Kanuma (sebelipase alfa) — HCPCS J2840

CareCost Estimate · Billing Cheat Sheet

Alexion / AstraZeneca Rare Disease 20 mg/10 mL single-dose vial IV infusion ≥2 hours LAL-D (Wolman & CESD) Reviewed: May 22, 2026 ASP: Q2 2026

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PHENOTYPE DETERMINES DOSING. Wolman (infant, rapidly progressive) = 1–5 mg/kg WEEKLY. CESD (late-onset) = 1 mg/kg EVERY 2 WEEKS. Mismatching frequency to phenotype on the PA triggers immediate denial.

HCPCS

J2840

1 mg = 1 unit

CESD dose

70 units

1 mg/kg q2w · 70 kg pt

Modifiers

JZ + JW

Only 20 mg vial → waste

Admin CPT

96365

+ 96366 (≥2-hr therapeutic IV)

PA gate

LAL assay + LIPA

#1 denial: missing enzyme assay

Codes & NDC

HCPCS	`J2840` — "Injection, sebelipase alfa, 1 mg" (permanent)
NDC	`25682-007-01` (Alexion labeler 25682)
Vial	20 mg/10 mL (2 mg/mL) solution, single-dose; refrigerate 2–8°C; do not shake
Dilution	0.9% NaCl; 0.2 micron low-protein-binding in-line filter
Benefit	Medical (provider buy-and-bill or specialty pharmacy → provider)
FDA approval	Dec 8, 2015 (BLA 125561) — first-in-class for LAL-D

Dosing matrix — Wolman vs CESD

Phenotype	Dose	Frequency
Wolman (rapidly progressive infantile LAL-D)	1 mg/kg → titrate to 3 mg/kg → 5 mg/kg	WEEKLY (~52 doses/yr)
CESD (late-onset LAL-D, children/adults)	1 mg/kg	EVERY 2 WEEKS (~26 doses/yr)

Wolman initiation in NICU/PICU. Severe disease + infusion reaction risk → intensive monitoring with emergency response capability. Inpatient stays = DRG-bundled, NOT separately billable.

Unit math examples

CESD adult (70 kg, 1 mg/kg q2w): 70 mg = 70 units JZ; 4×20 mg vials drawn; 10 units JW waste
Wolman infant (5 kg, 1 mg/kg wkly): 5 mg = 5 units JZ; 1×20 mg vial drawn; 15 units JW waste (75% of vial)
Wolman infant (5 kg, 3 mg/kg wkly): 15 mg = 15 units JZ; 1×20 mg vial drawn; 5 units JW waste
Wolman infant (5 kg, 5 mg/kg wkly): 25 mg = 25 units JZ; 2×20 mg vials drawn; 15 units JW waste

Administration & modifiers

Code	When
`96365`	Therapeutic IV initial, 1 hr (primary)
`96366`	Each additional hour (≥2-hr Kanuma infusion typical)
`96413`	NOT appropriate — ERT is non-chemo

Always bill BOTH lines: J2840/JZ for administered + J2840/JW for waste. On pediatric Wolman doses, waste can be 75% of the vial — do not omit.

ICD-10 — LAL-D

Code	For
`E75.5`	Primary — Other lipid storage disorders (LAL-D, both phenotypes; no unique ICD-10 code for LAL-D)
`K76.0`	Fatty change of liver (hepatic steatosis)
`K74.0 / K74.60`	Hepatic fibrosis / cirrhosis
`R16.0 / .1 / .2`	Hepato-/spleno-/hepatosplenomegaly
`E78.5 / .00 / .2`	Hyperlipidemia / hypercholesterolemia / mixed (CESD)
`D64.9`	Anemia, unspecified (Wolman)
`R62.51 / R62.7`	Failure to thrive (Wolman infants / adult FTT)
`E27.49`	Other adrenocortical insufficiency (Wolman adrenal calcification)

PA gate — #1 denial driver: LAL enzyme assay (dried blood spot + confirmatory) AND LIPA gene mutation analysis. Geneticist / hepatologist / metabolic specialist consult required.

Top denial drivers

#1 LAL enzyme assay missing from PA — order DBS + confirmatory before PA submission
#2 LIPA genotype missing — biallelic pathogenic variants typically required
#3 Phenotype/dose mismatch — Wolman weekly billed under CESD dx (or vice versa); document phenotype label in chart
#4 Missing JW waste line — especially on pediatric/Wolman doses (waste often 50–75% of vial)
#5 Inpatient NICU/PICU billed separately — bundled into DRG; use outpatient/observation for separate billing
#6 Home infusion for Wolman infant — not appropriate in early therapy; NICU/PICU/HOPD required

Payer requirements (May 2026)

Payer	PA	Notes
UnitedHealthcare	Yes	Rare disease ERT policy; LAL assay + LIPA + specialist; phenotype-matched dosing
Aetna	Yes	CPB on LAL-D; FDA label alignment, organ-system finding
BCBS plans	Yes	Plan-specific; specialty rare-disease pathway
Medicaid (state)	Yes	Often primary payer for Wolman infants; coordinate EPSDT
Medicare (MAC LCDs)	Documentation	Covered with E75.5 + diagnostic confirmation

Annual reauth: CESD → ALT/AST, lipid panel, hepatic imaging (US/MRE/FibroScan). Wolman → growth percentiles, transaminase trends, ferritin, hepatosplenic volume.

Site of care

Setting	POS	Notes
NICU/PICU (inpatient)	21	Wolman early therapy — DRG-bundled, not separately billable
NICU/PICU (outpatient/obs)	22/19	J2840 separately billable on UB-04
HOPD (on/off-campus)	22/19	Wolman maintenance; CESD initiation
Physician office (genetics/hep)	11	Preferred for stable CESD adults
Ambulatory infusion suite	49	Preferred for stable CESD adults
Patient home	12	Stable CESD adults only — NOT for Wolman infants in early therapy

Patient assistance — Alexion OneSource / AZ Access 360

Phone: 1-888-765-4747 (AZ Access 360, integrating Alexion OneSource post-2021 acquisition)
Kanuma Patient Support: case management, NICU/PICU coordination (Wolman), AIC/home transition (CESD), BI/PA help
Commercial copay: available for eligible commercially-insured (excludes Medicare/Medicaid/federal)
PAP: free drug for uninsured/underinsured
Foundations (Medicare/Medicaid): PAN, HealthWell, NORD — verify open LSD funds
Web: azaccess360.com · kanuma.com

Wolman urgency: Untreated Wolman fatal in first year. Engage AZ Access 360 at LAL assay result — do NOT wait for PA submission.

NO Boxed Warning. W&P only: hypersensitivity / anaphylaxis (most in Wolman infants), infusion-associated reactions, anti-drug antibody formation (more common in Wolman). Manage reactions w/ rate reduction, antihistamines, corticosteroids per label.

Sources: FDA label (BLA 125561, Dec 2015), ARISE trial (NEJM 2015), CMS ASP Q2 2026, NORD LAL-D, Alexion/AZ Access 360, UHC/Aetna rare-disease ERT policies. Pending SME review. carecostestimate.com/drugs/kanuma