HCPCS
J1931
1 unit = 0.1 mg
Phase 1
Identify what you're billing
Anaphylaxis prep first; then confirm dose, vial count, and the right MPS subtype.
BOXED WARNING — Anaphylaxis FDA label verified May 2026
Aldurazyme's most clinically significant safety signal — the warning that changes how every infusion is staffed and billed.
BOXED WARNING — Risk of anaphylaxis. Severe and life-threatening hypersensitivity
reactions, including anaphylaxis, have been observed in patients during and up to 3 hours after Aldurazyme
infusion. Anaphylaxis can be life-threatening; patients with compromised respiratory function or acute febrile
or respiratory illness at the time of infusion may be at increased risk. Aldurazyme must be administered in
a healthcare setting with appropriate resuscitation equipment (epinephrine, oxygen, IV fluids, airway
management) immediately available. Premedication with antihistamines (with or without antipyretics) is
recommended approximately 60 minutes prior to the start of each infusion.
Premedication regimen (typical)
- Diphenhydramine (or comparable H1 antihistamine) 60 minutes prior to infusion
- ± Acetaminophen as antipyretic if febrile history
- For patients with prior infusion-associated reactions: consider adding H2 blocker (e.g., ranitidine/famotidine) and/or corticosteroid per institutional protocol
Infusion-rate management
- Slow titration: start at low rate, titrate up per FDA label as tolerated
- Total infusion time typically 3–4 hours
- Stop or slow infusion if any signs of hypersensitivity (urticaria, flushing, dyspnea, hypotension, GI symptoms)
- Resuscitation equipment immediately available throughout AND for 1+ hour post-infusion observation
Site-of-care implication: The boxed warning makes home infusion clinically risky for many
MPS I patients, especially those with prior infusion reactions, severe Hurler phenotype, or compromised
respiratory function. Most payers require a healthcare facility (office, AIC, or HOPD) for at least the
first several infusions, and many require that level of care indefinitely.
Dosing & unit math FDA label verified May 2026
Weight-based dosing means partial-vial waste is the norm, not the exception.
Standard dose
- 0.58 mg/kg IV once weekly, lifelong enzyme replacement
- 1 unit of J1931 = 0.1 mg (so 1 mg = 10 units, 50 mg = 500 units)
- Vial: 5 mg / 5 mL single-dose vial = 50 units per vial
- Bill the actual mg administered (in 0.1 mg units); bill discarded vial portions on JW line
Worked example — 86 kg adult, 50 mg dose (clean math)
# Calculated dose
86 kg × 0.58 mg/kg = 49.88 mg ~ 50 mg
Vials needed: 50 mg / 5 mg per vial = 10 vials
Waste (50 mg dose, 50 mg drawn): 0 mg → bill JZ
Units billed: 50 mg / 0.1 mg = 500 units
# Year-1 totals (52 weekly infusions)
Total drug units billed: 26,000 (52 × 500)
Total drug cost (Q2 2026 ASP+6%): ~$1,038,804 before sequestration
86 kg × 0.58 mg/kg = 49.88 mg ~ 50 mg
Vials needed: 50 mg / 5 mg per vial = 10 vials
Waste (50 mg dose, 50 mg drawn): 0 mg → bill JZ
Units billed: 50 mg / 0.1 mg = 500 units
# Year-1 totals (52 weekly infusions)
Total drug units billed: 26,000 (52 × 500)
Total drug cost (Q2 2026 ASP+6%): ~$1,038,804 before sequestration
Worked example — 30 kg pediatric, 17.4 mg dose (waste math)
# Calculated dose
30 kg × 0.58 mg/kg = 17.4 mg
Vials needed: ceil(17.4 / 5) = 4 vials (= 20 mg drawn)
Administered: 17.4 mg = 174 units → bill on JZ-style admin line
Wasted: 20 mg - 17.4 mg = 2.6 mg = 26 units → bill on separate JW line
Total vials opened: 4 · Total units across both lines: 200
30 kg × 0.58 mg/kg = 17.4 mg
Vials needed: ceil(17.4 / 5) = 4 vials (= 20 mg drawn)
Administered: 17.4 mg = 174 units → bill on JZ-style admin line
Wasted: 20 mg - 17.4 mg = 2.6 mg = 26 units → bill on separate JW line
Total vials opened: 4 · Total units across both lines: 200
No premedication routinely required — FALSE for Aldurazyme
Unlike most monoclonal antibody therapeutics, Aldurazyme requires premedication per the FDA label and boxed warning: antihistamine (with or without antipyretic) approximately 60 minutes before each infusion. Document the premedication in the chart for audit defense.
NDC reference FDA NDC Directory verified May 2026
| NDC (10/11-digit) | Package | Use |
|---|---|---|
58468-0070-01 / 58468-070-01 |
5 mg / 5 mL (1 mg/mL) single-dose vial — 1 vial per carton | All Aldurazyme dosing — multiple vials drawn per dose |
Use the carton-level NDC on the claim form. Aldurazyme is a single-dose vial in a 1-vial
carton, so vial NDC and carton NDC are functionally the same here, but the correct format is the 11-digit
NDC with
N4 qualifier on CMS-1500 box 24A shaded area, with units of measure ML (or UN per
payer) and the total quantity drawn (sum of vial volumes opened, not just the volume infused).
Storage: Refrigerate 2–8°C (36–46°F). Do not freeze. Do not shake.
Single-dose vial — discard unused portion (which is reportable as wasted drug under the JW modifier).
MPS treatment landscape FDA verified May 2026
Each mucopolysaccharidosis subtype has its own enzyme replacement. Aldurazyme is one of five — do not cross-bill.
| MPS subtype | Brand | Generic | HCPCS | Manufacturer | ICD-10 |
|---|---|---|---|---|---|
| MPS I (Hurler / Hurler-Scheie / Scheie) | Aldurazyme (this page) | laronidase | J1931 (0.1 mg/unit) |
BioMarin / Sanofi | E76.01, E76.02, E76.03 |
| MPS II (Hunter syndrome) | Elaprase | idursulfase | J1743 |
Takeda (Shire legacy) | E76.1 |
| MPS IVA (Morquio A) | Vimizim | elosulfase alfa | J1322 |
BioMarin | E76.219 |
| MPS VI (Maroteaux-Lamy) | Naglazyme | galsulfase | J1458 |
BioMarin | E76.29 |
| MPS VII (Sly syndrome) | Mepsevii | vestronidase alfa | J3397 |
Ultragenyx | E76.29 |
Common cross-billing error: billing J1931 for a Hunter (MPS II) patient. Hunter is treated
with idursulfase (Elaprase, J1743), not laronidase. ICD-10
E76.1 with HCPCS J1931 will deny
for clinical mismatch. Always confirm the specific enzyme deficiency from the metabolic-genetics workup
before billing.
Diagnostic confirmation required. Most payers require documented α-L-iduronidase
enzyme assay deficiency AND/OR genetic testing (IDUA gene mutations) confirming MPS I in the medical record
before approving Aldurazyme PA. A clinical phenotype description alone is generally insufficient.
Phase 2
Code the claim
Therapeutic IV codes (96365 + 96366) — not chemo. Long infusion = stacked add-on hours.
Administration codes CPT verified May 2026
Aldurazyme is a non-chemo therapeutic protein. Use therapeutic IV codes, not chemo admin codes.
| Code | Description | When to use |
|---|---|---|
96365 |
Intravenous infusion, for therapy/prophylaxis/diagnosis; initial, up to 1 hour | Primary code for Aldurazyme. Bill 1 unit per encounter for the first hour. |
96366 |
IV infusion, each additional hour | Bill 2–3 units per encounter. Total infusion runs 3–4 hours, so add-on hours stack: 1 unit per additional hour beyond the first. |
96413 / 96415 |
Chemotherapy administration, IV infusion | NOT appropriate. Aldurazyme is enzyme replacement therapy, not chemotherapy or immunotherapy. CPT chemo admin codes do not apply. |
96367 |
IV infusion, additional sequential infusion of new drug/substance, up to 1 hour | If a separate non-Aldurazyme IV drug is given sequentially in the same encounter (rare for routine ERT visits). |
Why therapeutic IV (not chemo): CPT chemotherapy administration codes (96409–96425)
are reserved for cytotoxic drugs and complex monoclonal antibodies classified by AMA as chemo-admin.
Recombinant enzyme replacement therapies like laronidase fall under therapeutic IV codes (96365/96366).
Billing 96413 will trigger denial.
Document infusion time precisely. Add-on hour codes (96366) require documented infusion
time exceeding the prior hour by >30 minutes per AMA CPT guidelines. With a 3–4 hour Aldurazyme
infusion, a typical claim is 1× 96365 + 2× or 3× 96366. Stop/start time documentation in
the chart is the audit defense.
Modifiers CMS verified May 2026
JZ — required when there is no waste
Effective July 1, 2023, CMS requires the JZ modifier on all single-dose container claims when no drug is discarded. For Aldurazyme, JZ applies only when the calculated dose is an exact multiple of 5 mg (the vial size) — e.g., a patient dosed at exactly 50 mg uses 10 vials with zero waste.
JW — required when there is waste (the typical case)
Because Aldurazyme dosing is weight-based (0.58 mg/kg) and the vial is fixed at 5 mg, partial-vial waste
is the norm. Bill the administered units on the regular drug line and the discarded units on a separate
line with the JW modifier. One of JZ or JW must appear on every J1931 claim.
Common error: Forgetting to bill the discarded portion. CMS audits often catch this —
wasted drug is reimbursable but must be reported. Because a 50 mg dose can produce waste worth thousands of
dollars at J1931's ASP+6%, the JW line is material.
Modifier 25 — same-day E/M
Use modifier 25 on the E/M code when a significant, separately identifiable evaluation and management service is performed on the same day as the infusion (e.g., metabolic-genetics specialist consultation documenting clinical response or addressing infusion-reaction history). Routine pre-infusion clinical assessment is bundled.
340B modifiers (JG, TB)
For 340B-acquired Aldurazyme, follow your MAC's current 340B modifier policy (JG for hospital outpatient, TB for informational reporting where applicable). Sanofi's billing guide does not provide 340B-specific instructions for J1931.
ICD-10-CM — MPS I subtypes only FY2026 verified May 2026
Aldurazyme is approved only for MPS I. Other MPS subtypes have distinct enzyme replacements (see MPS landscape).
| ICD-10 | Description | Use with J1931? |
|---|---|---|
E76.01 | Hurler syndrome (severe MPS I) | Yes — primary indication; severe phenotype |
E76.02 | Hurler-Scheie syndrome (intermediate MPS I) | Yes — intermediate phenotype |
E76.03 | Scheie syndrome (attenuated MPS I) | Yes — attenuated phenotype |
E76.1 | Hunter syndrome (MPS II) | NO — use Elaprase (J1743) |
E76.219 | Morquio syndrome, unspecified (MPS IVA) | NO — use Vimizim (J1322) |
E76.29 | Other mucopolysaccharidoses (MPS VI Maroteaux-Lamy / MPS VII) | NO — use Naglazyme (J1458) or Mepsevii (J3397) |
E76.3 | Mucopolysaccharidosis, unspecified | Generally insufficient — payers require subtype-specific code with diagnostic confirmation |
Diagnostic confirmation in PA submission. Most payers require documentation of:
(1) deficient α-L-iduronidase enzyme activity from a CLIA-certified lab assay, and/or
(2) two confirmed pathogenic IDUA gene mutations from genetic testing. The ICD-10 code alone is not
sufficient for approval given the rare-disease and high-cost profile.
Site of care & place of service Verified May 2026
Aldurazyme's 3–4 hour infusion plus 1+ hour observation makes site-of-care selection a major operational decision. Most major payers run UM on lifelong ERT, but the boxed-warning anaphylaxis risk gives the provider strong leverage to keep care in a higher-acuity setting for at-risk patients.
| Setting | POS | Claim form | Payer steering |
|---|---|---|---|
| Physician/specialist office (genetics/metabolic) | 11 | CMS-1500 / 837P | Preferred by commercial UM (lower cost than HOPD) |
| Ambulatory infusion suite (AIC) | 49 | CMS-1500 / 837P | Preferred by commercial UM |
| Hospital outpatient (on-campus) | 22 | UB-04 / 837I | Acceptable for first several infusions or for patients with infusion-reaction history; some payers steer out long-term |
| Hospital outpatient (off-campus PBD) | 19 | UB-04 / 837I | Disfavored after stable tolerability established |
| Patient home | 12 | CMS-1500 (with home infusion) | Limited — boxed-warning anaphylaxis risk; only after multiple uneventful infusions, with appropriate vendor and medical-necessity documentation |
Home infusion clinical risk: Despite payer pressure to move ERT home, Aldurazyme's boxed
warning makes home infusion clinically risky. Many MPS I treatment centers keep at least the first
12–24 infusions in a healthcare facility and only consider home infusion after demonstrated
tolerability and with a vendor experienced in lysosomal-storage-disease ERT.
Claim form field mapping Sanofi/BioMarin 2026
From Sanofi Patient Connection HCP coding & coverage materials and BioMarin RareConnections.
| Information | CMS-1500 box | Notes |
|---|---|---|
| NPI | 17b | Rendering provider (typically metabolic geneticist or pediatric specialist) |
| NDC qualifier + 11-digit NDC + UoM + qty | 24A shaded area | N4 + 58468-0070-01 + ML + total mL drawn (e.g., 50 mL for 10 vials at 50 mg dose) |
| HCPCS J1931 + JZ (admin line, no waste case) | 24D (drug line) | Units = mg administered × 10 (since 1 unit = 0.1 mg) |
| HCPCS J1931 + JW (waste line, when applicable) | 24D (separate line) | Units = mg discarded × 10; on its own line, separate from administered units |
| Drug units | 24G | 500 for 50 mg adult dose; actual mg × 10 for pediatric |
| CPT 96365 (1st hour) | 24D (admin line) | 1 unit per encounter |
| CPT 96366 (each add'l hour) | 24D (admin line) | 2–3 units per encounter for typical 3–4 hour infusion |
| ICD-10 | 21 | E76.01, E76.02, or E76.03 (MPS I subtype-specific) |
| PA number | 23 | Required by all major payers |
Phase 3
Get paid
PA always required; diagnostic confirmation and specialist consultation are non-negotiable.
Payer policy snapshot + coverage criteria Reviewed May 2026
All major payers PA Aldurazyme. Diagnostic confirmation and specialist involvement are universal requirements.
| Payer | PA? | Required documentation | Site-of-care UM |
|---|---|---|---|
| UnitedHealthcare Lysosomal Storage Disease policy |
Yes | MPS I confirmed by α-L-iduronidase enzyme assay or genetic testing; metabolic-genetics specialist consultation; lifelong therapy commitment | Yes — preference for office/AIC over HOPD after first several infusions |
| Aetna CPB Medical Drug policy |
Yes | Diagnosis confirmed by enzyme assay AND/OR IDUA genetic testing; prescribed by/in consultation with metabolic specialist | Yes (separate Site-of-Care policy; ERTs steered toward office/AIC where clinically appropriate) |
| BCBS plans Vary by plan |
Yes | Generally aligned with FDA label, requiring confirmed MPS I diagnosis and specialist oversight | Plan-specific; many have ERT site-of-care steering |
| Medicare (LCDs / NCDs) | No formal PA, but coverage requires medical necessity documentation | FDA-approved indication (MPS I); no NCD specific to Aldurazyme; MAC LCDs cover for confirmed MPS I diagnosis | N/A (POS billing per setting) |
Universal PA criteria checklist
- Confirmed MPS I diagnosis by deficient α-L-iduronidase enzyme activity (CLIA-certified lab) AND/OR confirmed pathogenic IDUA gene mutations
- ICD-10 specifying subtype (E76.01 / E76.02 / E76.03)
- Prescription by, or in consultation with, a metabolic geneticist or other specialist experienced in MPS I management
- Lifelong therapy commitment (ERT is not curative; suspension typically results in clinical regression)
- For pediatric patients: age and weight; growth parameters often required for ongoing reauth
- Premedication regimen documented (for boxed-warning compliance)
Step therapy
Generally NOT applicable — Aldurazyme is the only FDA-approved enzyme replacement therapy for MPS I, so there is no equivalent biologic to step through. Hematopoietic stem cell transplant (HSCT) is sometimes used for severe Hurler phenotype in early infancy, but is not a "step" required before Aldurazyme by payer policy.
Medicare reimbursement CMS Q2 2026 (live)
Quarterly ASP from CMS Part B Drug Pricing File. Refreshes automatically each quarter.
Q2 2026 payment snapshot — J1931
Effective April 1 – June 30, 2026 · Based on 4Q25 ASP submissions
ASP + 6%
$39.954
per 0.1 mg unit
50 mg dose (~86 kg adult)
$19,977.00
500 units × ASP+6%
Annualized (52 weekly doses)
$1,038,804
~$1.04M/year per adult patient
Annualized cost: 50 mg weekly × 52 weeks = ~$1.04M/year (Medicare ASP+6%)
for a typical adult MPS I patient. After ~2% sequestration: ~$1.02M/year actual paid. Pediatric patients at
lower body weight have proportionally lower annual cost (e.g., ~$362K/year for the 17.4 mg pediatric example
above).
Coverage
No NCD specific to laronidase. Coverage falls under MAC LCDs for biologics + the generic drug-coverage framework for FDA-approved therapies. All MACs cover J1931 for FDA-approved MPS I indication with confirmed diagnosis (enzyme assay or genetic testing) and appropriate ICD-10 documentation.
Code history
- J1931 — permanent code, "Laronidase injection," 1 unit = 0.1 mg. Aldurazyme was FDA-approved April 30, 2003 (BLA 125058) as the first enzyme replacement therapy for MPS I.
Patient assistance — Sanofi Patient Connection / BioMarin RareConnections Sanofi verified May 2026
- Sanofi Patient Connection / Aldurazyme Patient Support: 1-866-906-6100 — benefits investigation, prior authorization assistance, copay support, free drug for eligible patients
- BioMarin RareConnections: dedicated rare-disease support program; nurse case managers experienced in MPS I; coordinates infusion-site setup and reauthorization
- Aldurazyme Co-Pay Assistance Program: commercial copay support for eligible commercially-insured patients (excludes Medicare, Medicaid, federal program patients per federal anti-kickback rules)
- Patient Assistance Program (PAP): free product for uninsured / underinsured patients meeting income requirements
- Foundations: for Medicare patients, refer to PAN Foundation, HealthWell, NORD, and the National MPS Society — verify open MPS/lysosomal-storage-disease funds quarterly
- National MPS Society: patient advocacy and family support — mpssociety.org
Need to model what a specific MPS I patient will actually pay after copay assistance, deductible, coinsurance,
and OOP max? Run a CareCost Estimate — J1931 pre-loaded.
Phase 4
Fix problems
JW omission, wrong admin code, and missing diagnostic confirmation are the top three.
Common denials & how to fix them
| Denial reason | Common cause | Fix |
|---|---|---|
| Diagnostic confirmation missing | PA submitted with ICD-10 but no enzyme assay or genetic testing report | Submit α-L-iduronidase enzyme assay result and/or IDUA genetic testing report; retroactive PA with documentation. |
| Wrong admin code (96413) | Chemo IV billed instead of therapeutic IV | Resubmit with 96365 (1st hour) + 96366 × 2–3 (add'l hours). Aldurazyme is non-chemo enzyme replacement. |
| JW missing on weight-based dose | Wasted drug not reported when calculated dose isn't an exact 5 mg multiple | Add JW line for discarded units. JZ on the administered units; JW on the wasted units. Required since 7/1/2023. |
| JZ missing on exact-multiple dose | Single-dose vial claim without JZ when no waste occurred | Resubmit with JZ. Required when no drug is discarded. |
| Wrong indication (E76.1 Hunter) | Aldurazyme billed for MPS II patient | Hunter syndrome is treated with idursulfase (Elaprase, J1743), not laronidase. Switch drug or correct ICD-10. |
| Specialist consultation not documented | PA missing metabolic-genetics specialist note | Submit consultation note from a metabolic geneticist or experienced MPS I clinician with the appeal. |
| Site of care (HOPD) | HOPD administration after stable tolerability on commercial plan with site-of-care UM | Move to office (POS 11) or AIC (POS 49). Submit medical necessity letter citing boxed-warning anaphylaxis risk if HOPD required for clinical reasons. |
| Add-on hours (96366) denied | Insufficient infusion time documentation | Submit chart note with stop/start times demonstrating >30 minutes beyond each prior hour per AMA CPT guidelines. |
Frequently asked questions
What is the HCPCS code for Aldurazyme?
Aldurazyme (laronidase) is billed under HCPCS J1931 — "Laronidase injection." Each
0.1 mg equals one billable unit. A typical 50 mg adult weekly dose is billed as 500 units.
J1931 is a permanent code; do not bill under unclassified J3490.
How many units do I bill for a typical Aldurazyme dose?
1 unit = 0.1 mg. For a patient weighing 86 kg at 0.58 mg/kg, the calculated dose is approximately 50 mg = 500 units of J1931. A 30 kg pediatric patient receives ~17.4 mg = 174 units. Always bill the actual mg administered (in 0.1 mg units), and bill discarded vial portions on a separate JW line.
What administration CPT do I use for Aldurazyme?
CPT 96365 — "Intravenous infusion, for therapy/prophylaxis/diagnosis; initial, up to 1
hour" — plus 96366 for each additional hour. Aldurazyme is non-chemo enzyme replacement
and does NOT use chemo administration codes (96413/96415). Standard infusion runs 3–4 hours, so
expect 1× 96365 + 2× or 3× 96366 per encounter.
Do I bill JZ or JW for Aldurazyme?
Both, depending on the dose. Aldurazyme comes in 5 mg single-dose vials and dosing is weight-based, so
partial-vial waste is the norm. Bill JZ when the dose is an exact multiple of 5 mg;
otherwise bill JW for the discarded portion on a separate line. One of JZ or JW
must be on every J1931 claim.
What is the Medicare reimbursement for J1931?
For Q2 2026, the Medicare Part B payment limit for J1931 is $39.954 per 0.1 mg unit (ASP + 6%). A 50 mg weekly adult dose reimburses at approximately $19,977.00 per infusion; annualized across 52 weekly doses, that is roughly $1,038,804 per adult patient at the median weight. Sequestration (~2%) reduces actual paid to roughly ASP + 4.3%.
What ICD-10 codes support an Aldurazyme claim?
MPS I subtypes only: E76.01 (Hurler syndrome), E76.02 (Hurler-Scheie syndrome),
E76.03 (Scheie syndrome). E76.1 (Hunter / MPS II) is NOT covered — that is
Elaprase (J1743). Diagnosis must be confirmed by α-L-iduronidase enzyme assay or IDUA gene testing
in the medical record.
Does Aldurazyme have a boxed warning?
Yes. Aldurazyme carries a BOXED WARNING for risk of anaphylaxis. Severe and life-threatening hypersensitivity reactions, including anaphylaxis, have been observed during and up to 3 hours after infusion. Premedication with antihistamines (with or without antipyretics) is required approximately 60 minutes before each infusion. Aldurazyme must be administered in a healthcare setting with appropriate resuscitation equipment immediately available.
What patient assistance is available?
Sanofi Patient Connection / Aldurazyme Patient Support / BioMarin RareConnections support most patients. Phone: 1-866-906-6100 (Sanofi). Programs include benefits investigation, PA assistance, copay support for commercially-insured patients, and free drug for eligible uninsured/underinsured patients. For Medicare patients, refer to PAN Foundation, HealthWell, NORD, and the National MPS Society.
Reference
Sources & methodology
Every claim on this page is sourced. Methodology and review history below.
Source documents
- FDA Aldurazyme (laronidase) Prescribing Information — full label including BOXED WARNING for anaphylaxis
- DailyMed — ALDURAZYME (laronidase) injection prescribing information
- CMS — Medicare Part B Drug ASP Pricing File
- SEER CanMED — HCPCS J1931 reference
- Sanofi Patient Connection — Aldurazyme patient support, 1-866-906-6100
- BioMarin Pharmaceutical — Aldurazyme product information and RareConnections support
- National MPS Society — patient advocacy and clinical resources for MPS I families
- UnitedHealthcare — Lysosomal Storage Disease enzyme replacement policies
- Aetna — Clinical Policy Bulletins for enzyme replacement therapies
- FDA National Drug Code Directory
About this page
We maintain this page as a living reference. Medicare ASP pricing is bound to our underlying CareCost data layer and refreshes automatically when CMS publishes new quarterly files. Coding and policy content is reviewed at least quarterly and updated whenever a source document changes.
Found an error? Email hello@carecostestimate.com.
Refresh cadence
| Element | Cadence | How it's refreshed |
|---|---|---|
| Medicare ASP pricing | Quarterly | Auto-bound to CareCost ASP layer; updates on CMS file release. |
| Payer policies (UHC, Aetna, BCBS) | Semi-annual | Manual review against published payer policy documents. |
| HCPCS / CPT / modifier rules | Annual | Reviewed against CMS HCPCS quarterly files and AMA CPT releases. |
| NDC, dosing, FDA label, MPS subtype assignments | Event-driven | Tied to manufacturer document version and FDA label revision date. Aldurazyme's label is stable; MPS I subtype dosing is unchanged. |
Reviewer
Pending SME review. This page is staff-authored from primary sources (FDA label, CMS,
Sanofi/BioMarin, payer documents — all linked above). Editorial review in progress. Until that review is complete, treat this as a draft reference and verify each cited
source for high-stakes claims, particularly around the boxed-warning anaphylaxis protocol.
Change log
- — Initial publication. ASP data: Q2 2026 ($39.954 per 0.1 mg unit). Manufacturer source: Sanofi Patient Connection / BioMarin RareConnections 2026 materials. FDA label verified May 2026 (BLA 125058, original approval April 30, 2003). Boxed warning for anaphylaxis prominently featured. MPS treatment landscape table includes the four other ERTs (Elaprase, Vimizim, Naglazyme, Mepsevii).
Methodology
Every claim on this page is sourced inline. Pricing reflects the current CMS Part B Drug ASP Pricing File. Payer policies are read directly from each payer's published medical/pharmacy policy documents. The boxed warning text and premedication guidance are taken from the FDA-approved label. We do not paraphrase from billing-software vendor blogs.