Parenteral Manual

Alglucosidase alfa (NON-FORMULARY)

Disclaimer: Official controlled document is the CHEO online copy. It is the responsibility of user to ensure that any paper copy version is the same as the online version before use.

Alternate Name(s): 
MYOZYME
Classification: 
Recombinant human acid alpha-glucosidase
Original Date: 
October 2011
Revised Date: 
August 2012
Indications: 
  • Patients with Pompe's Disease (GAA deficiency)
Reconstitution and Stability: 
VIAL SIZE STERILE WATER for INJECTION FINAL CONCENTRATION
   50 mg                          10.3 mL                     5 mg/mL
  • Refrigerate unreconstituted vials and protect from light
  • Allow vials to reach room temperature prior to reconstitution (about 30 minutes)
  • Reconstitute each vial with 10.3 mL of Sterile Water for Injection
  • Gently tilt and roll vial.  Do not invert, swirl, or shake
  • Reconstituted  vial stable up to 24 hours  refrigerated and protected from light
  • After reconstitution, dilute in 0.9% sodium chloride to a concentration of 4 mg/mL
  • Diluted solution stable 24 hours at room temperature or refrigerated
  • Minimize exposure to airspace to reduce particle formation due to sensitivity of drug to air-liquid interfaces
  • Withdraw myozyme solution from vial into syringe then QS with normal saline· DO NOT SHAKE
  • Diluted solution should be filtered through an in-line, low protein-binding 0.2 micron filter.  Final solution may contain a few particles in the form of thin,white strands or translucent fibers which are easily filtered during infusion
  • Syringe is stable up to 24 hours refrigerated or at room temperature and should be protected from light
  • Multiple syringes will be provided for doses greater than 200 mg

 

 

Compatibility: 
  • Solutions Compatible:  0.9% Normal Saline
  • Additives/Above Cassette Compatible: Not compatible
  • Y-site Compatible: Not compatible

Incompatible:  DO NOT infuse with any other medications

Administration: 

(For approved routes of administration by nursing personnel, refer to Policy for the Administration of Intravenous Medications.)

SC NO
IM NO
IV Direct NO
IV Intermittent Infusion YES

Dilution:  4 mg/mL

Infusion time:  approximately 4 hours

-Administer with a low-protein binding filter (pore size 0.2 µm)

 

Run at 1 mg/kg/hr x 30 minutes (= 0.125 mL/kg over 30 minutes), then 3 mg/kg/hr x 30 minutes (= 0.375 mL/kg over 30 minutes), then 5 mg/kg/hr x 30 minutes (= 0.625 mL/kg over 30 minutes), then 7 mg/kg/hr (= 1.75 mL/kg/hour) until finished

IV Continuous Infusion NO
Dosage: 

(For neonatal dosages, refer to Neonatal IV Drug Manual.)

·          20 mg/kg IV every 2 weeks (Round up to nearest 50 mg dose) over approximately 4 hours

Potential hazards of parenteral administration: 
  • Infusion reactions are common (13.3 to 51% of patients) – monitor vital signs every 30 minutes during infusion, prior to each rate increase, and for 1-2 hours following end of infusion
  • Monitor for infusion-related reactions:  anaphylaxis, rash, flushing, urticaria, facial edema, nausea/vomiting, cough, wheezing, decreased oxygen saturation, tachypnea, tachycardia, agitation, irritability, pruritis, tremor, hypertension, hypotension, and bronchospasm
  • If an infusion reaction occurs, decreasing the rate of infusion, temporarily stopping the infusion, and/or administration of antipyretics, corticosteroids, and antihistamines may lessen symptoms
Notes: 
  • Restricted to patients with approval from MOHLTC Exceptional Access Program

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