Velmanase alfa

Recommended Dosage and Administration Prior to LAMZEDE administration, consider pre-treating with antihistamines, antipyretics, and/or corticosteroids [see Warnings and Precautions ( 5.1 , 5.2 )] . The recommended dosage of LAMZEDE is 1 mg/kg (actual body weight) administered once every week as an intravenous infusion. The total volume of infusion is determined by the patient’s actual body weight and should be administered over a minimum of 60 minutes for patients weighing up to 49 kg.

Patients weighing 50 kg and greater should be infused at a maximum infusion rate of 25 mL/hour to control the protein load [ s ee Dosage and Administration] . If one or more doses are missed, restart the treatment as soon as possible, as long as it is at least 3 days from the next scheduled dose. If it is within 3 days from the next scheduled dose, give only the next dose per schedule. 2. 3 Dosage and Administration Modifications Due to Hypersensitivity Reactions and/or Infusion-Associated Reactions In the event of a severe hypersensitivity reaction (including anaphylaxis) or severe infusion-associated reaction (IAR), immediately discontinue LAMZEDE administration and initiate appropriate medical treatment.

For additional recommendations in the event of a severe hypersensitivity reaction or IAR, see Warnings and Precautions ( 5.1 , 5.2 ). In the event of a mild to moderate hypersensitivity reaction or a mild to moderate IAR, consider temporarily holding the infusion for 15 to 30 minutes, slowing the infusion rate to 25% to 50% of the recommended rate, and initiating appropriate medical treatment [see Warnings and Precautions ( 5.1 , 5.2 )] .

If symptoms: Persist despite temporarily holding or slowing the infusion, stop the infusion and monitor the patient. If symptoms continue to persist, discontinue the infusion, and consider re-initiating the infusion within 7 to 14 days at 25% to 50% of the recommended rate with appropriate pretreatment. Subside following holding or slowing the infusion, resume infusion at 25% to 50% the recommended rate.

If tolerated, increase the infusion rate by increments of 25% of the recommended rate until the recommended infusion rate is reached. Closely monitor the patient. 2. 4 Reconstitution Instructions Use aseptic technique during preparation. Reconstitute LAMZEDE in the following manner: Determine the number of LAMZEDE vials to be reconstituted based on the patient’s weight in kg and the recommended dose [see Dosage and Administration ] .

Round the number of vials up to the next whole number. Remove vials from the refrigerator and set aside for approximately 30 minutes to allow vials to come to room temperature. Reconstitute each vial by slowly injecting 5 mL of Sterile Water for Injection, down the inside wall of each vial. Avoid adding the Sterile Water for Injection to the vial forcefully or directly onto the lyophilized powder to minimize foaming.

Allow the reconstituted vials to stand on the table for 5 – 15 minutes. Then gently tilt and roll each vial for 15 – 20 seconds to enhance the dissolution process. Each vial will yield a concentration of 2 mg/mL. Do not invert, swirl, or shake the vials. Visually inspect the reconstituted solution in the vials for particulate matter and discoloration.

The solution should be clear to slightly opalescent. Due to the nature of LAMZEDE, the solution may occasionally contain some proteinaceous particles in the form of thin white strands or translucent fibers which will be removed by the in line filter during infusion. Discard if opaque particles are present or the solution is discolored.

Slowly withdraw the required volume from the vials with caution to avoid foaming in the syringe. If the volume of the solution exceeds one syringe capacity, prepare the required number of syringes in order to replace the syringe quickly during the infusion. Discard unused portion remaining in the vials. Storage of the Reconstituted Solution If the reconstituted LAMZEDE vial is not used immediately, store the vial refrigerated at 2°C to 8°C (36°F to 46°F) for up to 24 hours inclusive of infusion time.

Protect from light during refrigeration. Do not freeze. Reconstituted LAMZEDE vial must be infused within 10 hours after removal from the refrigerator, inclusive of total infusion time. Discard if not used within 10 hours. Infuse reconstituted solution within 24 hours from the time of preparation, which includes the storage time in the refrigerator, the time at room temperature, and the duration of the infusion. 2. 5 Administration Instructions Use an infusion set equipped with a pump and a low protein binding, 0.2-micron, in-line filter to administer LAMZEDE.

Do not shake the syringe. The total volume of infusion is determined by the patient’s actual body weight and should be administered over a minimum of 60 minutes for patients weighing up to 49 kg. Patients weighing 50 kg and greater should be infused at a maximum infusion rate of 25 mL/hour to control the protein load. When the last syringe is empty, replace the dosage syringe with a 20 mL syringe filled with 0.9% Sodium Chloride Injection, and then continue to infuse an additional 10 mL of 0.9% Sodium Chloride Injection through the infusion system to infuse the remaining fraction of LAMZEDE in the line to the patient.