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Agalsidase beta

Generic Name: agalsidase beta (a GAL sih daze BAY tah)
Brand Name: Fabrazyme

Medically reviewed on January 29, 2018

What is agalsidase beta?

Agalsidase beta 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.

Agalsidase beta is used in the treatment of Fabry disease.

Agalsidase beta may also be used for purposes other than those listed here.

Important Information

Many people have a severe reaction to agalsidase beta. Tell your caregiver right away if you have a side effect such as: 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 feeling light-headed.

Before taking this medicine

To make sure agalsidase beta is safe for you, tell your doctor if you have:

  • heart problems; or

  • if you have ever had an allergic reaction to agalsidase beta or have antibodies to the medication.

FDA pregnancy category B. Agalsidase beta is not expected to harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant during treatment.

It is not known whether agalsidase beta passes into breast milk or if it could harm a nursing baby. Tell your doctor if you are breast-feeding a baby.

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 agalsidase beta. Taking part in this registry is especially important if you are pregnant or breast-feeding.

How should I use agalsidase beta?

Agalsidase beta is injected into a vein through an IV. A healthcare provider will give you this injection.

Agalsidase beta is usually given once every 2 weeks. Follow your doctor's instructions.

While using agalsidase beta, you may need frequent medical tests.

What happens if I miss a dose?

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

What happens if I overdose?

Since this medication is given by a healthcare professional in a medical setting, an overdose is unlikely to occur.

What should I avoid while taking agalsidase beta?

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

Agalsidase beta side effects

Get emergency medical help if you have any of these signs of an allergic reaction:

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

  • trouble swallowing, chest discomfort, difficult breathing, feeling light-headed; or

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

Many people have a severe reaction to the agalsidase beta infusion (side effects that occur during the injection). Tell your caregiver right away if you have any of these signs of an infusion reaction:

  • fever, headache, chills, stuffy nose, muscle pain, back pain, dizziness, drowsiness, tired feeling;

  • pale skin, feeling hot or cold, itching, numbness or tingly feeling, swelling in your hands or feet;

  • nausea, vomiting, tight feeling in your throat, stomach pain, diarrhea;

  • chest pain, fast or slow heart rate, feeling short of breath; or

  • a light-headed feeling, like you might pass out.

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.

See also: Side effects (in more detail)

Agalsidase beta 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 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

What other drugs will affect agalsidase beta?

Other drugs may interact with agalsidase beta, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell each of your health care providers about all medicines you use now and any medicine you start or stop using.

Further information

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

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

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