By Counterforce Health Team in Zokinvy (lonafarnib) —

The Requirements Checklist to Get Zokinvy (lonafarnib) Covered by Blue Cross Blue Shield in New York

Answer Box

Getting Zokinvy (lonafarnib) covered by Blue Cross Blue Shield in New York requires prior authorization with genetic confirmation of HGPS or processing-deficient progeroid laminopathies. Submit via Availity Essentials or your plan's provider portal with genetic test results, specialist prescription, and BSA calculation (≥0.39 m²). If denied, New York's external appeal process through the Department of Financial Services offers binding review within 4 months. First step today: Contact your BCBS plan to verify current PA requirements and download the application form.

Table of Contents

  1. Who Should Use This Guide
  2. Member & Plan Basics
  3. Clinical Criteria Checklist
  4. Coding & Billing Requirements
  5. Documentation Packet
  6. Submission Process
  7. Specialty Pharmacy Routing
  8. After Submission
  9. Denial Prevention Tips
  10. Appeals Process in New York
  11. Printable Checklist

Who Should Use This Guide

This checklist applies if you're seeking coverage for Zokinvy (lonafarnib) through any Blue Cross Blue Shield plan in New York State. You'll need this if:

  • Your child or patient has confirmed Hutchinson-Gilford Progeria Syndrome (HGPS) or processing-deficient progeroid laminopathies
  • You're at least 12 months old with body surface area ≥0.39 m²
  • Your doctor wants to prescribe this FDA-approved treatment
  • You've received a denial and need to appeal

Expected outcome: With proper documentation, most HGPS cases meeting FDA criteria receive approval. Appeal success rates are high in New York when genetic confirmation and specialist involvement are documented.

Member & Plan Basics

Active Coverage Requirements

✓ Current BCBS enrollment: Verify your member ID and plan type
✓ Plan includes prescription benefits: Check if specialty drugs are covered
✓ Deductible status: High-deductible plans may require meeting deductible first
✓ Prior authorization awareness: Zokinvy requires PA across all BCBS plans

Note: Blue Cross Blue Shield operates as 33 independent plans. While PA requirements are consistent, submission processes may vary slightly by your specific BCBS entity.

Plan Type Considerations

Plan Type Typical Requirements Contact Method
Commercial/Employer PA via Availity Essentials Provider portal or 1-800 number on ID card
Individual/ACA Same PA process Member services on ID card
Medicare Advantage May have additional step therapy Plan-specific process

Clinical Criteria Checklist

FDA-Approved Indications

Zokinvy is approved for patients ≥12 months old to:

  • Reduce mortality risk in Hutchinson-Gilford Progeria Syndrome (HGPS)
  • Treat processing-deficient progeroid laminopathies with specific genetic mutations

Required Clinical Criteria

Patient Eligibility:

  • Age ≥12 months
  • Body surface area (BSA) ≥0.39 m² (calculate using Mosteller formula: √(height[cm] × weight[kg]/3600))
  • Confirmed genetic diagnosis (see genetic requirements below)

Genetic Confirmation Required:

  • HGPS: Heterozygous LMNA c.1824C>T (G608G) mutation (~90% of cases)
  • Non-classic HGPS: LMNA exon/intron 11 variant with progerin accumulation (~10% of cases)
  • Processing-deficient progeroid laminopathy: Homozygous or compound heterozygous ZMPSTE24 mutations

Prescriber Requirements:

  • Prescribed by or in consultation with a specialist (genetics, metabolism, or progeria specialist)
  • Documentation of no contraindications to therapy
  • Monitoring plan established

Exclusion Criteria (must be absent):

  • Processing-proficient progeroid laminopathies
  • Other progeroid syndromes not specified in FDA labeling
  • Severe renal, hepatic, pulmonary, or immune dysfunction

Coding & Billing Requirements

ICD-10 Diagnosis Code

  • Q87.1: Congenital malformation syndromes predominantly associated with short stature

HCPCS J-Code

  • J8499: Prescription drug, oral, non-chemotherapeutic, not otherwise specified

NDC Numbers

Contact Sentynl Therapeutics at 1-888-251-2800 for current NDC numbers for 50 mg and 75 mg capsules.

Dosing for Documentation

  • Initial: 115 mg/m² PO twice daily with food
  • After 4 months: Increase to 150 mg/m² PO twice daily (maximum dose)
  • Document BSA calculation and weight-based dosing rationale

Documentation Packet

Provider Letter of Medical Necessity

Your specialist should include these elements:

Patient Information:

  • Full name, date of birth, member ID
  • Current height, weight, and BSA calculation
  • Confirmed diagnosis with genetic test results

Clinical Justification:

  • "Patient has confirmed [HGPS/processing-deficient progeroid laminopathy] via genetic testing"
  • "Meets all FDA criteria: age ≥12 months, BSA ≥0.39 m²"
  • "No contraindications present; monitoring plan established"
  • "FDA-approved therapy to reduce mortality risk"

Treatment Plan:

  • Starting dose and titration schedule
  • Monitoring plan (labs, ophthalmology exams)
  • Duration of therapy requested (typically 12 months initial)

Required Attachments

  • Genetic test report confirming HGPS or PDPL diagnosis
  • Specialist consultation note or prescription
  • Recent growth chart and vital signs
  • Baseline laboratory results (CBC, comprehensive metabolic panel, liver function tests)
  • Documentation ruling out contraindications
Tip: The Progeria Research Foundation offers free genetic testing. Contact them if you need confirmation of diagnosis.

Submission Process

BCBS New York Plans Submission Methods

Anthem Blue Cross Blue Shield NY:

  • Submit via Availity Essentials
  • Use Patient Registration > Authorization application for outpatient services
  • Include all required documentation in single submission

Other BCBS NY Plans:

  • Check your specific plan's provider portal
  • Contact provider services number on member ID card for submission instructions

Common Submission Errors to Avoid

  • Incomplete genetic documentation
  • Missing BSA calculation
  • Lack of specialist involvement documentation
  • Submitting without baseline labs
  • Using outdated PA forms

Counterforce Health can help streamline this process by analyzing your denial letter and plan policy to create targeted, evidence-backed appeals that address specific PA criteria and denial reasons. Learn more about our platform.

Specialty Pharmacy Routing

BCBS Preferred Specialty Pharmacy Networks

BCBS Plan Preferred Vendor Contact
Blue Shield of California Network Specialty Pharmacy Locator online
BCBS Michigan Curant Health (required) 1-866-460-8040
General BCBS Varies by plan Check member portal

Manufacturer Support

Sentynl Cares: 1-888-251-2800 (Mon-Fri 8AM-8PM ET)

  • Connects patients to authorized Zokinvy suppliers
  • Provides bridge therapy if PA is delayed
  • Offers financial assistance information

Transfer Process

  1. Verify current pharmacy's ability to dispense limited distribution drugs
  2. If transfer needed, have prescriber fax new prescription to preferred specialty pharmacy
  3. Call Sentynl Cares to facilitate transfer to authorized supplier
  4. Confirm PA approval before shipment

After Submission

Timeline Expectations

  • Standard PA review: 15 business days
  • Expedited review: 72 hours (if urgent medical need)
  • Confirmation: Request confirmation number upon submission

Status Monitoring

  • Record submission confirmation number and date
  • Check status via provider portal weekly
  • Follow up if no response within expected timeframe
  • Document all communications for potential appeals

What to Record

  • Submission date and method
  • Confirmation number
  • Review timeline provided
  • Status check dates and responses
  • Any additional information requests

Denial Prevention Tips

Five Common Pitfalls and Solutions

  1. Incomplete Genetic Documentation
    • Problem: Submitting without confirmed genetic test results
    • Solution: Ensure genetic report clearly states LMNA or ZMPSTE24 mutations
  2. Missing Specialist Involvement
    • Problem: Primary care prescription without specialist consultation
    • Solution: Obtain genetics, metabolism, or progeria specialist consultation
  3. Incorrect BSA Calculation
    • Problem: Patient appears too small based on age rather than BSA
    • Solution: Use Mosteller formula and document calculation clearly
  4. Insufficient Monitoring Plan
    • Problem: No documentation of safety monitoring protocols
    • Solution: Include detailed monitoring schedule in provider letter
  5. Wrong Indication Documentation
    • Problem: Unclear diagnosis or non-FDA approved indication
    • Solution: Clearly state "HGPS" or "processing-deficient progeroid laminopathy" with genetic confirmation

Appeals Process in New York

If your initial PA is denied, New York offers robust appeal rights through the Department of Financial Services.

Internal Appeal (First Step)

  • File with BCBS within 45 days of denial
  • Include additional documentation addressing denial reasons
  • Decision within 30 days (expedited: 72 hours for urgent cases)

External Appeal (Binding Review)

If internal appeal fails, you can request external review:

Timeline: File within 4 months of BCBS final adverse determination
Cost: Maximum $25 (waived for financial hardship or Medicaid)
Process: Submit External Appeal Application online, by email ([email protected]), fax (800-332-2729), or mail

Required Documents:

  • Completed External Appeal Application
  • Patient-signed consent for medical records release
  • Copy of denial notice
  • Supporting medical documentation

Decision Timeline:

  • Standard: 30 days
  • Expedited (non-formulary drugs): 24-72 hours
  • Decision is binding on BCBS

When to Request Expedited Review

  • Patient's health would be seriously jeopardized by delay
  • Current treatment is ineffective
  • Delay would subject patient to severe pain

Community Health Advocates provides free assistance with appeals in New York: 888-614-5400

Printable Checklist

Before You Start

  • Current BCBS member ID card
  • Genetic test results confirming HGPS or PDPL
  • Specialist consultation or prescription
  • Height and weight for BSA calculation
  • Baseline lab results

Documentation Requirements

  • Letter of medical necessity from specialist
  • Genetic confirmation report
  • BSA calculation (≥0.39 m²)
  • Age verification (≥12 months)
  • Monitoring plan documentation
  • Baseline safety labs

Submission

  • Submit via correct portal/method for your BCBS plan
  • Include all required attachments
  • Request confirmation number
  • Note submission date and expected review timeline

Follow-Up

  • Check status weekly
  • Respond promptly to information requests
  • Prepare appeal documentation if denied
  • Contact Sentynl Cares for specialty pharmacy coordination

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage decisions depend on your specific plan benefits and clinical circumstances. Always consult with your healthcare provider and insurance plan directly for personalized guidance.

For additional help with insurance appeals in New York, contact:

  • New York Department of Financial Services: dfs.ny.gov
  • Community Health Advocates: 888-614-5400
  • Counterforce Health: counterforcehealth.org - for automated appeal assistance

Sources & Further Reading

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