Haegarda (C1 esterase inhibitor SC) — HCPCS J0599
CareCost Estimate · Billing Cheat Sheet
CSL Behring
2,000 IU & 3,000 IU lyophilized vials
SC injection q3–4 days (HAE prophylaxis)
Reviewed: May 2, 2026
ASP: Q2 2026 (pending)
Modifiers
JZ + JW
Partial-vial waste common
Admin CPT
96372
SC inj (NOT 96365 IV)
Medicare ASP+6%
Pending
CMS J0599 not yet listed
PROPHYLAXIS ONLY — NOT for acute HAE attacks. For acute attacks use Berinert (J0597), Ruconest (J0596), Firazyr SC, or Kalbitor SC. Submitting J0599 with acute-attack documentation will deny.
1 unit = 10 IU (biller error trap). Total IU ÷ 10 = J0599 units. Example: 80 kg × 60 IU/kg = 4,800 IU = 480 units J0599 (NOT 4,800).
Codes & vials
| HCPCS | J0599 — "C-1 esterase inhibitor, haegarda, 10 units" (Haegarda-specific) |
|---|
| Vials | 2,000 IU and 3,000 IU lyophilized; reconstitute with 4 mL or 6 mL sterile water → 500 IU/mL |
|---|
| Route | Subcutaneous (abdomen preferred); NOT IV |
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| FDA approval | June 2017 (BLA 125598) — first SC C1-INH for HAE prophylaxis |
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| Benefit | Medical for clinic admin; specialty pharmacy for self-admin (typical maintenance) |
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Dosing by weight
| Weight | Dose (60 IU/kg) | Vials | Units (JZ) | Waste (JW) |
|---|
| 50 kg | 3,000 IU | 1 × 3,000 | 300 | 0 |
| 60 kg | 3,600 IU | 2 × 2,000 | 360 | 40 |
| 70 kg | 4,200 IU | 2,000 + 3,000 | 420 | 80 |
| 80 kg | 4,800 IU | 2,000 + 3,000 | 480 | 20 |
| 90 kg | 5,400 IU | 2 × 3,000 | 540 | 60 |
| 100 kg | 6,000 IU | 2 × 3,000 | 600 | 0 |
SC vs IV C1-INH — same molecule, different billing
Haegarda (SC, J0599) and Cinryze (IV, J0598) are the same plasma-derived human C1-INH molecule from CSL Behring. Match by brand AND route, not active ingredient.
| Haegarda | Cinryze |
|---|
| HCPCS | J0599 | J0598 |
| Route | SC | IV |
| Dose | 60 IU/kg q3–4d | 1,000 IU q3–4d |
| Admin CPT | 96372 | 96365/66 |
| Self-admin | Norm after training | Limited |
HAE class quick reference
| Drug | HCPCS | Route | Use |
|---|
| Haegarda | J0599 | SC | Prophy |
| Cinryze | J0598 | IV | Prophy |
| Berinert | J0597 | IV | Acute |
| Ruconest | J0596 | IV | Acute |
| Takhzyro | J0593 | SC | Prophy (mAb) |
ICD-10 (HAE Type I & II only)
| Code | For |
|---|
D84.1 | Primary — defects in complement system (HAE Type I & II) |
D89.49 | Other complement disorder (some HAE variants) |
T78.3xxxA-S | Angioneurotic edema — pair with D84.1, never alone |
| HAE Type III (HAE-nC1INH) | NOT covered — off-label |
Self-admin vs clinic billing
| Scenario | Pathway | POS |
|---|
| First dose / training | Medical (J0599 + 96372 + E/M w/ mod 25) | 11 / 49 |
| Maintenance — home self-admin | Specialty pharmacy (NCPDP) | 12 |
| Maintenance — in-clinic | Medical (J0599 + 96372) | 11 / 49 |
| Home health (nurse) | Home health / medical | 12 |
Don't double-bill. If specialty pharmacy is dispensing for home self-admin, providers should NOT submit J0599 on a medical claim for the same doses.
Payer requirements (May 2026)
| Payer | PA | Key criteria |
|---|
| UnitedHealthcare | Yes | HAE Type I/II labs; ≥1 attack/mo; allergy/imm Rx |
| Aetna | Yes | HAE Type I/II lab confirmation; specialist Rx |
| Cigna | Yes | Step through Takhzyro may apply |
| BCBS | Yes | Plan-specific; aligned w/ US HAEA / WAO guidelines |
| Medicare Part B | Generally no | FDA on-label use; MAC LCD |
Site-of-care: First dose in clinic OK; maintenance steered to home self-admin (POS 12) via specialty pharmacy.
Medicare reimbursement (Q2 2026)
| Field | Value |
|---|
| ASP + 6% | Pending CMS publication for J0599 (Q2 2026 file) |
| Fallback | MAC reimbursement at invoice cost / WAC less applicable % |
| Verify | cms.gov/medicare/payment/part-b-drugs/asp-pricing-files |
| Coverage | No NCD; MAC LCD for biologics + HAE local policy |
Patient assistance — CSL Behring AssureSupport
- Phone (Haegarda): 1-855-999-3273
- Phone (CSL AssureSupport): 1-877-355-IGIV (4448)
- Co-Pay Program: $0 first dose for eligible commercial patients (excludes Medicare/Medicaid/federal)
- PAP: free product for uninsured / underinsured meeting income criteria
- Foundations (Medicare): PAN HAE fund, HealthWell HAE fund, NORD HAE fund — verify quarterly
- Web: haegarda.com / cslbehring.com
Top denial traps: 96365 IV (use 96372 SC) · J0598 instead of J0599 (Cinryze vs Haegarda) · missing JW on weight-based waste · acute-attack indication on prophy code · unit conversion error (4,800 IU billed as 4,800 units instead of 480).