Imiglucerase is a man-made form of an enzyme that occurs naturally in the body. It is used as an enzyme replacement in people with Type I Gaucher disease.

Gaucher disease is a genetic condition in which the body lacks the enzyme needed to break down certain fatty materials (lipids). Lipids can build up in the body, causing symptoms such as easy bruising or bleeding, weakness, anemia, bone or joint pain, enlarged liver or spleen, or weakened bones that are easily fractured.

Imiglucerase may improve the condition of the liver, spleen, bones, and blood cells in people with Type I Gaucher disease. However, imiglucerase is not a cure for this condition.

Imiglucerase may also be used for purposes other than those listed in this medication guide.

Follow all directions on your medicine label and package. Tell each of your healthcare providers about all your medical conditions, allergies, and all medicines you use.

Imiglucerase is injected into a vein through an IV. A healthcare provider will give you this injection.

Imiglucerase is usually given every 2 weeks, but you may need the medicine more often when you first start using it. Follow your doctor's dosing instructions very carefully.

You may be given other medicines to prevent certain side effects of imiglucerase. Take these medicines exactly as directed.

Tell your doctor if you have any changes in weight. Imiglucerase doses are based on weight.

Usual Adult Dose for Gaucher Disease:

Initial dose: May range from 2.5 units/kg 3 times a week IV infusion to 60 units/kg IV infusion once every 2 weeks

Comments:
-The majority of data available is for the 60 units/kg every 2 weeks dose.
-Disease severity may dictate that treatment be initiated at a relatively high dose or relatively frequent administration.
-Dose adjustments should be made on an individual basis and may increase or decrease based on achievement of therapeutic goals as assessed by routine evaluations of the patient's clinical manifestations.

Use: For long-term enzyme replacement therapy in patients with a confirmed diagnosis of Type 1 Gaucher disease that results in 1 or more of the following conditions: anemia, thrombocytopenia, bone disease, hepatomegaly, or splenomegaly

Usual Pediatric Dose for Gaucher Disease:

2 years or older:
-Initial dose: May range from 2.5 units/kg 3 times a week IV infusion to 60 units/kg IV infusion once every 2 weeks

Comments:
-The majority of data available is for the 60 units/kg every 2 weeks dose.
-Disease severity may dictate that treatment be initiated at a relatively high dose or relatively frequent administration.
-Dose adjustments should be made on an individual basis and may increase or decrease based on achievement of therapeutic goals as assessed by routine evaluations of the patient's clinical manifestations.

Use: For long-term enzyme replacement therapy in patients with a confirmed diagnosis of Type 1 Gaucher disease that results in 1 or more of the following conditions: anemia, thrombocytopenia, bone disease, hepatomegaly, or splenomegaly

You should not use imiglucerase if you are allergic to it.

To make sure imiglucerase is safe for you, tell your doctor if you have:

• a breathing problem such as pneumonia or pulmonary hypertension.

FDA pregnancy category C. It is not known whether imiglucerase will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant while using imiglucerase.

It is not known whether imiglucerase passes into breast milk or if it could harm a nursing baby. Tell your doctor if you are breast-feeding a baby.

Imiglucerase should not be given to a child younger than 2 without a doctor's advice.

Call your doctor for instructions if you miss a dose of imiglucerase.

Since imiglucerase is given by a healthcare professional in a medical setting, an overdose is unlikely to occur.

Follow your doctor's instructions about any restrictions on food, beverages, or activity.

Get emergency medical help if you have any of these signs of an allergic reaction: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Some side effects may occur during or shortly after the injection. Tell your caregiver right away if you feel dizzy, itchy, light-headed, sweaty, or have chest pain, cough, trouble breathing, or flushing (warmth, redness, or tingly feeling).

Imiglucerase may cause serious side effects. Call your doctor at once if you have:

• fever, chills, cough with yellow or green mucus;

• stabbing chest pain, wheezing, feeling short of breath; or

• worsening or no improvement in your Gaucher disease symptoms.

Common side effects of imiglucerase may include:

• stomach pain, nausea, vomiting, diarrhea;

• fast heartbeats;

• headache, dizziness;

• back pain;

• fever, chills, tired feeling;

• mild rash; or

• itching, burning, swelling, or other discomfort around the IV needle.

Other drugs may interact with imiglucerase, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell each of your health care providers about all medicines you use now and any medicine you start or stop using.