LAMZEDE Dosage & Rx Info | Uses, Side Effects

Lamzede Generic Name & Formulations

Legal Class

General Description

Velmanase alfa-tycv 10mg; per vial; lyophilized pwd for IV infusion after reconstitution; preservative-free.

Pharmacological Class

Recombinant human lysosomal alpha-mannosidase.

How Supplied

Single-dose vials—1, 5, 10

Storage

Store refrigerated at 2°C to 8°C (36°F to 46°F) in the original carton to protect from light. Do not freeze.

Manufacturer

Chiesi USA, Inc.

Generic Availability

Mechanism of Action

Alpha-mannosidase catalyzes the degradation of accumulated mannose-containing oligosaccharides. The deficiency of alpha-mannosidase causes an intra-lysosomal accumulation of mannose-rich oligosaccharides in various tissues. Velmanase alfa-tycv provides an exogenous source of alpha-mannosidase. Velmanase alfa-tycv is internalized via binding to the mannose-6-phosphate receptor on the cell surface and transported into lysosomes where it is thought to exert enzyme activity.

Lamzede Indications

Indications

Non-central nervous system manifestations of alpha-mannosidosis.

Lamzede Dosage and Administration

Adults and Children

Consider pretreatment with antihistamines, antipyretics, and/or corticosteroids. ≤49kg: Give as IV infusion over ≥60mins. ≥50kg: max infusion rate of 25mL/hr. 1mg/kg once weekly; flush line with NaCl 0.9% inj. Dosage modifications due to hypersensitivity and/or infusion-related reactions: see full labeling.

Lamzede Contraindications

Not Applicable

Lamzede Boxed Warnings

Boxed Warning

Severe hypersensitivity reactions.

Lamzede Warnings/Precautions

Warnings/Precautions

Have cardiopulmonary resuscitation equipment readily available. Discontinue immediately and treat if severe hypersensitivity or infusion-associated reaction (eg, anaphylaxis) occurs; consider withholding dose for 15–30mins or slowing rate (25–50%) if mild to moderate reaction occurs. Embryo-fetal toxicity. Advise females of reproductive potential to use effective contraception during and for 14 days after the last dose. Pregnancy: exclude status prior to initiation. Nursing mothers.

Lamzede Pharmacokinetics

Metabolism

Catabolic pathways.

Elimination

Half-life: 33.6 hours (in those with alpha-mannosidosis).

Lamzede Interactions

Not Applicable

Lamzede Adverse Reactions

Adverse Reactions

Hypersensitivity reactions, anaphylaxis, infusion-associated reactions, nasopharyngitis, pyrexia, headache, arthralgia.

Lamzede Clinical Trials

Clinical Trials

The approval was based on efficacy and safety data from multiple clinical trials including a phase 3 study (ClinicalTrials.gov Identifier: NCT01681953) and a phase 2 trial (ClinicalTrials.gov Identifier: NCT02998879).

The phase 3 trial included adult and pediatric patients with alpha-mannosidosis who were randomly assigned to receive either velmanase alfa 1mg/kg weekly as an intravenous infusion (n=15) or placebo (n=10) over 52 weeks. Compared with placebo, treatment with velmanase alfa demonstrated improvements in the 3-minute stair climbing test, 6-minute walking test, and forced vital capacity at 12 months. Moreover, the efficacy of velmanase alfa was supported by a reduction in serum oligosaccharide concentration.

The single-arm phase 2 trial evaluated velmanase alfa 1mg/kg given once weekly as intravenous infusion in 5 patients less than 6 years of age with alpha-mannosidosis. At 24 months, the mean absolute and percentage changes from baseline for serum oligosaccharides were -7.7 (4.27) μmol/L and -65.8% (23.1%), respectively.

Lamzede

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