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

Therapeutic Classification: replacement enzyme

Pharmacologic Classification: enzymes

• Fabry disease.

• Replaces a deficient enzyme.

• Prevents damage from accumulation of globotriaosylceramide in capillary endothelium.

Absorption: IV administration results in complete bioavailability.

Distribution: Widely distributed.

Metabolism/Excretion: Unknown.

Half-life: 45–102 min.

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.

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

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

• Assess for infusion reactions (fever, rigors, chest tightness, hypertension, hypotension, pruritus, myalgia, dyspnea, urticaria, abdominal pain, headache, nausea, vomiting). Pretreat patients with antipyretics. If an infusion reaction occurs, regardless of pretreatment, decrease infusion rate, temporarily stop the infusion, and/or administer additional antipyretics, antihistamines, and/or oral corticosteroids. Infusion reactions decline in frequency with continued use; serious reactions may still occur with extended of therapy.
• Monitor for signs and symptoms of hypersensitivity reactions (bronchospasm, urticaria, hypotension, swelling of the face, mouth, and throat, bronchospasm, hypotension, generalized urticaria, dysphagia, rash, dyspnea, flushing, chest discomfort, pruritus, nasal congestion). If anaphylactic or severe hypersensitivity reactions occur, immediately discontinue infusion and start emergency treatment. Ensure medical support measures are readily available with each infusion.
• Monitor cardiac function during treatment in patients with previously compromised cardiac function.

• Testing for IgE antibodies are available for patients who experienced hypersensitivity reactions. There are no marketed tests for antibodies against Fabrazyme. If testing is warranted, contact Genzyme Corporation at 1-800-745-4447. Patients who have tested positive for Fabrazyme-specific IgE antibody have been rechallenged with Fabrazyme using a rechallenge protocol.

IV Administration:

• Administer antipyretics prior to infusion.
• Intermittent Infusion: Allow refrigerated agalsidase and diluent to reach room temperature prior to reconstitution (30 min). Determine number of 35 mg and 5 mg vials needed for dose. Reconstitution: Reconstitute each vial by slowly injecting 7.2 mL and 1.1 mL of Sterile Water for Injection into each 35 mg vial and 5 mg vial, respectively. Inject down the inside wall of vial. Roll and tilt each vial gently. Solution is clear and colorless; do not administer solutions that are discolored or contain particulate matter.
  Diluent: Remove an equal volume of 0.9% NaCl from a 500 mL infusion bag, then dilute further with 0.9% NaCl for a final volume of 500 mL. Inject reconstituted agalsidase directly into 0.9% NaCl, do not inject into air space. Discard unused portion of vial. Gently invert bag to mix solution, avoid vigorous shaking or agitation. Diluted solution may be filtered through an in-line low protein-binding 0.2 micrometer filter during administration. Use reconstituted and diluted solutions immediately or within 24 hrs..
• Rate: Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr). Infusion rate may be slowed if infusion reaction occurs. After tolerance to rate is well established, infusion may be increased in increments of 0.05–0.08 mg/min (increments of 3–5 mg/hr) each subsequent infusion.
• Y-Site Incompatibility: Do not infuse agalsidase in the same IV line with other products.

• 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.

• Reduction of globotriaosylceramide deposition in capillary endothelium of the kidney and certain other cell types.

Fabrazyme

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