Dosing and administration
NAGLAZYME® (galsulfase) is supplied as a sterile, nonpyrogenic, colorless to pale yellow, clear to opalescent solution. Each 5-mL single-use vial provides 5 mg of galsulfase (expressed as protein content).
Dosage recommendations
The recommended dose of NAGLAZYME is 1 mg/kg of body weight administered once weekly as an IV infusion over no less than 4 hours.
Preparation:
- Determine the number of vials needed using the 2-step formula below. Round to the nearest whole vial. Remove the required number of vials from the refrigerator and allow them to reach room temperature. Do not allow vials to remain at room temperature longer than 24 hours prior to dilution. Do not heat or microwave vials
- Step 1: Patient’s weight (kg) x 1 mL/kg NAGLAZYME =
total mL NAGLAZYME
- Step 2: Total mL NAGLAZYME ÷ 5 mL per vial =
Total vials needed
Visually inspect each vial for particulate matter and discoloration. The solution should be clear to slightly opalescent and colorless to pale yellow. A few translucent particles may be present. Do not use if the solution is discolored or if there is particulate matter in the solution
Determine the total infusion volume. All patients in the MPS VI study, including those with weights as low as 14 kg, were infused using the 250 mL total volume. Consider using a 100 mL infusion bag for patients who are 20 kg and under or susceptible to fluid overload due to pulmonary disease, cardiac valvular disease, or congestive heart failure
- For a 250 mL infusion bag. Withdraw and discard a volume of the 0.9% Sodium Chloride, USP, bag, equal to the volume of NAGLAZYME to be added
- For a 100 mL infusion bag. Withdrawing and discarding of dose volume is not necessary. Add the dose volume directly to the infusion bag
Slowly withdraw the calculated dose of NAGLAZYME from the appropriate number of vials, using caution to avoid excessive agitation, bubbles and foaming. Agitation may render NAGLAZYME biologically inactive
Add the NAGLAZYME to the 0.9% Sodium Chloride USP bag, angling the needle tip to ensure slow addition
Gently rotate the infusion bag to mix; do not shake
Label the infusion bag per your institution’s policy. Do not mix NAGLAZYME with other medicinal products
NAGLAZYME does not contain any preservatives; therefore, after dilution with saline in the infusion bag, any unused product or waste materials should be discarded and disposed of in accordance with local requirements.
Administration
Refer to the package insert, the prescribing physician’s orders and your institution’s policies and procedures for additional information and guidance.
- Premedication with antihistamines with or without antipyretics 30 minutes to 1 hour prior to infusion is recommended
- NALGLAZYME should be administered with a PVC infusion set equipped with an in-line low-protein-binding 0.2 µm filter
- Either a 100 mL or a 250 mL infusion bag may be used; 100 mL infusion bags should be considered in patients 20 kg and under who are susceptible to fluid volume overload
- The total dose of NAGLAZYME should be delivered over no less than 4 hours by controlled IV infusion using an infusion pump
- Examples:
- 250 mL: 6 mL for the first hour. If the infusion is well tolerated, the infusion rate can be increased to 81 mL/hour for approximately 3 hours
- 120 mL: 3 mL/hour for the first hour. If the infusion is well tolerated, the infusion rate can be increased to 39 mL/hour for approximately 3 hours
- Patient vital signs should be monitored for signs of infusion reactions
Please download our complete Dosage and Administration Guide for special considerations, recommended equipment and storage information. Or, receive training right from your computer with our Infusion Training Video.
Next: Infusion training video