Myozyme

Myozyme Manufacturers

• Genzyme Corporation
  

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  Myozyme | Genzyme Corporation

  

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  2.1 Recommended Dose

  The recommended dosage regimen of MYOZYME is 20 mg/kg body weight administered every 2 weeks as an intravenous infusion.

  2.2 Instructions for Use

  MYOZYME does not contain any preservatives.  Vials are single-use only.  Any unused product should be discarded.

  The total volume of infusion is determined by the patient’s body weight and should be administered over approximately 4 hours.  

  Infusions should be administered in a step-wise manner using an infusion pump. The initial infusion rate should be no more than 1 mg/kg/hr. The infusion rate may be increased by 2 mg/kg/hr every 30 minutes, after patient tolerance to the infusion rate is established, until a maximum rate of 7 mg/kg/hr is reached. Vital signs should be obtained at the end of each step. If the patient is stable, MYOZYME may be administered at the maximum rate of 7 mg/kg/hr until the infusion is completed. The infusion rate may be slowed and/or temporarily stopped in the event of infusion reactions. See Table 1 below for the rate of infusion at each step, expressed as mL/hr based on the recommended infusion volume by patient weight.

  Table 1: Recommended Infusion Volumes and Rates Patient Weight Range (kg)   Total infusion volume (mL) 

  Step 1

  1 mg/kg/hr

  (mL/hr) 

  Step 2

  3 mg/kg/hr

  (mL/hr) 

  Step 3

  5 mg/kg/hr 

  (mL/hr) 

  Step 4

  7 mg/kg/hr

  (mL/hr)  1.25 - 10 50 3 8 13 18 10.1 - 20 100 5 15 25 35 20.1 - 30 150 8 23 38 53 30.1 - 35 200 10 30 50 70 35.1 - 50 250 13 38 63 88 50.1 - 60 300 15 45 75 105 60.1 - 100 500 25 75 125 175 100.1 - 120 600 30 90 150 210 120.1 - 140 700 35 105 175 245 140.1 - 160 800 40 120 200 280 160.1 - 180 900 45 135 225 315 180.1 - 200 1000 50 150 250 350 Reconstitution, dilution and administration

  MYOZYME should be reconstituted, diluted and administered by a healthcare professional.

  
  

  Use aseptic technique during preparation. Do not use filter needles during preparation.

  
  Determine the number of vials to be reconstituted based on the individual patient’s weight and the recommended dose of 20 mg/kg.    
  
  Patient weight (kg) x dose (mg/kg) = patient dose (in mg)
  
  Patient dose (in mg) divided by 50 mg/vial = number of vials to reconstitute. If the number of vials includes a fraction, round up to the next whole number.
  
  Example: Patient weight (16 kg) x dose (20 mg/kg) = patient dose (320 mg)
  
  320 mg divided by 50 mg/vial = 6.4 vials; therefore, 7 vials should be reconstituted
  
  Remove the required number of vials from the refrigerator and allow them to reach room temperature prior to reconstitution (approximately 30 minutes).

  
  

  Reconstitute each MYOZYME vial by slowly injecting 10.3 mL of Sterile Water for Injection, USP to the inside wall of each vial. Each vial will yield 5 mg/mL. The total extractable dose per vial is 50 mg per 10 mL. Avoid forceful impact of the water for injection on the powder and avoid foaming. This is done by slow drop-wise addition of the water for injection down the inside of the vial and not directly onto the lyophilized cake. Tilt and roll each vial gently. Do not invert, swirl, or shake. 

  
  

  The reconstituted MYOZYME solution should be protected from light.

  
  

  Perform an immediate visual inspection on the reconstituted vials for particulate matter and discoloration. If upon immediate inspection opaque particles are observed or if the solution is discolored do not use. The reconstituted solution may occasionally contain some alglucosidase alfa particles (typically less than 10 in a vial) in the form of thin white strands or translucent fibers subsequent to the initial inspection. This may also happen following dilution for infusion. These particles have been shown to contain alglucosidase alfa and may appear after the initial reconstitution step and increase over time. Studies have shown that these particles are removed via in-line filtration without having a detectable effect on the purity or strength.

  
  

  MYOZYME should be diluted in 0.9% Sodium Chloride for Injection, USP, immediately after reconstitution, to a final MYOZYME concentration of 0.5 to 4 mg/mL. See Table 1 for the recommended total infusion volume based on patient weight.

  
  

  Slowly withdraw the reconstituted solution from each vial. Avoid foaming in the syringe.

  
  

  Remove airspace from the infusion bag to minimize particle formation due to the sensitivity of MYOZYME to air-liquid interfaces.

  
  

  Add the reconstituted MYOZYME solution slowly and directly into the sodium chloride solution. Do not add directly into airspace that may remain within the infusion bag. Avoid foaming in the infusion bag.

  
  

  Gently invert or massage the infusion bag to mix. Do not shake. 

  
  

  Administer MYOZYME using an in-line low protein-binding 0.2 µm filter. 

  MYOZYME should not be infused in the same intravenous line with other products.

  MYOZYME does not contain any preservatives. Vials are single-use only. Discard any unused product.

  
  

  2.1 Recommended Dose

  The recommended dosage regimen of MYOZYME is 20 mg/kg body weight administered every 2 weeks as an intravenous infusion.

  2.2 Instructions for Use

  MYOZYME does not contain any preservatives.  Vials are single-use only.  Any unused product should be discarded.

  The total volume of infusion is determined by the patient’s body weight and should be administered over approximately 4 hours.  

  Infusions should be administered in a step-wise manner using an infusion pump. The initial infusion rate should be no more than 1 mg/kg/hr. The infusion rate may be increased by 2 mg/kg/hr every 30 minutes, after patient tolerance to the infusion rate is established, until a maximum rate of 7 mg/kg/hr is reached. Vital signs should be obtained at the end of each step. If the patient is stable, MYOZYME may be administered at the maximum rate of 7 mg/kg/hr until the infusion is completed. The infusion rate may be slowed and/or temporarily stopped in the event of infusion reactions. See Table 1 below for the rate of infusion at each step, expressed as mL/hr based on the recommended infusion volume by patient weight.

  Table 1: Recommended Infusion Volumes and Rates Patient Weight Range (kg)   Total infusion volume (mL) 

  Step 1

  1 mg/kg/hr

  (mL/hr) 

  Step 2

  3 mg/kg/hr

  (mL/hr) 

  Step 3

  5 mg/kg/hr 

  (mL/hr) 

  Step 4

  7 mg/kg/hr

  (mL/hr)  1.25 - 10 50 3 8 13 18 10.1 - 20 100 5 15 25 35 20.1 - 30 150 8 23 38 53 30.1 - 35 200 10 30 50 70 35.1 - 50 250 13 38 63 88 50.1 - 60 300 15 45 75 105 60.1 - 100 500 25 75 125 175 100.1 - 120 600 30 90 150 210 120.1 - 140 700 35 105 175 245 140.1 - 160 800 40 120 200 280 160.1 - 180 900 45 135 225 315 180.1 - 200 1000 50 150 250 350 Reconstitution, dilution and administration

  MYOZYME should be reconstituted, diluted and administered by a healthcare professional.

  
  

  Use aseptic technique during preparation. Do not use filter needles during preparation.

  
  Determine the number of vials to be reconstituted based on the individual patient’s weight and the recommended dose of 20 mg/kg.    
  
  Patient weight (kg) x dose (mg/kg) = patient dose (in mg)
  
  Patient dose (in mg) divided by 50 mg/vial = number of vials to reconstitute. If the number of vials includes a fraction, round up to the next whole number.
  
  Example: Patient weight (16 kg) x dose (20 mg/kg) = patient dose (320 mg)
  
  320 mg divided by 50 mg/vial = 6.4 vials; therefore, 7 vials should be reconstituted
  
  Remove the required number of vials from the refrigerator and allow them to reach room temperature prior to reconstitution (approximately 30 minutes).

  
  

  Reconstitute each MYOZYME vial by slowly injecting 10.3 mL of Sterile Water for Injection, USP to the inside wall of each vial. Each vial will yield 5 mg/mL. The total extractable dose per vial is 50 mg per 10 mL. Avoid forceful impact of the water for injection on the powder and avoid foaming. This is done by slow drop-wise addition of the water for injection down the inside of the vial and not directly onto the lyophilized cake. Tilt and roll each vial gently. Do not invert, swirl, or shake. 

  
  

  The reconstituted MYOZYME solution should be protected from light.

  
  

  Perform an immediate visual inspection on the reconstituted vials for particulate matter and discoloration. If upon immediate inspection opaque particles are observed or if the solution is discolored do not use. The reconstituted solution may occasionally contain some alglucosidase alfa particles (typically less than 10 in a vial) in the form of thin white strands or translucent fibers subsequent to the initial inspection. This may also happen following dilution for infusion. These particles have been shown to contain alglucosidase alfa and may appear after the initial reconstitution step and increase over time. Studies have shown that these particles are removed via in-line filtration without having a detectable effect on the purity or strength.

  
  

  MYOZYME should be diluted in 0.9% Sodium Chloride for Injection, USP, immediately after reconstitution, to a final MYOZYME concentration of 0.5 to 4 mg/mL. See Table 1 for the recommended total infusion volume based on patient weight.

  
  

  Slowly withdraw the reconstituted solution from each vial. Avoid foaming in the syringe.

  
  

  Remove airspace from the infusion bag to minimize particle formation due to the sensitivity of MYOZYME to air-liquid interfaces.

  
  

  Add the reconstituted MYOZYME solution slowly and directly into the sodium chloride solution. Do not add directly into airspace that may remain within the infusion bag. Avoid foaming in the infusion bag.

  
  

  Gently invert or massage the infusion bag to mix. Do not shake. 

  
  

  Administer MYOZYME using an in-line low protein-binding 0.2 µm filter. 

  MYOZYME should not be infused in the same intravenous line with other products.

  MYOZYME does not contain any preservatives. Vials are single-use only. Discard any unused product.

  
  

  Reconstitution, dilution and administration

  MYOZYME should be reconstituted, diluted and administered by a healthcare professional.

  
  

  Use aseptic technique during preparation. Do not use filter needles during preparation.

  
  Determine the number of vials to be reconstituted based on the individual patient’s weight and the recommended dose of 20 mg/kg.    
  
  Patient weight (kg) x dose (mg/kg) = patient dose (in mg)
  
  Patient dose (in mg) divided by 50 mg/vial = number of vials to reconstitute. If the number of vials includes a fraction, round up to the next whole number.
  
  Example: Patient weight (16 kg) x dose (20 mg/kg) = patient dose (320 mg)
  
  320 mg divided by 50 mg/vial = 6.4 vials; therefore, 7 vials should be reconstituted
  
  Remove the required number of vials from the refrigerator and allow them to reach room temperature prior to reconstitution (approximately 30 minutes).

  
  

  Reconstitute each MYOZYME vial by slowly injecting 10.3 mL of Sterile Water for Injection, USP to the inside wall of each vial. Each vial will yield 5 mg/mL. The total extractable dose per vial is 50 mg per 10 mL. Avoid forceful impact of the water for injection on the powder and avoid foaming. This is done by slow drop-wise addition of the water for injection down the inside of the vial and not directly onto the lyophilized cake. Tilt and roll each vial gently. Do not invert, swirl, or shake. 

  
  

  The reconstituted MYOZYME solution should be protected from light.

  
  

  Perform an immediate visual inspection on the reconstituted vials for particulate matter and discoloration. If upon immediate inspection opaque particles are observed or if the solution is discolored do not use. The reconstituted solution may occasionally contain some alglucosidase alfa particles (typically less than 10 in a vial) in the form of thin white strands or translucent fibers subsequent to the initial inspection. This may also happen following dilution for infusion. These particles have been shown to contain alglucosidase alfa and may appear after the initial reconstitution step and increase over time. Studies have shown that these particles are removed via in-line filtration without having a detectable effect on the purity or strength.

  
  

  MYOZYME should be diluted in 0.9% Sodium Chloride for Injection, USP, immediately after reconstitution, to a final MYOZYME concentration of 0.5 to 4 mg/mL. See Table 1 for the recommended total infusion volume based on patient weight.

  
  

  Slowly withdraw the reconstituted solution from each vial. Avoid foaming in the syringe.

  
  

  Remove airspace from the infusion bag to minimize particle formation due to the sensitivity of MYOZYME to air-liquid interfaces.

  
  

  Add the reconstituted MYOZYME solution slowly and directly into the sodium chloride solution. Do not add directly into airspace that may remain within the infusion bag. Avoid foaming in the infusion bag.

  
  

  Gently invert or massage the infusion bag to mix. Do not shake. 

  
  

  Administer MYOZYME using an in-line low protein-binding 0.2 µm filter. 

  MYOZYME should not be infused in the same intravenous line with other products.

  MYOZYME does not contain any preservatives. Vials are single-use only. Discard any unused product.

  
  

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