agalsidase beta (Rx)

Brand and Other Names:Fabrazyme
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

injection, lyophilized powder for reconstitution

  • 5mg/vial
  • 35mg/vial

Fabry Disease

Indicated for confirmed Fabry disease

1 mg/kg IV q2Weeks  

Dosage Forms & Strengths

injection, lyophilized powder for reconstitution

  • 5mg/vial
  • 35mg/vial

Fabry Disease

Indicated for confirmed Fabry disease in adults and pediatric patients aged ≥2 years

<2 years: Safety and efficacy not established

≥2 years: 1 mg/kg IV q2Weeks  

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Interactions

Interaction Checker

and agalsidase beta

No Results

     activity indicator 
    No Interactions Found
    Interactions Found

    Contraindicated

      Serious - Use Alternative

        Significant - Monitor Closely

          Minor

            All Interactions Sort By:
             activity indicator 

            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)

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              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

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              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
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              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.

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              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)

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              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
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              Images

              No images available for this drug.
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              Patient Handout

              Patient Education
              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) 2021 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.

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              Formulary

              FormularyPatient Discounts

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              • View the formulary and any restrictions for each plan.
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              • Access your plan list on any device – mobile or desktop.

              The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

              Tier Description
              1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
              2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
              3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
              4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
              5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
              6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
              NC NOT COVERED – Drugs that are not covered by the plan.
              Code Definition
              PA Prior Authorization
              Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
              QL Quantity Limits
              Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
              ST Step Therapy
              Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
              OR Other Restrictions
              Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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              Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.