Alglucosidase Alfa
Pronunciation: (al-gloo-KOSE-i-dase Alfa)Class: Enzyme
Trade Names:Myozyme- Powder for injection 50 mg
Pharmacology
Exogenous source of alpha-glucosidase (GAA).
Pharmacokinetics
Absorption
Following a single IV infusion of 20 mg/kg, C max and AUC were 162 mcg/mL and 811 mcg•h/mL, respectively.
Elimination
Following a single IV infusion of 20 mg/kg, Cl and t ½ were 25 mL/h/kg and 2.3 h, respectively.
Indications and Usage
Treatment of Pompe disease (GAA deficiency).
Contraindications
Standard considerations.
Dosage and Administration
AdultsIV 20 mg/kg administered by IV infusion over approximately 4 h every 2 wk. Administer in a step-wise manner, starting with no more than 1 mg/kg/h. The infusion rate may be increased by 2 mg/kg/h every 30 min, after tolerance to infusion rate is established (max rate, 7 mg/kg/h).
General Advice
- Obtain vital signs at the end of each step of dose titration.
- Slow or temporarily stop the infusion in the event of an infusion reaction.
- Because alglucosidase alfa does not contain preservatives, vials are single-use only; discard unused portion.
- Remove required number of vials from the refrigerator and allow to reach room temperature (59° to 86°F) for approximately 30 min prior to reconstitution.
- Reconstitute each vial by slowly injecting 10.3 mL of sterile water for injection, which will yield 5 mg/mL.
- Protect reconstituted solution from light.
- Perform an immediate inspection on reconstituted vials for particulate matter and discoloration. Do not use discolored solution. Reconstituted solution may contain some alglucosidase alfa particles in the form of thin, white strands or translucent fibers. These particles are removed by inline filtration without affecting purity or strength.
- Immediately dilute reconstituted solution with sodium chloride 0.9% for injection to a final concentration of 0.5 to 4 mg/mL.
- Slowly withdraw reconstituted solution to avoid foaming in syringe.
- Remove airspace from infusion bag to minimize particle formation and add reconstituted alglucosidase solution slowly and directly into sodium chloride solution. Avoid foaming in the infusion bag.
- Gently invert or massage infusion bag to mix. Do not shake.
- Filter diluted solution through a 0.2 mcm, low protein-binding, inline filter during administration to remove any visible particles.
- Do not infuse in the same IV line with other products.
Storage/Stability
Refrigerate between 36° and 46°F. Administer reconstituted and diluted solution without delay. If immediate use is not possible, reconstituted and diluted solution is stable for up to 24 h at 36° to 46°F. Protect from light. Do not freeze or shake.
Drug Interactions
None well documented.
Laboratory Test Interactions
None well documented.
Adverse Reactions
Cardiovascular
Tachycardia (23%); bradycardia (21%).
CNS
Pyrexia (92%).
Dermatologic
Rash (54%); diaper dermatitis (36%); urticaria (23%); flushing (21%).
EENT
Otitis media (44%); pharyngitis (36%); ear infection (33%); nasopharyngitis (23%).
GI
Diarrhea (62%); vomiting (49%); gastroenteritis (41%); oral candidiasis (31%); gastroesophageal reflux disease (26%); constipation (23%).
Hematologic-Lymphatic
Anemia (31%).
Lab Tests
Positive test for immunoglobulin G antibodies (89%); decreased oxygen saturation (41%).
Local
Catheter-related infection (28%).
Respiratory
Cough, pneumonia (46%); upper respiratory tract infection (44%); respiratory distress (33%); respiratory failure (31%); rhinorrhea (28%); bronchiolitis, tachypnea (23%).
Miscellaneous
Infusion reactions (51%); postprocedural pain (26%).
Precautions
WarningsLife-threatening anaphylactic reactions, including anaphylactic shock, have been observed in patients during alglucosidase alfa infusion. Appropriate medical support measures should be readily available. |
Pregnancy
Category B .
Lactation
Undetermined.
Children
Safety and efficacy not established in juvenile-onset Pompe disease population.
Cardiac arrhythmia
Cardiac arrhythmia, including bradycardia, ventricular fibrillation, and ventricular tachycardia, resulting in cardiac arrest or defibrillation has occurred. These events were in infantile-onset Pompe disease patients with cardiac hypertrophy, associated with general anesthesia used for placement of central venous catheter for alglucosidase infusion.
Cardiorespiratory failure
Acute cardiorespiratory failure requiring intubation and inotropic support has been observed after infusion with alglucosidase.
Infusion reactions
Severe infusion reactions may occur.
Overdosage
Symptoms
No reports of overdosage.
Patient Information
- Inform patient and caregiver that a registry for patients with Pompe disease has been established to better understand the variability and progression of the disease and monitor and evaluate treatment.
Copyright © 2009 Wolters Kluwer Health.
- Alglucosidase Alfa MedFacts Consumer Leaflet (Wolters Kluwer)
- Myozyme Prescribing Information (FDA)
- Myozyme Consumer Overview