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 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.