Dosing & Uses
Dosage Forms & Strengths
injection, lyophilized powder for reconstitution
- 5mg/vial
- 35mg/vial
Fabry Disease
Indicated for confirmed Fabry disease
Interactions
Interaction Checker
No Results

Contraindicated
Serious - Use Alternative
Significant - Monitor Closely
Minor

Contraindicated (1)
- chloroquine
chloroquine decreases effects of agalsidase beta by pharmacodynamic antagonism. Contraindicated.
Serious - Use Alternative (2)
- amiodarone
amiodarone decreases effects of agalsidase beta by pharmacodynamic antagonism. Contraindicated.
- gentamicin
gentamicin decreases effects of agalsidase beta by pharmacodynamic antagonism. Contraindicated.
Monitor Closely (1)
- tobramycin inhaled
tobramycin inhaled and agalsidase beta both increase nephrotoxicity and/or ototoxicity. Modify Therapy/Monitor Closely. Avoid concurrent or sequential use to decrease risk for ototoxicity
Minor (0)
Adverse Effects
>10%
Adults
- Upper respiratory tract infection (53%)
- Chills (49%)
- Pyrexia (39%)
- Headache (39%)
- Cough (33%)
- Paresthesia 31%)
- Fatigue (24%)
- Peripheral edema (21%)
- Dizziness (21%)
- Rash (20%)
- Pain in extremity (19%)
- Myalgia (18%)
- Lower respiratory tract infection (18%)
- Pain (16%)
- Back pain (16%)
- Hypertension (14%)
Pediatric patients
- Headache (>20%)
- Abdominal pain (>20%)
- Pharyngitis (>20%)
- Fever (>20%)
- Nausea (>20%)
- Vomiting (>20%)
- Rhinitis (>20%)
- Diarrhea (>20%)
- Arthralgia (>20%)
- Dizziness (>20%)
1-10%
Adults
- Pruritus (10%)
- Tachycardia (9%)
- Excoriation (9%)
- Increased blood creatinine (9%)
- Tinnitus (8%)
- Dyspnea (8%)
- Fall (6%)
- Burning sensation (6%)
- Anxiety (6%)
- Depression (6%)
- Wheezing (6%)
- Hypoacusis (5%)
- Chest discomfort (5%)
- Fungal infection (5%)
- Viral infection (5%)
- Hot flush (5%)
Postmarketing Reports
Cardiovascular: Cardiorespiratory arrest, cardiac failure, myocardial infarction, palpitations
Infections: Sepsis and pneumonia
Infusion-associated reactions: Anaphylaxis, localized angioedema (eg, auricular swelling, eye swelling, dysphagia, lip swelling, edema, pharyngeal edema, face swelling, swollen tongue), and bronchospasm
General: Hyperhidrosis, asthenia, infusion site reaction
Lymphatic: Lymphadenopathy
Musculoskeletal: Arthralgia
Nasopharyngeal: Rhinorrhea
Neurologic: Cerebrovascular accident, hypoesthesia, oral hypoesthesia
Ophthalmologic: Increased lacrimation
Pulmonary: Respiratory failure, hypoxia
Renal: Renal failure
Dermatologic: Erythema
Vascular: Leukocytoclastic vasculitis
Warnings
Contraindications
None
Cautions
Anaphylaxis and hypersensitivity reactions
- In clinical trials and postmarketing safety experience, anaphylactic or severe hypersensitivity reactions reported
- Higher incidences of hypersensitivity reactions were observed in adults with persistent anti-Fabrazyme antibodies and with high antibody titer compared to that in antibody negative adults
- Consider testing for IgE antibodies in patients who experienced suspected hypersensitivity reactions
- Consider risks and benefits of continued treatment in patients with anti-Fabrazyme IgE antibodies
- No marketed tests for antibodies; if testing is warranted, contact Genzyme Corporation at 1-800-745-4447
- Patients who have had a positive skin test or have tested positive for Fabrazyme-specific IgE antibody have been rechallenged using a rechallenge protocol
- Rechallenge under direct supervision of qualified personnel, with appropriate medical support measures readily available
Infusion-associated reactions
- Infusion-associated reactions, some severe, reported
- Incidence was higher in patients positive for anti-Fabrazyme antibodies compared with those who were negative
- Severe infusion-associated reactions included chills, vomiting, hypotension, and paresthesia
- Other infusion-associated reactions included pyrexia, feeling hot or cold, dyspnea, nausea, flushing, headache, fatigue, pruritus, pain in extremity, hypertension, chest pain, throat tightness, abdominal pain, dizziness, tachycardia, nasal congestion, diarrhea, edema peripheral, myalgia, urticaria, bradycardia, and somnolence
- Ensure appropriate medical support measures are readily available during administration
- Closely monitor with history infusion-associated reactions
- Advanced Fabry disease may compromise cardiac function, which may potentially increase risk of severe complications from infusion-associated reactions; closely monitor with compromised cardiac function
Pregnancy & Lactation
Pregnancy
Registry available and monitors drug effects on pregnant women and their offspring
For more information, visit www.registrynxt.com or call 1-800-745-4447, extension 15500
Encourage patients to enroll for the registry
Available data from postmarketing case reports and case series with use in pregnant women have not identified a drug-associated risk of major birth defects, miscarriage or adverse maternal or fetal outcomes
Animal data
- Reproduction studies performed in rats at doses up to 68 times the human dose have revealed no evidence of effects on embryo-fetal development
Lactation
There are no data on the presence of agalsidase beta in either human or animal milk, the effects of the drug on the breastfed infant, or on milk production
Developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for therapy and any potential adverse effects on breastfed child from drug or from underlying maternal condition
Encourage lactating women to enroll in the registry
Pregnancy Categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk. C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done. D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk. X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist. NA: Information not available.Pharmacology
Mechanism of Action
Recombinant alpha-galactosidase A, an enzyme (deficient in Fabry disease) involved in the metabolism of glycosphingolipids (eg, GL-3); corrects abnormal glycosphingolipid metabolism resulting in reduced accumulation of glycosphingolipids and improvement of associated clinical manifestations; reduces GL-3 deposition in capillary endothelium
Absorption
Peak plasma concentration: 0.6-29.7 mcg/mL (0.3-1 mg/kg-dose)
Distribution
Vd (steady-state): 81-330 mL/kg (0.3-1 mg/kg-dose)
Elimination
Half-life: 45-102 min (0.3-1 mg/kg-dose)
Clearance: 0.8-4.6 mL/min/kg (0.3-1 mg/kg-dose)
Administration
IV Incompatibilities
Do not in same IV line with other products
IV Compatibilities
0.9% NaCl
IV Preparation
Calculate dose based on patient’s body weight and select a combination of 35-mg and 5-mg vials
Remove vials from refrigerator and allow them to reach room temperature before reconstituting (~30 min)
Reconstitution
- 35-mg vials: Reconstitute with 7.2 mL of sterile water for injection (SWI) (total extractable solution per vial is 7 mL)
- 5-mg vials: Reconstitute with 1.1 mL of SWI (total extractable solution per vial is 1 mL)
- Roll and tilt each vial gently to yield a 5 mg/mL clear, colorless solution
- Visually inspect for particulate matter and discoloration; discard if there is particulate matter or discoloration
- Discard any unused product
- Avoid shaking or agitating this product; do not use filter needles during preparation
Dilution
- Further dilute reconstituted solution with 0.9% NaCl to a total volume based on patient weight
- Slowly withdraw reconstituted solution to required dose volume and inject directly into 0.9% NaCl
- Do not inject in airspace within infusion bag; discard any vial with unused reconstituted solution
- Gently invert infusion bag to mix, avoiding vigorous shaking and agitation
-
Minimum total infusion volume based on patient’s weight
- ≤35 kg: 50 mL
- 35.1-70 kg: 100 mL
- 70.1 to 100 kg: 250 mL
- >100 kg: 500 mL
IV Administration
Administer as an IV infusion using an in-line low protein binding 0.2-micron filter
Do not infuse in the same IV line with other products
Appropriate medical support measures should be readily available
Administer antipyretics before infusion
Infusion rate
- 0.25 mg/min (15 mg/hr) initially; may increase rate based on patient’s weight once tolerance to infusion is well established
- >30 kg: Increase rate in increments of 0.05-0.08 mg/min (increments of 3-5 mg/hr) with each subsequent infusion; minimum infusion duration is 1.5 hr (based on tolerability)
- <30 kg: Maximum infusion rate is 0.25 mg/min (15 mg/hr)
- Slow infusion rate in event of an infusion-associated reaction
Rechallenge procedure
- Patients who have had a positive skin test or tested positive for anti-Fabrazyme IgE may be successfully rechallenged
- Initial rechallenge administration should be a low dose at a lower infusion rate (eg, ½ the therapeutic dose [0.5 mg/kg] at 1/25 of initial standard rate [0.01 mg/min])
- Once tolerated, may increase dose to 1 mg/kg and slowly titrate rate up (doubled q30min up to a maximum rate of 0.25 mg/min), as tolerated
Storage
Does not contain preservatives
Unused vials
- Single use only
- Refrigerate at 2-8ºC (36-46ºF); do not use after expiration date on vial
Reconstituted vials and diluted solutions
- Use immediately
- If immediate use is not possible, refrigerate at 2-8ºC (36-46ºF) for up to 24 hr
Images
Patient Handout
agalsidase beta intravenous
AGALSIDASE BETA - INJECTION
(a-GAL-si-dase BAY-ta)
COMMON BRAND NAME(S): Fabrazyme
USES: This medication is used to treat a certain inherited disorder (Fabry disease) that causes buildup of a certain fat substance (GL-3) in some parts of your body. Worsening of this disorder can lead to kidney disease, heart attack, or stroke. Agalsidase beta works by replacing an enzyme that your body lacks, which helps break down the fat substance (GL-3) in your body. This can help prevent symptoms such as pain (especially in the hands and feet), dark red spots on the skin, a decreased ability to sweat, cloudiness of the front part of the eye, stomach/intestinal problems, ringing in the ears (tinnitus), and hearing loss.
HOW TO USE: This medication is given by slow injection into a vein as directed by your doctor, usually every 2 weeks. The injection is given by a health care professional. The dosage is based on your medical condition, weight, and response to treatment.To help prevent reaction during the injection, your doctor will prescribe medications (such as acetaminophen, antihistamine) for you before the injection. Carefully follow your doctor's directions for taking all your medications. Tell your doctor right away if you have symptoms during the injection, such as chills, flushing, itching, nausea, vomiting, dizziness, numbness/tingling, shortness of breath, headache, or chest/abdominal pain. Your doctor may stop or slow the injection for a time, and/or give other medications if needed.Use this medication regularly to get the most benefit from it. It may help to mark your calendar when to receive each dose.Tell your doctor if your condition does not get better or if it gets worse.
SIDE EFFECTS: See also How to Use section.Injection reactions commonly occur. Pain at the injection site, fever, tiredness, muscle pain, dizziness, or cold symptoms (such as runny/stuffy nose, cough) may also occur. If any of these effects last or get worse, tell your doctor or pharmacist promptly.Remember that this medication has been prescribed because your doctor has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.Tell your doctor right away if you have any serious side effects, including: swelling feet/legs/hands.A very serious allergic reaction to this drug is rare. However, get medical help right away if you notice any symptoms of a serious allergic reaction, including: rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, trouble breathing.This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.In the US -Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.
PRECAUTIONS: Before using agalsidase beta, tell your doctor or pharmacist if you are allergic to it; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.Before using this medication, tell your doctor or pharmacist your medical history, especially of: heart problems.This drug may make you dizzy. Alcohol or marijuana (cannabis) can make you more dizzy. Do not drive, use machinery, or do anything that needs alertness until you can do it safely. Limit alcoholic beverages. Talk to your doctor if you are using marijuana (cannabis).Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products).Tell your doctor if you are pregnant before using this medication.It is unknown if this medication passes into breast milk. Consult your doctor before breast-feeding.
DRUG INTERACTIONS: Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.
OVERDOSE: If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call their local poison control center at 1-800-222-1222. Canada residents can call a provincial poison control center.
NOTES: Lab and/or medical tests may be done while you are using this medication. Keep all medical and lab appointments.
MISSED DOSE: It is important to get each dose of this medication as scheduled. If you miss a dose, ask your doctor or pharmacist right away for a new dosing schedule.
STORAGE: Not applicable. This medication is given in a hospital or clinic or doctor's office and will not be stored at home.
Information last revised August 2021. Copyright(c) 2023 First Databank, Inc.
IMPORTANT: HOW TO USE THIS INFORMATION: This is a summary and does NOT have all possible information about this product. This information does not assure that this product is safe, effective, or appropriate for you. This information is not individual medical advice and does not substitute for the advice of your health care professional. Always ask your health care professional for complete information about this product and your specific health needs.
Formulary
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