By Counterforce Health Team in Orfadin (nitisinone) —

Get Orfadin (Nitisinone) Covered by Cigna in Pennsylvania: Complete Prior Authorization Guide

Answer Box: Getting Orfadin Covered by Cigna in Pennsylvania

Yes, Cigna covers Orfadin (nitisinone) for hereditary tyrosinemia type 1 (HT-1) in Pennsylvania, but prior authorization is required. The fastest path to approval: (1) Ensure your metabolic specialist documents confirmed HT-1 diagnosis with elevated succinylacetone and/or FAH gene testing, (2) Submit PA through Cigna's provider portal with dietary management plan, and (3) If denied, use Pennsylvania's new external review process—about 50% of appeals are overturned. Start today: Contact your doctor to gather diagnostic labs and begin the PA process through Cigna's provider portal.

Table of Contents

Coverage Basics

Is Orfadin Covered by Cigna?

Cigna covers Orfadin (nitisinone) for patients with confirmed hereditary tyrosinemia type 1, but it requires prior authorization and must be dispensed through specialty pharmacy channels like Accredo. The medication is typically placed on specialty tiers with higher cost-sharing.

Which Cigna Plans Cover Orfadin?

Most Cigna commercial plans, Medicare Advantage, and employer-sponsored plans cover Orfadin when medically necessary. However, coverage varies by:

  • Formulary tier placement (usually specialty tier)
  • Step therapy requirements (some employer plans require trying Nityr first)
  • Quantity limits based on approved dosing
  • Site of care restrictions (specialty pharmacy only)
Note: Medicaid managed care plans through Cigna may have different criteria—verify with your specific plan.

Prior Authorization Process

Step-by-Step: Fastest Path to Approval

  1. Gather Diagnostic Documentation (Patient/Family)
    • Collect all HT-1 lab results (succinylacetone levels, tyrosine)
    • Obtain genetic testing results (FAH gene analysis)
    • Request newborn screening reports if available
    • Timeline: 1-2 days
  2. Schedule Specialist Consultation (Patient)
    • Must be prescribed by or in consultation with a metabolic disease specialist
    • Ensure dietary management plan is documented
    • Timeline: 1-2 weeks (depending on availability)
  3. Submit Prior Authorization (Provider)
    • Use Cigna's provider portal or designated PA form
    • Include all required clinical documentation
    • Timeline: Same day submission
  4. Cigna Review Process (Insurer)
    • Standard review: 5-7 business days
    • Expedited review: 24-72 hours if urgent
    • Timeline: Up to 14 calendar days maximum
  5. Approval and Specialty Pharmacy Setup (All parties)
    • Transfer prescription to Accredo or designated specialty pharmacy
    • Coordinate first shipment and ongoing delivery
    • Timeline: 2-3 business days after approval

Required Documentation Checklist

  • ✅ Confirmed HT-1 diagnosis with lab evidence
  • ✅ Genetic testing results (FAH gene mutations)
  • ✅ Metabolic specialist's clinical notes
  • ✅ Dietary management plan documentation
  • ✅ Previous treatment history (if applicable)
  • ✅ Current lab values and monitoring plan

Timeline and Urgency

Standard Processing Times

Review Type Cigna Timeline Pennsylvania Rights
Standard PA 5-7 business days 14 calendar days maximum
Expedited PA 24-72 hours 72 hours for urgent cases
Internal Appeal 30 calendar days Up to 60 days
External Review N/A (after internal) 45 days via PA Insurance Dept

When to Request Expedited Review

Request expedited processing if:

  • Patient is currently hospitalized
  • Delay would seriously jeopardize health
  • Current supply will run out before standard review completes
  • Patient is experiencing acute symptoms requiring immediate treatment

Medical Necessity Criteria

Cigna's Specific Requirements

Based on Cigna's coverage policy, Orfadin approval requires:

Requirement Details Documentation Needed
Confirmed HT-1 Diagnosis Biochemical or genetic evidence Elevated succinylacetone OR FAH gene mutations
Dietary Management Tyrosine/phenylalanine restriction Dietitian notes or nutrition plan
Specialist Prescribing Metabolic disease specialist involvement Specialist consultation notes
No Drug Duplication Cannot use with other nitisinone products Medication history review
Employer Plan Step Therapy May require Nityr trial first Prior therapy documentation if applicable

Common Denial Reasons and Solutions

Denial Reason How to Overturn Required Evidence
"Diagnosis not confirmed" Submit complete lab workup Succinylacetone levels, genetic testing
"Not prescribed by specialist" Get metabolic specialist involved Consultation notes, referral
"Missing dietary plan" Document nutrition management Dietitian assessment, meal plans
"Step therapy not completed" Show medical necessity for Orfadin Intolerance/failure documentation

Clinician Corner: Medical Necessity Letter

When submitting a medical necessity letter, include:

  • Patient demographics and confirmed HT-1 diagnosis
  • Clinical presentation and symptoms
  • Laboratory evidence (succinylacetone, tyrosine levels)
  • Genetic testing results if available
  • Dietary management plan and compliance
  • Treatment goals and expected outcomes
  • Monitoring plan for safety and efficacy
  • Citations to FDA labeling and clinical guidelines

Costs and Patient Assistance

Understanding Your Costs

Orfadin is typically placed on specialty tiers, meaning:

  • Commercial plans: 20-40% coinsurance after deductible
  • Medicare plans: Variable based on coverage gap
  • High-deductible plans: Full cost until deductible met

Manufacturer Support Programs

Orfadin4U Patient Support Program:

  • Eligibility: HT-1 patients with commercial insurance
  • Benefit: As little as $0 copay per month for eligible patients
  • Exclusions: Medicare Part D beneficiaries not eligible for copay assistance
  • How to apply: Contact through Orfadin support website

Additional Financial Resources

  • Foundation grants for uninsured/underinsured patients
  • State pharmaceutical assistance programs in Pennsylvania
  • Specialty pharmacy payment plans through Accredo
Tip: Apply for manufacturer assistance before filling your first prescription to maximize savings.

Denials and Appeals

Pennsylvania's Advantage: Strong External Review

Pennsylvania launched its Independent External Review Program in January 2024, giving patients powerful appeal rights. Key statistics: About 50% of appealed denials are overturned, with 259 successful appeals in the program's first year.

Appeals Process Overview

  1. Internal Appeal with Cigna (Required first step)
    • Timeline: 30 calendar days from denial
    • Process: Submit appeal through member portal or by mail
    • Required: Copy of denial letter, supporting clinical evidence
  2. Pennsylvania External Review (After internal appeal)
    • Timeline: 4 months from final internal denial to request
    • Process: Submit through PA Insurance Department portal
    • Decision: Independent review organization makes binding decision
    • Success rate: ~50% overturn rate statewide

Appeal Script for Patients

When calling Cigna customer service:

"I'm calling to appeal the denial of my Orfadin prescription. I have hereditary tyrosinemia type 1, which is a rare genetic disorder, and this is the only FDA-approved treatment. I have all the required documentation including genetic testing and specialist consultation. Can you help me start the internal appeal process and provide the forms I need?"

Renewals and Reauthorization

When to Reauthorize

  • Initial approval: Typically 6-12 months
  • Renewal requirements: Evidence of clinical benefit
  • Monitoring: Regular succinylacetone levels, liver function
  • Timeline: Submit renewal 30-45 days before expiration

Required Documentation for Renewal

  • Updated lab results showing treatment response
  • Specialist assessment of clinical improvement
  • Continued dietary management documentation
  • Any changes in dosing or administration

Specialty Pharmacy Requirements

Why Specialty Pharmacy is Required

Cigna requires Orfadin to be dispensed through specialty pharmacies like Accredo because:

  • Complex handling and storage requirements
  • Patient education and monitoring needs
  • Coordination with manufacturer support programs
  • Prior authorization management

Working with Accredo

What to expect:

  • Dedicated pharmacist and care team
  • Proactive outreach for refills and insurance issues
  • Coordination with manufacturer assistance programs
  • Temperature-controlled shipping and delivery tracking

Transfer process:

  • Accredo will coordinate with your current pharmacy
  • All PA documentation transfers automatically
  • No gap in therapy during transition
Note: Counterforce Health helps patients navigate specialty pharmacy transfers and resolve prior authorization issues more efficiently.

Troubleshooting Common Issues

Portal and Technical Problems

If Cigna's provider portal is down:

  • Submit PA via fax (verify current number with customer service)
  • Call provider services for alternative submission methods
  • Document submission date and method for appeal purposes

Missing Forms or Documentation

Common missing items:

  • Genetic testing results (contact original testing lab)
  • Specialist consultation notes (request from metabolic clinic)
  • Dietary assessment (coordinate with registered dietitian)
  • Prior therapy documentation (obtain from previous providers)

Communication Breakdowns

Between patient and clinic:

  • Designate one point person for PA coordination
  • Set up shared documentation system
  • Schedule regular check-ins during PA process

Between clinic and Cigna:

  • Use peer-to-peer review option for complex cases
  • Escalate to medical director if standard review insufficient
  • Document all communications with dates and names

FAQ

Q: How long does Cigna prior authorization take for Orfadin in Pennsylvania? A: Standard review takes 5-7 business days, with a maximum of 14 calendar days. Expedited review for urgent cases is completed within 24-72 hours.

Q: What if Orfadin is not on Cigna's formulary? A: You can request a formulary exception with medical necessity documentation. Pennsylvania's external review process can also address non-formulary denials.

Q: Can I request an expedited appeal in Pennsylvania? A: Yes, both Cigna internal appeals and Pennsylvania external reviews offer expedited processes for urgent health situations.

Q: Does step therapy apply if I've tried treatments outside Pennsylvania? A: Yes, previous treatment history from any location counts toward step therapy requirements. Ensure all documentation is transferred to your new provider.

Q: What happens if my appeal is denied? A: After exhausting Cigna's internal appeals, you can request Pennsylvania external review within 4 months. The state program has a 50% success rate for overturning denials.

Q: How much will Orfadin cost with Cigna coverage? A: Costs vary by plan, but specialty tier medications typically require 20-40% coinsurance. The Orfadin4U program can reduce costs to $0 for eligible commercial insurance patients.

Q: Can my pediatrician prescribe Orfadin? A: While pediatricians can write the prescription, Cigna requires involvement of a metabolic disease specialist for prior authorization approval.

Q: What if I'm switching from another nitisinone product? A: Document the medical reason for switching (e.g., formulation preference, dosing needs) and ensure no overlap in therapy to avoid duplication issues.

Counterforce Health specializes in turning insurance denials into successful appeals by creating targeted, evidence-backed submissions that align with each payer's specific requirements. Our platform helps patients, clinicians, and specialty pharmacies navigate complex prior authorization processes more effectively, reducing delays and improving approval rates for critical medications like Orfadin.

Sources and Further Reading

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 details. Coverage policies and requirements may change—verify current information with official sources before making treatment decisions.

For additional help navigating insurance appeals and prior authorizations, visit Counterforce Health for expert assistance with complex coverage challenges.

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