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.

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.

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.

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 Dose for Fabry Disease:

8 years or older: 1 mg/kg IV every 2 weeks

Comments:
-Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr).

Use: For treatment of patients with Fabry disease

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.

Call your doctor for instructions if you miss an appointment for your Fabrazyme injection.

Since this medication is given by a healthcare professional in a medical setting an overdose would be treated quickly.

Follow your doctor's instructions about any restrictions on food, beverages, or activity.

Get emergency medical help if you have signs of an allergic reaction to Fabrazyme:

• wheezing, chest tightness, trouble breathing, feeling light-headed;

• skin rash, hives, flushing (warmth, redness, or tingly feeling);

• trouble swallowing; or

• swelling of your face, lips, tongue, or throat.

Some side effects may occur during the injection. Tell your caregiver right away if you have any of these signs of an infusion reaction:

• chest pain, tightness in your throat, fast or slow heartbeats, pounding in your neck or ears;

• shortness of breath, stuffy nose, feeling hot or cold;

• tiredness, drowsiness, dizziness, feeling like you might pass out;

• rash, itching, numbness or tingling ;

• nausea, vomiting, stomach pain, diarrhea; or

• headache, muscle pain, swelling in your hands or feet.

Common Fabrazyme side effects may include:

• fever, chills, cough;

• dizziness;

• swelling in your hands or feet;

• numbness or tingling;

• feeling tired;

• rash; or

• cold symptoms such as stuffy nose, sneezing, sore throat.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Other drugs may interact with agalsidase beta, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell your doctor about all your current medicines and any medicine you start or stop using.