In the U.S.—

• Enzyme (alpha-galactosidase A) replenisher

Agalsidase beta (a-GAl-si-daze bay-ta) 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 available only with your doctor's prescription, in the following dosage forms:

Parenteral
• Injection (U.S.)

In deciding to use a medicine, the risks of receiving the medicine must be weighed against the good it will do. 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 unusual or allergic reaction to agalsidase beta. Also tell your doctor and pharmacist if you are allergic to any other substances, such as foods, preservatives, or dyes.

Pregnancy—Agalsidase beta has not been studied in pregnant women or animals.

Breast-feeding—It is not known whether agalsidase beta passes into human breast milk.

Children—Studies on this medicine have been done only in adult patients, and there is no specific information comparing use of agalsidase beta in children with use in other age groups.

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

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 health care professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

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

Dosing—

The dose of agalsidase beta will be different for different patients. Follow your doctor's orders. The following information 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 determined by your doctor. The usual dose is 1 mg per kilogram (kg) of body weight, injected slowly 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.

It is important that your doctor monitor your progress while you are receiving agalsidase beta to make sure that the medicine is working properly.

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

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

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 immediately if any of the following side effects occur:

• More common
  • Blurred vision;  chest pain or discomfort ;  confusion;  cough producing mucus;  difficulty breathing;  difficulty in moving;  dizziness;;  fatigue;  faintness or lightheadedness when getting up from a lying or sitting position suddenly;  feeling unusually cold shivering;  headache;  hives or welts;  irregular heartbeat ;  itching;  joint pain;  muscle aching or cramping;  muscle pains or 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 cardiac output;  difficulty in speaking;  double vision;  feeling of constant movement of self or surroundings;  high blood pressure;  inability to move arms, legs, or facial muscles;  inability to speak;  no blood pressure or pulse;  partial loss of hearing;  pounding or rapid pulse;  problems with muscle control or coordination;  sensation of spinning;  shakiness and unsteady walk;  slow speech ;  stopping of heart;  swelling of face;  swelling of the lip or ear;  throat tightness;  trembling;  unconsciousness 
  
  

Other 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. However, check with your doctor if any of the following side effects continue or are bothersome.

• 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 throat;  fear;  feeling sad or empty;  fever, not related to infusion;  heartburn;  hoarseness;  indigestion;  irritability;  lack of appetite;  loss of interest or pleasure;  nausea;  pain or tenderness around eyes and cheekbones;  paleness of skin;  runny nose;  sensation of 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  
  
  

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