By Counterforce Health Team in Haegarda (C1-esterase inhibitor, SC) —

Coding That Helps Get Haegarda Covered by UnitedHealthcare in Texas: ICD-10, J-Code, and NDC Requirements

Answer Box: Getting Haegarda Covered by UnitedHealthcare in Texas

Fastest Path: Submit prior authorization with ICD-10 code D84.1 (hereditary angioedema), specialist documentation, and lab evidence of C1-INH deficiency. Use J-code J0599 for billing (10 units per code). UnitedHealthcare requires specialist prescriber and confirmed HAE diagnosis—no absolute step therapy mandate as of 2025. First step today: Contact your allergist/immunologist to gather C4 and C1-INH lab results and request a medical necessity letter citing attack frequency and prophylaxis need.

Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit
  2. ICD-10 Mapping for HAE
  3. Product Coding: HCPCS, NDC, and Units
  4. Clean Request Anatomy
  5. Frequent Coding Pitfalls
  6. Verification with UnitedHealthcare
  7. Quick Audit Checklist
  8. Common Denial Reasons & Fixes
  9. Appeals Process in Texas

Coding Basics: Medical vs. Pharmacy Benefit

Haegarda (C1-esterase inhibitor, subcutaneous) typically falls under the medical benefit rather than pharmacy benefit due to its specialty nature and subcutaneous administration route. This means claims process through UnitedHealthcare's medical division, not OptumRx pharmacy.

Key Distinctions:

  • Medical benefit: Uses HCPCS J-codes, requires medical necessity documentation
  • Pharmacy benefit: Uses NDC codes primarily, processed through OptumRx
  • Prior authorization: Required regardless of benefit type through UnitedHealthcare's specialty drug program
Note: Some self-administered specialty drugs may be covered under pharmacy benefit. Always verify with UnitedHealthcare which benefit applies to your specific plan.

ICD-10 Mapping for HAE

The primary diagnosis code for hereditary angioedema is D84.1 (Defects in the complement system). This code specifically covers C1-esterase inhibitor deficiency.

Documentation Requirements for D84.1:

Lab Test Type I HAE Type II HAE HAE with Normal C1-INH
C4 Level Low Low Normal
C1-INH Antigenic Low Normal/High Normal
C1-INH Functional Low Low Normal
Genetic Testing Optional Optional Required

Supporting Documentation Words:

  • "Recurrent angioedema without urticaria"
  • "Family history of hereditary angioedema"
  • "C1-esterase inhibitor deficiency confirmed"
  • "Failed response to antihistamines and corticosteroids"
  • "Prophylaxis indicated due to attack frequency"

Source: UnitedHealthcare HAE Treatment Policy

Product Coding: HCPCS, NDC, and Units

HCPCS J-Code

J0599 - Injection, C1 esterase inhibitor (human), HAEGARDA, 10 units

NDC Codes

  • 63833-0828-02 (2000 units per carton)
  • 63833-0829-02 (3000 units per carton)

Convert to 11-digit format for billing: 63833-00828-02 and 63833-00829-02

Units Calculation

  • J0599 represents 10 units of Haegarda
  • Example: 2000 units administered = 200 billing units (2000 ÷ 10 = 200)
  • Always report total units administered on the claim

Administration Details

  • Route: Subcutaneous injection
  • Frequency: Typically every 3-4 days (60 IU/kg twice weekly)
  • Self-administered: Patient or caregiver injection after training

Source: Haegarda Reimbursement and Coding Guide

Clean Request Anatomy

Prior Authorization Submission Checklist:

Patient Information:

  • Member ID and policy number
  • Date of birth and contact information
  • Prescribing physician details

Clinical Documentation:

  • ICD-10 code: D84.1
  • Lab results: C4, C1-INH antigenic and functional levels
  • Attack history with dates and severity
  • Specialist consultation notes (allergist/immunologist)

Prescription Details:

  • Drug name: Haegarda
  • NDC: 63833-00828-02 or 63833-00829-02
  • Strength and quantity requested
  • Dosing frequency and administration route

Medical Necessity:

  • Confirmed HAE diagnosis
  • Attack frequency documentation
  • Prior therapy failures (if applicable)
  • Quality of life impact assessment

Example Claim Line:

Field 24D: J0599
Field 24A: HAEGARDA NDC 63833-00828-02; Subcutaneous Injection; 2000 units
Units: 200 (2000 ÷ 10)
Diagnosis: D84.1

Frequent Coding Pitfalls

Common Mistakes to Avoid:

  1. Incorrect Unit Conversion
    • Wrong: Billing 2000 units instead of 200 units of J0599
    • Right: Divide total units by 10 for J0599 billing
  2. NDC Format Errors
    • Wrong: Using 10-digit NDC (63833-0828-02)
    • Right: Converting to 11-digit format (63833-00828-02)
  3. Missing Route of Administration
    • Always specify "subcutaneous injection" in claim details
  4. Incorrect Diagnosis Coding
    • Wrong: Using angioedema codes (T78.3)
    • Right: Using hereditary angioedema code (D84.1)
  5. Incomplete Prior Authorization
    • Missing specialist documentation
    • Lack of lab evidence for HAE diagnosis
    • No attack frequency documentation

Verification with UnitedHealthcare

Before Submitting:

  1. Check Coverage Status
    • Log into UnitedHealthcare provider portal
    • Verify patient eligibility and benefits
    • Confirm prior authorization requirements
  2. Review Current Policies
    • Access latest HAE treatment policy
    • Check for formulary updates
    • Verify step therapy requirements
  3. Contact Information for Verification:
    • OptumRx: 888-397-8129
    • UnitedHealthcare Provider Services: 1-800-842-3211
    • Prior Authorization Fax: (verify current number through provider portal)

Key Questions to Ask:

  • Is Haegarda covered under medical or pharmacy benefit?
  • What documentation is required for prior authorization?
  • Are there quantity limits or step therapy requirements?
  • What is the expected timeline for PA decision?

Quick Audit Checklist

Pre-Submission Review:

  • ICD-10 code D84.1 documented
  • Lab results confirming HAE diagnosis attached
  • Specialist (allergist/immunologist) prescription
  • Correct J-code J0599 with proper unit calculation
  • NDC in 11-digit format
  • Route of administration specified (subcutaneous)
  • Medical necessity letter includes attack frequency
  • Prior therapy history documented (if applicable)
  • Patient training plan for self-injection
  • All required forms completed and signed

Documentation Completeness:

  • C4 level results
  • C1-INH antigenic and functional assay results
  • Family history of HAE (if relevant)
  • Attack log with dates, severity, and treatment
  • Quality of life impact assessment
  • Contraindications to alternative therapies (if applicable)

Common Denial Reasons & Fixes

Denial Reason How to Overturn Required Documentation
No confirmed HAE diagnosis Submit complete lab workup C4, C1-INH levels, genetic testing if indicated
Lack of specialist involvement Get allergist/immunologist consultation Specialist evaluation and prescription
Insufficient attack frequency Document attack history Attack log, ER visits, acute treatment records
Step therapy not met Document contraindications to alternatives Medical reasons why Takhzyro/Orladeyo inappropriate
Quantity limits exceeded Justify weight-based dosing Body weight, BSA calculations, dosing rationale
Training plan missing Submit injection training documentation Training completion certificate, competency assessment

Step Therapy Considerations:

While UnitedHealthcare doesn't mandate absolute step therapy for Haegarda as of 2025, some plans may prefer trials of:

  • Lanadelumab (Takhzyro)
  • Berotralstat (Orladeyo)
  • IV C1-INH (Cinryze)

Document medical reasons if these alternatives are inappropriate.

Source: UnitedHealthcare PA Notification for Haegarda

Appeals Process in Texas

Internal Appeals Timeline:

  • Standard: 30 days for pre-service requests
  • Expedited: 72 hours for urgent cases
  • Deadline to file: 180 days from denial date

External Review (IRO) Process:

Texas law provides independent external review for denied specialty drugs:

  • Timeline: 20 days for standard review, 5 days for expedited
  • Cost: Free to patient (insurer pays)
  • Deadline: 4 months from final internal denial
  • Decision: Binding on UnitedHealthcare

How to Request External Review:

  1. Complete internal appeals process first
  2. Request IRO form from UnitedHealthcare or Texas Department of Insurance
  3. Submit within 4-month deadline
  4. Include all medical documentation and denial letters

Texas Department of Insurance Contact:

  • Phone: 1-800-252-3439
  • Website: tdi.texas.gov
  • IRO Information Line: 1-866-554-4926

Expedited Appeals:

Available when delay could jeopardize health or ability to regain function. Include physician statement of urgency and clinical risk.

From Our Advocates: "We've seen Haegarda denials overturned most successfully when the appeal includes a detailed attack log showing frequency and severity, complete lab workup confirming HAE diagnosis, and a strong medical necessity letter from an allergist explaining why prophylaxis is essential. The key is demonstrating that attacks significantly impact quality of life and that Haegarda is the most appropriate prophylactic option for the patient's specific situation."

Counterforce Health: Streamlining Your Appeals

When facing a Haegarda denial from UnitedHealthcare, Counterforce Health helps patients, clinicians, and specialty pharmacies turn insurance denials into targeted, evidence-backed appeals. The platform analyzes denial letters and plan policies to identify the specific denial basis—whether PA criteria, step therapy, or "not medically necessary"—and drafts point-by-point rebuttals aligned to UnitedHealthcare's own rules.

For Haegarda appeals, Counterforce Health pulls the right evidence—FDA labeling, HAE treatment guidelines, and specialty literature—and weaves them into appeals with required clinical facts like HAE diagnosis confirmation, attack frequency documentation, and specialist recommendations. The system produces letters that meet UnitedHealthcare's procedural requirements while tracking Texas-specific deadlines and required documentation.

FAQ

Q: How long does UnitedHealthcare prior authorization take for Haegarda in Texas? A: Standard PA decisions typically take 2-4 weeks. Expedited reviews for urgent cases are decided within 72 hours.

Q: What if Haegarda is non-formulary on my UnitedHealthcare plan? A: Request a formulary exception with medical necessity documentation. Include specialist letter explaining why formulary alternatives are inappropriate.

Q: Can I request an expedited appeal in Texas? A: Yes, both internal and external expedited appeals are available when delay could jeopardize health. Include physician statement of urgency.

Q: Does step therapy apply if I've tried other HAE drugs outside Texas? A: Previous therapy trials from any location count. Document dates, duration, and reasons for failure or intolerance.

Q: What happens if UnitedHealthcare denies my external review request? A: The IRO decision is binding. If approved, UnitedHealthcare must cover Haegarda. If denied, consider working with Counterforce Health to identify other coverage pathways.

Q: Are there cost assistance programs for Haegarda? A: Yes, contact Haegarda Connect at 1-844-424-4273 for information about patient assistance programs and copay support.

Q: How do I find an allergist/immunologist for HAE diagnosis? A: Use UnitedHealthcare's provider directory or contact the US Hereditary Angioedema Association (HAEA) for specialist referrals.

Q: Can I appeal multiple times if my first appeal is denied? A: Yes, UnitedHealthcare typically allows 1-2 levels of internal appeals before external review. Each level has specific timelines and requirements.

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 company for specific coverage decisions. Coverage policies and requirements may change. Verify all information with official sources before submitting claims or appeals.

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