Fabrazyme

— THERAPEUTIC CATEGORIES —
  • Inborn errors of metabolism

Fabrazyme Generic Name & Formulations

General Description

Agalsidase beta 5mg, 35mg; per vial; lyophilized cake or pwd for IV infusion after reconstitution and dilution; preservative-free.

Pharmacological Class

Recombinant human alpha-galactosidase A enzyme.

How Supplied

Single-use vial—1

Storage

Store vials at 2°C to 8°C (36°F to 46°F).

Manufacturer

Generic Availability

NO

Mechanism of Action

Agalsidase beta provides an exogenous source of α-galactosidase A in Fabry disease patients. Agalsidase beta is internalized and transported into lysosomes where it exerts enzymatic activity and reduces accumulated GL-3.

Fabrazyme Indications

Indications

Fabry disease.

Fabrazyme Dosage and Administration

Adults and Children

<2yrs: not established. Pretreat with antihistamines, antipyretics, and/or corticosteroids. Give by IV infusion. ≥2yrs: 1mg/kg every 2 weeks. Initially infuse at a rate of 0.25mg/min; if tolerated, may increase by increments of 0.05–0.08mg/min with each subsequent infusion. <30kg: max rate 0.25mg/min. ≥30kg: minimum duration is 1.5hrs. Slow rate if infusion reactions occur. Rechallenge: initially give a low dose and infusion rate (eg, 0.5mg/kg at 0.01mg/min); once tolerated, may increase dose to 1mg/kg, titrate rate slowly upwards (doubled every 30mins up to max 0.25mg/min); see full labeling.

Fabrazyme Contraindications

Not Applicable

Fabrazyme Boxed Warnings

Not Applicable

Fabrazyme Warnings/Precautions

Warnings/Precautions

Have appropriate medical support measures readily available; discontinue immediately if anaphylaxis or severe hypersensitivity reactions occur. Consider testing for IgE antibodies in patients who experienced suspected hypersensitivity reactions; if positive, may be rechallenged. Risk of infusion-associated reactions; if mild or moderate reaction occurs, temporarily withhold, slow the infusion rate, and/or reduce dose; if severe reactions occur, discontinue immediately. Compromised cardiac function; monitor closely. Pregnancy. Nursing mothers.

Fabrazyme Pharmacokinetics

See Literature

Fabrazyme Interactions

Not Applicable

Fabrazyme Adverse Reactions

Adverse Reactions

Upper RTI, chills, pyrexia, headache, cough, paresthesia, fatigue, peripheral edema, dizziness, rash.

Fabrazyme Clinical Trials

See Literature

Fabrazyme Note

Notes

Register pregnant patients at www.registrynxt.com or call (800) 745-4447.

Fabrazyme Patient Counseling

See Literature