Patient Monitoring & Management

Life-threatening anaphylactic reactions and severe allergic reactions have been observed in some patients during and up to three hours after Lumizyme® (alglucosidase alfa) infusions. Patients with acute underlying respiratory illness or compromised cardiac and/or respiratory function may be at risk of serious exacerbation of their cardiac or respiratory compromise during infusions.1

Patients should be observed during and after the completion of each infusion by appropriate medical personnel familiar with Pompe disease and potential reactions to treatment. Some patients may require prolonged observation times. The appropriate length of post-infusion monitoring should be determined by the treating physician based on the individual patient’s clinical status and infusion history.

During Infusions1

Following are some guidelines to follow during Lumizyme infusions:

  • Infusions should be administered in a step-wise manner, taking the patient's vital signs at the end of each step
  • Patients should be observed during and after the completion of each infusion by appropriate medical personnel familiar with Pompe disease and potential reactions to treatment
  • Appropriate medical support and monitoring measures should be readily available when Lumizyme is administered

Adverse Reactions & Follow-Up Care1

If an infusion reaction occurs, temporarily stop the infusion, and/or administer antihistamines and/or antipyretics.

If anaphylaxis or other severe allergic reactions occur, immediate discontinuation of the administration of Lumizyme should be considered, and appropriate medical treatment should be initiated. In some cases of anaphylaxis, epinephrine has been administered.  Appropriate medical treatment, may include administering antihistamines, corticosteroids, intravenous fluids, and/or oxygen, when clinically indicated.

The risks and benefits of re-adminstering 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.

Severe cutaneous and systemic immune mediated reactions have been reported with Lumizyme treatment, occurring from several weeks to three years after infusions. Therefore patients receiving Lumizyme should be monitored for the development of systemic immune mediated reactions involving skin and other organs.

Anaphylaxis and Allergic Reactions

Anaphylaxis and hypersensitivity reactions have been observed in patients during and up to 3 hours after alglucosidase alfa infusion. Some of the reactions were life-threatening and included anaphylactic shock, cardiac arrest, respiratory arrest, respiratory distress, hypoxia, apnea, dyspnea, bradycardia, tachycardia, bronchospasm, throat tightness, hypotension, angioedema (including tongue or lip swelling, periorbital edema, and face edema), and urticaria. Other accompanying reactions included chest discomfort/pain, wheezing, tachypnea, cyanosis, decreased oxygen saturation, convulsions, pruritus, rash, hyperhidrosis, nausea, dizziness, hypertension/increased blood pressure, flushing/feeling hot, erythema, pyrexia, pallor, peripheral coldness, restlessness, nervousness, headache, back pain, and paresthesia. Some of these reactions were IgE-mediated.

If anaphylaxis or severe hypersensitivity reactions occur, immediately discontinue administration of alglucosidase alfa, and initiate appropriate medical treatment. Severe reactions are generally managed with infusion interruption, administration of antihistamines, corticosteroids, intravenous fluids, and/or oxygen, when clinically indicated. In some cases of anaphylaxis, epinephrine has been administered. Appropriate medical support, including cardiopulmonary resuscitation equipment, should be readily available when alglucosidase alfa is administered. The risks and benefits of re-administering alglucosidase alfa following an anaphylactic or hypersensitivity reaction should be considered. Some patients have been rechallenged and have continued to receive alglucosidase alfa under close clinical supervision. Extreme care should be exercised, with appropriate resuscitation measures available, if the decision is made to re-administer the product .

Be sure to report any suspected adverse reactions to Sanofi Genzyme by:

  • Calling 800-745-4447, option 2

You can also report directly to the FDA's MedWatch program: call 800-FDA-1088 or visit the MedWatch site.

Monitoring for Immunogenicity

As with all therapeutic proteins, Lumizyme treatment poses potential for immunogenicity, so patients should be monitored regularly for antibody formation.

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

References

  1. Lumizyme Full Prescribing Information, Genzyme Corporation

Reporting Adverse Reactions

You can report suspected adverse reactions to Sanofi Genzyme by either:

Calling 800-745-4447, option 2 OR
Sending us a detailed description by fax at 617-761-8506 or by email

You can also report directly to the FDA's MedWatch program: call 800-FDA-1088 or visit the MedWatch site.

Contact Us

Sanofi Genzyme
50 Binney Street
Cambridge, MA 02142

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