CurrentAvMedPolicy Pub. 100-2

Lamzede (velmanase alfa-tycv) medical prior authorization

Authorization form and medical necessity criteria governing coverage of Lamzede (velmanase alfa-tycv) for treatment of non-CNS manifestations of alpha-mannosidosis for AvMed members; affects prescribers and infusion sites requesting medical benefit coverage.

Policy Summary

PayerAvMed

PolicyLamzede (velmanase alfa-tycv) medical prior authorization

Policy CodePolicy Pub. 100-2

Change TypeNo material changes

Effective Date

Next Review Date

Key ActionSubmit prior authorization using the AvMed PA/step-edit request form and include required clinical documentation (diagnostic test results, baseline serum oligosaccharides, and baseline functional measures).

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

J0217HCPCS code listed for Lamzede

12 monthsInitial authorization period

1 mg/kg weeklyMax dose/frequency

>=3 yrsMinimum age

Coverage Criteria for Lamzede (velmanase alfa)

Initial Authorization

Covered when ALL of the following are met

Initial authorization criteria: Member is at least 3 years of age; prescribed by or in consultation with a geneticist or metabolic specialist; definitive diagnosis of alpha-mannosidosis confirmed by either identification of deficient acid alpha-mannosidase enzyme activity in peripheral blood leukocytes or other nucleated cells (e.g., fibroblasts) of <11% of normal activity, OR identification of biallelic pathogenic variants in MAN2B1 by molecular genetic testing; provider has submitted baseline serum oligosaccharides lab results obtained within the last 30 days; provider has submitted baseline age-appropriate values for at least ONE functional measure (6-minute walk test [6-MWT], 3-minute stair climb test [3-MSCT], pulmonary function tests such as forced vital capacity [FVC], or a motor function test e.g., BOT-2) where applicable; confirmed negative pregnancy test in females of reproductive potential; medication is being used to treat non-central nervous system manifestations of alpha-mannosidosis (e.g., skeletal abnormalities, myopathy, motor function disturbances, immunodeficiency); member does NOT have a history of hematopoietic stem cell transplant (HSCT); dosing does not exceed the recommended maximum of 1 mg/kg IV administered no more frequently than weekly;

All documentation supporting each line must be provided or request may be denied.

Reauthorization/Continuation

Covered when ALL of the following are met

Policy Summary

PayerAvMed

PolicyLamzede (velmanase alfa-tycv) medical prior authorization

Policy CodePolicy Pub. 100-2

Change TypeNo material changes

Effective Date

Next Review Date

SourceLink

On This Page

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Reauthorization criteria: Member continues to meet all initial authorization criteria; member has experienced an absence of unacceptable toxicity from the drug (e.g., anaphylaxis or severe allergic/infusion-associated reactions); provider submits current documentation showing a decrease in serum oligosaccharide concentration from baseline after initial authorization, or stabilization in concentration for subsequent reauthorizations; AND the provider submits ONE of the following: stability or improvement in 6-MWT, stability or improvement in 3-MSCT, stability or improvement in FVC (% predicted), or stabilization/slowing in the rate of disease progression or clinical decline.

All documentation must be provided or request may be denied.

This policy applies only to treatment of non-central nervous system (non‑CNS) manifestations of alpha‑mannosidosis. Requests must document that the medication is being used to treat systemic features such as skeletal abnormalities, myopathy, motor function disturbances, or immunodeficiency; treatment of CNS manifestations is not an approved indication under these criteria.

Members with a prior history of hematopoietic stem cell transplant (HSCT) are excluded from coverage under these criteria and are not eligible for approval.

Initiation of therapy using drug samples does not satisfy the step‑edit or preauthorization requirements and therefore does not meet criteria for authorization; prior therapies will be verified through pharmacy paid claims or submitted chart notes.

Initial Therapy Dosing and Duration

Initial therapy dosing and duration

Initial therapy dosing and duration: Approved maximum dose: 1 mg/kg (actual body weight) administered by intravenous infusion no more frequently than every week; initial authorization period: 12 months; member must meet required diagnostic and baseline testing criteria as listed in Initial Authorization.1 mg/kg weekly

Provider must submit baseline labs and functional measures; authorization may be delayed if the request is incomplete.

Reauthorization and Continuation Requirements

Reauthorization requirements

Reauthorization requires continued benefit and safety monitoring

Reauthorization requirements: Absence of unacceptable toxicity (e.g., anaphylaxis or severe allergic/infusion reactions); decreased or stabilized serum oligosaccharide concentration from baseline and documentation of stability or improvement in at least one functional measure (6-MWT, 3-MSCT, or FVC % predicted), or evidence of stabilization/slowing of disease progression or clinical decline.

Documentation (lab results, diagnostics, and/or chart notes) supporting each item must be submitted or the request may be denied.

Coding and Diagnostic Thresholds

Covered HCPCS CodesHCPCSCovered

J0217

Injection, velmanase alfa-tycv

Enzyme activity diagnostic threshold

Enzyme activity diagnostic threshold<11% of normal activity

Provider Requirements, Documentation, and Submission

Prior Authorization

Prior Authorization Required

Prior Authorization is required for J0217. Submit the completed AvMed PA/Step-Edit form and supporting documentation to AvMed Pre-Authorization via fax to 1-844-668-1550. Ensure all contact information is correct to avoid delays.

Drug: Lamzede (velmanase alfa-tycv) J0217
Submit AvMed PA/Step-Edit form

Step Therapy

Samples and Prior Therapy Verification

Use of samples to initiate therapy does not meet step edit / preauthorization criteria. Prior therapies will be verified through pharmacy paid claims or submitted chart notes; provide documentation of prior treatment attempts when applicable.

Samples cannot be used to satisfy prior authorization or step-therapy requirements
Provide documentation of prior therapies (pharmacy claims or chart notes)

Documentation Required

Required Clinical Documentation

Provide complete clinical documentation to support each checked criterion. Include diagnostic test results, laboratory values, and baseline functional testing as specified on the form. If documentation is not provided for any criterion indicated, the request may be denied.

Test results confirming diagnosis: enzyme activity <11% OR biallelic pathogenic MAN2B1 variants
Baseline serum oligosaccharides lab results (within 30 days)
At least one baseline age-appropriate functional measure: 6-MWT, 3-MSCT, pulmonary function tests (FVC), or motor function test (e.g., BOT-2)
Negative pregnancy test for females of reproductive potential

Documentation Required

Administrative Documentation

The prescribing physician must sign and clearly print their name on the request (preprinted stamps are not valid). Include complete prescriber and office contact information: prescriber name, signature, date, office contact name, phone number, fax number, and NPI. For the administering location, include location/site name and NPI or DEA number if applicable.

Prescriber signature and printed name (hand-signed; stamps not acceptable)
Prescriber date
Office contact name, phone number, and fax number
Prescriber NPI
Administering location name and NPI or DEA # (if applicable) or specialty pharmacy information

Denial Risk

Incomplete or Illegible Request

Incomplete, incorrect, or illegible requests may delay or result in denial of authorization. Ensure every field required on the PA form is completed legibly and accurately before submission.

Verify all required fields on the PA form are completed
Ensure documentation (labs, diagnostics, chart notes) is legible and attached
Confirm fax number and contact information are correct

Denial Risk

Missing Supporting Clinical Documentation

If laboratory results, diagnostic testing, or chart notes supporting any checked clinical criteria are missing, the request is at risk for denial. Provide the specific test reports cited on the form (e.g., enzyme assay, genetic testing, serum oligosaccharides, functional test reports) to substantiate each criterion.

Include enzyme assay or genetic test report when used to confirm diagnosis
Attach serum oligosaccharide lab results and date obtained
Attach reports for functional assessments used to meet baseline or reauthorization criteria

Background

Alpha‑mannosidosis is a rare lysosomal storage disorder caused by deficient acid alpha‑mannosidase activity. The enzyme deficiency leads to systemic manifestations including skeletal abnormalities, myopathy, motor disturbances, and immunodeficiency. Velmanase alfa (Lamzede) is an enzyme replacement therapy administered by intravenous infusion for the treatment of non‑CNS manifestations of alpha‑mannosidosis.

Definitions and Diagnostic Criteria

Definitive diagnosis of alpha-mannosidosis

Definitive diagnostic methodsIdentification of deficient acid alpha-mannosidase enzyme activity in peripheral blood leukocytes or other nucleated cells (<11% of normal activity) or identification of biallelic pathogenic MAN2B1 variants by molecular genetic testing

Required supporting documentationSubmit test results confirming enzyme assay or molecular genetic testing

Specimen types for enzyme assayPeripheral blood leukocytes or other nucleated cells (e.g., fibroblasts)

Quantity Limits and Dosing Caps

velmanase alfa (Lamzede) dosing limits

Maximum dose and frequency1 mg/kg (actual body weight) administered by intravenous infusion no more frequently than weekly

Recommended dosage formIntravenous infusion

Authorization dosing noteDosing must meet recommended maximum of 1 mg/kg IV no more frequently than weekly for approval

Step Therapy and Sample Use

Step	Requirement
1	Use of samples to initiate therapy does not meet step edit/preauthorization criteria; prior therapies will be verified through pharmacy paid claims or submitted chart notes.

Site of Care and Dispensing

Billing Rule

Specify infusion center or specialty pharmacy and provide administering location NPI/DEA

Indicate the location/site of drug administration or select the specialty pharmacy option (Proprium Rx) on the form and include the NPI or DEA number of the administering location.

Check the box for location/site of drug administration and provide the administering location name and NPI or DEA #, OR
Check the Specialty Pharmacy box and select Proprium Rx when applicable.