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Agalsidase Beta

Medically reviewed on Nov 15, 2018


(aye GAL si days BAY ta)

Index Terms

  • Alpha-Galactosidase-A (Recombinant)
  • r-h α-GAL

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Solution Reconstituted, Intravenous [preservative free]:

Fabrazyme: 5 mg (1 ea); 35 mg (1 ea) [contains mouse (murine) and/or hamster protein]

Brand Names: U.S.

  • Fabrazyme

Pharmacologic Category

  • Enzyme


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.


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


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

Use: Labeled Indications

Fabry disease: For use in patients with Fabry disease. Agalsidase beta reduces globotriaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types.


There are no contraindications listed within the manufacturer's labeling.

Dosing: Adult

Fabry disease: IV: 1 mg/kg every 2 weeks

Dosing: Geriatric

Refer to adult dosing.

Dosing: Pediatric

Fabry disease: Children ≥8 years and Adolescents: IV: Refer to adult dosing

Dosing: Renal Impairment

No dosage adjustment required.

Dosing: Hepatic Impairment

There are no dosage adjustments provided in the manufacturer's labeling.

Dosing: Adjustment for Toxicity

Patient with IgE antibodies or a positive skin test to agalsidase beta (rechallenge): IV: 0.5 mg/kg every 2 weeks at an initial maximum infusion rate of 0.01 mg/minute; may gradually escalate dose (to maximum of 1 mg/kg every 2 weeks) and/or infusion rate (doubling the infusion rate every 30 minutes to a maximum rate of 0.25 mg/minute) as tolerated.


IV: Allow vials and diluent to reach room temperature prior to reconstitution (~30 minutes). Each 35 mg vial should be reconstituted with 7.2 mL SWFI; reconstitute 5 mg vials with 1.1 mL SWFI; inject down internal side wall of vial; roll and tilt gently; do not shake. Resulting solution contains 5 mg/mL. Do not use filter needle to prepare. To make final infusion solution, add the desired amount of reconstituted solution to NS to make a final volume based on patient weight (see table for dilution volumes). Prior to adding the volume of agalsidase beta dose to the NS, remove an equal volume of NS. Avoid vigorous shaking or agitation.

Recommended Minimum Volumes for Dilution

Patient Weight


Minimum Total Volume




35.1 - 70


70.1 - 100




Table has been converted to the following text:

Recommended minimum total volume for dilution

Patient weight ≤35 kg: 50 mL

Patient weight 35.1 - 70 kg: 100 mL

Patient weight 70.1 - 100 kg: 250 mL

Patient weight >100 kg: 500 mL


IV: Antipyretics should be administered prior to infusion. Infuse through a low protein binding 0.2 micron in-line filter. Initial infusion rate should not exceed 0.25 mg/minute (15 mg/hour). Interrupt or decrease rate in the event of an infusion reaction; may be restarted after resolution of symptoms and/or after administration of antipyretics, antihistamines, and/or steroids. After patient tolerance to the infusion is established, rate may be increased in increments of 0.05-0.08 mg/minute (3-5 mg/hour) with each subsequent infusion. Maximum infusion rate: Patients <30 kg: 0.25 mg/minute; patients ≥30 kg: Infuse over at least 1.5 hours. An initial maximum infusion rate of 0.01 mg/minute should be used for rechallenge in patients with IgE antibodies or who have had a positive skin test to agalsidase beta; may increase infusion rate (doubling the infusion rate every 30 minutes) to a maximum rate of 0.25 mg/minute as tolerated.


Store intact vials between 2°C and 8°C (36°F and 46°F). Reconstituted solutions and solutions diluted in NS are stable for 24 hours at 2°C and 8°C (36°F and 46°F).

Drug Interactions

Amiodarone: May diminish the therapeutic effect of Agalsidase Beta. Avoid combination

Chloroquine: May diminish the therapeutic effect of Agalsidase Beta. Avoid combination

Gentamicin (Systemic): May diminish the therapeutic effect of Agalsidase Beta. Avoid combination

Adverse Reactions


Cardiovascular: Peripheral edema (21%), hypertension (14%)

Central nervous system: Chills (43%), headache (39%), paresthesia (31%), procedural pain (25%), fatigue (24%), dizziness (21%), pain (16%), sensation of cold (11%)

Dermatologic: Skin rash (20%)

Immunologic: Development of IgG Antibodies (69% to 79%)

Local: Infusion site reaction (50% to 55%, severe ≥5%)

Neuromuscular & skeletal: Limb pain (19%), back pain (16%), myalgia (14%)

Respiratory: Upper respiratory tract infection (44%), cough (33%), nasal congestion (19%), lower respiratory tract infection (18%)

Miscellaneous: Fever (39%)

1% to 10%:

Cardiovascular: Tachycardia (9%), bradycardia (≥5%), chest discomfort (≥5%), chest pain (≥5%), facial edema (≥5%), flushing (≥5%), hypotension (≥5%), ventricular hypertrophy (5%)

Central nervous system: Hypoesthesia (9%), anxiety (6%), burning sensation (6%), depression (6%), falling (6%)

Dermatologic: Pruritus (10%), excoriation (9%), pallor (≥5%), urticaria (≥5%), thermal injury (4%)

Gastrointestinal: Toothache (6%), abdominal pain (≥5%), diarrhea (≥5%), nausea (≥5%), vomiting (≥5%), xerostomia (4%)

Hematologic & oncologic: Bruise (4%)

Hypersensitivity: Anaphylaxis, hypersensitivity reaction

Infection: Fungal infection (5%), viral infection (5%), localized infection (4%)

Neuromuscular & skeletal: Muscle spasm (5%)

Otic: Tinnitus (8%), hypoacusis (5%)

Renal: Increased serum creatinine (9%)

Respiratory: Sinusitis (9%), dyspnea (8%), respiratory congestion (8%), pharyngitis (6%), wheezing (6%), pharyngeal edema (≥5%)

Miscellaneous: Procedural complications (postprocedure, 10%)

Frequency not defined:

Cardiovascular: Cardiac arrhythmia, cerebrovascular accident, low cardiac output

Central nervous system: Ataxia, vertigo

Genitourinary: Nephrotic syndrome

<1%, postmarketing, and/or case reports: Anaphylactic shock, angioedema (including auricular edema, dysphagia, lip edema, ocular edema, pharyngeal edema, tongue edema), arthralgia, bronchospasm, cardiac failure, decreased oxygen saturation, erythema, hyperhidrosis, hypersensitivity angiitis, hypoxia, increased lacrimation, lymphadenopathy, myocardial infarction, palpitations, pneumonia, renal failure, respiratory failure, rhinorrhea, sepsis, weakness


Concerns related to adverse effects:

• Anaphylaxis/allergic reactions: Life-threatening anaphylactic and severe allergic reactions have been reported. Reactions may include angioedema, bronchospasm, chest discomfort, dysphagia, dyspnea, flushing, hypotension, nasal congestion, pruritus, rash, and urticaria. Stop infusion if severe reactions occur; immediate medical support should be readily available. Use caution when administering to patients with history of an anaphylactic or severe allergic reaction.

• Antibody formation: Development of IgG antibodies is common and has been observed within 3 months from the onset of therapy. Some patients may also develop IgE antibodies or skin test reactivity; consider IgE testing in patients with allergic reaction. Rechallenge of patients with IgE-mediated reaction or who have had a positive skin test may be done with caution.

• Infusion reactions: Infusion-related reactions are common, and may be severe (chills, vomiting, hypotension, paresthesia); pretreatment with antipyretics and antihistamines is advised. Decrease infusion rate, temporarily discontinue infusion, and/or administer additional antipyretics, antihistamines, and/or steroids to manage infusion reactions. Immediate discontinuation of infusion should be considered for severe reactions. Appropriate medical support for the management of infusion reactions should be readily available. Infusion reactions have occurred despite premedication. Use with caution when readministering to patients with history of infusion reactions.

Disease-related concerns:

• Cardiovascular disease: Use with caution in patients with cardiovascular disease; may have increased risk of complications from infusion reactions; monitor closely.

Other warnings/precautions:

• Registry: A registry has been created to monitor therapeutic responses and adverse effects during long-term treatment, as well as effects on pregnant and breast-feeding women and their offspring; patients should be encouraged to register ( or 1-800-745-4447).

Monitoring Parameters

Development of IgG or IgE antibodies in patients with suspected allergic reactions (test available from manufacturer). Monitor for infusion-related reactions.

Pregnancy Risk Factor


Pregnancy Considerations

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

Patient Education

• Discuss specific use of drug and side effects with patient as it relates to treatment. (HCAHPS: During this hospital stay, were you given any medicine that you had not taken before? Before giving you any new medicine, how often did hospital staff tell you what the medicine was for? How often did hospital staff describe possible side effects in a way you could understand?)

• Patient may experience common cold symptoms, cough, anxiety, or back pain. Have patient report immediately to prescriber signs of allergic reaction with organ failure (fever, rash, fatigue, flu-like signs, nausea, vomiting, diarrhea, abdominal pain, pharyngitis, cough, or difficulty breathing), signs of depression (suicidal ideation, anxiety, emotional instability, or confusion), hearing impairment, tinnitus, severe dizziness, passing out, difficulty swallowing, or signs of infusion reactions (HCAHPS).

• Educate patient about signs of a significant reaction (eg, wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat). Note: This is not a comprehensive list of all side effects. Patient should consult prescriber for additional questions.

Intended Use and Disclaimer: Should not be printed and given to patients. This information is intended to serve as a concise initial reference for healthcare professionals to use when discussing medications with a patient. You must ultimately rely on your own discretion, experience and judgment in diagnosing, treating and advising patients.

Further information

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