Xenpozyme (olipudase alfa-rpcp) — HCPCS J0218

CareCost Estimate · Billing Cheat Sheet

Sanofi Genzyme 4 mg / 20 mg lyophilized SDV IV ~3 hours, q2w (lifelong) Reviewed: May 2, 2026 ASP: Q2 2026

★ FIRST AND ONLY FDA-APPROVED ASMD TREATMENT (Aug 2022) — no class peer, no biosimilar, no alternative ERT. Treats acid sphingomyelinase deficiency (Niemann-Pick type B / type A–B). NOT for type A neurologic manifestations.

HCPCS

J0218

1 mg = 1 unit

Maintenance

210 units

3 mg/kg q2w · 70 kg pt

Modifiers

JZ + JW

Fixed 4/20 mg vials → waste

Admin CPT

96365

+ 96366 ×2 (~3 hr IV)

Medicare ASP+6%

$395.598

/mg · $83,075.58/dose

Codes & vials

HCPCS	`J0218` — "Inj olipudase alfa-rpcp 1mg" (permanent, post-Aug 2022 FDA approval)
Vials	4 mg lyophilized SDV (→ 0.5 mg/mL); 20 mg lyophilized SDV (→ 1 mg/mL); reconstitute with sterile water for injection
Indication	ASMD (Niemann-Pick type B and type A–B) — non-CNS manifestations only; NOT for type A neurologic
Benefit	Medical (provider buy-and-bill); rare-disease pathway

Maintenance dosing

3 mg/kg IV q2w after Day 98 (post-escalation)
Lifelong therapy
26 doses/year on q2w schedule
70 kg pt: 210 mg (= 210 units) per dose
Vial mix: 10 × 20 mg + 3 × 4 mg = 212 mg → ~2 mg waste (JW)
Year-1 mg volume is materially lower (escalation phase)

14-week dose escalation (mandatory)

Day	mg/kg	70 kg pt
1	0.03	~2 mg
15	0.1	~7 mg
29	0.3	~21 mg
43	0.3	~21 mg
57	0.6	~42 mg
71	1.0	~70 mg
85	2.0	~140 mg
99+	3.0 (maint)	~210 mg

Cannot skip or compress. Per FDA label — mitigates cytokine release / infusion reactions. Document schedule for PA.

Premedication checklist (escalation phase)

Administer 30–60 min before each infusion:

Antihistamine (H1) — diphenhydramine, cetirizine, fexofenadine
Antipyretic — acetaminophen
Corticosteroid — methylprednisolone or hydrocortisone (per protocol)
Antiemetic optional (e.g., ondansetron) per symptom history
Document premedication on every infusion encounter
May taper corticosteroid once stable on maintenance

Administration & modifiers

Code	When
`96365`	Therapeutic IV, initial hour (primary)
`96366`	Each additional hour — bill 2 units (~3-hr infusion)
`96413`	NOT appropriate — non-chemo
`JZ`	Administered units (e.g., 210 for 70 kg)
`JW`	Discarded mg from 4/20 mg vials (separate line)

ICD-10 — ASMD / Niemann-Pick

Code	For
`E75.249`	Most common — Niemann-Pick unspecified (ASMD)
`E75.242`	Niemann-Pick type B (confirmed)
`E75.241`	Type A — caution; non-CNS only
`E75.243`	NOT for Xenpozyme — type C1 (different disease)
`D69.6 / D64.9`	Thrombocytopenia / anemia (secondary)
`K76.89 / R16.x`	Liver / hepatosplenomegaly (secondary)
`J84.10`	Pulmonary fibrosis (interstitial lung disease)
`E78.5`	Hyperlipidemia (secondary)

Confirmation required: acid sphingomyelinase enzyme assay + SMPD1 gene mutation analysis.

Payer requirements (May 2026)

Payer	PA	Requirements
UnitedHealthcare	Yes	Enzyme assay + SMPD1 + specialist + 14-wk titration plan
Aetna	Yes	Same + at least one organ-system finding
BCBS plans	Yes	Plan-specific; aligned with FDA label

No step therapy possible. Xenpozyme is the only ASMD treatment. Annual reauth: current organ size, hematologic, pulmonary, lipid data.

Medicare reimbursement (Q2 2026)

Field	Value
ASP + 6%	$395.598 / mg (eff. 4/1 – 6/30/2026)
3 mg/kg, 70 kg pt	$83,075.58 (210 × $395.598)
Annual maintenance (26 doses)	~$2.16M (admin only, before waste)
After ~2% sequestration	~$2.13M/year actual paid
Year 1 (escalation included)	Materially less than maintenance year

Site of care

Setting	POS	Notes
Physician office	11	Stable maintenance pts
Ambulatory infusion suite	49	Preferred — fits 3-hr
Hospital outpatient	19/22	Common during escalation
Patient home	12	Stable pts only; experienced vendor

Patient assistance — Sanofi Patient Connection

Phone: 1-800-745-4447 (Sanofi Patient Connection)
Benefits investigation + PA assistance + appeal support
Commercial copay assistance: for eligible commercial pts (excludes Medicare/Medicaid/federal)
PAP: free drug for uninsured / underinsured
Foundations (Medicare): National Niemann-Pick Disease Foundation, PAN Foundation, HealthWell
Web: sanofipatientconnection.com

W&P (no Boxed): hypersensitivity / anaphylaxis (premedicate during escalation), infusion reactions / cytokine release (rationale for 14-wk titration), elevated transaminases (monitor LFTs — temporary elevation expected), embryo-fetal toxicity.

Pending SME review. Staff-authored from FDA label, CMS Q2 2026 ASP, Sanofi materials, payer policies. Verify high-stakes claims against linked sources.

Sources: FDA label Xenpozyme (BLA, Aug 2022 first-in-class), CMS ASP Q2 2026, Sanofi Patient Connection 2025, UHC/Aetna/BCBS rare-disease ERT policies, GeneReviews ASMD, NNPDF. carecostestimate.com/drugs/xenpozyme