Agalsidase Beta Dosage
Medically reviewed by Drugs.com. Last updated on Jun 1, 2023.
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
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
- 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)
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.
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