CurrentCentenePolicy CP PHAR.161

Galsulfase (Naglazyme)

Clinical coverage policy for galsulfase (Naglazyme) describing FDA indication (MPS VI), initial and continuation approval criteria, dosing limits, approvals durations by line of business, contraindications/boxed warning, required documentation, and billing code guidance for Centene-affiliated health plans.

Policy Summary

PayerCentene

PolicyGalsulfase (Naglazyme)

Policy CodePolicy CP PHAR.161

Change Type2Q 2025 annual review: clarified (boxed warning added)

Effective Date02.01.16

Next Review Date

Key ActionProvider must submit office chart notes, lab results or other clinical information supporting diagnosis confirmation (enzyme assay or DNA testing) and member's current weight; documentation of clinical response required for renewals.

POLICY UPDATE CHANGES

2Q 2025 annual review: added new Boxed Warning per label; references reviewed and updated.

2Q 2024 annual review: no significant changes; references reviewed and updated.

2Q 2022 annual review: added requirement for documentation of member's purposes; references reviewed and updated.

1FDA-approved Indications

1 mg/kg/weekMaximum Dose

J1458Primary HCPCS Code

Boxed warningSerious Safety Warning

Coverage Summary

Galsulfase (Naglazyme) is covered with criteria for the treatment of mucopolysaccharidosis VI (MPS VI) per the FDA-labeled indication across Centene-affiliated Commercial, HIM, and Medicaid lines of business. Coverage follows the policy's initial and continued therapy criteria (including diagnostic confirmation, weight documentation, and dosing limits). A boxed warning for risk of life‑threatening hypersensitivity reactions is noted in the latest review and included in the policy.

Quick facts & thresholds

Maximum dose1 mg/kg/week

Primary HCPCS CodeJ1458

FDA-approved indications1 (MPS VI)

Serious Safety WarningBoxed warning (risk of life-threatening hypersensitivity reactions)

Shown clinical benefitImproves walking and stair-climbing capacity

Vial strength5 mg/5 mL vial

Initial Therapy Criteria

Initial Approval Criteria

Naglazyme is medically necessary when ALL of the following are met:

ALL of the following

Diagnosis of MPS VI
- ONE of
  - Enzyme assay demonstrating a deficiency in N-acetylgalactosamine 4-sulfatase (arylsulfatase B) activity
  - DNA testing confirming MPS VI
Weight documentation: Documentation of member's current weight (in kg)

Continuation Therapy Criteria

Continued Therapy (A: MPS VI)

Continued therapy for MPS VI is authorized when ALL of the following are met:

ALL of the following

Continuity requirement: Member is currently receiving medication via Centene benefit or member has previously met initial approval criteria
continuity requirement
Response to therapy: Member is responding positively to therapy as evidenced by improvement in the individual member's MPS VI manifestation profile (examples in Appendix D)
Weight documentation: Documentation of member's current weight (in kg)

Unapproved Indications / Exclusions

Applicable Codes

HCPCS - Drug J-codeHCPCSCovered

J1458

Injection, galsulfase, 1 mg

Provider Actions & Billing

Prior Authorization

Documentation required for prior authorization

Providers must submit office chart notes, laboratory results, or other clinical information supporting diagnosis confirmation (enzyme assay or DNA testing) and documentation of the member's current weight. Documentation of clinical response is required for renewals.

Affected code: J1458
Required documents: office chart notes
Required documents: lab results (e.g., enzyme assay or DNA testing)
Required documents: current weight (kg)
Required for renewals: documentation of clinical response

Billing Rule

Dose billing unit

Clinical Evidence

Evidence summarized in this policy references the FDA Prescribing Information: Naglazyme Prescribing Information, BioMarin; September 2024 (accessed March 10, 2025). The label and background evidence report that Naglazyme has demonstrated improvements in walking and stair‑climbing capacity in patients with MPS VI.

Background

Background: Galsulfase (Naglazyme) is an enzyme replacement therapy indicated for mucopolysaccharidosis VI (MPS VI; Maroteaux‑Lamy syndrome). This policy applies to Centene-affiliated Commercial, HIM, and Medicaid lines of business. Definition: MPS VI = Mucopolysaccharidosis VI (Maroteaux‑Lamy syndrome).

Revision History

05.25.2025materialLatest

2Q 2025 annual review: added new Boxed Warning per label; references reviewed and updated.

05.24.2024revised

2Q 2024 annual review: no significant changes; references reviewed and updated.

02.26.2022revised

2Q 2022 annual review: template changes applied and requirement added for documentation of member's purposes; references reviewed and updated.

Policy Summary

PayerCentene

PolicyGalsulfase (Naglazyme)

Policy CodePolicy CP PHAR.161

Change Type2Q 2025 annual review: clarified (boxed warning added)

Effective Date02.01.16

Next Review Date

On This Page

Age note: Age — implied applicable per FDA label; policy references age (example text shows '23 months; Age')

Dose limit: Dose does not exceed 1 mg/kg per week<= 1 mg/kg/week

Provider documentation: Provider must submit documentation (office chart notes, lab results or other clinical information) supporting that member has met all approval criteria

Required documentation for approval

Dose limit: Dose does not exceed 1 mg/kg per week<= 1 mg/kg/week

Continued Therapy (B: Other diagnoses/indications)

Other diagnoses/indications (must meet one of the following):

ANY of the following

Formulary/PDL uses: For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid): follow the no coverage criteria policy for the relevant line of business (CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, CP.PMN.255 for Medicaid)
Non-formulary uses: For drugs NOT on the formulary or PDL: follow the non-formulary policy for the relevant line of business (CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, CP.PMN.16 for Medicaid)
Off-label referral: If requested use is not listed and criterion 1 does not apply: refer to the off-label use policy for the relevant line of business (CP.CPA.09 commercial, HIM.PA.154 marketplace, CP.PMN.53 Medicaid)

Bill HCPCS code J1458 per 1 mg of galsulfase as described in the HCPCS descriptor. Ensure the ordered dose does not exceed 1 mg/kg per week.

Bill J1458 per 1 mg of galsulfase (HCPCS descriptor)
Verify total weekly dose ≤ 1 mg/kg/week prior to billing
Reference most current professional coding guidance when submitting claims

Documentation Required

Weight documentation

The member's current weight in kilograms must be documented at the time of initial approval and at each renewal to support dosing and continued eligibility.

Denial Risk

Non-FDA indications

Requests for non‑FDA approved indications will be denied unless there is sufficient documentation of efficacy and safety according to the applicable off‑label use policy or evidence of coverage documents (see referenced off‑label policy codes).

Relevant off‑label policy codes: CP.CPA.09 (commercial), HIM.PA.154 (marketplace), CP.PMN.53 (Medicaid)