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What to Expect from Fabrazyme Enzyme Replacement Therapy (ERT)

If you are starting treatment for the first time, there may be a significant amount of GL-3 in your body's cells. Regular intravenous (IV) infusions with Fabrazyme® (agalsidase beta) are necessary to keep GL-3 levels reduced. For more on this, see Continuing Treatment.

Dosage

The recommended Fabrazyme dosage is 1.0 milligram (mg) for each kilogram (kg) of body weight given once every 2 weeks as an intravenous (IV) infusion. For instance, a man weighing 70 kg (approximately 154 pounds) would receive 70 mg of Fabrazyme.

How Fabrazyme is Administered

Fabrazyme is administered by IV infusion. This is a process that involves injecting the drug into a vein, directly into the bloodstream. Fabrazyme cannot be taken orally as a pill or liquid because it is an enzyme that is destroyed in the digestive system. Infusion into the bloodstream bypasses the body's digestive processes and allows Fabrazyme to work in cells throughout the body.

The Infusion Process

Before the infusion begins, your health care professional will give you anti-fever medication (e.g. ibuprofen or acetaminophen) to help prevent reactions your body may have to Fabrazyme.

Your infusion time may be approximately 4 to 5 hours when you first start treatment because your doctor will want to see how your body responds to Fabrazyme. However, that time may decrease once your body becomes used to the infusion. In a clinical study, over time a majority of patients completed one or more infusions in approximately 2 hours. Your results with Fabrazyme infusion may vary. Talk to your doctor about what you can expect for your infusion time.

During the infusion, you can do things such as read a book, talk on the phone, write in your journal, visit with friends or family who are with you, or use the restroom--even take a nap if you wish.

With Fabrazyme treatment, it is important not to miss a dose. Go to Infusion Scheduler for a tool to help you remember when your treatment is scheduled.


Important Safety Information

Fabrazyme (agalsidase beta) is indicated for use in patients with Fabry disease.  Fabrazyme reduces globotriaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types. The reduction of GL-3 inclusions suggests that Fabrazyme may ameliorate disease expression; however, the relationship of GL-3 inclusion reduction to specific clinical manifestations of Fabry disease has not been established.

The most serious and most common adverse reactions reported with Fabrazyme are infusion reactions.  Serious and/or frequently occurring related adverse reactions consisted of one or more of the following events: chills, pyrexia, feeling hot or cold, dyspnea, nausea, flushing, headache, vomiting, paresthesia, fatigue, pruritus, pain in extremity, hypertension, chest pain, throat tightness, abdominal pain, dizziness, tachycardia, nasal congestion, diarrhea, edema peripheral, myalgia, back pain, pallor, bradycardia, urticaria, hypotension, face edema, rash, and somnolence. The occurrence of somnolence can be attributed to clinical trial specified pre-treatment with antihistamines. 
 
Other reported serious adverse events included stroke, pain, ataxia, bradycardia, cardiac arrhythmia, cardiac arrest, decreased cardiac output, vertigo, hypoacousia, and nephrotic syndrome.  These adverse events also occur as manifestations of Fabry disease; an alteration in frequency or severity cannot be determined from the small numbers of patients studied.

Infusion reactions occurred in many patients treated with Fabrazyme and some of the reactions were severe.  Patients should be given antipyretics prior to infusion.  Infusion reactions occurred in some patients after receiving pretreatment with antipyretics, antihistamines, and oral steroids.  Infusion reactions declined in frequency with continued use of Fabrazyme.  However, infusion reactions may still occur despite extended duration of Fabrazyme treatment.  Because of the potential for severe infusion reactions, appropriate medical support measures should be readily available when Fabrazyme is administered. 

Patients with compromised cardiac function should be monitored closely if the decision is made to administer Fabrazyme.

Most patients develop IgG antibodies to Fabrazyme.  A few patients developed IgE or skin test reactivity specific to Fabrazyme.  Physicians should consider testing for IgE in patients who experienced suspected allergic reactions and consider the risks and benefits of continued treatment in patients with anti- Fabrazyme IgE.   Patients with Fabrazyme- specific IgE antibody have been treated using a rechallenge protocol.  Rechallenge of these patients should only occur under the direct supervision of qualified personnel, with appropriate medical support measures readily available.

The safety and efficacy in patients younger than 8 years of age have not been evaluated.  IgE immunologic responses in pediatric patients may differ from those in adults, as IgG seroconversion was associated with prolonged half-life concentrations of Fabrazyme, which is rarely observed in adult patients.

Fabrazyme is available by prescription only. Side effects should be reported promptly to Genzyme Medical Information at 800-745-4447, option 2. To learn more, please see the full prescribing information (PDF) or contact Genzyme at 1-800-745-4447.
 
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