Codes & NDC
| HCPCS | J2840 — "Injection, sebelipase alfa, 1 mg" (permanent) |
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| NDC | 25682-007-01 (Alexion labeler 25682) |
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| Vial | 20 mg/10 mL (2 mg/mL) solution, single-dose; refrigerate 2–8°C; do not shake |
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| Dilution | 0.9% NaCl; 0.2 micron low-protein-binding in-line filter |
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| Benefit | Medical (provider buy-and-bill or specialty pharmacy → provider) |
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| FDA approval | Dec 8, 2015 (BLA 125561) — first-in-class for LAL-D |
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Dosing matrix — Wolman vs CESD
| Phenotype | Dose | Frequency |
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| 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 admin (no modifier); 4×20 mg vials drawn; 10 units JW waste
- Wolman infant (5 kg, 1 mg/kg wkly): 5 mg = 5 units admin (no modifier); 1×20 mg vial drawn; 15 units JW waste (75% of vial)
- Wolman infant (5 kg, 3 mg/kg wkly): 15 mg = 15 units admin (no modifier); 1×20 mg vial drawn; 5 units JW waste
- Wolman infant (5 kg, 5 mg/kg wkly): 25 mg = 25 units admin (no modifier); 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 |
Bill BOTH lines when waste exists: J2840 administered units (no wastage modifier) + J2840/JW for waste on a separate line. On pediatric Wolman doses, waste can be 75% of the vial — do not omit. JZ only on zero-waste claims.
ICD-10 — LAL-D
| Code | For |
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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 |
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| 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.