Galsulfase Dosage
Medically reviewed by Drugs.com. Last updated on Dec 11, 2019.
Applies to the following strengths: 1 mg/mL
Usual Adult Dose for:
Usual Pediatric Dose for:
Additional dosage information:
Usual Adult Dose for Mucopolysaccharidosis Type VI
1 mg/kg IV once a week
Comments:
-Clinical studies did not include patients older than 29 years and it is therefore unknown if they respond differently than younger patients.
Use: For the treatment of patients with Mucopolysaccharidosis VI (MPS VI [Maroteaux-Lamy syndrome])
Usual Pediatric Dose for Mucopolysaccharidosis Type VI
5 years or older: 1 mg/kg IV once a week
Use: For the treatment of patients with MPS VI (Maroteaux-Lamy syndrome)
Renal Dose Adjustments
Data not available
Liver Dose Adjustments
Data not available
Precautions
Safety and efficacy have not been established in patients younger than 5 years.
Consult WARNINGS section for additional precautions.
Dialysis
Data not available
Other Comments
Administration advice:
-For IV infusion only
-Pretreatment is recommended 30 to 60 minutes prior to start of infusion and may include antihistamines with or without antipyretics.
-Prepare using low protein binding containers and administer diluted solution using a low protein binding infusion set with an in-line, low-protein-binding 0.2 micrometer filter; do not administer with other products in infusion tubing.
-Dilute to a final volume of 250 mL and infuse total volume over no less than 4 hours by controlled IV infusion using an infusion pump; initial infusion rate is 6 mL/hr for the first hour; if well tolerated, may increase to 80 mL/hr for the remaining 3 hours; extend infusion time up to 20 hours if infusion reaction occurs.
-For patients weighing 20 kg or less, or those susceptible to fluid overload, consider diluting in a volume of 100 mL and decreasing infusion rate so that the total infusion duration remains no less than 4 hours.
Storage requirements:
-Store vials at 2C to 8C (36F to 46F); protect from light; do not freeze or shake.
-Use diluted solution immediately; if cannot use immediately, may store at 2C to 8C (36F to 46F) and administered within 48 hours from time of dilution to completion of administration.
Reconstitution/preparation techniques:
-The manufacturer product information should be consulted.
IV compatibility:
-Compatible with 0.9% Sodium Chloride Injection, USP
General:
-This drug has been shown to improve walking and stair climbing capacity.
Monitoring:
-Hypersensitivity: Signs/symptoms of allergic reactions and/or infusion-related reactions (during and after infusion)
Patient advice:
-Seek medical attention if you experience any adverse reactions, including allergic reactions and anaphylaxis.
-You are encouraged to register in the MPS VI Clinical Surveillance Program. For additional information: 1-800-983-4587.
Further information
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.
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Consumer resources
Other brands: Naglazyme