CurrentAvMedPolicy N/A

Naglazyme (Medical)

Prior authorization form and clinical criteria for medical-benefit coverage of Naglazyme (galsulfase) IV infusion (J1458) for treatment of Mucopolysaccharidosis VI (MPS VI) including initial and continuation approval requirements, documentation and quantity limit guidance.

Policy Summary

PayerAvMed

PolicyNaglazyme (Medical)

Policy CodePolicy N/A

Change TypeNo material change

Effective Date

Next Review Date

Key ActionPrescriber must complete and sign the prior authorization form and fax to 1-844-668-1550; approval durations: initial 6 months, continuation 6 months.

SourceLink

POLICY UPDATE CHANGES

No material changes to clinical coverage criteria or policy were reported.

6 moInitial Approval Duration

6 moContinuation Approval Duration

1 mg/kg q7dMaximum Approvable Dose

>=5Minimum Age

Coverage Summary

Scope: This prior authorization form and clinical criteria cover Naglazyme (galsulfase) IV infusion (HCPCS J1458) for treatment of Mucopolysaccharidosis VI (MPS VI).

Initial Therapy Criteria (Medically Necessary)

Initial Authorization / Medically Necessary

Covered when ALL of the following are met:

ALL of the following

Provider is a metabolic geneticist or other specialist in treatment of this disease
Member is 5 years of age or older>= 5 years
Current weight must be noted and chart notes documenting member's current weight must be submitted
Definitive diagnosis of MPS VI
- ONE of
  - Detection of pathogenic mutations in ARSB gene by molecular genetic testing
    Must submit lab result documentation
  - Arylsulfatase B (ASB) enzyme activity of <10% of the lower limit of normal in cultured fibroblasts or isolated leukocytes<10% of lower limit of normal
    Must submit lab result documentation
- Member has normal enzyme activity of a different sulfatase (excluding members with Multiple Sulfatase Deficiency [MSD])
  Must submit lab result documentation
- Member has an elevated urinary glycosaminoglycan (uGAG) level (i.e. dermatan sulfate or chondroitin sulfate)above the upper limit of normal by the reference laboratory
  Must submit lab result documentation
Baseline functional and laboratory measures
- Provider has attached documented baseline 12-minute walk test (12-MWT) or 3-minute stair climb test
  Baseline performance documentation required
- Provider has attached documented baseline pulmonary function tests (e.g., FEV1, FVC; etc.)
  Baseline performance documentation required
- Provider has attached documented baseline lab value of urinary glycosaminoglycan (uGAG)
  Baseline performance documentation required

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Continuation Therapy Criteria (Medically Necessary)

Continuation Authorization / Medically Necessary

Covered when ALL of the following are met:

ALL of the following

Member continues to meet all initial authorization criteria
Member's current weight must be noted
Chart notes documenting member's current weight must be submitted
Member has absence of unacceptable toxicity from the drug
Examples: anaphylaxis or hypersensitivity reactions, immune-mediated reactions, acute respiratory complications, acute cardiorespiratory failure, severe infusion reactions, spinal or cervical cord compression; etc.
AND (response criteria: ALL of the following, with alternates)
- Reduction in uGAG level by ≥50% from baseline or maintenance of level at ≥50% below baseline>= 50% reduction or maintenance at ≥50% below baseline
- Improvement in or stability of pulmonary function testing (e.g., FEV1, FVC; etc.)
- Functional performance (either)
  - Improvement in or stability of 12-minute walk test (12-MWT) from last approval
  - Improvement in or stability of 3-minute stair climb test from last approval

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Provider Actions and Prior Authorization

Prior Authorization

Prior authorization required

Prescriber must complete and sign the prior authorization form (preprinted stamps not valid) and fax to 1-844-668-1550. Initial approvals are for 6 months; continuation approvals are for 6 months.

Documentation Required

Submission of diagnostic and baseline tests

Submit documentation of diagnostic and baseline tests listed below with the request; all documentation (including lab results, diagnostics, and chart notes) must be provided or the request may be denied.

Genetic testing showing pathogenic ARSB mutations or ASB enzyme assay results (<10% of lower limit of normal) (lab result documentation required)
Baseline urinary glycosaminoglycan (uGAG) lab value (must submit lab result)
Baseline 12-minute walk test (12-MWT) or 3-minute stair climb test (must attach documented baseline)
Baseline pulmonary function tests (e.g., FEV1, FVC) (must attach documented baseline)
Chart notes documenting member's current weight

Documentation Required

Continuation documentation

For renewal requests submit current weight, documentation of absence of unacceptable toxicity, and objective evidence of clinical response as specified below.

Current weight noted with supporting chart notes
Documentation of absence of unacceptable toxicity (e.g., anaphylaxis, hypersensitivity, immune-mediated reactions, acute respiratory or cardiorespiratory complications, severe infusion reactions, spinal/cervical cord compression)
Objective evidence of clinical response: reduction in uGAG ≥50% from baseline or maintenance at ≥50% below baseline, or stability/improvement in pulmonary function testing and 12-MWT or 3-minute stair climb

Billing Rule

Quantity limit

Quantity limit: maximum approvable dose is 1 mg/kg infused every 7 days. Billing and coding: use HCPCS J1458 (Injection, galsulfase, 1 mg).

Denial Risk

Incomplete documentation may delay or deny authorization

Authorization can be delayed or denied if required documentation (labs, diagnostics, or chart notes) is incomplete, incorrect, or illegible.

Step Therapy

Samples do not meet prior authorization

Use of samples to initiate therapy does not meet step edit/preauthorization criteria and does not satisfy prior authorization requirements.

Background

This prior authorization form establishes the clinical criteria for outpatient medical-benefit administration of Naglazyme (galsulfase) for patients with confirmed MPS VI. It requires documentation of a definitive diagnosis (genetic or enzyme confirmation), baseline functional assessments, and baseline laboratory measures prior to approval.

Diagnostic confirmation must include either detection of pathogenic ARSB gene mutations by molecular genetic testing or Arylsulfatase B (ASB) enzyme activity below diagnostic threshold, plus corroborating lab evidence such as elevated urinary glycosaminoglycan (uGAG). The form requires submission of baseline 12-minute walk test (12-MWT) or 3-minute stair climb, baseline pulmonary function tests (e.g., FEV1, FVC), baseline uGAG lab value, and chart notes documenting current weight.

The form follows Medicare Part B guidance where applicable and provides administrative instructions for prescribers (including signature and faxing to the specified prior authorization fax number). It also specifies quantity limits, approval durations (initial and continuation 6 months), and that incomplete or illegible documentation may delay or deny authorization.

uGAG: Urinary glycosaminoglycan laboratory measurement used to support diagnosis and as a treatment response biomarker (e.g., dermatan sulfate or chondroitin sulfate above the reference laboratory upper limit of normal).

ASB: Arylsulfatase B enzyme; diagnostic testing includes enzyme activity measurement (diagnostic threshold specified elsewhere in the form).

12-MWT: 12-minute walk test, a baseline functional performance test required for initial authorization and used for monitoring response on continuation.

OpenPayer

Naglazyme (Medical)

Coverage Summary

Initial Therapy Criteria (Medically Necessary)

Continuation Therapy Criteria (Medically Necessary)

Provider Actions and Prior Authorization

Applicable Codes

Background

Medicare Determinations

Revision History