ModifiedCentenePolicy CP.PHAR.601

Velmanase Alfa-tycv (Lamzede)

Medical necessity policy governing coverage criteria, dosing, and authorization requirements for velmanase alfa-tycv (Lamzede) for treatment of non-CNS manifestations of alpha‑mannosidosis for Centene lines of business.

Policy Summary

PayerCentene

PolicyVelmanase Alfa-tycv (Lamzede) clinical coverage criteria

Policy CodePolicy CP.PHAR.601

Change TypeMaterial revision — authorization durations and criteria clarifications

Effective DateFeb 16, 2023

Next Review Date

Key ActionSubmit prior authorization with clinical documentation including current actual body weight in kg and evidence the member meets all approval criteria.

SourceLink

POLICY UPDATE CHANGES

Updated initial and continued approval durations from 6 months to 12 months for Medicaid/HIM and added standard authorization duration language for Commercial; added ICHRA line of business.

For Initial Approval added examples of CNS manifestations of AM that were previously outlined in Appendix D into the criteria; for Continued Therapy added examples of positive treatment response that were previously outlined in Appendix D into the criteria.

1 mg/kg/weekMax weekly dose

12 moMedicaid/HIM/ICHRA approval

6 moCommercial approval

J0217

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Coverage Criteria for Velmanase alfa-tycv (Lamzede)

Initial Therapy — covered when ALL of the following are met

Covered when ALL of the following are met:

Initial Approval for Alpha-Mannosidosis: 1) Diagnosis of AM confirmed by EITHER: a) reduced AM activity < 10% of normal in leukocytes or fibroblasts OR b) genetic testing revealing biallelic MAN2B1 mutation; 2) Prescribed by or in consultation with an endocrinologist, neurologist, ophthalmologist, clinical geneticist, or specialist familiar with lysosomal storage disorders; 3) Member does NOT have central nervous system manifestations of AM such as mental retardation, speech delay, sensorineural hearing loss, dysostosis multiplex, genu valgum, hypotonia, motor and coordination disturbances, ataxia, ocular manifestations with strabismus and acute psychotic manifestations, occipital white matter signal aberrations, delayed myelination, or hydrocephalus; 4) Member is able to ambulate independently; 5) Member has not previously received bone marrow transplant or hematopoietic stem cell transplantation; 6) Documentation of current actual body weight in kg; 7) Dose does not exceed 1 mg/kg (actual body weight) per week.

See Appendix D for examples of CNS and non-CNS manifestations.

Continuation Therapy — covered when ALL of the following are met

Covered when ALL of the following are met:

Continued Therapy for Alpha-Mannosidosis: 1) Member meets one of the following: a) currently receiving medication via Centene benefit or has previously met initial approval criteria OR b) member is currently receiving medication and is in a state/product with continuity of care regulations; 2) Member is responding positively to therapy as evidenced by stabilization or improvement in one or more parameters such as serum oligosaccharides, 3-minute stair climb test, 6-minute walk test, Bruininks-Oseretsky motor proficiency test, forced vital capacity, coarse facial features, frequency of infections due to immune deficiency, or skeletal abnormalities; 3) Documentation of current actual body weight in kg; 4) If request is for a dose increase, new dose does not exceed 1 mg/kg (actual body weight) per week.

Positive response examples were added from Appendix D.

Non–FDA‑approved (off‑label) indications that are not specifically addressed in this policy are not authorized unless the provider supplies sufficient documentation of efficacy and safety in accordance with the applicable off‑label use policy for the member’s line of business: CP.CPA.09 (Commercial), HIM.PA.154 (Health Insurance Marketplace/ICHRA), or CP.PMN.53 (Medicaid), or unless coverage is supported by other evidence of coverage documents.

When state Medicaid coverage provisions conflict with the coverage provisions in this clinical policy, state Medicaid provisions take precedence. Providers should consult the applicable state Medicaid manual for specific state requirements and coverage rules.

This policy does not authorize use of velmanase alfa‑tycv for members who have central nervous system manifestations of alpha‑mannosidosis; examples of CNS manifestations include cognitive and neurologic findings such as mental retardation, speech delay, sensorineural hearing loss, ataxia, motor and coordination disturbances, delayed myelination, hydrocephalus, and related neurologic or ocular findings. Additionally, clinicians should consider the drug’s safety profile, including the boxed warning for severe hypersensitivity reactions including anaphylaxis when determining medical necessity.

Coding and Dosing Information

HCPCS CodesHCPCS

J0217

Injection, velmanase alfa-tycv, 1 mg

Coding ImplicationsHCPCS

HCPCS (updated)

Reference to an updated HCPCS code for velmanase alfa noted in 2Q2024 (exact code number not present in this part of the document)

inv-08: Maximum weekly dose — dosing limit (≤ 1 mg/kg/week)

Maximum weekly doseDose does not exceed 1 mg/kg (actual body weight) per week; dosing regimen: 1 mg/kg IV once weekly

Dose calculation basisUse current actual body weight in kilograms for dose calculation

Administration route and frequencyIntravenous (IV) administration once every week

Documentation requirementProvider must document current actual body weight in kg when requesting authorization

Provider Actions, Authorization & Documentation Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Lamzede. Initial and continued approvals vary by line of business.

Initial Approval: see clinical criteria in Initial Approval section (diagnosis confirmation, specialist involvement, absence of CNS manifestations, ambulation ability, no prior HSCT, weight documentation, dose ≤ 1 mg/kg/week).
Approval duration: Medicaid/HIM/ICHRA — 12 months; Commercial — 6 months or to member's renewal date, whichever is longer (updated 2Q2026: Commercial standard auth duration language and Medicaid/HIM changed to 12 months).
Continued Therapy: see Continued Therapy criteria (current receipt or prior approval, positive response evidence, weight documentation, dose increase limit ≤ 1 mg/kg/week).

Note

Authorization Duration Updates (2Q2026)

Standard authorization durations were updated in 2Q2026. Initial and continued approval durations for Medicaid/HIM/ICHRA were set to 12 months; Commercial retains the standard authorization language of 6 months or to the member's renewal date, whichever is longer. Other editorial clarifications were added to criteria examples and coding references were reviewed.

Background

Alpha‑mannosidosis (AM) is a lysosomal storage disorder caused by deficiency of alpha‑mannosidase. Clinical manifestations vary and can include non‑CNS features such as coarse facial features, recurrent infections related to immune deficiency, and skeletal abnormalities. Velmanase alfa‑tycv (Lamzede) is a recombinant human alpha‑mannosidase enzyme replacement therapy indicated for the treatment of non‑central nervous system manifestations of AM in adult and pediatric patients. Coverage under this policy is limited to members with documented non‑CNS disease manifestations and who meet the specified diagnostic, specialist involvement, functional, and dosing criteria.

Definitions and Abbreviations

inv-18: AM — definition and manifestations of alpha-mannosidosis

Definition of AMAlpha‑mannosidosis (AM) is a lysosomal storage disorder treated with velmanase alfa-tycv for non‑CNS manifestations

Common non‑CNS manifestationsCoarse facial features; frequent infections due to immune deficiency; skeletal abnormalities

Central nervous system (CNS) manifestations (examples)Mental retardation, speech delay, sensorineural hearing loss, dysostosis multiplex, genu valgum, hypotonia, motor and coordination disturbances, ataxia, ocular manifestations with strabismus, acute psychotic manifestations, occipital white matter signal aberrations, delayed myelination, hydrocephalus

inv-19: ICHRA — line of business definition added to policy applicability

ICHRA added to policy applicabilityIndividual Coverage Health Reimbursement Arrangement (ICHRA) was added as a line of business in the 2Q 2026 annual review