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Nexviazyme Dosage

Generic name: AVALGLUCOSIDASE ALFA 100mg in 10mL
Dosage form: injection, powder, lyophilized, for solution
Drug class: Lysosomal enzymes

Medically reviewed by Drugs.com. Last updated on Sep 26, 2023.

Recommendations Prior to NEXVIAZYME Treatment

  • Prior to NEXVIAZYME administration, consider pretreating with antihistamines, antipyretics, and/or corticosteroids [see Warnings and Precautions (5.1, 5.2)].
  • NEXVIAZYME must be reconstituted and diluted prior to use [see Dosage and Administration (2.4)].
  • ​Appropriate medical monitoring and support measures, including cardiopulmonary resuscitation equipment, should be readily available during NEXVIAZYME administration.

Recommended Dosage and Administration

  • NEXVIAZYME is administered as intravenous infusion. For patients weighing:
  • ​The initial recommended infusion rate is 1 mg/kg/hour. Gradually increase the infusion rate every 30 minutes if there are no signs of infusion-associated reactions (IARs) [see Dosage and Administration (2.6)]. If one or more doses are missed, restart NEXVIAZYME treatment as soon as possible, maintaining the 2 week interval between infusions thereafter.

Administration Modifications Due to Hypersensitivity Reactions and/or Infusion-Associated Reactions

  • In the event of a severe hypersensitivity reaction (e.g., anaphylaxis) or severe infusion-associated reaction (IAR), immediately discontinue NEXVIAZYME 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 30 minutes or slowing the infusion rate by 50% [see Dosage and Administration (2.6)] and initiating appropriate medical treatment [see Warnings and Precautions (5.1, 5.2)].
    • If symptoms persist for longer than 30 minutes despite holding or slowing the infusion, stop the infusion and monitor the patient. Consider re-initiating the infusion on the same day when symptoms subside at 50% of the rate at which the reaction occurred with appropriate pretreatment.
    • If symptoms subside after holding the infusion, resume infusion at 50% of the rate at which the reaction occurred, and subsequently increase the infusion rate every 15 to 30 minutes by 50% as tolerated. Alternatively, if symptoms subside after slowing the infusion, complete the infusion at the reduced rate as tolerated.
    • Starting with the next infusion, increase the infusion rate until the infusion rate at which the reaction occurred is reached. Consider continuing to increase the infusion rate in a stepwise manner until reaching the recommended infusion rate. Closely monitor the patient.

Reconstitution and Dilution Instructions

Reconstitute and dilute NEXVIAZYME in the following manner. Use aseptic technique during preparation.

Reconstitute the Lyophilized Powder

  1. Determine the number of NEXVIAZYME vials to be reconstituted based on actual body weight in kg and the recommended dose [see Dosage and Administration (2.2)]. Round the number of vials up to the next whole number.
  2. Remove the required number of NEXVIAZYME vials from the refrigerator and allow the vials to sit for 30 minutes at room temperature 20°C to 25°C (68°F to 77°F) before use.
  3. Reconstitute each vial by injecting 10 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.
  4. Gently tilt and roll each vial to enhance the dissolution process. Do not invert, swirl, or shake the vial. Allow the solution to become dissolved. Each vial will yield a concentration of 100 mg/10 mL (10 mg/mL) of avalglucosidase alfa-ngpt.
  5. Visually inspect the reconstituted solution in the vials for particulate matter and discoloration. The reconstituted solution should be clear, colorless to pale-yellow. Discard if particles are present or the solution is discolored.

Dilute the Reconstituted Solution

  1. Select an appropriate size 5% Dextrose Injection infusion bag and prepare by removing a volume equal to the required NEXVIAZYME volume and any overfill to achieve a fixed total volume per Table 1 based on actual body weight.
  2. Slowly withdraw the required volume of reconstituted solution from the NEXVIAZYME vial(s). Discard any unused reconstituted solution remaining in the vial.
  3. Gently inject the NEXVIAZYME reconstituted solution into the port of the 5% Dextrose Injection bag. Avoid foaming or agitation of the infusion bag and avoid introducing air into the infusion bag.
  4. Gently invert the infusion bag to mix the solution. Do not shake. After dilution, the solution will have a final concentration of 0.5 to 4 mg/mL of avalglucosidase alfa-ngpt.

Administer the diluted solution without delay. The recommended infusion duration is between 4 to 7 hours [see Dosage and Administration (2.6)]. Discard any unused diluted solution after 9 hours.

Table 1: Projected Intravenous Infusion Volume for NEXVIAZYME Administration According to Actual Body Weight
Patient Actual Body Weight Range (kg) Total Infusion Volume (mL) for 20 mg/kg Total Infusion Volume (mL) for 40 mg/kg
5 to 9.9 kg N/A 100 mL
10 to 19.9 kg N/A 200 mL
20 to 29.9 kg N/A 300 mL
30 to 34.9 kg 200 mL N/A
35 to 49.9 kg 250 mL N/A
50 to 59.9 kg 300 mL N/A
60 to 99.9 kg 500 mL N/A
100 to 119.9 kg 600 mL N/A
120 to 140 kg 700 mL N/A

Storage Instructions for the Reconstituted and Diluted Product

Storage of the Reconstituted Solution

  • Dilute the reconstituted solution without delay. If the reconstituted solution is not diluted immediately, refrigerate at 2°C to 8°C (36°F to 46°F) for up to 24 hours.
  • Do not freeze.

Storage of the Diluted Solution

  • If the diluted solution is not used immediately, refrigerate the diluted solution at 2°C to 8°C (36°F to 46°F) for up to 24 hours.
  • The diluted solution must be infused within 9 hours after removal from the refrigerator, inclusive of infusion time, or discarded.
  • Once the diluted solution is removed from the refrigerator, it must not be restored back into the refrigerator.
  • Do not freeze.

Administration Instructions

  1. If the diluted solution was refrigerated, allow solution to equilibrate to room temperature for 30 minutes prior to infusion.
  2. It is recommended to use an in-line, low protein-binding, 0.2 micron filter during administration.
  3. Administer the infusion incrementally, as determined by the patient's response and comfort.
    When the recommended dose is 20 mg/kg
    • Initial and Subsequent Infusions: The initial recommended infusion rate is 1 mg/kg/hour (see Table 2). If there are no signs of hypersensitivity or infusion-associated reactions (IARs), gradually increase the infusion rate every 30 minutes in each of the following three steps: 3 mg/kg/hour, 5 mg/kg/hour, and then 7 mg/kg/hour; then, maintain the infusion rate at 7 mg/kg/hour until the infusion is complete. The approximate total infusion duration is 4 to 5 hours.
    Table 2: Recommended Infusion Rates at 20 mg/kg Dose
    Dose Step 1 Step 2 Step 3 Step 4 Step 5
    Start infusion at step 1 and in absence of infusion-associated reaction increase infusion rate sequentially per the steps of infusion every 30 minutes until completion (total time approximately 4 to 5 hours).
    20 mg/kg 1 mg/kg/hour 3 mg/kg/hour 5 mg/kg/hour 7 mg/kg/hour Continue 7 mg/kg/hour

    When the recommended dose is 40 mg/kg
    • Initial Infusion: The initial recommended infusion rate is 1 mg/kg/hour (see Table 3). If there are no signs of hypersensitivity or IARs, gradually increase the infusion rate every 30 minutes in each of the following three steps: 3 mg/kg/hour, 5 mg/kg/hour, and then 7 mg/kg/hour; then, maintain the infusion rate at 7 mg/kg/hour until the infusion is complete (4-step process). The approximate total infusion duration is 7 hours.
    • Subsequent Infusions: The initial recommended infusion rate is 1 mg/kg/hour (see Table 3) with gradual increase in infusion rate every 30 minutes if there are no signs of hypersensitivity or IARs. The process may use either the above 4-step process or the following 5-step process: 3 mg/kg/hour, 6 mg/kg/hour, 8 mg/kg/hour, and then 10 mg/kg/hour; then, maintain the infusion rate at 10 mg/kg/hour until the infusion is complete. The approximate total 5-step infusion duration is 5 hours.
    Table 3: Recommended Infusion Rates at 40 mg/kg Dose
    Dose Step 1 Step 2 Step 3 Step 4 Step 5
    Start infusion at step 1 and in absence of infusion-associated reaction increase infusion rate sequentially per the steps of infusion every 30 minutes until completion. Total time for initial infusion approximately 7 hours and can optionally increase rate of subsequent infusions to decrease total duration to 5 hours.
    40 mg/kg Initial infusion rate 1 mg/kg/hour 3 mg/kg/hour 5 mg/kg/hour 7 mg/kg/hour Continue 7 mg/kg/hour
    Subsequent infusions (optional) 1 mg/kg/hour 3 mg/kg/hour 6 mg/kg/hour 8 mg/kg/hour Continue 10 mg/kg/hour
  4. After the infusion is complete, flush the intravenous line with 5% Dextrose Injection.
  5. Do not infuse NEXVIAZYME in the same intravenous line with other products.

Further information

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