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

1 mg/kg IV q2Weeks  

Also see Administration

Dosage Forms & Strengths

injection, lyophilized powder for reconstitution

  • 5mg/vial
  • 35mg/vial

Fabry Disease

<8 years: Safety and efficacy not established

≥8 years: 1 mg/kg IV q2Weeks  

Also see Administration

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Interactions

Interaction Checker

and agalsidase beta

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    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%)

            Serum 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%)

            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

            Hypersensitivity to product or components

            Cautions

            Life-threatening anaphylactic and severe allergic reactions observed; if anaphylactic or severe allergic reactions occur, immediately discontinue treatment and initiate necessary emergency treatment; appropriate medical support measures should be readily available when drug is administered

            In clinical trials, 59% of patients experienced infusion-associated reactions during administration, some of which were severe; severe reactions are generally managed with administration of antihistamines, corticosteroids, intravenous fluids, and/or oxygen, when clinically indicated

            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

            Patients with advanced Fabry disease may have compromised cardiac function, which may predispose them to a higher risk of severe complications from infusion-associated reactions; closely monitor patients with compromised cardiac function during administration

            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

            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

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

            IV Preparation

            Reconstitute with 7.2 mL of SWI (35-mg vial) and 1.1 mL SWI (5-mg vial) to yield a 5 mg/mL clear, colorless solution

            Visually inspect reconstituted vials for particulate matter and discoloration

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

            Recommended minimum total volume per patient's weight

            • ≤35 kg: 50 mL
            • 35.1-70 kg: 100 mL
            • 70.1-100 kg: 250 mL
            • >100 kg: 500 mL

            Remove dose amount of NS from infusion bag and replace with 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

            Unused vials:

            Reconstituted and diluted solutions

            • Use reconstituted and diluted solutions immediately
            • If immediate use is not possible, store for ≤24 hr at 2-8°C (36-46°F)
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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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            Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.