Prepare NAGLAZYME® (galsulfase)
The recommended dose of NAGLAZYME® (galsulfase) is 1 mg/kg of body weight administered once weekly as an IV infusion over no less than 4 hours. Calculate the patient’s NAGLAZYME dose as follows:
- Determine the number of vials needed using the 2‐step algorithm below
- Round up to the next 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
NAGLAZYME dosing algorithm1
Before withdrawing NAGLAZYME from the vial, visually inspect each vial for particulate matter and discoloration.
The solution should be clear to slightly opalescent and colorless to pale yellow. Some translucency may be present in the solution. Do not use if the solution is discolored or if there is particulate matter in the solution. Do not freeze or shake vial.1
Calculate total infusion volume
All MPS VI study patients, including those with weights as low as 14 kg, were infused using 250-mL total volume.
Consider using a 100‐mL infusion bag for patients who are1:
- 20 kg and under
- Susceptible to fluid overload due to pulmonary disease, cardiac valvular disease, or congestive heart failure
Prepare the NAGLAZYME infusion
For a 250‐mL infusion bag:
- Withdraw and discard a volume of the 0.9% Sodium Chloride Injection, 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
Using an 18‐gauge non‐filter needle, slowly withdraw the calculated dose of NAGLAZYME from the appropriate number of vials, using caution to avoid excessive agitation, bubbles, and foaming.
Slowly add the NAGLAZYME to the 0.9% Sodium Chloride Injection, USP, bag, angling the needle tip.
Gently rotate the infusion bag to mix—do not shake. Agitation may denature NAGLAZYME, rendering it biologically inactive. Therefore:
- Use care to avoid agitation of the solution
- Do not use a filter needle
- Do not shake the solution
- Do not use a pneumatic tube delivery system
- Do not use Volutrol or Buretrol straight IV tubing
- Avoid generating bubbles or foam
Label the infusion bag per your institution’s policy. Do not mix NAGLAZYME with other medicinal products.1
NAGLAZYME does not contain any preservatives; therefore, after dilution with saline in the infusion bag, any unused product or waste material should be discarded and disposed of in accordance with local requirements.1