CurrentBlue Cross Blue Shield - TennesseePolicy N/A

Naglazyme (galsulfase) — Coverage Criteria and Prior Authorization

Coverage criteria and prior authorization requirements for Naglazyme (galsulfase) to treat Mucopolysaccharidosis VI (MPS VI) for BlueCross BlueShield of Tennessee members.

Policy Summary

PayerBlue Cross Blue Shield - Tennessee

PolicyNaglazyme (galsulfase) — Coverage Criteria and Prior Authorization

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateID_CHS_2026

Next Review DateN/A

Key ActionSubmit prior authorization with baseline chart notes and confirmatory enzyme assay or genetic testing; authorization may be granted for up to 12 months when criteria are met.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsauthorization period

MPS VIcovered indication

FDA-approved onlyother indications

Specialist requiredprescriber requirement

Coverage and Medical Necessity Criteria

Initial Therapy

Policy Summary

PayerBlue Cross Blue Shield - Tennessee

PolicyNaglazyme (galsulfase) — Coverage Criteria and Prior Authorization

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateID_CHS_2026

Next Review DateN/A

Key ActionSubmit prior authorization with baseline chart notes and confirmatory enzyme assay or genetic testing; authorization may be granted for up to 12 months when criteria are met.

SourceLink

On This Page

Initial Therapy for MPS VI: Member exhibits clinical signs and symptoms of MPS VI at baseline (examples: decreased growth velocity, coarse facial features, skeletal deformities, frequent upper-airway infections, enlarged liver and spleen, hearing loss, joint stiffness, coarse hair).

Examples listed in policy

Diagnostic Confirmation: Diagnosis of MPS VI confirmed by enzyme assay demonstrating deficiency of N-acetylgalactosamine-4-sulfatase (arylsulfatase B) enzyme activity or by genetic testing.

Continuation Therapy

Covered when ALL of the following are met

Continued Indication: Member is receiving treatment for an indication listed in the Coverage Criteria (MPS VI).

Clinical Benefit: Documentation of clinically positive response to therapy, defined as improvement, stabilization, or slowing of disease progression (examples: growth rate, 12-minute walk test, 3-minute stair climb test, urinary GAG levels).

Authorization may be granted for 12 months.

Clinical Response Measures and Coding Notes

Clinical response measures — improvement, stabilization, or slowing of disease progression

Clinical response definitionClinically positive response defined as improvement, stabilization, or slowing of disease progression

Example measure — growthChange in growth rate documented in chart notes

Example measure — 12-minute walk testImprovement, stabilization, or slowing of decline on the 12-minute walk test

Example measure — 3-minute stair climb testImprovement, stabilization, or slowing of decline on the 3-minute stair climb test

Example measure — urinary biomarkerUrinary glycosaminoglycan (GAG) levels showing improvement, stabilization, or slowing of disease progression

Prior Authorization, Documentation, and Provider Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Naglazyme (galsulfase) for treatment of MPS VI. Authorization of up to 12 months may be granted for initial therapy and for continuation when the coverage criteria are met.

Authorization duration: up to 12 months for initial and continuation requests

Documentation Required

Required Documentation

Initial and continuation requests must include supporting clinical documentation. Include chart notes documenting baseline clinical signs/symptoms and confirmatory testing for diagnosis, and for continuation requests include documentation of clinically positive response to therapy.

Initial requests: chart notes documenting baseline clinical signs/symptoms (e.g., decreased growth velocity, coarse facial features, skeletal deformities, frequent upper-airway infections, enlarged liver and spleen, hearing loss, joint stiffness, coarse hair).
Initial requests: confirmatory diagnostic testing — N-acetylgalactosamine-4-sulfatase (arylsulfatase B) enzyme assay OR genetic testing results supporting MPS VI diagnosis.
Continuation requests: chart notes documenting clinically positive response (improvement, stabilization, or slowing of disease progression) — examples: growth rate, 12-minute walk test, 3-minute stair climb test, urinary glycosaminoglycan (GAG) levels.

Step Therapy

No step therapy sequence is specified for this policy.

Diagnostic Confirmation and Definitions

Diagnostic confirmation — MPS VI

Required diagnostic test — enzyme assayEnzyme assay demonstrating deficiency of N-acetylgalactosamine-4-sulfatase (arylsulfatase B) activity

Alternative diagnostic test — genetic testingMolecular genetic testing consistent with MPS VI

Context — indicationDiagnosis required for FDA‑approved indication for Naglazyme (MPS VI)

Documentation requirementConfirmatory test results should be included in initial request chart notes

OpenPayer

Naglazyme (galsulfase) — Coverage Criteria and Prior Authorization

Coverage and Medical Necessity Criteria

Clinical Response Measures and Coding Notes

Prior Authorization, Documentation, and Provider Requirements

Diagnostic Confirmation and Definitions

Clinical Background