Agalsidase beta

Name: Agalsidase beta

Agalsidase Beta Interactions

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

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.

Indications

Fabrazyme (agalsidase beta) is indicated for use in patients with Fabry disease. Fabrazyme (agalsidase beta) reduces globo-triaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types (see Clinical Studies).

How supplied

Fabrazyme (agalsidase beta) is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder. Fabrazyme (agalsidase beta) 35 mg vials are supplied in single-use, clear Type I glass 20 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic purple flip-off cap. Fabrazyme (agalsidase beta) 5 mg vials are supplied in single use, clear Type I glass 5 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic gray flip-off cap.

35 mg vial: NDC 58468-0040-1
  5 mg vial: NDC 58468-0041-1

Fabrazyme (agalsidase beta) is manufactured and distributed by: Genzyme Corporation, 500 Kendall Street, Cambridge, MA 02142. 1-800-745-4447 (phone). Issued: September 29, 2006. FDA rev date: 2233

Overdose

There have been no reports of overdose with Fabrazyme (agalsidase beta) . In clinical trials, patients received doses up to 3 mg/kg body weight. The adverse reactions experienced by patients who received treatment with 3 mg/kg were similar to the adverse reactions experienced by patients who received treatment with 1 mg/kg.

Side Effects of Agalsidase beta

Serious side effects have been reported with agalsidase beta. See the "Drug Precautions" section.

Common side effects of agalsidase beta include:

  • fever
  • chills
  • shortness of breath
  • feeling hot or cold
  • flushing
  • vomiting
  • chest pain
  • stomach pain
  • dizziness
  • diarrhea
  • throat tightness
  • tiredness
  • changes in heart rate
  • swelling in the face and limbs
  • back and muscle pain
  • nasal congestion
  • changes in blood pressure
  • nausea
  • headache
  • itching
  • anxiety

This is not a complete list of agalsidase beta side effects. Ask your doctor or pharmacist for more information.

Tell your doctor if you have any side effect that bothers you or that does not go away.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

What should I avoid while taking agalsidase beta?

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

Actions

  • Provides exogenous source of α-galactosidase that catalyzes the hydrolysis of glycosphingolipids, including GL-3, in patients with Fabry’s disease.1 2 3 5

  • May reverse histologic abnormalities and decrease clinical manifestations of Fabry’s disease.2 5

  • Reduces GL-3 inclusions in the capillary endothelium of the kidney, heart, and skin.a

What do I need to tell my doctor BEFORE I take Agalsidase Beta?

  • If you have an allergy to agalsidase beta or any other part of agalsidase beta.
  • If you are allergic to any drugs like this one, any other drugs, foods, or other substances. Tell your doctor about the allergy and what signs you had, like rash; hives; itching; shortness of breath; wheezing; cough; swelling of face, lips, tongue, or throat; or any other signs.

This medicine may interact with other drugs or health problems.

Tell your doctor and pharmacist about all of your drugs (prescription or OTC, natural products, vitamins) and health problems. You must check to make sure that it is safe for you to take this medicine with all of your drugs and health problems. Do not start, stop, or change the dose of any drug without checking with your doctor.

How is this medicine (Agalsidase Beta) best taken?

Use this medicine as ordered by your doctor. Read all information given to you. Follow all instructions closely.

  • It is given as an infusion into a vein over a period of time.

What do I do if I miss a dose?

  • Call your doctor to find out what to do.

Pharmacologic Category

  • Enzyme

Pharmacology

Agalsidase beta is a recombinant form of the enzyme alpha-galactosidase-A, which is required for the hydrolysis of GL-3 and other glycosphingolipids. The compounds may accumulate (over many years) within the tissues of patients with Fabry disease, leading to renal and cardiovascular complications. In clinical trials of limited duration, agalsidase been noted to reduce tissue inclusions of a key sphingolipid (GL-3). It is believed that long-term enzyme replacement may reduce clinical manifestations of renal failure, cardiomyopathy, and stroke. However, the relationship to a reduction in clinical manifestations has not been established.

Distribution

Vdss: Children: 247 to 1097 mL/kg; Adults: 81 to 570 mL/kg

Excretion

Clearance: Children: 1.1 to 5.8 mL/minute/kg; Adults: 0.8 to 4.9 mL/minute/kg

Half-Life Elimination

Dose dependent: Children: 86 to 151 minutes; Adults: 45 to 119 minutes

Pregnancy Risk Factor B Pregnancy Considerations

Adverse events have not been observed in animal reproduction studies. Women of childbearing potential are encouraged to enroll in the Fabry registry (www.registrynxt.com or 1-800-745-4447).

Liver Dose Adjustments

Data not available

Precautions

Safety and efficacy have not been established in patients younger than 8 years.

Consult WARNINGS section for additional precautions.

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