IntravenousFabry diseaseAdult: As long-term enzyme replacement therapy: 1 mg/kg once every 2 weeks given via infusion at an initial rate of not more than 0.25 mg/min (15 mg/hour). For patients weighing ≥30 kg, after tolerance to the infusion is established, infusion rate may be gradually increased in increments of 0.05-0.08 mg/min (3-5 mg/hour) with each subsequent infusion; minimum infusion duration: 1.5 hours (based on individual tolerability). For patients weighing <30 kg, the Max infusion rate is 0.25 mg/min (15 mg/hour). Premedicate with antipyretics, antihistamines, and/or corticosteroids prior to infusion.
Child: ≥8 years Same as adult dose.
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Rechallenge in patients with IgE antibodies or positive skin test result to agalsidase beta: 0.5 mg/kg once every 2 weeks via IV infusion at an initial Max rate of 0.01 mg/min. Dose may be gradually increased to 1 mg/kg, and infusion rate (doubled every 30 minutes up to the Max rate of 0.25 mg/min), as tolerated.
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Reconstitute a vial labelled as 5 mg and 35 mg with 1.1 mL and 7.2 mL of sterile water for inj (SWFI) respectively, to make a solution containing 5 mg/mL. Slowly add the SWFI down on the inside wall of the vial. Gently roll and tilt the vial; do not invert, swirl or shake. Before adding the volume of reconstituted solution required for the patient dose, remove an equal volume of 0.9% NaCl solution for inj from the infusion bag. Further dilution may be done by slowly adding the required dose of reconstituted solution directly into the 0.9% NaCl solution for inj (not in the airspace) to prepare a final concentration between 0.05-0.7 mg/mL. Gently invert or lightly massage the infusion bag to mix. Avoid vigorous shaking or agitation. Do not use filter needles during preparation.
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Patient with compromised cardiac function. Children. Pregnancy and lactation.
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Significant: IgG and IgE antibody development, skin test reactivity, infusion-associated reactions (e.g. chills, vomiting, paraesthesia).
Cardiac disorders: Tachycardia, bradycardia, palpitation, chest pain.
Ear and labyrinth disorders: Tinnitus, hypoacusis, vertigo.
Eye disorders: Increased lacrimation.
Gastrointestinal disorders: Nausea, abdominal pain or discomfort, diarrhoea.
General disorders and administration site conditions: Fever, fatigue, lethargy, asthenia, pain, feeling hot or cold, hyperthermia.
Infections and infestations: Fungal or viral infection.
Investigations: Increased serum creatinine.
Musculoskeletal and connective tissue disorders: Arthralgia, myalgia, back pain, muscle spasm or tightness, pain in extremity, musculoskeletal stiffness.
Nervous system disorders: Headache, dizziness, somnolence, hypoaesthesia, burning sensation.
Psychiatric disorders: Anxiety, depression.
Respiratory, thoracic and mediastinal disorders: Cough, pharyngitis, rhinitis, upper or lower respiratory tract infection, wheezing.
Skin and subcutaneous tissue disorders: Skin excoriation, generalised pruritus, erythema, maculopapular rash.
Vascular disorders: Hypertension, syncope, hot flush, pallor.
Potentially Fatal: Anaphylactic and severe hypersensitivity reactions (e.g. angioedema, bronchospasm, dyspnoea, chest discomfort, hypotension, nasal congestion, flushing, dysphagia, pruritus, rash, generalised urticaria).
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Monitor for the development of IgG or IgE antibodies in patients with suspected allergic reactions; signs of infusion-associated reactions.
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Amiodarone, monobenzone, chloroquine, or gentamicin may diminish the therapeutic effect of agalsidase beta.
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Description: Agalsidase beta is a recombinant form of α-galactosidase A, an enzyme that catalyses the hydrolysis of globotriaosylceramide (GL-3) and other α-galactyl-terminated neutral glycosphingolipids. These substrates accumulate within the tissues of patients with Fabry disease, resulting in renal and CV complications. Agalsidase beta reduces the tissue inclusions of GL-3 and restores the level of enzymatic action sufficient to clear the accumulation of substrates in organ tissues, thereby preventing and stabilising the progressive decline of organ functions.
Pharmacokinetics:
Distribution: Volume of distribution: 81-570 mL/kg.
Metabolism: Metabolised via peptide hydrolysis.
Excretion: Elimination half-life (dose-dependent): Mean range: 45-119 minutes.
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Intact vial: Store between 2-8°C. Diluted solutions: Store between 2-8°C for up to 24 hours.
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A16AB04 - agalsidase beta ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.
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Anon. Agalsidase Beta. AHFS Clinical Drug Information [online]. Bethesda, MD. American Society of Health-System Pharmacists, Inc. https://www.ahfscdi.com. Accessed 01/03/2022. Anon. Agalsidase Beta. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 01/03/2022. Buckingham R (ed). Alpha Galactosidase A. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 01/03/2022. Fabrazyme (Sanofi-Aventis Singapore Pte. Ltd.). MIMS Singapore. http://www.mims.com/singapore. Accessed 01/03/2022. Fabrazyme 35 mg Powder for Concentrate for Solution for Infusion (Aventis Pharma Ltd Trading as: Sanofi Genzyme). MHRA. https://products.mhra.gov.uk. Accessed 01/03/2022. Fabrazyme 5 mg Powder for Concentrate for Solution for Infusion (Aventis Pharma Ltd Trading as: Sanofi Genzyme). MHRA. https://products.mhra.gov.uk. Accessed 01/03/2022. Fabrazyme Injection, Powder, Lyophilized, for Solution (Genzyme Corporation). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed. Accessed 01/03/2022. Joint Formulary Committee. Agalsidase Beta. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 01/03/2022.
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