CurrentCentenePolicy CP PHAR.601

Velmanase Alfa-tycv (Lamzede)

Defines medical necessity criteria, initial and continued therapy requirements, dosing limits, exclusions, documentation and coding implications for Velmanase alfa-tycv (Lamzede) across commercial, HIM, and Medicaid lines of business.

Policy Summary

PayerCentene

PolicyVelmanase Alfa-tycv (Lamzede)

Policy CodePolicy CP PHAR.601

Change TypeRevised/Clarified

Effective Date02.16.23

Next Review Date

Key ActionProvider must submit office chart notes, lab results, or other clinical information supporting that member meets all approval criteria.

POLICY UPDATE CHANGES

Policy updated per FDA labeling; added ambulatory requirement, exclusion for prior HSCT or bone marrow transplant, additional specialist prescribers, and dosing/max dose language.

Added requirement that member does not have central nervous system manifestations of AM.

Specified required laboratory threshold: Reduced AM activity defined as 10% of normal activity.

Added/updated HCPCS code J0217 for velmanase alfa-tycv.

6 monthsApproval Duration

1 mg/kg/weekMaximum Dose

J0217HCPCS Code

Coverage Summary & Key Limits

Coverage stance: covered_with_criteria for Velmanase alfa-tycv (Lamzede). Scope: policy defines medical necessity criteria, initial and continued therapy requirements, dosing limits, exclusions, documentation, and coding implications for Velmanase alfa-tycv (Lamzede) across commercial, HIM, and Medicaid lines of business. Status: CURRENT.

Key Thresholds

Approval Duration6 months

Maximum Dose1 mg/kg/week

Reduced AM activity threshold10% of normal activity

HCPCS CodeJ0217

Initial Therapy Criteria

Initial Approval Criteria

Lamzede is medically necessary when ALL of the following are met:

ALL of the following

Diagnosis of alpha-mannosidosis (AM)
Diagnosis confirmation (one of)
- Reduced AM activity defined as 10% of normal activity in leukocytes or fibroblast cells10% of normal activity
- Genetic testing revealing biallelic MAN2B1 gene mutation
Member does not have central nervous system manifestations of AM (see Appendix D)
Prescribed by or in consultation with an endocrinologist; neurologist; ophthalmologist; clinical geneticist; or specialist familiar with the treatment of lysosomal storage disorders
Member is able to ambulate independently
Member has not previously received a bone marrow transplant or hematopoietic stem cell transplantation
Documentation of current actual body weight in kg
Dose does not exceed 1 mg/kg (actual body weight) per weekDose does not exceed 1 mg/kg (actual body weight) per week
Approval duration: 6 months

ref

title

Other diagnoses/indications (initial)

If requested use is not covered in this policy or the drug has recent label changes not yet reflected, follow formulary/off-label policies:

ANY of the following

For drugs on formulary: follow no coverage criteria policies CP.CPA.190 (commercial), HIM.PA.33 (marketplace), CP.PMN.255 (Medicaid)
For drugs NOT on formulary: follow non-formulary/no coverage policies CP.CPA.190 (commercial), HIM.PA.103 (marketplace), CP.PMN.16 (Medicaid)
If requested use is not listed under Diagnoses/Indications not authorized and recent label change does not apply, refer to off-label use policies: CP.CPA.09 (commercial), HIM.PA.154 (marketplace), CP.PMN.53 (Medicaid)

Continuation (Renewal) Criteria

Continued Therapy (Renewal) Criteria

Member meets ALL of the following for continuation:

ALL of the following

Eligibility for continued therapy (one of)
- Member is currently receiving medication via Centene benefit
- Member has previously met initial approval criteria
- Member is enrolled in state/product with continuity of care regulations (per state addendums CC PHARM.03A and CC PHARM.03B)
Examples of parameters demonstrating response (one or more)
- Serum oligosaccharides levels
- 3-minute stair climb test
- 6-minute walk test
- Forced vital capacity
- Bruininks-Oseretsky test of motor proficiency
Documentation of current actual body weight in kg
If request is for a dose increase, new dose does not exceed 1 mg/kg (actual body weight) per weekDose does not exceed 1 mg/kg (actual body weight) per week
Approval duration: 6 months

Exclusions and Important Limits

Explicit limits/exclusions: member must not have central nervous system manifestations of AM (see Appendix D); member must not have previously received a bone marrow transplant or hematopoietic stem cell transplantation; member must be able to ambulate independently.

Numeric Thresholds

Reduced AM activity10% of normal activity in leukocytes or fibroblast cells

Maximum dose1 mg/kg (actual body weight) per week

Prior Authorization, Documentation & Billing Actions

Prior Authorization

Prior authorization required for Lamzede

Requests require documentation of diagnosis confirmation, reduced enzyme activity (10% of normal in leukocytes or fibroblasts), documentation of current actual body weight in kg, prescribing specialty (endocrinologist, neurologist, ophthalmologist, clinical geneticist, or specialist familiar with lysosomal storage disorders), ambulatory status (able to ambulate independently), prior transplant status (has not previously received bone marrow transplant or hematopoietic stem cell transplantation), and dosing not exceeding 1 mg/kg (actual body weight) per week. Approval duration: 6 months.

Documentation Required

Submit supporting documentation

Provider must submit office chart notes, lab results, or other clinical information supporting that the member meets all approval criteria.

Documentation Required

Monitor and document treatment response for continuation

For continuation, document stabilization or improvement in at least one relevant parameter such as serum oligosaccharide levels, 3-minute stair climb, 6-minute walk test, forced vital capacity, or Bruininks-Oseretsky test of motor proficiency; include documentation of current actual body weight in kg.

Billing Rule

Dose limit billing constraint

Ensure billed dose does not exceed 1 mg/kg (actual body weight) per week. Use HCPCS code J0217 (Injection, velmanase alfa-tycv, 1 mg) to report the administered dose (J0217 = 1 mg per unit).

Background

Background: Lamzede is indicated for treatment of non-central nervous system manifestations of alpha-mannosidosis in adults and pediatrics. Individual patient non-CNS manifestations may include coarse facial features, frequent infections due to immune deficiency, and skeletal abnormalities. Central nervous system manifestations are enumerated in Appendix D.

Revision History

05.23policy_revision

Policy updated per FDA labeling: added ambulatory requirement (member is able to ambulate independently), exclusion for prior HSCT or bone marrow transplant, added additional specialist prescribers, and dosing/max dose language (dose does not exceed 1 mg/kg actual body weight per week).

04.11.23policy_revision

Policy updated per FDA approval/labeling; references reviewed and updated; added ambulatory requirement, HSCT/bone marrow transplant exclusion, and additional specialties familiar with lysosomal storage disorders.

02.16.23policy_created

Clinical policy created pre-emptively around Lamzede (velmanase alfa-tycv) with initial effective date; includes indication for non-central nervous system manifestations of alpha-mannosidosis and baseline policy structure.

01.12.24annual_review

2Q 2024 annual review: no significant changes; added/updated HCPCS code J0217 and updated references.

03.11.25annual_reviewLatest

2Q 2025 annual review: no significant changes; references reviewed and updated.

labeling_update

Policy reflects FDA-approved indication: treatment of non-central nervous system manifestations of alpha-mannosidosis; added requirement that member does not have central nervous system manifestations (see Appendix D).

laboratory_threshold

Initial approval requires reduced AM activity defined as 10% of normal activity in leukocytes or fibroblast cells.

coding_update

Coding implications: HCPCS code J0217 added for injection, velmanase alfa-tycv, 1 mg (drug billing).