Fabry disease.

Cardiovascular -       cardiomyopathy 3%            hypotension 7%    edema dependent 3%

CNS-                          dizziness  7%       headache   38%     paraesthesia  7%     anxiety  17%
                                   depression   3%

GI -                            dyspepsia  3%      nausea  14%

Respiratory            bronchitis  3%               

Special Precautions-
Infusion reactions- occurred in many patients treated with agalasidase beta.
Some of the reactions included fever, rigors, chest tightness, hypertension, hypotension, pruritus,
myalgia, dyspnea, urticsaria, abdominal pain, and headache.

All the patients were pretreated with acetoaminophen and an antihistamine.
Patients should be given antipyretics prior to infusion.

Cardiac function- patients with Fabry  disease may have compromised cardiac function which
predisposes them to compromised cardiac function.
Patients with compromised cardiac function should be monitored closely if the decison is made
to administer agalasidase beta

Pregnancy- this drug should be used during pregnancy only if clearly needed

Lactation-     caution should be excecised when agalsidase beta is administered to a breast feeding
woman

Children- The safety and efficacy of agalsidase beta in pediatric patients have not been established

Indication-
Fabry disease.

Dosage-
1mg/kg body weight infused every 3 weeks as an IV infusion.

The initial infusion rate should be no more than 0.25mg/min ( 15mg/hr)
The infusion may be slowed in the event of infusion associated reaction.

After patient tolerance to the infusion is well established the infusion rate may be increased
in incrementof 0.05 to 0.08mg/min ( increments of 3 to 5mg/hr) each subsequent infusion.

1. Patients should be informed that a registry has been established in order understand the variability
    and progression of Fabry disease in the population as a whole and in women.

2. The registry will also monitor the effects of agalidase beta on pregnant woman and their offspring
     and determine if the drug is excreted in the breast milk.

3. Patients should be encouraged to participate and adviced that participation is voluntary and
   may invlove long term follow up

Pharmacology-
Fabry disease is an X-linked genetic disorder of glycosphingolid metabolism. 
Defeciency of the glycosomal enzyme alpha-galatosidase A leads to progressive accumulation
of glycosphingolipids  predominantly GL-3 in many  body tissues , occuring over a period of
years or decades.

Clinical manifestation of the disease include renal  failure, cardiomyopathy, and cerebrovascular
accidents. Accumulation of GL-3 in renal endothelial cells may play a role in renal failure

Pregnancy-
This drug should be used during pregnancy only if clearly needed

Lactation- 
Caution should be excecised when agalsidase beta is administered to a breast feeding
woman

Children-
The safety and efficacy of agalsidase beta in pediatric patients have not been established