Lumizyme® (alglucosidase alfa) is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder that must be reconstituted for intravenous administration. Certain measures, outlined below, should be followed to ensure proper product storage and preparation for infusion.
Lumizyme is available only in 50 mg units, supplied in single-use, clear Type I glass 20 mL (cc) vials. The vial's closure consists of a siliconized butyl stopper and an aluminum seal with a plastic flip-off cap. Lumizyme does not contain any preservatives and should be stored and handled according to these guidelines:
Lumizyme should be reconstituted, diluted, and administered by a health care professional using aseptic technique. Do not use filter needles during preparation. Reconstitution devices may be used to prepare successive vials of Lumizyme without the need to enter and withdraw from numerous vials of sterile water. Such devices are available from Sanofi Genzyme by contacting Medical Information at, option 2.
The recommended dosage of Lumizyme is 20 mg per kg of body weight (administered every two weeks). For more details on calculating vials needed per dose and recommended total infusion volume based on patient weight, see the Dosing & Administration page.
Clear and colorless
Strands/fibers of alglucosidase alfa
DO NOT USE
Opaque particles or solution discoloration
LUMIZYME® (alglucosidase alfa) is a hydrolytic lysosomal glycogen-specific enzyme indicated for patients with Pompe disease (GAA deficiency).
WARNING: Risk of anaphylaxis, hypersensitivity and immune-mediated reactions, and risk of cardiorespiratory failure
Anaphylaxis and Hypersensitivity Reactions: Life-threatening anaphylaxis and hypersensitivity reactions have been observed in some patients during and after treatment with alglucosidase alfa. If anaphylaxis or severe hypersensitivity reactions occur, immediately discontinue infusion and institute appropriate medical treatment. Appropriate medical support and monitoring measures should be available during infusion.
Immune-Mediated Reactions: Monitor patients for the development of systemic immune-mediated reactions involving skin and other organs.
Risk of Acute Cardiorespiratory Failure: Patients with acute underlying respiratory illness and compromised cardiac and/or respiratory function may be at risk of acute cardiorespiratory failure. Caution should be exercised when administering alglucosidase alfa to patients susceptible to fluid volume overload. Appropriate medical support and monitoring measures should be available during infusion and some patients may require longer observation times.
Risk of Cardiac Arrhythmia and Sudden Cardiac Death during General Anesthesia for Central Venous Catheter Placement: Caution should be used when administering general anesthesia for the placement of a central venous catheter intended for alglucosidase alfa infusion.
Risk of Antibody Development: As with all therapeutic proteins, there is potential for immunogenicity. There is some evidence to suggest that some patients who develop high and sustained IgG antibody titers may experience reduced clinical efficacy. Patients should be monitored for IgG antibody formation every 3 months for 2 years and then annually thereafter.
The most frequently reported adverse reactions (≥ 5%) in clinical trials were hypersensitivity reactions and included: anaphylaxis, rash, pyrexia, flushing/feeling hot, urticaria, headache, hyperhidrosis, nausea, cough, decreased oxygen saturation, tachycardia, tachypnea, chest discomfort, dizziness, muscle twitching, agitation, cyanosis, erythema, hypertension/increased blood pressure, pallor, rigors, tremor, vomiting, fatigue, and myalgia.
Pregnancy: Based on animal data, alglucosidase alfa may cause fetal harm.
Please see the Full Prescribing Information for complete details, including boxed WARNING.