Velaglucerase alfa

Intravenous
Gaucher disease type 1

Reconstitute a 400 units vial with 4.3 mL of sterile water for inj resulting in a 100 units/mL concentration. Gently mix; do not shake. Withdraw the appropriate volume of reconstituted solution and further dilute in 100 mL of 0.9% NaCl solution. Administer the diluted solution through an in-line low protein-binding 0.2- or 0.22-micrometre filter.

Hypersensitivity.

Patient with symptoms of hypersensitivity to other enzyme replacement therapies. May consider premedication with antihistamines and/or corticosteroids in patients with prior hypersensitivity reactions requiring symptomatic treatment. Children. Pregnancy and lactation.

Significant: Hypersensitivity reactions (including anaphylaxis); infusion-related reactions, IgG antibody formation.
Cardiac disorders: Tachycardia, chest discomfort.
Eye disorders: Blurred vision.
Gastrointestinal disorders: Abdominal pain, nausea, vomiting.
General disorders and administration site conditions: Fatigue, pyrexia.
Investigations: Prolonged aPTT.
Musculoskeletal and connective tissue disorders: Back pain, arthralgia, bone pain.
Nervous system disorders: Headache, dizziness.
Respiratory, thoracic and mediastinal disorders: Dyspnoea.
Skin and subcutaneous tissue disorders: Rash, pruritus, urticaria.
Vascular disorders: Hypertension, hypotension, flushing.

IV: B

Monitor CBC, IgG antibodies, liver enzymes; antibodies (in patients who developed antibodies to other enzyme replacement therapies). Perform bone density studies and computed tomography (CT) scan or MRI of liver and spleen.

Description:
Mechanism of Action: Velaglucerase alfa is a biosynthetic form of human β-glucocerebrosidase, a lysosomal enzyme that catalyses the hydrolysis of glucocerebroside to glucose and ceramide. It is used to replace the β-glucocerebrosidase enzyme that is deficient in Gaucher's disease, thereby reducing the accumulated glucocerebroside and correcting the pathophysiology of Gaucher's disease. As a result, it diminishes hepatosplenomegaly and improves thrombocytopenia, anaemia, and bone disease.
Pharmacokinetics:
Absorption: Time to peak plasma concentration: 40-60 minutes.
Excretion: Elimination half-life: 5-12 minutes.

Intact vials: Store between 2-8°C. Diluted solutions are stable for up to 24 hours when stored between 2-8°C. Do not freeze. Protect from light.

Other Agents Affecting Metabolism

A16AB10 - velaglucerase alfa ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.

Anon. Velaglucerase Alfa. AHFS Clinical Drug Information [online]. Bethesda, MD. American Society of Health-System Pharmacists, Inc. https://www.ahfscdi.com. Accessed 04/08/2022.

Anon. Velaglucerase Alfa. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 04/08/2022.

Buckingham R (ed). Velaglucerase Alfa. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 04/08/2022.

Joint Formulary Committee. Velaglucerase Alfa. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 04/08/2022.

Takeda New Zealand Limited. Vpriv 400 Units Powder for Solution for Infusion data sheet 19 October 2020. Medsafe. http://www.medsafe.govt.nz. Accessed 04/08/2022.

Vpriv 400 Units Powder for Solution for Infusion (Takeda Pharmaceuticals International AG Ireland Branch). MHRA. https://products.mhra.gov.uk. Accessed 04/08/2022.

Vpriv Injection, Powder, Lyophilized, for Solution (Takeda Pharmaceuticals America, Inc.). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed. Accessed 04/08/2022.