct NDC for prescribed strength
- Quantity matches BSA-based dosing
- 30-day supply specified
Submission Details:
- All required fields completed
- Prescriber signature and date
- Urgent designation if applicable
- Correct fax number or portal submission
Appeals Process in Illinois
If Aetna denies your Zokinvy prior authorization, Illinois provides strong appeal rights through the Health Carrier External Review Act.
Appeal Timeline and Process
Internal Appeal (Required First Step):
- File within 180 days of denial
- Aetna has 30 days for standard review, 72 hours for expedited
- Submit via member portal or written request
External Review (After Internal Denial):
- File within 4 months of Aetna's final adverse determination
- Illinois Department of Insurance assigns independent reviewer
- Decision within 45 days (standard) or 72 hours (expedited)
- IRO decision is binding on Aetna
Required Documentation for Appeals
- Original denial letter from Aetna
- Internal appeal denial (final adverse determination)
- Medical records authorization form
- Provider certification for urgent cases
- Clinical evidence supporting medical necessity
Illinois-Specific Advantage: The state's external review process is faster than many states, with IRO decisions required within 5 business days of receiving complete documentation.
At Counterforce Health, we help patients and clinicians turn insurance denials into successful appeals by identifying the specific denial basis and crafting targeted, evidence-backed rebuttals that align with each payer's own coverage rules.
FAQ
How long does Aetna (CVS Health) PA take for Zokinvy in Illinois? Standard prior authorization decisions typically take 15 business days. Expedited reviews for urgent cases are completed within 72 hours when properly documented.
What if Zokinvy is non-formulary on my Aetna plan? Request a formulary exception with your PA submission. Include documentation that formulary alternatives are ineffective or contraindicated for HGPS treatment.
Can I request an expedited appeal if denied? Yes, if the delay would jeopardize your health. Mark "urgent" on appeal forms and include provider certification of medical urgency.
Does step therapy apply to Zokinvy? Generally no, as Zokinvy is the only FDA-approved treatment for HGPS. However, document any prior supportive care attempts in your submission.
What happens if I miss the 4-month appeal deadline in Illinois? The external review window closes, but you may still file complaints with the Illinois Department of Insurance for procedural violations or request a new PA with additional documentation.
How much does Zokinvy cost without insurance? Cash prices approach $25,600 for a 30-day supply of 50mg capsules, with monthly costs potentially reaching $90,000 for higher doses.
Sources & Further Reading
- Aetna Precertification Requirements
- Illinois Department of Insurance External Review Process
- ICD-10 Code E34.8 Details
- CVS Specialty Pharmacy Prior Authorization
- Zokinvy FDA Prescribing Information
- Illinois Health Carrier External Review Act
Disclaimer: This guide provides general information about insurance coding and appeals processes. It is not medical advice. Always consult with your healthcare provider and insurance plan for specific coverage determinations. For personalized assistance with appeals, consider using tools like Counterforce Health that specialize in turning denials into targeted, evidence-backed appeals.
Illinois residents can contact the Illinois Department of Insurance Consumer Division at 877-527-9431 for additional support with insurance appeals and external reviews.
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