Coverage Policies

Effective Date:

a)    This policy will apply to all services performed on or after the above revision date which will become the new effective date.

b)    For all services referred to in this policy that were performed before the revision date, contact customer service for the rules that would apply.

1)    Lamzede (velmanase alfa-tycv) requires prior authorization.

2)    Lamzede is indicated for the treatment of non-central nervous system manifestations of alpha-mannosidosis (AM) in adult and pediatric patients.

3)    Lamzede is covered under the medical benefit.

I. Initial Approval Criteria

A. Alpha-Mannosidosis (must meet all):

1. Diagnosis of AM confirmed by one of the following (a or b):

a. Reduced AM activity defined as < 10% of normal activity in leukocytes or fibroblasts cells;

b. Genetic testing revealing biallelic MAN2B1 gene mutation;

2. Prescribed by or in consultation with an endocrinologist, neurologist, ophthalmologist, clinical geneticist, or specialist familiar with the treatment of lysosomal storage disorders;

3. Member does not have central nervous system manifestations of AM;

4. Member is able to ambulate independently;

5. Member has not previously received a 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.

Approval duration: 6 months

II. Continued Therapy

A. Alpha-Mannosidosis (must meet all):

1. Member is responding positively to therapy as evidenced by stabilization or improvement in, but not limited to, any of the following parameters:

a. Serum oligosaccharides levels;

b. 3-minute stair climb test;

c. 6-minute walk test;

d. Bruininks-Oseretsky test of motor proficiency;

e. Forced vital capacity;

2. Documentation of current actual body weight in kg;

3. If request is for a dose increase, new dose does not exceed 1 mg/kg (actual body weight) per week.

1. Lamzede Prescribing Information. Cary, NC: Chiesi USA, Inc. February 2023. Available at: https://resources.chiesiusa.com/Lamzede/LAMZEDE_PI.pdf. Accessed February 24, 2023.

2. Malm D, Nilseen O. Alpha-mannosidosis . National Library of Medicine. Available at: https://www.ncbi.nlm.nih.gov/books/NBK1396/. Accessed April 5, 2023.

3. European Medicine Agency. Lamzede: EPAR – Product Information; May 2022. Available at: https://www.ema.europa.eu/en/medicines/human/EPAR/Lamzede. Accessed September 21, 2022.

4. Borgwardt L, Guffon N, Amraoui Y, et al. Efficacy and safety of velmanase alfa in the treatment of patients with alpha-mannosidosis: results from the core and extension phase analysis of a phase III multicentre, double-blind, randomized, placebo-controlled trial. H Inherit Metab Dis. 2018; 41(6): 1215-1223. https://doi.org/10.1007/s10545-018-0185-0.

5. Lund AM, Borgwardt L, Cattaneo F, et al. Comprehensive long-term efficacy and safety of recombinant human alpha-mannosidase (velmanase alfa) treatment in patients with alpha-mannosidosis. J Inherit Metab Dis. 2018; 41(6): 1225-1233. https://doi.org/10.1007/s10545-018-0175-2.

6. Harmatz P, Cattaneo F, Ardigo D, et al. Enzyme replacement therapy with velmanase alfa (human recombinant alpha-mannosidase): Novel global treatment response model and outcomes in patients with alpha-mannosidosis. Molecular Genetics and Metabolism: 2018; 124(2): 152-160. https://doi.org/10.1016/j.ymgme.

7. Guffon N, Tylki-Szymanska A, Borgwardt L, et al. Recognition of alpha-mannosidosis in paediatric and adult patients: presentation of a diagnostic algorithm from an international working group. Molecular Genetics and Metabolism. 2019;126:470-4. https://doi.org/10.1016/j.ymgme.