By Counterforce Health Team in Orfadin (nitisinone) —

Getting Orfadin (Nitisinone) Covered by Blue Cross Blue Shield in Georgia: Complete Coding, Prior Authorization, and Appeals Guide

Answer Box: Getting Orfadin Covered by Blue Cross Blue Shield in Georgia

Blue Cross Blue Shield Georgia requires prior authorization for Orfadin (nitisinone) with specific coding and documentation. Use ICD-10 code E70.2 for hereditary tyrosinemia type 1, bill with HCPCS J8499, and submit biochemical proof (elevated succinylacetone) plus genetic testing results. If denied, you have 60 days to file an external review with the Georgia Department of Insurance. Start by gathering your HT-1 diagnosis documentation and contacting your specialty pharmacy for PA submission.

Table of Contents

Coding Basics: Medical vs. Pharmacy Benefit

Orfadin (nitisinone) for hereditary tyrosinemia type 1 is almost universally covered under the specialty pharmacy benefit rather than the medical benefit. This means you'll work with specialty pharmacies like Accredo or Express Scripts, not your clinic's buy-and-bill process.

Key differences:

  • Pharmacy benefit: Requires prior authorization through pharmacy channels, uses NDC codes, dispensed by specialty pharmacy
  • Medical benefit: Would use J-codes for clinic administration (not applicable for oral Orfadin)

Blue Cross Blue Shield Georgia follows this standard approach, routing Orfadin through their specialty pharmacy network with prior authorization requirements.

ICD-10 Mapping and Documentation Requirements

Primary Diagnosis Code

Use ICD-10 code E70.2 (Disorders of tyrosine metabolism) for all Orfadin claims and prior authorization requests. This is the specific code for hereditary tyrosinemia type 1.

Required Documentation

Blue Cross Blue Shield Georgia requires biochemical or genetic confirmation of HT-1 diagnosis:

Biochemical Evidence (Gold Standard):

  • Elevated succinylacetone (SA) in blood or urine
  • Laboratory report from accredited clinical lab using tandem mass spectrometry
  • Must be documented prior to starting therapy

Genetic Evidence:

  • Pathogenic mutations in the FAH gene
  • Official genetic testing report
  • Often required for regulatory programs and some payers

Supporting Clinical Documentation:

  • Newborn screening results (if applicable)
  • Liver function tests showing abnormalities
  • Elevated alpha-fetoprotein levels
  • Evidence of dietary tyrosine/phenylalanine restriction plan
Tip: Include both biochemical and genetic evidence when available. Some Blue Cross Blue Shield plans require both for comprehensive coverage approval.

Product Coding: HCPCS, NDC, and Units

HCPCS J-Code

Use HCPCS J8499 ("Prescription drug, oral, non-chemotherapeutic, NOS") because there is no specific J-code for nitisinone. Include documentation specifying:

  • Drug name (Orfadin/nitisinone)
  • Exact dosage strength
  • Route of administration (oral)

National Drug Codes (NDCs)

Always use the specific NDC for the strength dispensed. Common Orfadin NDCs include:

  • 2 mg capsules: 68135-0201-30
  • 5 mg capsules: 68135-0202-30
  • 10 mg capsules: 68135-0203-30
  • 20 mg capsules: 68135-0204-30
  • Oral suspension: 68135-0401-01
Note: NDCs may change. Always confirm current codes with your dispensing specialty pharmacy.

Billing Units

Bill in milligrams (mg) dispensed, not capsule count. For example:

  • 30 capsules of 10 mg = 300 mg total
  • Bill as 300 units with J8499

Clean Request Anatomy

Example Prior Authorization Request

Patient Information:

  • Member ID: [Policy number]
  • DOB: [Date of birth]
  • Primary diagnosis: E70.2 (Hereditary tyrosinemia type 1)

Prescriber Information:

  • Metabolic specialist, endocrinologist, or geneticist
  • NPI number and credentials
  • Contact information for peer-to-peer review

Clinical Documentation:

  • Laboratory confirmation of elevated succinylacetone
  • FAH gene mutation analysis (if available)
  • Evidence of dietary restriction plan
  • Baseline ophthalmologic examination results
  • Treatment goals and monitoring plan

Medication Details:

  • Drug: Orfadin (nitisinone)
  • Strength and formulation requested
  • Dosing: Maximum 2 mg/kg/day
  • NDC code for specific product
  • Quantity and days supply

Common Coding Pitfalls

Unit Conversion Errors

Wrong: Billing capsule count instead of milligrams Right: Calculate total mg dispensed (capsules × strength)

Mismatched Diagnosis Codes

Wrong: Using P74.5 (transient tyrosinemia of newborn) Right: E70.2 (hereditary tyrosinemia type 1)

Missing Documentation

Wrong: Submitting PA without biochemical proof Right: Include succinylacetone lab results and genetic testing

Incorrect Benefit Path

Wrong: Trying to bill through medical benefit with clinic J-codes Right: Route through specialty pharmacy benefit with NDC codes

Blue Cross Blue Shield Verification Steps

Before Submitting Your Request

  1. Check formulary status on the Blue Cross Blue Shield Georgia drug list
  2. Verify prior authorization requirements for your specific plan
  3. Confirm specialty pharmacy network providers
  4. Review current PA criteria (may require metabolic specialist involvement)

Key Verification Points

  • Is Orfadin on your plan's specialty tier?
  • Are there quantity limits for your prescribed strength?
  • Does your plan require step therapy (unlikely for HT-1)?
  • What documentation format does your plan prefer?

Appeals Process in Georgia

If Blue Cross Blue Shield denies your Orfadin prior authorization, Georgia law provides a structured appeals process with specific deadlines.

Internal Appeal (First Step)

  • Deadline: 180 days from denial date
  • Timeline: Decision within 30 days (expedited: 72 hours)
  • Required: Medical necessity documentation, prescriber attestation

External Review (Final Step)

  • Deadline: 60 days from final internal denial
  • Authority: Georgia Office of Commissioner of Insurance
  • Cost: Free to consumer
  • Timeline: 30 business days (expedited: 72 hours)
  • Outcome: Binding on Blue Cross Blue Shield

How to File External Review

  1. Contact Georgia DOI Consumer Services: 1-800-656-2298
  2. Complete external review application
  3. Submit supporting documentation:
    • Final denial letter from Blue Cross Blue Shield
    • Clinical evidence of medical necessity
    • Provider notes supporting Orfadin need
    • Laboratory/genetic testing results
Critical: Missing the 60-day deadline eliminates your right to state external review in Georgia.

Counterforce Health helps patients, clinicians, and specialty pharmacies navigate these complex appeals by turning insurance denials into targeted, evidence-backed appeals. The platform analyzes denial reasons and drafts point-by-point rebuttals aligned to each plan's specific requirements, potentially saving weeks of back-and-forth with insurers.

Pre-Submission Audit Checklist

Documentation Ready?

  • HT-1 diagnosis confirmed with E70.2 code
  • Elevated succinylacetone lab results attached
  • FAH gene mutation report (if available)
  • Dietary restriction plan documented
  • Baseline ophthalmologic exam completed
  • Metabolic specialist involvement confirmed

Coding Accurate?

  • Correct NDC for prescribed strength
  • J8499 HCPCS code used
  • Units calculated in milligrams
  • ICD-10 E70.2 applied consistently

Administrative Complete?

  • Blue Cross Blue Shield PA form completed
  • Prescriber NPI and credentials included
  • Member ID and policy details verified
  • Submission method confirmed (fax/portal)

For complex cases, Counterforce Health can streamline this process by automatically generating compliant appeals with the right clinical evidence and procedural requirements for your specific Blue Cross Blue Shield plan.

FAQ

How long does Blue Cross Blue Shield PA take in Georgia? Standard prior authorization decisions are made within 72 hours. Expedited reviews for urgent medical needs are processed within 24 hours.

What if Orfadin is non-formulary on my plan? Submit a formulary exception request with clinical justification showing why covered alternatives are inappropriate or ineffective for your HT-1 treatment.

Can I request an expedited appeal? Yes, if your medical condition could seriously deteriorate without Orfadin. Expedited appeals must be decided within 72 hours in Georgia.

Does step therapy apply to Orfadin? Generally no, since Orfadin is the only FDA-approved treatment for HT-1. However, some plans may require trying generic nitisinone before brand Orfadin.

What happens if I miss the 60-day appeal deadline? You lose the right to Georgia's external review process. However, federal rules may provide up to 4 months for certain plan types—consult with the Georgia DOI.

Who can file appeals on my behalf? Your healthcare provider, a family member, or an authorized representative can file with your written consent. Some advocacy platforms also provide professional appeal services.

Are there cost assistance programs for Orfadin? Yes, manufacturer Sobi offers patient assistance programs. Check eligibility requirements and income limits on their official website.

What if Blue Cross Blue Shield approves but limits the quantity? File a quantity limit exception if the approved amount is insufficient for your prescribed dose. Include prescriber justification for the higher quantity needed.

Sources & Further Reading

Medical Disclaimer: This information is for educational purposes only and does not constitute medical advice. Always consult with your healthcare provider and insurance plan for specific coverage decisions. Appeal deadlines and procedures may vary by plan type and can change over time. Verify current requirements with official sources before taking action.

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