Agalsidase Beta
Pronunciation
(aye GAL si days BAY ta)
U.S. Brand Names
Fabrazyme®
Synonyms
Alpha-Galactosidase-A (Human, Recombinant); r-h
-GAL
Generic Available
No
Use
Replacement therapy for Fabry disease
Pregnancy Risk Factor
B
Pregnancy Implications
There are no adequate and well-controlled studies in pregnant women. Women of childbearing potential are encouraged to enroll in Fabry registry.
Lactation
Excretion in breast milk unknown/use caution
Contraindications
No known contraindications
Warnings/Precautions
Infusion reactions are common, and may be severe; pretreatment with antipyretics is advised. Use caution in cardiovascular disease (risk related to infusion reactions may be increased). A registry has been created to monitor therapeutic responses and adverse effects during long-term treatment; patients should be encouraged to register (www.fabryregistry.com or 1-800-745-4447). Safety and efficacy in pediatric patients have not been established (studies limited to patients
16 years of age).
Adverse Reactions
Note:
The most common and serious adverse reactions are infusion reactions (symptoms may include fever, tachycardia, hypertension, throat tightness, dyspnea, chills, abdominal pain, pruritus, urticaria, vomiting).
>10%:
Cardiovascular: Edema (21%), chest pain (17%), hypotension (14%)
Central nervous system: Fever (48%), headache (45%), anxiety (28%), pain (21%), dizziness (14%), paresthesia (14%)
Dermatologic: Pallor (14%)
Gastrointestinal: Nausea (28%)
Miscellaneous: Infusion reactions (alteration of temperature sensation 17%)
Neuromuscular & skeletal: Rigors (52%), skeletal pain (21%)
Respiratory: Rhinitis (38%), pharyngitis (28%)
1% to 10%:
Cardiovascular: Cardiomegaly (10%), hypertension (10%)
Central nervous system: Depression (10%)
Gastrointestinal: Dyspepsia (10%)
Genitourinary: Testicular pain (7%)
Neuromuscular & skeletal: Arthrosis (10%)
Respiratory: Bronchitis (10%), bronchospasm (7%), laryngitis (7%), sinusitis (7%)
Other reported severe reactions (frequency not established): Arrhythmia, ataxia, bradycardia, cardiac arrest, cardiac output decreased, nephritic syndrome, stroke, vertigo
Drug Interactions
No formal drug interaction studies have been conducted.
Stability
Store vials between 2°C and 8 °C (36°F and 46°F). Final infusion should be used immediately if possible, but may be stored for up to 24 hours between 2°C and 8 °C (36°F and 46°F). Each vial should be reconstituted with 7.2 mL SWFI; inject down internal side wall of vial; roll and tilt gently. Resulting solution contains 5 mg/mL. To make final infusion, add the desired amount of reconstituted solution (based on patient weight) to make a final volume of 500 mL in NS (do not use filter needle to prepare). Avoid vigorous shaking or agitation.
Compatibility
Compatible
in NS. Should not be infused with other products.
Mechanism of Action
Agalsidase beta is a recombinant form of the enzyme alpha-galactosidase-A, which is required for the hydrolysis of GL-3 and other glycosphingolipids. The compounds may accumulate (over many years) within the tissues of patients with Fabry disease, leading to renal and cardiovascular complications. In clinical trials of limited duration, agalsidase been noted to reduce tissue inclusions of a key sphingolipid (GL-3). It is believed that long-term enzyme replacement may reduce clinical manifestations of renal failure, cardiomyopathy, and stroke. However, the relationship to a reduction in clinical manifestations has not been established.
Pharmacodynamics/Kinetics
Half-life elimination: 42-102 minutes (nonlinear)
Dosage
I.V.: Adults: 1 mg/kg every 2 weeks
Dosage adjustment in renal impairment:
No dosage adjustment required
Administration
Antipyretics should be administered prior to infusion. Initial infusion rate should not exceed 0.25 mg/minute (15 mg/hour). Decrease rate in the event of an infusion reaction. After patient tolerance to the infusion is established, rate may be increased in increments of 0.05-0.08 mg/minute (3-5 mg/hour) with each subsequent infusion. A 0.2 micron low protein-binding filter may be used.
Monitoring Parameters
Development of IgG or IgE antibodies in patients with suspected allergic reactions (test available from manufacturer).
Patient Education
This medication will not cure Fabry's Disease, rather it may help to reduce or slow the progression of symptoms. This medication can only be administered by infusion. You will be monitored during and after each dose. Report immediately any tightness in your throat or chest, difficulty breathing; chills, fever, vomiting; or acute headache at the time of infusion. Report any chest pain; swelling of extremities; headache, nausea, or vomiting; skeletal pain; infusion site redness or swelling; or other adverse reactions that occur between doses.
Pregnancy/breast-feeding precautions:
Notify prescriber if you are or intend to become pregnant. Consult prescriber if breast-feeding.
Dental Health: Effects on Dental Treatment
No significant effects or complications reported
Dental Health: Vasoconstrictor/Local Anesthetic Precautions
No information available to require special precautions
Mental Health: Effects on Mental Status
Anxiety and dizziness are common; may cause depression
Mental Health: Effects on Psychiatric Treatment
None reported
Dosage Forms
Injection, powder for reconstitution: 35 mg [contains mannitol 222 mg/vial; derived from Chinese hamster cells]
References
Eng C, Guffton N, Wilcox WR, et al, "Safety and Efficacy of Recombinant Human Alpha-Galactosidase A Replacement Therapy in Fabry's Disease,"
N Engl J Med
, 2001, 345(1):9-16.
International Brand Names
Fabrazyme® (AT, CH, DE, DK, ES, FR, IL, IT, NO, PL, SE)
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