By Counterforce Health Team in Orfadin (nitisinone) —

Getting Orfadin (Nitisinone) Covered by Cigna in New York: Coding, Appeals & Prior Authorization Guide

Answer Box: Getting Orfadin (Nitisinone) Covered by Cigna in New York

Orfadin (nitisinone) for hereditary tyrosinemia type 1 requires Cigna prior authorization using ICD-10 code E70.29 and HCPCS J8499. Key steps: 1) Confirm HT-1 diagnosis with elevated succinylacetone levels, 2) Submit PA with specialist documentation and dietary management plan, 3) If denied, file internal appeal within 180 days, then external appeal through NY Department of Financial Services within 4 months. Standard PA decisions take 72 hours; expedited reviews 24 hours.

Table of Contents

Coding Basics: Medical vs. Pharmacy Benefit

Orfadin (nitisinone) is typically covered under Cigna's specialty pharmacy benefit, managed through Express Scripts or Accredo. This means it follows pharmacy benefit rules rather than medical benefit procedures, which affects both coding and prior authorization requirements.

For specialty drugs like Orfadin, Cigna requires:

  • Prior authorization before dispensing
  • Specialty pharmacy distribution (not retail pharmacy)
  • Quantity limits based on FDA-approved dosing
  • Periodic reauthorization (typically every 12 months)

Understanding this pathway is crucial because it determines which forms to use, where to submit documentation, and what coding requirements apply.

ICD-10 Mapping for Hereditary Tyrosinemia Type 1

The primary diagnosis code for hereditary tyrosinemia type 1 is E70.29 - "Other disorders of tyrosine metabolism". This code must appear on all prior authorization requests and claims for Orfadin.

Documentation Requirements for E70.29

Your medical records must support this diagnosis with:

Laboratory Evidence:

  • Elevated succinylacetone in blood or urine (key diagnostic marker)
  • Elevated tyrosine levels
  • Abnormal amino acid metabolite patterns
  • Newborn screening results (if available)

Genetic Testing:

  • FAH gene mutation analysis showing pathogenic variants
  • Molecular testing confirming hereditary tyrosinemia type 1

Clinical Presentation:

  • Failure to thrive in infancy
  • Hepatomegaly or liver dysfunction
  • Jaundice or coagulopathy
  • Renal tubular dysfunction
Note: Avoid confusion with transitory tyrosinemia of newborn (P74.5), which is a different condition that doesn't require Orfadin therapy.

Product Coding: HCPCS, NDC, and Billing Units

HCPCS J-Code

Orfadin uses J8499 ("Prescription drug, oral, non-chemotherapeutic, NOS") since there's no specific J-code for nitisinone. This catch-all code requires additional documentation to specify the exact drug and strength.

NDC Numbers

Always use the specific NDC for the dispensed product:

Strength NDC Example Package Size
2 mg capsules 68135-0201-30 30 count
5 mg capsules 68135-0202-30 30 count
10 mg capsules 68135-0203-30 30 count
20 mg capsules 68135-0204-30 30 count
Oral suspension 68135-0401-01 60 mL bottle
Tip: Always verify the current NDC with your pharmacy, as these numbers can change with product revisions.

Billing Units and Dosing

Cigna bills Orfadin in milligrams (mg) rather than by capsule count. Calculate the total mg dispensed for accurate billing:

  • 30 capsules × 10 mg = 300 mg billed
  • Maximum dose: 2 mg/kg/day
  • For a 20 kg patient: maximum 40 mg/day allowed

Clean Prior Authorization Anatomy

A successful Orfadin prior authorization includes these essential elements:

Required Clinical Information

  1. Patient Demographics
    • Full name, DOB, Cigna member ID
    • Prescriber NPI and contact information
  2. Diagnosis Documentation
    • ICD-10 code E70.29
    • Laboratory results showing elevated succinylacetone
    • Genetic testing results (FAH gene mutations)
  3. Treatment Plan
    • Specific Orfadin dosing (mg/kg twice daily)
    • Dietary management plan (tyrosine/phenylalanine restriction)
    • Monitoring schedule (succinylacetone levels, liver function)
  4. Medical Necessity Justification
    • Why Orfadin is required for this patient
    • Expected clinical outcomes
    • Specialist consultation notes

Sample PA Request Format

Patient: [Name], DOB: [Date], Member ID: [Number]
Diagnosis: Hereditary tyrosinemia type 1 (E70.29)
Medication: Orfadin (nitisinone) [strength] mg capsules
Dosing: [X] mg twice daily ([X] mg/kg/day)
Duration: 12 months
Clinical Justification: Confirmed HT-1 with elevated succinylacetone [value] and FAH gene mutation. Patient on appropriate dietary restrictions. Orfadin required to prevent hepatic complications and improve long-term outcomes.

Common Coding Pitfalls to Avoid

Unit Conversion Errors

  • Wrong: Billing by capsule count
  • Right: Billing by total mg content
  • Example: 30 × 10mg capsules = 300 mg (not 30 units)

Mismatched Diagnosis Codes

  • Wrong: Using P74.5 (transitory tyrosinemia of newborn)
  • Right: Using E70.29 (hereditary tyrosinemia type 1)
  • Impact: Wrong code leads to automatic denial

Missing Documentation

  • Common error: Submitting PA without lab values
  • Required: Succinylacetone levels, genetic testing, specialist notes
  • Result: Delays approval by 1-2 weeks while gathering documents

Incorrect NDC Usage

  • Problem: Using expired or incorrect NDC numbers
  • Solution: Verify current NDC with pharmacy before submission
  • Check: Cross-reference with FDA Orange Book or manufacturer

Cigna Verification and Resources

Before submitting your Orfadin request, verify current requirements through these Cigna resources:

Online Verification Tools

  • Cigna Provider Portal: Check formulary status and PA requirements
  • Express Scripts: Verify specialty pharmacy requirements
  • Member Services: 1-800-Cigna24 for specific plan benefits

Pre-Submission Checklist

  • Confirmed E70.29 diagnosis with supporting labs
  • Obtained specialist consultation or referral
  • Documented dietary management plan
  • Verified current NDC and billing units
  • Completed all required PA forms
  • Attached clinical notes and test results
From our advocates: "We've found that including a one-page summary with key lab values and genetic testing results upfront can reduce back-and-forth requests for additional information. Cigna reviewers appreciate having the essential clinical data clearly presented rather than buried in lengthy chart notes."

Appeals Process in New York

When Cigna denies Orfadin coverage, New York patients have robust appeal rights through a two-tier system.

Internal Appeals with Cigna

Timeline: File within 180 days of denial Process:

  1. Submit written appeal with additional clinical documentation
  2. Request peer-to-peer review with Cigna medical director
  3. Include updated lab values or specialist letters
  4. Cigna responds within 30 days (expedited: 72 hours)

External Appeals through NY DFS

If Cigna upholds the denial, you can appeal to the New York State Department of Financial Services:

Eligibility: Denials for "not medically necessary" or "experimental/investigational" Timeline: Within 4 months of final Cigna denial Fee: $25 (waived for Medicaid or financial hardship) Process:

  1. Complete External Appeal Application
  2. Submit via fax: (800) 332-2729
  3. Include all medical records and Cigna denial letters
  4. Independent reviewer makes binding decision within 30 days

Expedited Appeals: Available for urgent cases with 72-hour decisions

Note: Medicare and fee-for-service Medicaid patients cannot use NY DFS external appeals but have federal appeal rights.

FAQ: Orfadin Coverage with Cigna

Q: How long does Cigna prior authorization take for Orfadin? A: Standard requests: 72 hours after receiving complete documentation. Expedited requests (for urgent cases): 24 hours.

Q: What if Orfadin isn't on Cigna's formulary? A: Request a formulary exception by demonstrating medical necessity and providing clinical justification for why formulary alternatives aren't appropriate.

Q: Can I get expedited review if my child needs Orfadin urgently? A: Yes. If delay could seriously jeopardize health, request expedited PA review (24-hour decision) or expedited appeal (72-hour decision).

Q: Does Cigna require step therapy for Orfadin? A: Generally no, since Orfadin is the primary treatment for HT-1. However, they may require documentation that dietary management alone was insufficient.

Q: What happens if I move to New York from another state while on Orfadin? A: Cigna typically honors existing authorizations, but you may need to transfer to a New York specialty pharmacy and update your provider network.

Q: How often do I need reauthorization? A: Typically every 12 months, with documentation of ongoing clinical response (stable or improved succinylacetone levels) and continued dietary compliance.

Getting specialized help with insurance appeals can make the difference between approval and denial. Counterforce Health helps patients, clinicians, and specialty pharmacies turn insurance denials into targeted, evidence-backed appeals. Their platform analyzes denial letters and plan policies to draft point-by-point rebuttals aligned with each payer's specific requirements, pulling the right clinical evidence and regulatory citations to support medical necessity arguments.

For complex cases like Orfadin appeals, having expert support can significantly improve approval rates. Counterforce Health's system understands payer-specific workflows and produces letters that meet procedural requirements while tracking deadlines and required documentation.

Sources & Further Reading

Disclaimer: This information is for educational purposes and doesn't constitute medical or legal advice. Coverage decisions depend on individual plan benefits and clinical circumstances. Always consult your healthcare provider for medical guidance and verify current policy details with Cigna directly.

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