agalsidase beta (Rx)

Brand and Other Names:Fabrazyme
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

powder for injection

  • 5mg/vial
  • 35mg/vial
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Fabry Disease

1 mg/kg IV infusion q2Weeks 

Infuse initial infusion over 4-6 hours; subsequent infusions may be administered at 3-5 mg/min

Dosage Forms & Strengths

powder for injection

  • 5mg/vial
  • 35mg/vial
more...

Fabry Disease

<8 years: Safety & efficacy not established

≥8 years: As adult; 1 mg/kg IV infusion 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

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            Adverse Effects

            >10%

            Infusion Reactions (50-55%)

            • Decrease frequency with continued treatment
            • Bradycardia, flushing, hypertension, hypotension, peripheral edema, tachycardia
            • Chills, dizziness, fatigue, feeling hot or cold, headache, pain in extremity, paresthesia, pyrexia, somnolence
            • Abdominal pain, diarrhea, nausea, vomiting
            • Pruritus, Urticaria
            • Myalgia
            • Chest pain, dyspnea, nasal congestion, throat tightness

            Upper respiratory tract infections (44%)

            Chills (43%)

            Headache (39%)

            Pyrexia (39%)

            Paresthesia (31%)

            Fatigue (24%)

            Dizziness (21%)

            Edema peripheral (21%)

            Cough (20%)

            Pain in extremity (19%)

            Lower respiratory tract infection (18%)

            Back pain (16%)

            Pain (16%)

            Myalgia (14%)

            Hypertension (14%)

            Feeling cold (11%)

            1-10%

            Pruitus (10%)

            Excoriation (9%)

            Sinusitis (9%)

            Sr creatinine increased (9%)

            Tachycardia (9%)

            Dyspnea (8%)

            Respiratory tract congestion (8%)

            Tinnitus (8%)

            Anxiety (6%)

            Burning sensation (6%)

            Depression (6%)

            Fall (6%)

            Pharyngitis (6%)

            Toothache (6%)

            Wheezing (6%)

            Chest discomfort (5%)

            Fungal infection (5%)

            Hot flash (5%)

            Hypoacusis (5%)

            Muscle spasms (5%)

            Ventricular wall thickening (5%)

            Viral infections (5%)

            Confusion (4%)

            Dry mouth (4%)

            Localized infection (4%)

            Thermal burn (4%)

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            Warnings

            Contraindications

            Hypersensitivity to product or components

            Cautions

            Moderate-to-severe HTN, renal impairment, febrile patients, patients with prior sensitivity to agalsidase products

            Severe reactions occur may occur despite premedication; decrease infusion rate or temporarily discontinue infusion and/or administer additional antihistamines, antipyretics, and/or steroids to manage infusion reactions; in severe reactions may consider immediate discontinuation of infusion

            Development of IgG antibodies reported within 3 months from the onset of therapy; some may also develop IgE antibodies

            Premedicate with antipyretics 1 hr before infusion

            Use caution in patients with cardiovascular diseases

            Fabry Disease Registry has been created to monitor therapeutic responses and adverse effects; www.fabryregistry.com or 1-800-745-4447

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            Pregnancy & Lactation

            Pregnancy Category: B

            Lactation: Not known if distributed into breast milk, use caution

            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

            Pharmacokinetics

            Half-Life: 86-151 min (children); 45-119 min (adults)

            Onset: 10-20 wk

            Vd: 247-1097 mL/kg; 81-570 mL/kg

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            Administration

            IV Incompatibilities

            Should not be infused in the same intravenous line with other products.

            IV Preparation

            Reconstitute 35 mg vial by injecting 7.2 mL of SWI & 5 mg vial by injecting 1.1 mL SWI to yield a 5 mg/mL clear, colorless solution

            Visually inspect the reconstituted vials for particulate matter & discoloration

            Do not use the reconstituted solution if there is particulate matter or if it is discolored

            The reconstituted solution should be further diluted with NS to a final total volume of 50-500 mL based on dosage

            Remove dose amount of NS from infusion bag & replace w/ drug solution

            IV Administration

            Reconstituted & diluted solns should be used immediately (drug contains no preservatives); however may be stored in fridge for up to 24 hr

            Initial infusion rate

            • Not to exceed 0.25 mg/min (15 mg/hr); may incr by 0.05-0.08 mg/min (3-5 mg/hr) for on each subsequent infusion
            • <30 kg: not to exceed 0.25 mg/min
            • >30 kg: infuse over at least 1.5 hr

            Do not shake or agitate product

            Do not use filter needles during the preparation of the infusion

            Storage

            Refrigerate

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            Formulary

            FormularyPatient Discounts

            Adding plans allows you to compare formulary status to other drugs in the same class.

            To view formulary information first create a list of plans. Your list will be saved and can be edited at any time.

            Adding plans allows you to:

            • View the formulary and any restrictions for each plan.
            • Manage and view all your plans together – even plans in different states.
            • Compare formulary status to other drugs in the same class.
            • 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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