Pronunciation:

aye-GAL-si-days BAY-ta

Brand Names:

• Fabrazyme

Dosage Forms:

• Powder for Solution

Classifications:

Therapeutic—

Endocrine-Metabolic Agent

Pharmacologic—

Enzyme

Uses of This Medicine:

Agalsidase beta injection is used to treat Fabry disease, which is an inherited disease caused by the lack of an enzyme called alpha-galactosidase A in the body. This enzyme is necessary for your body. Agalsidase beta is used to help replace this enzyme.

This medicine is to be given only by or under the supervision of your doctor.

Before Using This Medicine:

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

Allergies—

Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

Children—

Appropriate studies have not been performed on the relationship of age to the effects of agalsidase beta injection in children younger than 2 years of age. Safety and efficacy have not been established.

Older adults—

Appropriate studies have not been performed on the relationship of age to the effects of agalsidase beta injection in geriatric patients.

Breast-feeding—

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Other medicines—

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

Other interactions—

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.

Other medical problems—

The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:

• Heart problems—Use with caution. May be at a higher risk of severe complications from infusion reaction.

Proper Use of This Medicine:

A nurse or other trained health professional will give you this medicine in a medical facility. This medicine is given through an IV catheter that is placed in one of your veins. The medicine must be injected slowly, so the IV will need to stay in place for at least 1½ hours.

You or your child may also receive other medicines (eg, allergy medicine, fever medicine, steroids) before starting treatment with this medicine.

Precautions While Using This Medicine:

It is very important that your doctor check your or your child's progress closely while you are receiving this medicine to make sure that it is working properly. Blood tests may be needed to check for unwanted effects.

This medicine may cause serious allergic reactions, including anaphylaxis, which can be life-threatening and require immediate medical attention. Check with your doctor right away if you or your child have chest tightness, cough, difficulty swallowing, dizziness, fast heartbeat, hives, itching, puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue, skin rash, trouble breathing, or unusual tiredness or weakness.

This medicine may cause infusion-related reactions, which can be life-threatening and require immediate medical attention. Tell your doctor right away if you or your child start to have a fever, chills or shaking, chest pain, dizziness, flushing, headache, trouble breathing, hives, itching, or skin rash, lightheadedness, fainting, fast, pounding, or uneven heartbeat, or trouble breathing after receiving this medicine.

Side Effects of This Medicine:

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

Check with your doctor or nurse immediately if any of the following side effects occur:

More common

Blurred vision

chest pain, discomfort, or tightness

confusion

cough producing mucus

difficulty breathing

difficulty in moving

dizziness

faintness or lightheadedness when getting up suddenly from a lying or sitting position

feeling unusually cold shivering

headache

hives or welts, itching, skin rash

irregular heartbeat

joint pain

muscle ache, cramps, pains, or stiffness

nervousness

pounding in the ears

redness of the skin

stomach pain

sweating

swelling of the ankles, feet, and lower legs

swollen joints

unusual tiredness or weakness

Incidence unknown

Blue lips, fingernails, or skin

chills

cloudy or bloody urine

cough

decreased cardiac output

decreased frequency or amount of urine

difficulty in speaking

difficulty swallowing

dilated neck veins

double vision

fast heartbeat

feeling of constant movement of self or surroundings

fever

high blood pressure

inability to move the arms, legs, or facial muscles

increased thirst

irregular, fast, or slow, or shallow breathing

lack or loss of strength

large, hive-like swelling on the face, eyelids, lips, tongue, throat, hands, legs, feet, or sex organs

loss of appetite

lower back or side pain

nausea

no breathing

no pulse or blood pressure

noisy breathing

pain in the joints

pain or discomfort in the arms, jaw, back, or neck

problems with muscle control or coordination

puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue

seizures

sensation of spinning

severe or sudden headache

shakiness and unsteady walk

slow or slurred speech

swollen, painful, or tender lymph glands in the neck, armpit, or groin

temporary blindness

throat tightness

trembling

trouble breathing

unconsciousness

vomiting

weakness in the arm or leg on one side of the body, sudden and severe

weight gain

Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More Common

Acid or sour stomach

belching

body aches or pain

body produces substance that 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 soreness of the throat

fear

feeling sad or empty

fever, not related to infusion

heartburn

hoarseness

indigestion

irritability

loss of interest or pleasure

pain or tenderness around eyes and cheekbones

paleness of skin

runny nose

sensation of change in temperature

skeletal pain

sneezing

stuffy nose

trouble concentrating

trouble sleeping

voice changes

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.