CurrentAvMedPolicy Pub. 100-2

Naglazyme (galsulfase) IV infusion prior authorization

Defines AvMed medical prior authorization and continuation criteria for Naglazyme (galsulfase) IV infusion for treatment of Mucopolysaccharidosis VI (MPS VI) for AvMed members. Applies to providers requesting medical (infusion) coverage, including specialty pharmacies and infusion sites.

Policy Summary

PayerAvMed

PolicyNaglazyme (galsulfase) IV infusion prior authorization

Policy CodePolicy Pub. 100-2

Change TypeNo material change

Effective Date

Next Review Date

Key ActionSubmit a completed and signed prior authorization form with required diagnostic labs and weight documentation to request coverage.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

1 mg/kg weeklymax dose/frequency

6 monthsinitial auth

6 monthsrenewal auth

≥5 yrsmin age

uGAG ≥50%↓response threshold

Coverage Criteria for Naglazyme (galsulfase)

inv-01: Initial Authorization

Covered when ALL of the following are met

Provider qualifications: Prescribing provider is a metabolic geneticist or other specialist in treatment of this disease

checkbox on form

Age: Member is 5 years of age or older>=5 years

must submit chart notes documenting member's current weight

Definitive diagnosis

Diagnostic evidence (one required)

Policy Summary

PayerAvMed

PolicyNaglazyme (galsulfase) IV infusion prior authorization

Policy CodePolicy Pub. 100-2

Change TypeNo material change

Effective Date

Next Review Date

Key ActionSubmit a completed and signed prior authorization form with required diagnostic labs and weight documentation to request coverage.

SourceLink

On This Page

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inv-02: Continuation Authorization

Covered when ALL of the following are met

Ongoing eligibility: Member continues to meet all initial authorization criteria

checkbox on form

Weight documentation: Member's current weight must be noted and chart notes documenting weight must be submitted

required

Safety: Absence of unacceptable toxicity from the drug (e.g., anaphylaxis, hypersensitivity, immune-mediated reactions, acute respiratory complications, acute cardiorespiratory failure, severe infusion reactions, spinal or cervical cord compression)

must be documented

Clinical response: Member has had a clinically significant response to treatment since last approval as defined by reduction in uGAG by >=50% from baseline or maintenance at >=50% below baseline, AND improvement or stability of pulmonary function testing (e.g., FEV1, FVC), AND improvement or stability of 12-minute walk test from last approval OR improvement or stability of 3-minute stair climb test from last approvaluGAG reduction >=50% or maintenance

combination of objective measures required; all documentation must be provided

Members with Multiple Sulfatase Deficiency (MSD) are excluded from the diagnostic enzyme activity pathway for MPS VI. The diagnostic enzyme pathway requires demonstration of arylsulfatase B (ASB/ARSB) deficiency together with normal activity of a different sulfatase; a finding of multiple sulfatase deficiencies would therefore preclude use of isolated enzyme activity as confirmatory diagnostic evidence for MPS VI and should follow an alternative diagnostic pathway.

For prior authorization of Naglazyme (galsulfase) IV infusion, providers must submit laboratory documentation supporting the chosen diagnostic route (molecular ARSB mutation detection OR ASB enzyme activity <10% of the lower limit of normal in cultured fibroblasts or isolated leukocytes) and include evidence of normal activity of a different sulfatase to exclude MSD when enzyme testing is used.

inv-13: Confirmation of MPS VI diagnosis through detection of pathogenic ARSB mutations

Accepted diagnostic evidence: Detection of pathogenic mutations in the ARSB gene by molecular genetic testing qualifies as diagnostic confirmation of MPS VI

Molecular genetic testing for ARSB is accepted as diagnostic evidence for coverage of enzyme replacement therapy; submit laboratory result documentation

Alternative diagnostic pathway: Alternatively, ARSB enzyme activity <10% of the lower limit of normal in cultured fibroblasts or isolated leukocytes plus normal activity of a different sulfatase and elevated urinary GAG qualifies as diagnostic confirmationASB activity <10% of lower limit of normal

submit enzyme activity report, different sulfatase activity, and uGAG result; members with Multiple Sulfatase Deficiency are excluded from the enzyme activity pathway

Required Provider Actions and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for Naglazyme (galsulfase). The prescribing physician must sign and clearly print their name on the PA request form (preprinted stamps are not valid). The completed form and all supporting documentation must be faxed to 1-844-668-1550. If information provided is not complete, correct, or legible, authorization may be delayed or denied.

Fax completed PA form to 1-844-668-1550
Prescribing physician signature and printed name required (no preprinted stamps)
Include phone and fax numbers on the form

Denial Risk

Incomplete Documentation May Delay or Deny Request

Authorization may be delayed or denied if the request is incomplete, incorrect, or illegible. Provide all requested documentation including lab results, diagnostics, and chart notes. Ensure weight (with date obtained) and other required fields on the form are completed.

Incomplete/illegible forms or missing supporting documentation can result in delays or denials
Ensure all fields (including weight and date weight obtained) are completed
Provide clear chart notes and laboratory reports to support each criterion checked on the form

Documentation Required

Required Diagnostic and Baseline Documentation

Submit required diagnostic and baseline documentation to establish a definitive diagnosis and baseline measures. All listed items must be provided to support initial and continuation requests.

Molecular genetic testing showing pathogenic ARSB mutations OR Arylsulfatase B (ASB) enzyme activity <10% of the lower limit of normal in cultured fibroblasts or isolated leukocytes
Normal enzyme activity of a different sulfatase (to exclude Multiple Sulfatase Deficiency)
Elevated urinary glycosaminoglycan (uGAG) level (dermatan sulfate or chondroitin sulfate) above the reference lab upper limit
Baseline 12-minute walk test (12-MWT) OR documented 3-minute stair climb test
Baseline pulmonary function tests (e.g., FEV1, FVC)
Baseline laboratory value of urinary glycosaminoglycan (uGAG)

Documentation Required

Samples Not Allowed / Prior Therapy Verification

Use of samples to initiate therapy does not meet step edit/preauthorization criteria. Prior therapies and treatments will be verified via pharmacy paid claims or submitted chart notes.

Samples do not satisfy prior authorization requirements
Previous therapies will be verified through pharmacy claims or chart documentation

Definitions and Diagnostic Thresholds

inv-12: Definitive diagnosis of MPS VI

Definitive diagnostic options (molecular)Detection of pathogenic mutations in the ARSB gene by molecular genetic testing

Definitive diagnostic options (biochemical)Arylsulfatase B (ASB) enzyme activity <10% of the lower limit of normal in cultured fibroblasts or isolated leukocytes

Sulfatase specificity requirementNormal enzyme activity of a different sulfatase (to exclude Multiple Sulfatase Deficiency)

Supporting biochemical evidenceElevated urinary glycosaminoglycan (uGAG) level (dermatan or chondroitin sulfate) above reference lab upper limit of normal

Covered Indications

inv-13: Covered indication

Molecular genetic testing accepted: Molecular genetic testing demonstrating detection of pathogenic ARSB mutations is accepted as diagnostic evidence for coverage of enzyme replacement therapy (Naglazyme)

Submit molecular testing report as part of prior authorization documentation

Biochemical confirmation accepted: Enzyme activity testing showing ASB activity <10% of the lower limit of normal in cultured fibroblasts or isolated leukocytes, plus normal activity of a different sulfatase and elevated urinary GAG, is also accepted as diagnostic evidenceASB activity <10% of lower limit of normal

Submit enzyme activity and uGAG lab results; MSD excluded from this pathway

Background

Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome) is a lysosomal storage disorder caused by deficiency of arylsulfatase B (ARSB). Naglazyme (galsulfase) is enzyme replacement therapy administered by intravenous infusion for patients with a confirmed diagnosis of MPS VI. Confirmation may be by detection of pathogenic ARSB gene mutations or by biochemical demonstration of markedly reduced ASB enzyme activity in appropriate specimens, together with supportive biochemical and functional baseline assessments.

Required baseline documentation for initiation includes diagnostic laboratory evidence (molecular ARSB mutation OR ASB enzyme activity <10% of the lower limit of normal in cultured fibroblasts or isolated leukocytes), demonstration of normal activity of a different sulfatase to exclude MSD when enzyme testing is used, an elevated urinary glycosaminoglycan (uGAG) level above the reference laboratory upper limit of normal, and baseline functional measures such as a 12-minute walk test or 3-minute stair climb and baseline pulmonary function tests.

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