Elfabrio (pegunigalsidase alfa-iwxj) — HCPCS J2508
CareCost Estimate · Billing Cheat Sheet
Chiesi Global Rare Diseases (Protalix BioTherapeutics dev.)
20 mg / 10 mL single-dose vial (liquid, no reconstitution)
IV q2wk · 3–4 hr initial / 2–3 hr maintenance
Reviewed: May 2, 2026
ASP: Q2 2026
Premedication recommended. Acetaminophen + diphenhydramine (± corticosteroid) 30–60 min pre-infusion. Hypersensitivity / anaphylaxis can occur during infusion and up to 3 days post-infusion — administer in healthcare setting with resuscitation equipment. No boxed warning (W&P only: anaphylaxis, IARs, MPGN, embryo-fetal toxicity).
Adult dose
1 mg/kg
IV q2wk · ~70 units typical
Modifier
JW + JZ
Wt-based → frequent waste
Admin CPT
96365 + 96366
Therapeutic IV (3–4 hr initial)
Medicare ASP+6%
$223.40
/mg · $15,638/70 mg dose
Codes & NDC
| HCPCS | J2508 — "Pegunigalsidase alfa-iwxj" (permanent; 1 unit = 1 mg) |
|---|
| Vial | 20 mg / 10 mL (2 mg/mL) single-dose liquid; no reconstitution; refrigerate 2–8°C; do not freeze/shake |
|---|
| NDC | Verify carton NDC with Chiesi Total Care & wholesaler invoice (11-digit padded format on CMS-1500 line 24A; N4 qualifier) |
|---|
| FDA approval | May 2023 (BLA 761161) — first PEGylated α-Gal A ERT |
|---|
| Benefit | Medical (provider buy-and-bill); rare-disease nurse case mgmt via Chiesi Total Care |
|---|
Dosing & vial math (20 mg vial)
| Wt | Dose | Vials | JW waste |
|---|
| 50 kg | 50 mg | 3 (60 mg) | 10 mg JW |
| 60 kg | 60 mg | 3 (60 mg) | 0 — bill JZ |
| 70 kg | 70 mg | 4 (80 mg) | 10 mg JW |
| 80 kg | 80 mg | 4 (80 mg) | 0 — bill JZ |
| 92 kg | 92 mg | 5 (100 mg) | 8 mg JW |
| 100 kg | 100 mg | 5 (100 mg) | 0 — bill JZ |
Administration & modifiers
| Code | When |
|---|
96365 | Therapeutic IV, 1st hour (primary) — non-chemo |
96366 | Each addtl hour (×2–3 initial; ×1–2 maintenance) |
96374 | IV push for premeds (diphenhydramine, methylprednisolone) |
96413 | Not appropriate — pegunigalsidase alfa is non-chemo ERT |
JZ/JW required: One must be on every J2508 claim per CMS 7/1/2023 SDV policy. Wt-based dosing across 20 mg vials → frequent waste → JW line is the norm.
Premedication checklist
- Acetaminophen 650–1000 mg PO — 30–60 min pre
- Diphenhydramine 25–50 mg PO/IV — 30–60 min pre (J1200 if IV)
- Methylprednisolone 20–100 mg IV — optional, 30–60 min pre (J2920/J2930)
- H2 blocker (famotidine 20–40 mg IV) — some centers
- Bill IV premeds separately + 96374 IV push
- Document premed order set + post-infusion observation on infusion record
ICD-10 — Fabry primary + organ Dx
| Code | For |
|---|
E75.21 | Fabry disease (primary; required) |
I43 | Cardiomyopathy (Fabry) |
N18.1–N18.6 | CKD stage-specific |
G93.89 / I63.x | CNS / cerebrovascular involvement |
G60.8 | Small-fiber neuropathy / acroparesthesias |
Diagnostic confirmation required: α-Gal A enzyme assay AND/OR GLA gene mutation analysis in chart for PA approval (both typically required — heterozygous females may have normal enzyme).
Fabry treatment landscape
| Drug | HCPCS | Class / Schedule |
|---|
| Elfabrio (pegunigalsidase alfa-iwxj) | J2508 | PEGylated ERT IV q2wk; adult only |
| Fabrazyme (agalsidase beta) | J0180 | Recombinant ERT IV q2wk; adult + ped ≥2 yr |
| Galafold (migalastat) | Pharmacy | Oral chaperone every other day; amenable mutations only |
| Replagal (agalsidase alfa) | — | NOT FDA-approved (EU only) |
Elfabrio vs Fabrazyme
| Elfabrio J2508 | Fabrazyme J0180 |
|---|
| Approved | 2023 | 2003 |
| Class | PEGylated | Non-PEG |
| Antibody | Designed reduced | Most seroconvert 3–6 mo |
| Pediatric | No (adult only) | Yes ≥2 yr |
| Vial | 20 mg liquid | 5 / 35 mg lyophilized |
| Dose / sched | 1 mg/kg q2wk | 1 mg/kg q2wk (same) |
| ASP+6%/mg (Q2 2026) | ~$223.40 | ~$227.58 |
Switch pathway: Documented Fabrazyme intolerance / anaphylaxis / high anti-drug antibody titers is the cleanest justification for Elfabrio when Fabrazyme is the contracted preferred ERT.
Payer requirements (May 2026)
| Payer | PA | Key criteria |
|---|
| UnitedHealthcare | Yes | Confirmed Fabry Dx + specialist (geneticist/cardiology/nephrology) consult + lifelong commitment |
| Aetna | Yes | Enzyme + GLA confirmation; combined Fabry ERT policy |
| BCBS plans | Yes | FDA label-aligned; plan-specific preferred ERT |
| Medicare (LCDs) | No formal PA | FDA-approved Fabry indication; medical necessity |
Step therapy possible: Some payers require trial of contracted preferred Fabry ERT (or Galafold for amenable mutations) first. Verify amenability + plan preference before submitting Elfabrio PA.
Medicare reimbursement (Q2 2026)
| Field | Value |
|---|
| ASP + 6% | $223.40 / mg (eff. 4/1 – 6/30/2026) |
| 70 mg dose (70 kg) | $15,638.00 (70 × $223.40) |
| 80 mg dose (80 kg, no waste) | $17,872.00 (80 × $223.40) |
| Annual (26 q2wk doses, 70 kg) | ~$406,588 admin / ~$464,672 inc. JW waste |
Site of care
| Setting | POS | Notes |
|---|
| Specialist office | 11 | Preferred (commercial UM) |
| Ambulatory infusion suite | 49 | Preferred (commercial UM) |
| Hospital outpatient | 19/22 | OK early; UHC/Aetna steer out long-term |
| Patient home | 12 | Limited — 3-day post-infusion anaphylaxis window; needs documented in-clinic tolerability |
Patient assistance — Chiesi Total Care
- Chiesi Total Care / Elfabrio Patient Support: 1-833-444-FABRY (1-833-444-3227)
- Benefits investigation, PA assistance, appeal support, rare-disease nurse case mgmt
- Commercial copay assistance (excludes Medicare/Medicaid/federal per AKS)
- PAP: free product for uninsured/underinsured eligible
- Foundations (Medicare): PAN, HealthWell, NORD, National Fabry Disease Foundation
- Patient advocacy: fabrydisease.org
Pending SME review. Staff-authored from FDA label (BLA 761161, May 2023), CMS Q2 2026 ASP, Chiesi/Protalix materials, and major payer policies. Verify Elfabrio carton NDC and current Chiesi Total Care PAP terms before high-stakes claims.