Pronunciation: (aye-GAL-sih-dace BAY-tah)Class: Enzyme replacement therapy
Trade Names:Fabrazyme- Powder for injection, lyophilized 5.5 mg (5 mg/mL when reconstituted)- Powder for injection, lyophilized 37 mg (5 mg/mL when reconstituted)
Provides exogenous source of α-galactosidase A.
Terminal t ½ ranges from 45 to 102 min.
Indications and Usage
Treatment of Fabry disease.
Dosage and AdministrationAdults
IV 1 mg/kg infused every 2 wk.
- Allow vials of powder for injection and diluent to reach room temperature before reconstitution.
- Reconstitute 35 mg vial with 7.2 mL sterile water for injection. Slowly inject diluent down inside wall of vial then roll and tilt vial gently until powder has dissolved. Final concentration of reconstituted solution is 5 mg/mL.
- Reconstitute 5 mg vial with 1.1 mL sterile water for injection. Slowly inject diluent down inside wall of vial then roll and tilt vial gently until powder has dissolved. Final concentration of reconstituted solution is 5 mg/mL.
- Do not shake or agitate vials during reconstitution or dilution. Do not use filter needles during preparation of infusion.
- Further dilute reconstituted solution with sodium chloride 0.9% injection to a final volume of 500 mL by removing equal volume of sodium chloride injection before adding prescribed volume of reconstituted solution.
- When adding reconstituted solution to normal saline injection, inject reconstituted solution directly into saline solution and gently invert infusion bag to mix the solution. Do not inject reconstituted solution into the airspace within the infusion bag or vigorously shake or agitate the infusion bag to mix solution.
- Inspect solution visually before administration. Do not administer if solution is cloudy, discolored, or contains particulate matter.
- Discard any unused product. Vials are for single-use only. Do not save medication for future use.
- Initial IV infusion rate should be no more that 0.25 mg/min (15 mg/h). After patient tolerance to the infusion has been established each subsequent infusion may be increased in increments of 0.05 to 0.08 mg/min (3 to 5 mg/h).
- Infusion may be filtered through in-line low protein-binding 0.2 mcm filter during administration.
- Do not administer in same IV line with other products.
Store unopened vials in refrigerator (36° to 46°F). Use reconstituted and diluted solutions immediately. If immediate use is not possible, store the reconstituted and diluted solutions for up to 24 h in refrigerator.
None well documented.
Do not infuse in the same IV line with other products.
Laboratory Test Interactions
None well documented.
Dependent edema (21%); hypotension (14%); cardiomegaly, hypertension (10%); bradycardia, cardiac arrest, cardiac arrhythmia, decreased cardiac output, stroke.
Headache (45%); anxiety (28%); dizziness, paresthesia (14%); ataxia, depression, vertigo.
Pharyngitis (28%); laryngitis (7%); hypoacousia.
Nausea (28%); dyspepsia (10%).
Nephrotic syndrome, testicular pain (7%).
Rhinitis (38%); bronchitis (10%); bronchospasm, sinusitis (7%).
Rigors (52%); infusion reactions (eg, tachycardia, hypertension, throat tightness, chest pain/tightness, dyspnea), fever (48%); pain, skeletal pain (21%); chest pain, sensations of temperature change (17%); pallor (14%); arthrosis (10%); abdominal pain, chills/rigors, lip or ear edema, nausea, pruritus, rash, urticaria, vomiting.
Category B .
Safety and efficacy not established.
Most patients develop IgG antibodies and some develop IgE antibodies to treatment. Consider testing for IgE if a suspected allergic reaction develops.
Patients with advanced Fabry disease may have compromised cardiac function, that may predispose them to higher risk of severe complications from infusion reactions. Monitor cardiac function closely in patients with compromised cardiac function.
Infusion-related hypersensitivity reactions may occur. Some reactions may be severe. Reactions may include fever, chills, rigors, throat and chest tightness, chest pain, rash, pruritus, hives, myalgia, dyspnea, abdominal pain, headache, hypotension, and hypertension. Prior to infusion, patients should receive antipyretics. If an infusion reaction occurs, decreasing the infusion rate, temporarily stopping the infusion, and/or administration of additional antipyretics, antihistamines, and/or steroids may ameliorate the symptoms.
- Advise patient, family, or caregiver that medication will be prepared and administered by a health care provider in a health care setting and that medication will not be administered at home.
- Advise patient, family, or caregiver that an antipyretic (eg, acetaminophen), an antihistamine (eg, diphenhydramine), and possibly a corticosteroid (eg, prednisone), will be administered before each treatment to prevent or reduce severity of infusion reactions.
- Advise patient, family, or caregiver to immediately report any signs or symptoms of infusion reaction (eg, fever, chills, or rigors; throat tightness; chest pain or tightness; rash; itching; hives; muscle aches; difficulty breathing; stomach pain; headache).
- Encourage patient to enroll and participate in the Fabry Registry as noted in the manufacturer's product information.
Copyright © 2009 Wolters Kluwer Health.
- Agalsidase Beta MedFacts Consumer Leaflet (Wolters Kluwer)
- agalsidase beta Intravenous Advanced Consumer (Micromedex) - Includes Dosage Information
- Fabrazyme Prescribing Information (FDA)
- Fabrazyme Consumer Overview