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Fabrazyme

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Fabrazyme

Indications
Dosage
How Supplied

INDICATIONS

Fabrazyme (agalsidase beta) is indicated for use in patients with Fabry disease. Fabrazyme (agalsidase beta) reduces globo-triaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types (see Clinical Studies).

DOSAGE AND ADMINISTRATION

The recommended dosage of Fabrazyme (agalsidase beta) is 1.0 mg/kg body weight infused every 2 weeks as an IV infusion. Patients should receive antipyretics prior to infusion (see WARNINGS: Infusion Reactions).

The initial IV infusion rate should be no more than 0.25 mg/min (15 mg/hr). The infusion rate may be slowed in the event of infusion reactions. After patient tolerance to the infusion is well established, the infusion rate may be increased in increments of 0.05 to 0.08 mg/min (increments of 3 to 5 mg/hr) with each subsequent infusion. For patients weighing < 30 kg, the maximum infusion rate should remain at 0.25 mg/min (15 mg/hr). For patients weighing ^ 30 kg, the administration duration should not be less than 1.5 hours (based on individual patient tolerability).

Patients who have had a positive skin test to Fabrazyme (agalsidase beta) or who have tested positive for anti-Fabrazyme (agalsidase beta) IgE may be successfully rechallenged with Fabrazyme (agalsidase beta) . The initial rechallenge administration should be a low dose at a lower infusion rate, e.g., ½ the therapeutic dose (0.5 mg/kg) at ½5 the initial standard recommended rate (0.01 mg/min). Once a patient tolerates the infusion, the dose may be increased to reach the approved dose of 1.0 mg/kg and the infusion rate may be increased by slowly titrating upwards (doubled every 30 minutes up to a maximum rate of 0.25 mg/min), as tolerated.

Instructions for Use

Fabrazyme (agalsidase beta) does not contain any preservatives. Vials are for single-use only. Any unused product should be discarded.

Shaking or agitation of this product should be avoided. Do not use filter needles during the preparation of the infusion.

Reconstitution and Dilution (using Aseptic Technique)

1. Fabrazyme (agalsidase beta) vials and diluent should be allowed to reach room temperature prior to reconstitution (approximately 30 minutes). The number of 35 mg and 5 mg vials needed is based on the patient's body weight (kg) and the recommended dose of 1.0 mg/kg.

Select a combination of 35 mg and 5 mg vials so that the total number of mg is equal to or greater than the patient's number of kg of body weight.

2. Reconstitute each 35 mg vial of Fabrazyme (agalsidase beta) by slowly injecting 7.2 mL of Sterile Water for Injection, USP down the inside wall of each vial. Roll and tilt each vial gently. Each vial will yield a 5.0 mg/mL clear, colorless solution (total extractable amount per vial is 35 mg, 7.0 mL).

Reconstitute each 5 mg vial of Fabrazyme (agalsidase beta) by slowly injecting 1.1 mL of Sterile Water for Injection, USP down the inside wall of each vial. Roll and tilt each vial gently. Each vial will yield a 5.0 mg/mL clear, colorless solution (total extractable amount per vial is 5 mg, 1.0 mL).

3. Visually inspect the reconstituted vials for particulate matter and discoloration. Do not use the reconstituted solution if there is particulate matter or if it is discolored.

4. The reconstituted solution should be further diluted with 0.9% Sodium Chloride Injection, USP to total volume based on patient weight specified in Table 4 below. Prior to adding the volume of reconstituted Fabrazyme (agalsidase beta) required for the patient dose, remove an equal volume of 0.9% Sodium Chloride for Injection, USP from the infusion bag.

Table 4

Patient Weight (kg) Minimum Total Volume
≤ 35 50
35.1 - 70 100
70.1 - 100 250
> 100 500

Patient dose (in mg) t 5 mg/mL = Number of mL of reconstituted Fabrazyme (agalsidase beta) required for patient dose

Example: Patient dose = 80 mg
80 mg / 5 mg/mL = 16 mL of Fabrazyme (agalsidase beta)

Slowly withdraw the reconstituted solution from each vial up to the total volume required for the patient dose. Inject the reconstituted Fabrazyme (agalsidase beta) solution directly into the Sodium Chloride solution. Do not inject in the airspace within the infusion bag. Discard any vial with unused reconstituted solution.

5. Gently invert infusion bag to mix the solution, avoiding vigorous shaking and agitation.

6. Fabrazyme (agalsidase beta) should not be infused in the same intravenous line with other products.

7. The diluted solution may be filtered through an in-line low protein-binding 0.2 urn filter during administration.

Storage

Store Fabrazyme (agalsidase beta) under refrigeration between 2°-8°C (36°-46°F). DO NOT USE Fabrazyme (agalsidase beta) after the expiration date on the vial.

Reconstituted and diluted solutions of Fabrazyme (agalsidase beta) should be used immediately. This product contains no preservatives. If immediate use is not possible, the reconstituted and diluted solution may be stored for up to 24 hours at 2°-8°C (36°-46°F).

HOW SUPPLIED

Fabrazyme (agalsidase beta) is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder. Fabrazyme (agalsidase beta) 35 mg vials are supplied in single-use, clear Type I glass 20 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic purple flip-off cap. Fabrazyme (agalsidase beta) 5 mg vials are supplied in single use, clear Type I glass 5 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic gray flip-off cap.

35 mg vial: NDC 58468-0040-1
  5 mg vial: NDC 58468-0041-1

Fabrazyme (agalsidase beta) is manufactured and distributed by: Genzyme Corporation, 500 Kendall Street, Cambridge, MA 02142. 1-800-745-4447 (phone). Issued: September 29, 2006. FDA rev date: 2233

Last reviewed on RxList: 2/26/2009
This monograph has been modified to include the generic and brand name in many instances.

Indications
Dosage
How Supplied
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