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Agalsidase Beta Dosage

Medically reviewed on January 29, 2018.

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 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.

Further information

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

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