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 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. View more

DOSING & ADMINISTRATION

The recommended dosage of Lumizyme® (alglucosidase alfa) is 20 mg per kg of body weight, administered every two weeks as an intravenous infusion. The total volume of infusion is determined by the patient’s body weight and should be administered over approximately 4 hours. If severe infusion or allergic reactions occur, immediate discontinuation of the administration of Lumizyme should be considered, and appropriate medical treatment should be initiated. Appropriate medical support should be readily available when Lumizyme is administered.

Calculating Dosages

A patient's dose and the vials required for that dose can be calculated with the following formulas:

  • Lumizyme dose in mg = Patient weight in kg X 20 mg/kg
  • Number of vials to be reconstituted = Lumizyme dose in mg ÷ 50 mg/vial
    (round result up to next whole number)

For example, if a patient weighs 68 kg:

  • Lumizyme dose in mg = 68 kg X 20 mg/kg = 1,360 mg
  • Number of vials to be reconstituted = 1,360 mg ÷ 50 mg/vial = 27.2 vials, rounded up = 28 vials
    Actual body weight was used as the basis for dosing in Lumizyme clinical trials. Physicians who have questions about dosing should contact Sanofi Genzyme Medical Information (800-745-4447, option 2).

Reconstitution & Dilution

Lumizyme is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder that must first be reconstituted before administration. For more details on Lumizyme reconstitution and dilution, see the Handling & Preparation page.

Administering Infusions

Once prepared, the diluted Lumizyme solution should be filtered through a 0.2 or 0.22 μm (micron), low protein-binding, in-line filter during administration to remove any visible particles. These filters are NOT provided in the product package.

Lumizyme should not be infused in the same intravenous line with other products, and the solution should be protected from light by using light-shielding covers over the infusion bag.

Filter

Recommended Infusion Volumes and Rates

Infusions should be administered in a step-wise manner using an infusion pump. The initial infusion rate should be no more than 1 mg/kg/hr. The infusion rate may be increased by 2 mg/kg/hr every 30 minutes, after patient tolerance to the infusion rate is established, until a maximum rate of 7 mg/kg/hr is reached. Consult the following chart for infusion rates at each step:

Patient Weight Range (kg) Total Infusion Volume (ml) Step 1
1 mg/kg/hr (mL/hr)
Step 2
3 mg/kg/hr (mL/hr)
Step 3
5 mg/kg/hr (mL/hr)
Step 4
7 mg/kg/hr (mL/hr)

1.25 – 10

50

3

8

13

18

10.1 – 20

100

5

15

25

35

20.1 -30

150

8

23

38

53

30.1 - 35

200

10

30

50

70

35.1 - 50

250

13

38

63

88

50.1 - 60

300

15

45

75

105

60.1 -100

500

25

75

125

175

100.1 -120

600

30

90

150

210

120.1 - 140

700

35

105

175

245

140.1 - 160

800

40

120

200

280

160.1 - 180

900

45

135

225

315

180.1 - 200

1000

50

150

250

350

Infusion Safety Guidelines1

  • Take the patient's vital signs at the end of each step. If the patient is stable, Lumizyme may be administered at the maximum rate of 7 mg/kg/hr until the infusion is completed.
  • In the event of infusion reactions, the infusion rate may be slowed and/or temporarily stopped.
  • If severe infusion or allergic reactions occur, consider immediately discontinuing the infusion, and initiate appropriate medical treatment.
  • Have appropriate medical support, including cardiopulmonary resuscitation equipment, readily available whenever Lumizyme is administered.
  • The risks and benefits of re-administering Lumizyme following an anaphylactic or severe allergic reaction should be considered. Some patients have been rechallenged and have continued to receive Lumizyme under close clinical supervision; however, extreme care should be exercised, with appropriate resuscitation measures available, if the product is re-administered.
 

Resources

Full Prescribing Information (PDF)

Dosing & Administration Guide

Find details on dose calculation, infusion volumes and rates, and step-by-step product preparation:

Lumizyme Fact Sheet (PDF)

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 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.

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.

References

  1. Lumizyme Full Prescribing Information, Genzyme Corporation