• Fabry disease.

Contraindicated in:

• None noted.

Use Cautiously in:

• Compromised cardiac function (may have ↑ risk of infusion reactions)
• OB: Use during pregnancy only if potential maternal benefit justifies potential fetal risk
• Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant
• Pedi: Children <2 yr (safety and effectiveness not established).

CV: edema, hypotension, cardiomegaly, hypertension.

Derm: pallor.

GI: dyspepsia, nausea.

GU: testicular pain.

MS: skeletal pain, arthrosis.

Neuro: anxiety, depression, dizziness, headache.

Resp: bronchospasm.

Misc: infusion reactions , rigors, fever, HYPERSENSITIVITY REACTIONS (INCLUDING ANAPHYLAXIS).

Drug-Drug:

• None reported.

• Lyophilized powder for injection: 5 mg/vial; 35 mg/vial;

• IV (Adults and Children ≥2 yr): 1 mg/kg every 2 wk.

Fabrazyme

• Replaces a deficient enzyme.

• Prevents damage from accumulation of globotriaosylceramide in capillary endothelium.

Therapeutic Classification: replacement enzyme

Pharmacologic Classification: enzymes

Absorption: IV administration results in complete bioavailability.

Distribution: Widely distributed.

Metabolism/Excretion: Unknown.

Half-life: 45–102 min.

• Inform patient of purpose for agalsidase beta.
• Inform patients that a Registry has been established to better understand the variability and progression of Fabry disease and to monitor the long-term effects of Fabrazyme. Encourage patients to participate; participation is voluntary. For information visit www.registrynxt.com or call 800-745-4447 extension 15500.
• Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding. Encourage pregnant women and women of reproductive potential to enroll in Fabry patient registry to monitor the effect of Fabrazyme on pregnant women and their offspring.

agalsidase beta: uh-GAL-si-dase BAY-ta

NDC Code^*

• 58468- Genzyme Corporation
  • 58468-0040- Fabrazyme
    • 58468-0040-1- 1 VIAL, GLASS in 1 CARTON (58468-0040-1) > 7 mL in 1 VIAL, GLASS

• 58468- Genzyme Corporation
  • 58468-0041- Fabrazyme
    • 58468-0041-1- 1 VIAL, GLASS in 1 CARTON (58468-0041-1) > 1 mL in 1 VIAL, GLASS