Alternate Name(s):
MYOZYME
Classification:
Recombinant human acid alpha-glucosidase
Original Date:
October 2011
Revised Date:
April 2022
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 (= 0.25 mL/kg/hr over 30 minutes), then 3 mg/kg/hr x 30 minutes (= 0.75 mL/kg/hr over 30 minutes), then 5 mg/kg/hr x 30 minutes (= 1.25 mL/kg/hr 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