Agalsidase beta


Concise Prescribing Info
Indications/Uses
Fabry disease.
Dosage/Direction for Use
Adult : IV 1 mg/kg every 2 wk.
Dosage Details
Intravenous
Fabry disease
Adult: 1 mg/kg once every 2 wk via infusion at an initial rate of ≤0.25 mg/min, if tolerated, infusion rate may be increased in increments of 0.05-0.08 mg/min w/ each subsequent infusion. Max infusion rate: <30 kg: 0.025 mg/min; ≥30 kg: Based on individual tolerability, infused over at least 1.5 hr.  Pre-medicate w/ antipyretics and antihistamine.
Child: ≥8 yr Same as adult dose.
Reconstitution
Add 7.2 mL or 1.1 mL of sterile water for inj to the vial containing 35 mg or 5 mg, respectively, to provide a soln containing approx 5 mg/mL. Gently roll and tilt vial, do not shake. Further dilute the required amount of reconstituted soln w/ NaCl 0.9% inj to a final concentration of 0.05-0.7 mg/mL. Determine the total volume of NaCl 0.9% soln for infusion (between 50 and 500 mL) based on individual dose. Prior to adding the volume of reconstituted soln required for the patient dose, remove an equal volume of NaCl 0.9% soln from the infusion bag.
Contraindications
Hypersensitivity.
Special Precautions
Patient w/ CV disease. Childn. Pregnancy and lactation.
Adverse Reactions
Significant: Infusion reactions (e.g. fever, rigors, chest tightness, HTN, pruritus, myalgia, dyspnoea, urticaria, abdominal pain, headache, nausea, vomiting, lip or ear oedema, rash), IgG antibodies formation.
Nervous: Asthenia, fatigue, flu-like symptoms, dizziness, neuropathic pain, paraesthesia, sleep disturbance, tremor.
CV: Bradycardia, palpitations, syncope, tachycardia.
GI: GI disturbance.
Resp: Cough, nasopharyngitis, parosmia, rhinorrhoea.
Musculoskeletal: Arthralgia, muscle spasms.
Otic: Tinnitus.
Ophthalmologic: Eye irritation.
Dermatologic: Acne, skin discolouration.
Others: Cold extremities.
Potentially Fatal: Severe allergic/anaphylactic reactions (e.g. angioedema, bronchospasm, chest discomfort, dyspnoea, flushing, hypotension, nasal congestion, pruritus, rash, and urticaria).
Patient Counseling Information
This drug may cause dizziness, somnolence, vertigo and syncope, if affected, do not drive or operate machinery.
MonitoringParameters
Monitor for infusion-related reactions, development of IgG or IgE antibodies in patients w/ suspected allergic reactions.
Drug Interactions
Amiodarone, chloroquine, and gentamicin may diminish the therapeutic effect of agalsidase beta.
Action
Description: Agalsidase beta is a recombinant form of α-galactosidase A, an enzyme that metabolises globotriaosylceramide (GL-3) and other glycosphingolipids. It is used to replace enzyme to restore enzymatic activity sufficient to clear the accumulation of substrate in the organ tissues, hence, preventing, stabilising or reversing the progressive decline in function of these organs before irreversible damage occur.
Pharmacokinetics:
Metabolism: Metabolised via peptide hydrolysis.
Excretion: Elimination half-life: 45-119 min.
Storage
Store between 2-8°C.
ATC Classification
A16AB04 - agalsidase beta ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.
References
Anon. Agalsidase Beta. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 07/06/2017.

Buckingham R (ed). Alpha Galactosidase A. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 07/06/2017.

Fabrazyme (Genzyme Corporation). U.S. FDA. https://www.fda.gov/. Accessed 07/06/2017.

Fabrazyme Injection, Powder. Lyophilized, for Solution (Genzyme Corporation). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed/. Accessed 07/06/2017.

Joint Formulary Committee. Agalsidase Beta. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 07/06/2017.

McEvoy GK, Snow EK, Miller J et al (eds). Agalsidase Beta. AHFS Drug Information (AHFS DI) [online]. American Society of Health-System Pharmacists (ASHP). https://www.medicinescomplete.com. Accessed 07/06/2017.

Disclaimer: This information is independently developed by MIMS based on Agalsidase beta from various references and is provided for your reference only. Therapeutic uses, prescribing information and product availability may vary between countries. Please refer to MIMS Product Monographs for specific and locally approved prescribing information. Although great effort has been made to ensure content accuracy, MIMS shall not be held responsible or liable for any claims or damages arising from the use or misuse of the information contained herein, its contents or omissions, or otherwise. Copyright © 2020 MIMS. All rights reserved. Powered by MIMS.com
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