CurrentEmblemHealthPolicy MG.MM.PH.31O

Vimizim (elosulfase alfa) intravenous infusion

Clinical coverage criteria for use of Vimizim (elosulfase alfa) for treatment of Mucopolysaccharidosis type IVA (Morquio A syndrome), including initial approval, renewal, dosing limits, and codes. Applies to EmblemHealth and ConnectiCare medical benefits.

Policy Summary

PayerEmblemHealth

PolicyVimizim (elosulfase alfa) intravenous infusion

Policy CodePolicy MG.MM.PH.31O

Change TypeAdded baseline assessmentsadded KS/GAG response metricdosing and age updates

Effective Date

Next Review Date

Key ActionTreating physician must submit documentation that the member meets the criteria (age, diagnostic confirmation, baseline assessments, specialist prescribing or consultation) to EmblemHealth or ConnectiCare for preauthorization review.

SourceLink

POLICY UPDATE CHANGES

Documented baseline for ONE or more of the following: endurance tests (6-MWT, T25FW, 3-MSCT), pulmonary function tests (MVV, percent predicted FVC, etc.), urine keratan sulfate (KS) or urine glycosaminoglycan (GAG) levels.

Renewal criteria: Stability or reduction in urine keratan sulfate (KS) or urine glycosaminoglycan (GAG) levels as evidence of response.

Updated dosing limits and added age restriction (patient must be at least 5 years of age).

2 mg/kgmaximum recommended dose per infusion

every 7 daysmaximum dosing frequency

12initial authorization length

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>=5minimum age for initiation

Medical Necessity Criteria for Vimizim (elosulfase alfa)

Initial Criteria Mucopolysaccharidosis Type IVA (Morquio A Syndrome)

Approve when ALL of the following are met:

ALL of the following

Patient is at least 5 years of age>= 5 years
Diagnosis confirmation (ONE of)
- Laboratory test demonstrating deficient N-acetylgalactosamine-6-sulfatase activity in leukocytes or fibroblasts
- Molecular genetic test demonstrating N-acetylgalactosamine-6-sulfatase gene mutation
- ANY of the following
  - Endurance tests (e.g., six-minute walk test [6-MWT], timed 25-foot walk test [T25FW], three-minute stair climb test [3-MSCT])
  - Pulmonary function tests (e.g., maximum voluntary ventilation [MVV], percent predicted forced vital capacity [FVC], etc.)
  - Urine keratan sulfate (KS) or urine glycosaminoglycan (GAG) levels
Vimizim is prescribed by or in consultation with a geneticist, endocrinologist, a metabolic disorder sub-specialist, or a physician who specializes in the treatment of lysosomal storage disorders

Renewal Criteria

Approve renewal when ALL of the following are met:

ALL of the following

Patient continues to meet indication-specific criteria as specified in the Initial Criteria
Absence of unacceptable toxicity from the drug (examples: anaphylaxis, hypersensitivity reactions, acute respiratory complications, spinal/cervical cord compression)
- ANY of the following
  - Stability or improvement in endurance tests (e.g., 6-MWT, T25FW, 3-MSCT)
  - Stability or improvement in pulmonary function tests (e.g., FVC)

Relevant Billing and Diagnosis Codes

Applicable HCPCS/J-codeHCPCSCovered

J1322

Injection, elosulfase alfa, 1 mg

Applicable NDCsNDCCovered

68135-0100-01

Vimizim SMG/SML Solution

Applicable ICD-10 DiagnosesICD-10Covered

E76.210

Morquio A mucopolysaccharidoses

What Providers Must Do

Prior Authorization

Submit clinical evidence for preauthorization

The treating physician must submit clinical evidence that the member meets all coverage criteria for Vimizim preauthorization, including age (≥ 5 years), diagnostic confirmation (enzyme assay or molecular genetic test), documented baseline assessments, and that the drug is prescribed by or in consultation with an appropriate specialist. Applicable billing/diagnosis codes: J1322 (Injection, elosulfase alfa, 1 mg) and E76.210 (Morquio A mucopolysaccharidoses).

Submit documentation of age ≥ 5 years and diagnostic confirmation (enzyme assay or genetic test).
Include documented baseline assessment(s) (endurance test, pulmonary function test, or urine KS/GAG).
Document specialist prescribing or consultation (geneticist, endocrinologist, metabolic disorder subspecialist, or lysosomal storage disorder specialist).

Documentation Required

Baseline and follow-up assessments required

Document baseline and follow-up results for at least one of the following to support initiation and renewal: an endurance test (e.g., 6-MWT, T25FW, 3‑MSCT), pulmonary function tests (e.g., MVV, percent predicted FVC, FVC), or urine keratan sulfate (KS) / urine glycosaminoglycan (GAG) levels.

Key Numeric and Clinical Thresholds

Dosing and Authorization Thresholds

Age>= 5 years

Dose per infusion2 mg/kg

Maximum billable units per 7 days230 mg (230 billable units) every 7 days

Authorization length12 months

Clinical Background

Vimizim (elosulfase alfa) is a recombinant human N-acetylgalactosamine-6-sulfatase indicated for patients with Mucopolysaccharidosis type IVA (Morquio A syndrome). It is produced via recombinant DNA technology in Chinese hamster ovary cells and functions as a lysosomal enzyme replacement therapy that is taken up by lysosomes and hydrolyzes sulfate from the nonreduced ends of the glycosaminoglycans keratan sulfate and chondroitin-6-sulfate. The clinical indication addressed by this policy is intravenous Vimizim for members with confirmed MPS IVA who meet the age, diagnostic confirmation, and baseline assessment requirements described in the coverage criteria.

Vimizim is administered intravenously under the medical benefit with dosing limits of up to 2 mg/kg per infusion no more frequently than once weekly (maximum billable units: 230 mg every 7 days). Coverage authorizations are issued for 12 months with renewal contingent on documentation of continued benefit and absence of unacceptable toxicity.

Defined Terms

Definitions

6-MWTSix-minute walk test — an endurance assessment measuring distance walked in six minutes.

T25FWTimed 25-foot walk test — a timed measure of gait speed over 25 feet.

3-MSCTThree-minute stair climb test — a measure of endurance and functional capacity.

KSKeratan sulfate

GAGGlycosaminoglycan

Policy Revision Timeline

2025-02-06Annual ReviewLatest

Initial criteria — added documented baseline assessments (endurance tests, pulmonary function tests, urine KS/GAG); Renewal criteria — added stability or reduction in urine KS/GAG levels.

2024-01-02Annual Review

Updated dosing limits and added age restriction (patient must be at least 5 years of age); added renewal criteria.

2023-04-07Template transfer/retirement

Policy Summary

PayerEmblemHealth

PolicyVimizim (elosulfase alfa) intravenous infusion

Policy CodePolicy MG.MM.PH.31O

Change TypeAdded baseline assessmentsadded KS/GAG response metricdosing and age updates

Effective Date

Next Review Date

SourceLink

On This Page

Stability or reduction in urine keratan sulfate (KS) or urine glycosaminoglycan (GAG) levels

Denial Risk

Denial risk for unacceptable toxicity or lack of response

Renewal may be denied for unacceptable toxicity or lack of response; examples of unacceptable toxicity include anaphylaxis, hypersensitivity reactions, acute respiratory complications, and spinal/cervical cord compression. Renewal may also be denied if there is no stability or improvement in the specified response measures compared to pretreatment baseline.

Examples of unacceptable toxicity: anaphylaxis, severe hypersensitivity, acute respiratory complications, spinal/cervical cord compression.
Response measures include stability or improvement in endurance tests, pulmonary function tests, or stability/reduction in urine KS/GAG versus baseline.

Transferred from CCUM template to Co-Branded Medical template and retired prior policy MG.MM.PH.178.

Revision history supplemental details (changes recorded; material status noted):

• Inserted into Initial Criteria as required baseline assessments. (Material: yes) — "Documented baseline for ONE or more of the following: endurance tests (6‑MWT, T25FW, 3‑MSCT), pulmonary function tests (MVV, percent predicted FVC, etc.), urine keratan sulfate (KS) or urine glycosaminoglycan (GAG) levels."

• Added to Renewal Criteria as an additional response metric. (Material: yes) — "Renewal criteria: Stability or reduction in urine keratan sulfate (KS) or urine glycosaminoglycan (GAG) levels as evidence of response."

• Dosing limits set and age restriction added to Initial Criteria. (Material: yes) — "Updated dosing limits and added age restriction (patient must be at least 5 years of age)."