Fabrazyme (Agalsidase Beta) - Indications and Dosage

INDICATIONS AND USAGE

Fabrazyme® (agalsidase beta) is indicated for use in patients with Fabry disease. Fabrazyme® reduces globotriaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types (see CLINICAL STUDIES).

INSTRUCTIONS FOR USE

Fabrazyme® 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® 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 mgs is equal to or greater than the patient's number of kg of body weight.
2. Reconstitute each vial of 35 mg Fabrazyme® 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 vial of 5 mg Fabrazyme® 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 a final total volume of 500 mL. Prior to adding the volume of reconstituted Fabrazyme® required for the patient dose, remove an equal volume of 0.9% Sodium Chloride for Injection, USP from the 500 mL infusion bag.
   Patient dose (in mg) ÷ 5 mg/mL = Number of mL of reconstituted Fabrazyme® required for patient dose
   Example: Patient dose = 80 mg
   80 mg ÷ 5 mg/mL = 16 mL of Fabrazyme®
   Slowly withdraw the reconstituted solution from each vial up to the total volume required for the patient dose. Inject the reconstituted Fabrazyme® 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® 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 µm filter during administration.

STORAGE

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

Reconstituted and diluted solutions of Fabrazyme® 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).

DOSAGE AND ADMINISTRATION

The recommended dosage of Fabrazyme® is 1.0 mg/kg body weight infused every 2 weeks as an IV infusion.

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-associated 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) each subsequent infusion. Thirty-one of 58 (53%) patients have received infusions at rates >/= 33 mg/hr.

Patients should receive antipyretics prior to infusion (see WARNINGS).

HOW SUPPLIED

Fabrazyme® is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder. Fabrazyme® 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® 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

Rx Only

U.S. Patent Number: 5,356,804

Fabrazyme® is manufactured and distributed by:

Genzyme Corporation

500 Kendall Street

Cambridge, MA 02142 USA

1-800-745-4447 (phone)

U.S. License Number: 1596

Issued: October 10, 2003

5031 (3/04)