Agalsidase Beta Dosage

Medically reviewed by Drugs.com. Last updated on Jun 1, 2023.

Usual Adult Dose for Fabry Disease

1 mg/kg via IV infusion every 2 weeks

INFUSION RATE: Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr); once tolerance to the infusion is well established, may increase infusion rate in increments of 0.05 to 0.08 mg/min with each subsequent infusion
Maximum infusion rate (weight less than 30 kg): 0.25 mg/minute
Minimum infusion duration (weight 30 kg or greater): 1.5 hours (based on individual patient tolerability)

Comments:

• Antipyretics are recommended prior to infusions; anti-histamines should also be given in patients who have had an infusion-associated reaction.
• For patients who have had a positive skin test, see dose adjustment section for rechallenge administration.

Use: For treatment of patients with confirmed Fabry disease.

Usual Pediatric Dose for Fabry Disease

2 years or older:
1 mg/kg via IV infusion every 2 weeks

INFUSION RATE: Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr); once tolerance to the infusion is well established, may increase infusion rate in increments of 0.05 to 0.08 mg/min with each subsequent infusion
Maximum infusion rate (weight less than 30 kg): 0.25 mg/minute
Minimum infusion duration (weight 30 kg or greater): 1.5 hours (based on individual patient tolerability)

Comments:

• Antipyretics are recommended prior to infusions; anti-histamines should also be given in patients who have had an infusion-associated reaction.
• For patients who have had a positive skin test, see dose adjustment section for rechallenge administration.

Use: For treatment of patients 2 years or older with confirmed Fabry disease.

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

Rechallenge Protocol: Patients who have had a positive skin test or who have tested positive for anti-drug IgE may be successfully rechallenged.

• Initial rechallenge: Administer low dose at a lower infusion rate, for example: 0.5 mg/kg at 0.01 mg/minute
• Once patient tolerates infusion, slowly increase infusion rate, for example: double infusion rate every 30 minutes up to a maximum rate of 0.25 mg/minute as tolerated

Precautions

CONTRAINDICATIONS:

• None

Safety and efficacy have not been established in patients younger than 2 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration Advice:

• For IV infusion only; due to potential for severe infusion-associated reactions, administration should occur in a setting with appropriate medical support measures readily available
• Pretreat with antipyretics prior to start of infusion; anti-histamines should also be given in patients who have had an infusion-associated reaction
• Administer diluted solution using an in-line low-protein-binding 0.2 micrometer filter; do not administer with other products in infusion tubing

INFUSION RATES:

• Initial infusion rate: No more than 0.25 mg/min (15 mg/hr)
• Once tolerance to infusion is well established, may increase in increments of 0.05 to 0.08 mg/min (3 to 5 mg/hr) with each subsequent infusion
• For patients weighing less than 30 kg: Maximum infusion rate of 0.25 mg/min (15 mg/hr)
• For patients weighing 30 kg or more: Minimum administration duration should not be less than 1.5 hours (based on individual patient tolerability)

RECHALLENGE (Following a positive skin test or positive test for IgE antibodies): See Dosage Adjustments
INFUSION-ASSOCIATED-REACTIONS:

• If an infusion associated reaction occurs, decrease the infusion rate, temporarily stop the infusion, and/or administer additional antipyretics, antihistamines, and/or steroids
• If a severe infusion-related reaction occurs, immediately discontinue infusion and treat appropriately

Reconstitution/preparation techniques:

• Single use only; discard unused product
• Avoid shaking or agitating during preparation; do not use filter needles during preparation of infusion
• Allow vials and diluent to reach room temperature (approximately 30 minutes)
• Reconstitute vials with appropriate volume of sterile water for injection; roll and tilt vial gently
• Further dilute with 0.9% sodium chloride to a total volume based on patient weight; consult manufacturer product information for specific instructions

Storage Requirements:

• Store vials at 2C to 8C (36F to 46F); do not shake or agitate
• Use diluted and reconstituted solution immediately; if cannot use immediately, may store for up to 24 hours at 2C to 8C (36F to 46F)

IV Compatibility:

• Compatible with 0.9% Sodium Chloride Injection
• Do not infuse this drug in the same IV line with other products

General:

• There are no marketed tests for agalsidase beta-specific IgE antibodies; if testing is warranted, contact your local Genzyme representative or Genzyme Corporation at 1-800-745-4447.

Monitoring:

• Monitor for signs/symptoms of allergic reactions (including anaphylaxis) and/or infusion-related reactions

Patient Advice:

• Patients should understand that due to potential for severe infusion-associated reactions, administration should only occur in a setting with appropriate medical support measures readily available.
• Patients should be informed of the registry that has been established to better understand the variability and progression of Fabry disease in the population as a whole and in women; patients are encouraged to participate; for information, visit www.registrynxt.com or call 1-800-745-4447, extension 15500.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

Medical Disclaimer