ModifiedmhnPolicy CP.PHAR.161

Clinical Policy: Galsulfase (Naglazyme)

Defines medical necessity, authorization criteria, dosing limits, and coverage durations for Naglazyme (galsulfase) for members diagnosed with mucopolysaccharidosis VI and other indications as governed by Centene-affiliated health plans.

Policy Summary

Payermhn

PolicyClinical Policy: Galsulfase (Naglazyme)

Policy CodePolicy CP.PHAR.161

Change TypeSafety updatedocumentation requirement

Effective Date02.01.16

Next Review Date

Key ActionProvider must submit documentation (diagnosis, current weight in kg, and clinical evidence) to support that the member meets approval criteria.

SourceLink

POLICY UPDATE CHANGES

Added new Boxed Warning per label.

Requirement for documentation of member's current weight for dose calculation purposes.

Policy identifier revised from HIM.PHAR.21 to HIM.PA.154.

1 mg/kg/weekMaximum dose

6 monthsInitial approval

J1458HCPCS

5 mg/5 mLPresentation

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Coverage Criteria

Initial Therapy

Covered when ALL of the following are met

Initial MPS VI criteria: 1. Diagnosis of MPS VI (Maroteaux-Lamy syndrome) confirmed by ONE of the following: (a) enzyme assay demonstrating deficiency of N-acetylgalactosamine 4-sulfatase (arylsulfatase B) activity OR (b) DNA testing; 2. Age ≥ 3 months; 3. Documentation of member's current weight (in kg); 4. Dose does not exceed 1 mg/kg per week.1 mg/kg per week

Continuation Therapy

Covered when ALL of the following are met

Continuation MPS VI criteria: 1. Member currently receiving medication via Centene benefit or previously met initial approval criteria OR member is receiving medication and enrolled in continuity of care as applicable; 2. Member is responding positively to therapy as evidenced by improvement in the individual's MPS VI manifestation profile (see Appendix D for examples); 3. Documentation of member's current weight (in kg); 4. If request is for a dose increase, the new dose does not exceed 1 mg/kg per week.1 mg/kg per week

Approval duration: Medicaid/HIM - 12 months; Commercial - 6 months or to the member's renewal date, whichever is longer. See Appendix D for examples of clinical response.

Other Indications

For requests not meeting the above

Other indications handling: 1. If the drug has had a label change within the last 6 months that is not yet reflected in this policy, refer to the appropriate formulary or no-coverage/non-formulary policy for the relevant line of business (see policy references). OR 2. If the requested use is not specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) and criterion 1 does not apply, refer to the off-label use policy for the relevant line of business.

Refer to CP.CPA.190, HIM.PA.33, CP.PMN.255, HIM.PA.103, CP.PMN.16, CP.CPA.09, HIM.PA.154, CP.PMN.53 as applicable.

This policy does not authorize coverage for non‑FDA approved indications that are not specifically addressed in the policy unless the provider supplies sufficient documentation of efficacy and safety in accordance with the applicable off‑label use policy (for Commercial: CP.CPA.09; Health Insurance Marketplace: HIM.PA.154; Medicaid: CP.PMN.53) or as allowed by the member’s evidence of coverage documents.

Where a state Medicaid program’s coverage provisions conflict with this clinical policy, the state Medicaid provisions take precedence over the policy to the extent of the conflict. Providers should follow the applicable state Medicaid requirements when administering benefits for Medicaid members.

Requests for treatment of indications that are not FDA‑approved and are not addressed in this policy will be denied unless the request includes sufficient documentation of efficacy and safety consistent with the applicable off‑label use policy (see CP.CPA.09, HIM.PA.154, CP.PMN.53) or the member’s evidence of coverage. Documentation such as clinical rationale, supporting literature, and relevant clinical records should be provided to substantiate off‑label use.

Coding and Dose Limits

HCPCS CodesHCPCS

J1458

Injection, galsulfase, 1 mg

HCPCS CodesHCPCS

J1458

Injection, galsulfase, 1 mg

inv-09: Maximum dose — key/value showing 1 mg/kg per week dosing limit

Maximum weekly doseDosing limit: 1 mg/kg per week (dose does not exceed 1 mg/kg/week)

Dosing frequencyAdministered IV once weekly per dosing regimen for MPS VI (1 mg/kg IV once weekly).

Applies toInitial and continuation therapy criteria for MPS VI include the 1 mg/kg/week maximum.

inv-10: Member weight documentation — key/value noting requirement for current weight for dose calculation

Required documentationProvider must document the member's current weight (in kg) for dose calculation.

PurposeUsed to verify weekly dosing does not exceed the 1 mg/kg limit.

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior Authorization Required. Prior authorization is required for Naglazyme (galsulfase) prior to administration. Approval durations: Initial approvals — Medicaid/HIM: 6 months; Commercial: 6 months or to the member's renewal date, whichever is longer. Continuation approvals — Medicaid/HIM: 12 months; Commercial: 6 months or to the member's renewal date, whichever is longer.

Initial approval durations: Medicaid/HIM – 6 months; Commercial – 6 months or to the member's renewal date, whichever is longer.
Continuation approval durations: Medicaid/HIM – 12 months; Commercial – 6 months or to the member's renewal date, whichever is longer.

Billing Rule

HCPCS Coding and Prior Authorization Note

HCPCS coding guidance: Providers should reference current professional coding guidance prior to claim submission. The HCPCS code commonly used for galsulfase is J1458 — Injection, galsulfase, 1 mg. Inclusion here is informational and does not guarantee coverage.

Background

Mucopolysaccharidosis VI (MPS VI, Maroteaux‑Lamy syndrome) is a lysosomal storage disorder caused by a deficiency of the enzyme arylsulfatase B, which leads to accumulation of glycosaminoglycans in multiple tissues. Clinical manifestations vary but commonly include reduced endurance and mobility, respiratory compromise, organomegaly, and progressive somatic involvement. Enzyme replacement with galsulfase (Naglazyme) supplies the deficient hydrolytic lysosomal enzyme and has been shown to improve functional measures such as walking and stair‑climb performance and other disease manifestations; monitoring of individual clinical response is required for continuation of therapy (see Appendix D for examples).

Definitions

inv-20: Naglazyme (galsulfase) — define the drug and indication

DrugGalsulfase (brand name: Naglazyme)

Drug classHydrolytic lysosomal glycosaminoglycan-specific enzyme (enzyme replacement therapy)

IndicationIndicated for treatment of mucopolysaccharidosis VI (MPS VI; Maroteaux-Lamy syndrome).

inv-21: Clinical policy — define what a clinical policy is and its limitations

DefinitionClinical policy: a guide to medical necessity used to assist in making coverage decisions and administering benefits.

LimitationsDoes not constitute a contract or guarantee of payment; not medical advice or a directive for clinical practice.

Initial Therapy Criteria

Initial Therapy

Initial therapy criteria and dosing

Initial therapy nodes: Diagnosis confirmed by enzyme assay or DNA testing; age ≥ 3 months; documentation of current weight (kg); dosing 1 mg/kg IV once weekly, not to exceed 1 mg/kg per week.1 mg/kg per week

Vial size 5 mg/5 mL

Continuation Therapy Criteria

Continuation Therapy Requirements

Continuation approvals require evidence of positive response

Continuation evidence of response: Member is responding positively to therapy as evidenced by improvement in MPS VI manifestations (examples include improved 12-minute walk distance, 3-minute stair climb rate, endurance, respiratory symptoms, and reduction in organomegaly).

See Appendix D for examples of clinical response.

Step Therapy

Step	Guidance
If indication is not listed	Follow the applicable formulary/non‑formulary policy or the off‑label use policy for the member's line of business (examples: CP.CPA.190, HIM.PA.103, CP.PMN.16 for non‑formulary; CP.CPA.09, HIM.PA.154, CP.PMN.53 for off‑label).

Quantity Limits

inv-25: Galsulfase (Naglazyme) — dose calculated by weight; vial size and reminder to ensure weekly dose does not exceed 1 mg/kg

Dose calculationDose is calculated by patient weight: 1 mg/kg per week (1 mg/kg IV once weekly for MPS VI).

Vial size / presentationProduct available as vial: 5 mg/5 mL.

Dose check reminderEnsure total weekly dose based on current weight does not exceed 1 mg/kg/week when preparing doses from 5 mg vials.

Site of Care

Note

No site‑of‑care restrictions specified

This portion of the policy does not specify any site‑of‑care restrictions; infusion center administration is addressed without site‑of‑care limitations in this policy.

No site‑of‑care restrictions are specified in this portion of the policy.