NAGLAZYME is indicated for patients with Mucopolysacchardosis VI (MPS VI). NAGLAZYME has been shown to improve walking and stair-climbing capacity.

The recommended dosage regimen of NAGLAZYME is mg/g of body weight administered once weekly as an intravenous infusion.

Pretreatment with antihistamines with or without antipyretics is recommended 30 to 60 minutes prior to the start of the infusion (see WARNINGS: Infusion Reactions).

The total volume of the infusion should be delivered over no less than 4 hours. NAGLAZYME should be reconstituted in 0. Sodium Chloride Injection, USP, to a final volume of 250 mL and delivered by controlled IV infusion using an infusion pump. The initial infusion rate should be 6 mL/h for the first hour. If the infusion is well tolerated, the rate of infusion may be increased to 80 mL/h for the remaining 3 hours. The infusion time can be extended up to 20 hours if infusion reactions occur.

For patients 20 kg and under who are susceptible to fluid volume overload, physicians may consider diluting NAGLAZYME in a volume of 100 mL. The infusion rate (mL/min) should be decreased so that the total infusion duration remains no less than 4 hours.

Each vial of NAG LAZY ME provides 5 mg of galsulfase (expressed in protein content) in 5 mL of solution and is intended for single use only. Do not use the vial more than one time. The concentrated solution for infusion must be diluted in 0.9% Sodium Chloride Injection, USP, using aseptic techniques. NAGLAZYME should be prepared using PVC containers and administered with a PVC infusion set equipped with an in-line, low-protein-binding 0.2 micrometer (µm) filter. There is no information on the compatibility of diluted NAGLAZYME with glass containers.

1. Determine the number of vials to be diluted based on the individual patient' s weight and the recommended dose of 1mg/Kg:

Patient's weight (kg) x 1 mL/kg of NAG LAZYME = Total # mL of NAG LAZYME

Total # of mL of NAGLAZYME ¸ 5 mL per vial = Total # of vials

Round to the nearest whole vial. Remove the required number of vials from the refrigerator to 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.

2. Before withdrawing the NAGLAZYME from the vial visually inspect each vial for particulate matter and discoloration. The NAGLAZYME 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.

3. From a 250 mL infusion bag of 0.9% Sodium Chloride Injection, USP, withdraw and discard a volume equal to the volume of NAG LAZY ME to be added. If using a 100mL infusion bag, this is not necessary.

4. Slowly withdraw the calculated volume of NAGLAZYME from the appropriate number of vials using caution to avoid excessive agitation. Do not use a filter needle, as this may cause agitation. Agitation may denature NAGLAZYME rendering it biologically inactive.

5. Slowly add the NAGLAZYME solution to the 0.9% Sodium Chloride Injection, USP, using care to avoid agitation ofthe solutions. Do not use a filter needle.

6. Gently rotate the infusion bag to ensure proper distribution of NAGLAZYME. Do not shake the solution.

NAGLAZYME does not contain preservatives; therefore, after dilution with saline in the infusion bags, any unused product or waste material should be discarded and disposed of in accordance with local requirements.

NAGLAZYME must not be infused with other products in the infusion tubing. The compatibility of NAG LAZY ME in solution with other products has not been evaluated.

STORAGE

Store NAGLAZYME under refrgeration at 2° C to 8° C (36° F to 46° F). DO NOT FREEZE OR SHAKE. DO NOT USE NAGLAZYME after the expiration date on the vial. This product contains no preservatives. The diluted solution should be used immediately. If immediate use is not possible, the diluted solution should be stored refrgerated at 2° C to 8° C (36° F to 46° F). Storage after dilution should not exceed 48 hours from the time of preparation to completion of administration. Room temperature storage of diluted solution, other than during infusion, is not recommended.

NAGLAZYME is supplied as a sterile solution in clear Type I glass 5 mL vials (5 mg galsulfase (expressed as protein content per 5 mL). The closure consists of a siliconized chlorobutyl rubber stopper and an aluminum seal with a plastic flip-off cap.

NDC 68135-020-01

NAGLAZYME is manufactured and distributed by: BioMarin Pharmaceutical Inc., 105 Digital Drive Novato, CA 94949, US License Number 1649, 866-906-6100 (phone). NAGLAZYME^TM is a trademark of BioMarin.

Bookmark this page: