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Generic name: agalsidase betaa-GAL-sih-daze-BAY-tah ]
Drug class: Lysosomal enzymes

Medically reviewed by Philip Thornton, DipPharm. Last updated on Jun 22, 2023.

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.


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

-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

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

Use: For treatment of patients with Fabry disease

What happens if I miss a dose?

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

What happens if I overdose?

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

What should I avoid while receiving Fabrazyme?

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

Fabrazyme side effects

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.

What other drugs will affect Fabrazyme?

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.

Popular FAQ

Fabrazyme is given by intravenous infusion every two weeks by a healthcare provider. How long the infusion of Fabrazyme takes depends on how well you tolerate Fabrazyme and your weight. Fabrazyme can cause infusion-associated reactions, such as chills, vomiting, low blood pressure, or paresthesia (burning or prickling sensations, usually in the hands), so your healthcare provider will start the infusion off slowly, at around 15mg per hour. This rate may be increased if you tolerate the infusion well and you weigh more than 30kg. The minimum infusion duration for people weighing more than 30kg is 1.5 hours. Continue reading

Fabrazyme is an enzyme replacement therapy that works by replacing the enzyme missing in people with Fabry disease, called alpha-galactosidase A (or alpha-GAL). Fabrazyme is a man-made form of alpha-GAL, called agalsidase beta, that works exactly the same as naturally occurring alpha-GAL, to break down and clear a fatty substance called globotriaosylceramide (GL-3) that accumulates in the body of people with Fabry disease. It is given by intravenous infusion every two weeks. Continue reading

Further information

Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use Fabrazyme only for the indication prescribed.

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.