Agalsidase Beta Dosage
Medically reviewed by Drugs.com. Last updated on Jan 22, 2021.
Applies to the following strengths: 35 mg; 5 mg
Usual Adult Dose for:
Usual Pediatric Dose for:
Additional dosage information:
Usual Adult Dose for Fabry Disease
1 mg/kg via IV infusion every 2 weeks
-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 Fabry disease; this drug reduced globotriaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types,.
Usual Pediatric Dose for Fabry Disease
8 years or older:
1 mg/kg via IV infusion every 2 weeks
-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 Fabry disease; this drug reduced globotriaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types,.
Renal Dose Adjustments
Data not available
Liver Dose Adjustments
Data not available
Dose Adjustments
Rechallenge Protocol:
-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 8 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 1-800745-4447, extension 15500.
More about agalsidase beta
- Side Effects
- During Pregnancy or Breastfeeding
- Drug Interactions
- En Español
- Drug class: lysosomal enzymes
Consumer resources
- Other brands
- Fabrazyme
Professional resources
Related treatment guides
Further information
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.