Agalsidase Beta Dosage

Usual Adult Dose for Fabry Disease

1 mg/kg IV every 2 weeks

Comments:
-Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr).
-Clinical studies did not include a sufficient number of patients aged 65 years or older to determine if they respond differently than younger patients.

Use: For treatment of patients with Fabry disease

Usual Pediatric Dose for Fabry Disease

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

Comments:
-Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr).

Use: For treatment of patients with Fabry disease

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

For patients being rechallenged following a positive skin test to this drug or positive for antidrug IgE antibodies:
-Initial dose: 0.5 mg/kg IV every 2 weeks; once the patient tolerates the infusion, may increase to 1 mg/kg IV every 2 weeks

Precautions

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
-Pretreatment with antipyretics is recommended prior to start of infusion.
-Administer the diluted solution using an in-line low-protein-binding 0.2 micrometer filter; do not administer with other products in infusion tubing.
-Infuse initially at a rate of 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.
-Infusion rate may be slowed or stopped in the event of an infusion reaction; pretreatment with antipyretics and antihistamines is then recommended.

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:
-Administration duration not less than 1.5 hours (based on individual patient tolerability)

For patients being rechallenged following a positive skin test to this drug or positive for antidrug IgE antibodies:
-Initial infusion rate of 0.01 mg/min; once tolerability is established, may increase by slowly titrating upwards (doubled every 30 minutes) to a maximum rate of 0.25 mg/min, as tolerated

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

Reconstitution/Preparation Techniques:
-The manufacturer product information should be consulted.

IV Compatibility:
-Compatible with 0.9% Sodium Chloride Injection

General:
-Clinical studies did not include a sufficient number of patients aged 65 years or older to determine if they respond differently than younger patients.

Monitoring:
-Immunologic: Signs/symptoms of allergic reactions (including anaphylaxis) and/or infusion-related reaction

Patient Advice:
-You are encouraged to participate in the Fabry registry and should visit fabryregistry.com for more information.