Fabrazyme
Generic name:agalsidase beta [ a-GAL-sih-daze-BAY-tah ]
Drug class:Lysosomal enzymes
What is Fabrazyme?
Fabrazyme is a man-made form of the naturally-occurring alpha-galactosidase A enzyme. A deficiency of this enzyme is called Fabry disease. Agalsidase beta reduces deposits of globotriaosylceramide (GL-3) in the kidneys and certain other cells in the body.
Fabrazyme is used in the treatment of Fabry disease (a deficiency of alpha-galactosidase A enzyme) in adults and children at least 2 years old.
Fabrazyme may also be used for purposes other than those listed here.
Warnings
Many people have a severe reaction to Fabrazyme. Tell your caregiver if you have a skin rash or hives, fever, chills, headache, muscle pain, dizziness, numbness, swelling, vomiting, diarrhea, chest pain, trouble swallowing or breathing, fast or slow heart rate, or severe dizziness.
Most patients treated with Fabrazyme develop antibodies to agalsidase beta and many will develop symptoms of an infusion reaction. A slow rate of injection of the medication and pretreatment with other medications may decrease the severity of these symptoms. Emergency medical attention may be required if a severe allergic reaction is experienced.
Before taking this medicine
To make sure Fabrazyme is safe for you, tell your doctor if you have ever had:
heart problems; or
an allergic reaction to agalsidase beta or have antibodies to the medication.
Tell your doctor if you are pregnant or breastfeeding.
Your name may be listed on a Fabry disease patient registry. This is to track the progress of your disease and to evaluate the treatment effects of Fabrazyme. Taking part in this registry is especially important if you are pregnant or breastfeeding.
How should I use Fabrazyme?
Fabrazyme is given as an infusion into a vein. A healthcare provider will give you this injection.
Fabrazyme is usually given once every 2 weeks. Follow your doctor's instructions.
Doses are based on weight. Your dose may change if you gain or lose weight.
While using agalsidase beta, you may need frequent medical tests.
Dosing information
Usual Adult Dose for Fabry Disease:
1 mg/kg IV every 2 weeks
Comments:
-Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr).
-Clinical studies did not include a sufficient number of patients aged 65 years or older to determine if they respond differently than younger patients.
Use: For treatment of patients with Fabry disease
Usual Pediatric Do
