AGALSIDASE BETA (Systemic)?

Some commonly used brand names are:

In the U.S.?

• Fabrazyme

? Not commercially available in Canada.

Category

• Enzyme (alpha-galactosidaseA) replenisher

Description

Agalsidase beta (a-GAl-si-daze bay-ta)is used to treat Fabry disease which is an inherited disease caused by thelack of an enzyme called alpha-galactosidase A, in the body. This enzyme isnecessary for your body.

Agalsidase beta is available only with your doctor's prescription, in thefollowing dosage forms:

Parenteral
• Injection (U.S.)

Before Using This Medicine

In deciding to use a medicine,the risks of receiving the medicine must be weighed against the good it willdo. This is a decision you and your doctor will make. For agalsidase beta,the following should be considered:

Allergies?Tell your doctor if you have ever had any unusualor allergic reaction to agalsidase beta. Also tell your doctor and pharmacistif you are allergic to any other substances, such as foods, preservatives,or dyes.

Pregnancy?Agalsidase beta has not been studied in pregnantwomen or animals.

Breast-feeding?It is not known whether agalsidase beta passesinto human breast milk.

Children?Studies on this medicine have been done only inadult patients, and there is no specific information comparing use of agalsidasebeta in children with use in other age groups.

Older adults?Many medicines have not been studied specificallyin older people. Therefore, it may not be known whether they work exactlythe same way they do in younger adults or if they cause different side effectsor problems in older people. There is no specific information comparing useof agalsidase beta in the elderly with use in other age groups.

Other medicines?Although certain medicines should not be usedtogether at all, in other cases two different medicines may be used togethereven if an interaction might occur. In these cases, your doctor may want tochange the dose, or other precautions may be necessary. Tell your health careprofessional if you are taking any other prescription or nonprescription (over-the-counter[OTC]) medicine.

Other medical problems?The presence of other medicalproblems may affect the use of agalsidase beta. Make sure you tell your doctorif you have any other medical problems, especially:

• Heart problems?May be at a higher risk of severe complicationsfrom infusion reaction.

Proper Use of This Medicine

Dosing?The dose of agalsidase beta will be different for differentpatients. Follow your doctor's orders. The followinginformation includes only the average doses of agalsidase beta.

• For Fabry disease:
  • For injection dosage form:
    • Adults?The dose is based on body weight and must be determinedby your doctor. The usual dose is 1 mg per kilogram (kg) of body weight, injectedslowly into a vein. The dose may be repeated once every 2 weeks.
    • Children?Use and dose must be determined by your doctor.

Missed dose?Contact your doctor as soon as possible for instructions.

Precautions While Using This Medicine

It is important that yourdoctor monitor your progress while you are receiving agalsidase beta to makesure that the medicine is working properly.

It is important to take medicine for treatment of infusion reaction priorto receiving your infusion.

Importance of receiving agalsidase beta in health care facility with medicalsupport for severe infusion reactions.

Side Effects of This Medicine

Along with its needed effects, a medicinemay cause some unwanted effects. Although not all of these side effects mayoccur, if they do occur they may need medical attention.

Check with your doctorimmediately if any of the following side effects occur:

More common

Blurred vision; chest pain or discomfort; confusion; cough producingmucus; difficulty breathing; difficulty in moving; dizziness;; fatigue; faintness or lightheadedness when gettingup from a lying or sitting position suddenly; feelingunusually cold shivering; headache; hives or welts; irregular heartbeat; itching; joint pain; muscle aching or cramping; muscle painsor stiffness; nervousness; pain; pounding in the ears; redness of skin; shortness of breath; skin rash; stomach pain; sweating; swelling of ankles, feet,and lower legs; swollen joints; tightness in chest; unusual tiredness or weakness; wheezing

Incidence unknown

Cloudy or bloody urine; decreased cardiacoutput; difficulty in speaking; double vision; feeling of constant movement of selfor surroundings; high blood pressure; inability to move arms, legs, or facial muscles; inability to speak; no blood pressureor pulse; partial loss of hearing; pounding or rapid pulse; problems with muscle controlor coordination; sensation of spinning; shakiness and unsteady walk; slow speech; stopping of heart; swellingof face; swelling of the lip or ear; throat tightness; trembling; unconsciousness

Other side effects may occur that usuallydo not need medical attention. These side effects may go away during treatmentas your body adjusts to the medicine. However, check with your doctor if anyof the following side effects continue or are bothersome.

More Common

Acid or sour stomach; belching; body aches or pain; body produces substancethat can bind to drug making it less effective or cause side effects; burning, crawling, itching, numbness, prickling, "pins and needles", or tingling feelings; congestion; discouragement; dryness or sorenessof throat; fear; feelingsad or empty; fever, not related to infusion; heartburn; hoarseness; indigestion; irritability; lack of appetite; loss of interest orpleasure; nausea; pain ortenderness around eyes and cheekbones; paleness ofskin; runny nose; sensationof change in temperature; skeletal pain; sneezing; stuffy nose; swelling of testes; tender, swollen glands in neck; trouble concentrating; trouble sleeping; trouble in swallowing; voice changes

Otherside effects not listed above may also occur in some patients. If you noticeany other effects, check with your doctor.