Imiglucerase

Imiglucerase is a man-made version of an enzyme that is normally found in the body. It is used as a replacement enzyme for people with Type I Gaucher disease.

Gaucher disease is a genetic disorder where the body doesn't have enough of the enzyme needed to break down certain fatty substances (lipids). When lipids build up in the body, they can cause symptoms like easy bruising or bleeding, weakness, anemia, bone or joint pain, an enlarged liver or spleen, or fragile bones that can break easily.

Imiglucerase may help improve the health 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 other purposes not mentioned in this medication guide.

Imiglucerase is given as an injection into a vein through an IV. A healthcare provider will administer this injection.

Imiglucerase is typically given every 2 weeks, but you may need to receive it more often when you first start treatment. Follow your doctor's dosing instructions very carefully.

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

Inform your doctor if you experience any changes in weight. Doses of imiglucerase are determined by your weight.

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.

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

To ensure imiglucerase is safe for you, inform your doctor if you have:

• a breathing problem such as pneumonia or pulmonary hypertension.

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

It is not known if imiglucerase passes into breast milk or if it could harm a nursing baby. Inform 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.

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.

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 weeksComments:-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 weeksComments:-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

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

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

Follow your doctor's instructions regarding any restrictions on food, drinks, or activities.

Other drugs may interact with imiglucerase, including prescription and over-the-counter medicines, vitamins, and herbal products. Inform each of your health care providers about all the medicines you are currently using and any medicines you begin or stop using.