By Counterforce Health Team in Orladeyo (berotralstat) —

How to Get Orladeyo (Berotralstat) Covered by Cigna in Pennsylvania: Complete Appeals Guide

Answer Box: Getting Orladeyo (Berotralstat) Covered by Cigna in Pennsylvania

Fastest path to approval: Orladeyo requires prior authorization from Cigna with documented hereditary angioedema (HAE) diagnosis using ICD-10 code D84.1, lab confirmation of C1-INH deficiency, and evidence of recurrent attacks. If denied, Pennsylvania's Independent External Review program has a 50% success rate overturning specialty drug denials. First step today: Have your doctor submit a prior authorization request through Cigna's provider portal with complete HAE documentation and any prior treatment failures. If you receive a denial, you have 180 days for internal appeals and four months for Pennsylvania's external review after exhausting internal options.

Table of Contents

  1. Coverage Requirements at a Glance
  2. ICD-10 Coding and Documentation Essentials
  3. Product Coding: NDC Numbers and Billing Units
  4. Step-by-Step: Fastest Path to Approval
  5. Common Denial Reasons and How to Fix Them
  6. Appeals Playbook for Cigna in Pennsylvania
  7. Costs and Patient Assistance Options
  8. When to Escalate to Pennsylvania Regulators

Coverage Requirements at a Glance

Requirement What It Means Where to Find It Status
Prior Authorization Required for all Orladeyo prescriptions Cigna Provider Portal Always Required
ICD-10 Code D84.1 (Hereditary angioedema) Medical records, lab reports Essential
Age Requirement 12 years or older FDA labeling Must document
Step Therapy May require trial of preferred HAE therapy first Plan-specific formulary Plan dependent
Quantity Limit Typically 30 capsules per 30 days Express Scripts criteria Standard limit
Diagnosis Confirmation Lab-confirmed C1-INH deficiency C1-INH antigen/function tests Required

ICD-10 Coding and Documentation Essentials

Primary diagnosis code: D84.1 ("Defects in the complement system") is the correct ICD-10 code for hereditary angioedema due to C1 esterase inhibitor deficiency. This code covers both HAE Type I and Type II and is valid through September 2025.

Critical distinction: D84.1 is specifically for hereditary angioedema, not allergic angioedema (T78.3). Documentation must clearly state "hereditary angioedema" to support this coding.

Required documentation elements:

  • Lab confirmation: Low C1-INH antigen levels (Type I) or low C1-INH functional activity (Type II)
  • Supporting labs: Low C4 levels during attacks or baseline
  • Clinical history: Recurrent episodes of non-pitting edema affecting face, extremities, genitals, or larynx
  • Family history: When available, document familial pattern
  • Attack frequency: Document baseline attack rate and severity

Documentation language that supports approval:

  • "Patient has confirmed hereditary angioedema Type [I/II] with laboratory evidence of C1-INH deficiency"
  • "Recurrent angioedema attacks despite conventional therapy with antihistamines and corticosteroids"
  • "Attacks involve [specific sites] with frequency of [X attacks per month/year]"
  • "Quality of life significantly impacted by unpredictable attack pattern"

Product Coding: NDC Numbers and Billing Units

Orladeyo is an oral medication billed through the pharmacy benefit (not medical benefit) using NDC codes:

NDC Numbers:

  • 150 mg capsules: NDC 72769-101-01
  • 110 mg capsules: NDC 72769-102-01

Billing units: Always bill as "each capsule" - not total milligrams. For a 30-day supply at standard dosing (one 150mg capsule daily), submit as 30 units.

Common billing errors to avoid:

  • Submitting total mg instead of capsule count
  • Billing under medical benefit instead of pharmacy benefit
  • Exceeding quantity limits (typically 30 capsules per 30 days)
  • Missing prior authorization before submission
Note: Orladeyo does not have a HCPCS J-code because it's an oral medication. All claims should use NDC codes through pharmacy benefits.

Step-by-Step: Fastest Path to Approval

1. Gather Essential Documentation (Patient/Clinic)

Timeline: 1-2 days

  • Insurance card and member ID
  • Complete medical records showing HAE diagnosis
  • Lab results (C1-INH levels, C4 levels)
  • Prior treatment history and outcomes
  • Current attack frequency documentation

2. Submit Prior Authorization Request (Prescriber)

Timeline: Same day

  • Complete Cigna's prior authorization form through their provider portal
  • Include ICD-10 code D84.1 on all documentation
  • Attach lab results and clinical notes
  • Submit via: Cigna Provider Portal or Express Scripts authorization system

3. Cigna Review Process (Insurer)

Timeline: 15 business days (standard) or 72 hours (expedited)

  • Medical necessity review by pharmacy benefit managers
  • Clinical criteria assessment against HAE guidelines
  • Step therapy evaluation if applicable

4. Receive Determination (Patient/Prescriber)

Timeline: Within review period

  • Approval: Prescription can be filled immediately
  • Denial: Review denial letter for specific reasons and appeal rights

5. If Denied: Internal Appeal (Patient/Prescriber)

Timeline: Must file within 180 days of denial

  • Submit additional clinical documentation
  • Request peer-to-peer review if available
  • Include updated attack logs or treatment failures

6. External Review Option (Patient)

Timeline: Must file within 4 months of final internal denial

Common Denial Reasons and How to Fix Them

Denial Reason How to Overturn Required Documentation
Insufficient HAE diagnosis Provide complete lab workup C1-INH antigen/function, C4 levels, genetic testing if available
Age requirement not met Confirm patient ≥12 years Birth certificate or medical record verification
Step therapy required Document failure/intolerance of preferred therapy Treatment logs, adverse reaction notes, efficacy data
Not medically necessary Strengthen clinical rationale Attack frequency logs, quality of life impact, specialist consultation
Quantity limit exceeded Justify higher dose/frequency Clinical notes supporting dose adjustment, weight-based calculations
Use for acute treatment Clarify prophylactic indication Treatment plan showing preventive use, not acute attack management
From our advocates: "We've seen the strongest appeals combine attack diaries with before-and-after quality of life measures. Patients who document specific functional limitations—missed work days, emergency room visits, activity restrictions—tend to have more successful appeals than those relying solely on clinical lab values."

Appeals Playbook for Cigna in Pennsylvania

Internal Appeals Process

First Level Internal Appeal

  • Deadline: 180 days from denial notice
  • Timeline: 15 business days for standard, 72 hours for expedited
  • How to submit: Cigna member portal, phone, or written request
  • Required: Denial letter, additional medical records, prescriber statement

Second Level Internal Appeal

  • Automatic: If first appeal is denied
  • Timeline: Additional 15 business days
  • Review: Independent medical reviewer not involved in original decision

Pennsylvania External Review

Pennsylvania launched its Independent External Review Program in January 2024, with impressive results for patients:

Key statistics:

  • 50% of external reviews overturned insurer denials in 2024
  • 259 out of 517 cases ruled in favor of patients
  • Average timeline: 45 days for standard review, 72 hours for expedited

Eligibility requirements:

  • Must complete internal appeals first
  • Receive Final Adverse Benefit Determination letter
  • Submit within 4 months of final denial
  • Plan must not be self-insured (verify with HR department)

How to submit:

  • Online: PA Insurance Department External Review Portal
  • By mail: Pennsylvania Insurance Department, External Review Program
  • Additional information: You can submit supporting documents within 15 days of assignment to independent review organization
Tip: Pennsylvania's external review program has specific expertise in rare disease cases. Include published treatment guidelines and peer-reviewed studies supporting Orladeyo's use in HAE prophylaxis.

Costs and Patient Assistance Options

Wholesale Acquisition Cost: Approximately $44,484 per 28-day supply (January 2025), though patient out-of-pocket costs vary significantly based on insurance coverage and assistance programs.

Patient assistance programs:

  • BioCryst Patient Access Program: Copay assistance for eligible commercially insured patients
  • Foundation support: Patient Access Network Foundation and other rare disease foundations
  • State programs: Pennsylvania's Medicaid program covers Orladeyo with prior authorization

Counterforce Health helps patients navigate complex prior authorization requirements and turn insurance denials into successful appeals. Their platform analyzes denial letters and creates targeted, evidence-backed appeals that align with payer-specific requirements, significantly improving approval rates for specialty medications like Orladeyo.

When to Escalate to Pennsylvania Regulators

Contact the Pennsylvania Insurance Department if:

  • Cigna fails to respond within required timelines
  • You suspect improper denial practices
  • Appeals process isn't followed correctly
  • You need assistance with external review process

Pennsylvania Insurance Department Contact:

  • Phone: Consumer Services hotline (verify current number on pa.gov)
  • Online: File a complaint through PA.gov
  • Consumer Assistance: Pennsylvania Health Law Project provides free help with appeals

Frequently Asked Questions

Q: How long does Cigna prior authorization take for Orladeyo in Pennsylvania? A: Standard reviews take up to 15 business days. Expedited reviews (for urgent cases) are completed within 72 hours.

Q: What if Orladeyo is not on Cigna's formulary? A: You can request a formulary exception with clinical documentation showing medical necessity and lack of suitable alternatives on formulary.

Q: Can I get an expedited appeal if I'm having frequent HAE attacks? A: Yes, if your health is at immediate risk, both Cigna internal appeals and Pennsylvania external reviews offer expedited timelines.

Q: Does step therapy apply if I've tried other HAE medications outside Pennsylvania? A: Yes, prior treatment history from any location counts toward step therapy requirements if properly documented.

Q: What happens if Pennsylvania's external review rules in my favor? A: Cigna must provide coverage immediately, including retroactive coverage if you paid out-of-pocket during the appeal.

Q: Are there income limits for BioCryst's patient assistance program? A: Income limits vary by program. Contact BioCryst directly or visit their website for current eligibility criteria.

Checklist: What to Gather Before You Start

For patients:

  • Current Cigna insurance card and member ID
  • Complete list of HAE symptoms and attack frequency
  • Records of previous treatments tried and outcomes
  • Emergency room visits or hospitalizations related to HAE
  • Impact on work, school, or daily activities

For prescribers:

  • Lab results: C1-INH antigen and functional levels
  • C4 levels (baseline and during attacks if available)
  • Genetic testing results (if performed)
  • Documentation of HAE attack pattern and severity
  • Prior treatment failures or contraindications
  • Current clinical assessment and treatment goals

Sources and Further Reading

Medical Disclaimer: This information is for educational purposes only and does not constitute medical advice. Always consult with qualified healthcare professionals and verify current insurance policies and procedures. Coverage criteria and appeal processes may change. For personalized assistance with complex prior authorization and appeal processes, Counterforce Health provides specialized support for patients and providers navigating insurance challenges for specialty medications.

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