CurrentCignaPolicy N/A

Naglazyme (galsulfase) prior authorization form — coverage criteria

Form and requirements for prior authorization of Naglazyme (galsulfase) for treatment of Mucopolysaccharidosis Type VI (Maroteaux-Lamy syndrome); intended for prescribers and provider staff submitting requests to Cigna Pharmacy Services.

Policy Summary

PayerCigna

PolicyNaglazyme (galsulfase) prior authorization form — coverage criteria

Policy CodePolicy N/A

Change TypeNo material change

Effective Date

Next Review Date

Key ActionPrior authorization is required; submit completed form with dose, frequency, duration, ICD-10, patient weight, prescriber specialty/consultation details, and supporting documentation (enzyme assay or molecular genetic testing, chart notes, labs).

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

5 business daysstandard response time

800.88.CIGNAurgent prior auth phone

855.840.1678fax to submit form

Accredopreferred specialty pharmacy

Coverage Criteria for Naglazyme (galsulfase)

Initial Therapy

Covered when ALL of the following are met

Initial authorization requirements: 1) Patient has a diagnosis of Mucopolysaccharidosis Type VI (Maroteaux-Lamy Syndrome); 2) Diagnosis documented by either: laboratory demonstration of deficient ARSB (N-acetylgalactosamine 4-sulfatase) activity in leukocytes or fibroblasts OR molecular genetic testing (with documentation of biallelic pathogenic or likely pathogenic ARSB variants if genetic testing performed); 3) Medication order includes medication name (Naglazyme), dose, frequency, duration, current patient weight (lb/kg), and ICD-10 code; 4) Medication is prescribed by or in consultation with a geneticist, endocrinologist, metabolic disorder subspecialist, or a physician who treats lysosomal storage disorders; 5) Supportive documentation (genetic testing report, chart notes, laboratory test results) is attached to the request.

Form lists required items and attestation

Continuation Therapy

Covered when ALL of the following are met

Continuation authorization requirements: 1) Indicate 'Continuation of therapy' on the form (select continuation); 2) Provide additional pertinent information including prior therapy, disease stage, performance status, and names/doses/schedules of any concurrent agents; 3) Supply updated clinical information and supportive documentation as requested to demonstrate ongoing clinical benefit; 4) Include current medication order details (dose, frequency, duration), patient weight, and ICD-10 code.

Policy Summary

PayerCigna

PolicyNaglazyme (galsulfase) prior authorization form — coverage criteria

Policy CodePolicy N/A

Change TypeNo material change

Effective Date

Next Review Date

SourceLink

On This Page

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Continuation-specific documentation requested on form

The prior authorization form is specific to treatment of Mucopolysaccharidosis Type VI (Maroteaux‑Lamy syndrome). If the patient does not have a diagnosis of MPS VI and no supportive documentation is provided, the form indicates that therapy for this indication is not appropriate and authorization is unlikely to be granted. Providers should therefore ensure the submitted diagnosis matches MPS VI and attach the required diagnostic evidence when requesting coverage for galsulfase (Naglazyme).

Requests that do not include required diagnostic confirmation or evidence of specialist involvement may be considered incomplete or not medically necessary. The form requires either a laboratory demonstration of deficient ARSB enzyme activity in leukocytes or fibroblasts or molecular genetic testing (with documentation of biallelic pathogenic or likely pathogenic ARSB variants when genetic testing is used), and asks whether the medication is prescribed by or in consultation with an appropriate specialist (geneticist, endocrinologist, metabolic disorder subspecialist, or physician experienced in lysosomal storage disorders). Lack of these items and accompanying supportive documentation (chart notes, lab reports, genetic reports) may lead to denial or delay.

Initial Authorization Requirements

Initial Therapy — Initial authorization requirements

Initial authorization requirements

Initial: Provide a diagnosis of Mucopolysaccharidosis Type VI confirmed by either ARSB enzyme activity testing or molecular genetic testing (with documentation of biallelic pathogenic or likely pathogenic ARSB variants if genetic testing performed); indicate prescriber specialty or consultation; include medication requested (Naglazyme), dose, frequency, duration, current weight (lb/kg), and ICD-10; attach supporting documentation (genetic reports, chart notes, labs).

Required on form

Continuation Authorization Requirements

Continuation Therapy — Requirements to continue therapy

Requirements to continue therapy

Continuation: Provider must indicate 'Continuation of therapy' on the form and supply updated clinical information including prior therapy, disease stage, performance status, and names/doses/schedules of any concurrent agents; provide current medication details (dose, frequency, duration), patient weight, and ICD-10; ongoing benefit may be subject to reviewer request and must be demonstrated with attached supporting documentation.

See Clinical Information section

Coding and Billing Information

Medicationmixed

Naglazyme vial

Medication requested (brand name listed on form)

inv-06: Weight — provider to supply current weight in lb/kg

Weight requirementProvider must supply the patient's current weight on the form (blank labeled 'What is your patient's current weight? ___________________ lb/kg').

Units acceptedWeight may be reported in pounds (lb) or kilograms (kg) as indicated on the form.

PurposeWeight is required to calculate dose and document medication order (dose, frequency, duration) for Naglazyme.

Provider Actions and Submission Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Naglazyme. Include dose, frequency, duration, ICD-10 diagnosis, and patient weight. Complete all required patient and provider identification fields (all items marked with *). Incomplete or missing fields may prevent timely review or cause the request to be returned.

Indicate whether request is new start or continuation.
Provide urgency (standard or urgent) if applicable.

Note

Site-of-Care Consideration

Infusions should be scheduled in the least intensive, medically appropriate setting when possible. Indicate planned site of administration (home, physician office, hospital outpatient, or other) and whether the patient is a candidate for redirection to an alternate site or for home infusion.

Per some Cigna plans infusion MUST occur in the least intensive, medically appropriate setting.

Documentation Required

Required Supportive Documentation

Attach required supportive documentation with the prior authorization request. This medication requires documentation such as laboratory testing, genetic testing reports, and pertinent clinical chart notes to establish the diagnosis and medical necessity.

Laboratory demonstration of deficient N-acetylgalactosamine 4-sulfatase (arylsulfatase B) activity in leukocytes or fibroblasts.
Molecular genetic testing showing biallelic pathogenic or likely pathogenic ARSB variants (if available).
Documentation that prescribing or consulting clinician is a geneticist, endocrinologist, metabolic disorder specialist, or lysosomal storage disorder specialist.
Additional clinical information: prior therapies, disease stage, performance status, and current medications/dosing schedule.

Documentation Required

Complete Required Form Fields

Complete all asterisked patient and provider identification fields on the form (e.g., physician name, specialty, DEA/NPI/TIN, patient name, Cigna ID, date of birth, contact information). Missing required clinical documentation or incomplete form fields may delay review or result in denial.

Patient and provider identification fields marked with * must be completed.
Prescriber signature and date required on submission.
Save time by submitting via CoverMyMeds or SureScripts when available.

Denial Risk

Missing Required Clinical Documentation — Denial Risk

Lack of the required supportive documentation (lab results, genetic report, specialist consultation notes) or incomplete clinical justification may trigger denial of the request.

If genetic testing is not provided, include explanation and other confirmatory documentation.
Attach charts, lab reports, and any prior authorization notes that substantiate ongoing therapy for continuation requests.

Site of Care and Infusion Setting

Note

Choose least intensive appropriate site: home, physician office, hospital outpatient, or infusion center; specialty pharmacy noted

Select and document the planned administration setting; infusion should occur in the least intensive, medically appropriate setting (home, physician's office, hospital outpatient, infusion center). The form notes Accredo as the nationally preferred specialty pharmacy for dispensing.

Mark planned administration site: Patient's Home, Physician's Office, Hospital Outpatient, or Other
If obtaining medication via specialty pharmacy, consider Accredo (nationally preferred)

Step	Requirement / Instruction
1	Consider redirection to an alternate infusion site (patient's home, physician's office, hospital outpatient, or other) with assistance from a Specialty Care Options Case Manager when appropriate. Document medical necessity rationale if declining redirection.

Definitions and Diagnostic Tests

inv-13: ARSB enzyme activity testing — Lab test demonstrating deficient arylsulfatase B activity in leukocytes or fibroblasts

DefinitionLaboratory test demonstrating deficient N-acetylgalactosamine 4-sulfatase (arylsulfatase B) activity in leukocytes or fibroblasts.

Use on formForm asks whether diagnosis is documented by this laboratory test and requests supportive documentation be provided.

Role in confirmationThis enzyme assay is an accepted method to document Mucopolysaccharidosis Type VI (MPS VI) on the prior authorization form.

inv-14: Genetic confirmation of MPS VI — Molecular genetic testing demonstrating biallelic pathogenic or likely pathogenic ARSB gene variants

DefinitionMolecular genetic testing demonstrating biallelic pathogenic or likely pathogenic ARSB gene variants.

Form requirementThe form requests indication if molecular genetic testing was performed and asks whether documentation shows biallelic pathogenic or likely pathogenic ARSB variants (Yes/No).

Use for diagnosisGenetic confirmation is an accepted method to document MPS VI diagnosis for prior authorization when supportive genetic reports are attached.

Background

Mucopolysaccharidosis Type VI (Maroteaux‑Lamy syndrome) is a lysosomal storage disorder caused by deficiency of arylsulfatase B (ARSB). Galsulfase (Naglazyme) is an enzyme replacement therapy indicated for this condition. The prior authorization form requires documentation confirming the diagnosis—either an ARSB enzyme activity assay demonstrating deficient N‑acetylgalactosamine 4‑sulfatase activity in leukocytes or fibroblasts, or molecular genetic testing (with documentation of biallelic pathogenic or likely pathogenic ARSB variants when applicable)—and notes that supportive materials such as genetic reports, chart notes, and lab results must be attached to the request.