Handling & Preparation

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.

Product Storage

Product packaging

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:

  • Do not use Lumizyme after the expiration date on the package
  • Store the unopened product vials under refrigeration at 2°-8° C (36°-47° F)
  • Allow vials to reach room temperature before reconstituting
  • Once reconstituted and diluted, Lumizyme should be administered without delay
  • If immediate use is not possible, the reconstituted solution may be stored for up to 24 hours under refrigeration at 2°-8° C (36°-47° F); do not freeze or store it at room temperature
  • Do not shake the reconstituted solution, and protect it from light using light-shielding covers during infusion
  • Once the infusion is complete, discard any unused product

Preparing Lumizyme for Infusion

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 800-745-4447, option 2.

Before You Begin

Remove Lumizyme vials from refrigeration and allow them to reach room temperature (about 30 minutes) before starting reconstitution. Organize a clean work area and prepare the following supplies:

  1. Lumizyme 50 mg vials
  2. Sterile Water for Injection, USP
  3. 0.9% Sodium Chloride for Injection, USP
  4. Syringes for reconstitution and dilution
  5. Needles (do not use filter needles during preparation)
  6. Intravenous administration set
  7. In-line low protein-binding 0.2 μm filter

You may need additional supplies, depending on the IV infusion protocol of your facility or practice.

Dosing Guidelines

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.

Reconstitution & Dilution

1.  Remove the required number of Lumizyme vials from refrigeration and let them reach room temperature (about 30 minutes).
2.  Remove the plastic protective caps from each vial, and then slowly inject 10.3 mL of Sterile Water for Injection, USP, to the inside wall of each vial.

  • Each vial will yield a concentration of 5 mg/mL; the total extractable dose per vial is 50 mg per 10 mL.
  • Avoid forceful impact of water on the powder, and avoid foaming by slowly adding the water for injection in a drop-wise addition down the inside of the vial rather than directly onto the lyophilized cake. Tilt and roll each vial gently—do not invert, swirl, or shake.
  • Once reconstituted, protect the solution from light.

3.  Perform an immediate visual inspection on the reconstituted vials for particulate matter and discoloration. Some strands or fibers of alglucosidase alfa may appear and are acceptable. Opaque particles or discolored solution are not acceptable, and the product should be discarded—report any such vials to Sanofi Genzyme Medical Information (800-745-4447, option 2).


Acceptable
Clear and colorless


Acceptable
Strands/fibers of alglucosidase alfa


DO NOT USE
Opaque particles or solution discoloration

4.  Prepare the infusion bag with 0.9% Sodium Chloride for Injection, USP, to be used for diluting the reconstituted Lumizyme solution. NOTE: The final Lumizyme concentration should be 0.5 to 4 mg/mL
5.  Slowly withdraw the reconstituted solution from each vial, taking care to avoid foaming in the syringe.
6.  Remove airspace from the infusion bag to minimize particle formation due to the sensitivity of Lumizyme to air-liquid interfaces.
7.  Add the reconstituted Lumizyme solution slowly and directly into the sodium chloride solution. Do not add directly into airspace that may remain within the infusion bag, and avoid foaming in the infusion bag.
8.  Gently invert or massage the infusion bag to mix. Do not shake it. Label the infusion bag per your institution’s protocol, and hand-carry it to the infusion administrator. Do not use a pneumatic tube delivery system
9.  The reconstituted and diluted solution should be protected from light. Do not freeze or shake.

INDICATION

LUMIZYME® (alglucosidase alfa) is a hydrolytic lysosomal glycogen-specific enzyme indicated for patients with Pompe disease (GAA deficiency).

IMPORTANT SAFETY INFORMATION

WARNING: RISK OF ANAPHYLAXIS, HYPERSENSITIVITY AND IMMUNE-MEDIATED REACTIONS, and RISK OF CARDIORESPIRATORY FAILURE
  • Life-threatening anaphylactic reactions and severe hypersensitivity reactions, presenting as respiratory distress, hypoxia, apnea, dyspnea, bradycardia, tachycardia, bronchospasm, throat tightness, hypotension, angioedema (including tongue or lip swelling, periorbital edema, and face edema), and urticaria, have occurred in some patients during and after alglucosidase alfa infusions. Immune-mediated reactions presenting as proteinuria, nephrotic syndrome, and necrotizing skin lesions have occurred in some patients following alglucosidase alfa treatment. Closely observe patients during and after alglucosidase alfa administration and be prepared to manage anaphylaxis and hypersensitivity reactions. Inform patients of the signs and symptoms of anaphylaxis, hypersensitivity reactions, and immune-mediated-reactions and have them seek immediate medical care should signs and symptoms occur.
  • Infantile-onset Pompe disease patients with compromised cardiac or respiratory function may be at risk of serious acute exacerbation of their cardiac or respiratory compromise due to fluid overload, and require additional monitoring.

WARNINGS AND PRECAUTIONS

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 immunemediated 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.

ADVERSE REACTIONS

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.

USE IN SPECIFIC POPULATIONS

Pregnancy: Based on animal data, alglucosidase alfa may cause fetal harm.

Please see the Full Prescribing Information, for complete details, including boxed WARNING

Contact Us

Sanofi Genzyme
50 Binney Street
Cambridge, MA 02142

Tel : 617-768-9000
Fax : 617 252 7600
Toll free : 800-745-4447