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.

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.

• 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.

• The manufacturer product information should be consulted.

• Compatible with 0.9% Sodium Chloride Injection, USP

• This drug has been shown to improve walking and stair climbing capacity.

• Hypersensitivity: Signs/symptoms of allergic reactions and/or infusion-related reactions (during and after infusion)

• 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.