Fabrazyme (agalsidase beta)

Common side effects of Fabrazyme include signs of allergic reaction such as:

• difficulty breathing
• closing of the throat
• hives
• rash
• itching
• fever
• shaking
• chest tightness
• high or low blood pressure
• fast heartbeats
• muscle pain
• stomach pain
• nausea or vomiting
• dizziness
• numbness or tingling, and
• headache

Recommended Dosage

• The recommended dosage of Fabrazyme is 1 mg/kg body weight infused every two weeks as an intravenous infusion.
• Infusion rate:
  • The initial intravenous infusion rate is 0.25 mg/min (15 mg/hour). Slow the infusion rate in the event of infusion-associated reactions.
  • For patients > 30 kg, after patient tolerance to the infusion is well established, increase the infusion rate in increments of 0.05 to 0.08 mg/min (increments of 3 to 5 mg/hour) with each subsequent infusion. The minimum infusion duration is 1.5 hours (based on individual patient tolerability).
  • For patients weighing < 30 kg, the maximum infusion rate is 0.25 mg/minute (15 mg/hour).
• Because of the potential for severe infusion-associated reactions, appropriate medical support measures should be readily available when Fabrazyme is administered.
• Administer antipyretics prior to infusion of Fabrazyme.
• Rechallenge: Patients who have had a positive skin test to Fabrazyme or who have tested positive for anti-Fabrazyme IgE may be successfully rechallenged with Fabrazyme. 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 1/25 of 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 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/minute), as tolerated.

• Pregnant women and women of reproductive potential should be encouraged to enroll in the Fabry patient registry.
• The registry will monitor the effect of Fabrazyme on pregnant women and their offspring.
• For more information, visit www.registrynxt.com or call 1-800-745-4447, extension 15500.
• There are no data on the presence of agalsidase beta in either human or animal milk, the effects of the drug on the breastfed infant, or on milk production.
• The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for Fabrazyme and any potential adverse effects on the breastfed child from Fabrazyme or from the underlying maternal condition.
• Lactating women with Fabry disease treated with Fabrazyme should be encouraged to enroll in the Fabry registry.