Imiglucerase (Intravenous)

Overview

Gaucher's disease is a rare genetic disease that is caused by the body's lack of the enzyme glucocerebrosidase. Without this enzyme, the body is unable to properly break down and make use of fats. This causes a problem where fats build up in the body. Symptoms of the disease include excessive bruising, bleeding, weakness, anemia, enlargement of the liver or spleen, weakened bones and bone and joint pain. Infusion with imiglucerase helps patients' bodies to break down fats.

The use of this medicine does not cure Gaucher's disease, but it does help to relieve its symptoms. Patients treated with imiglucerase see improvements in the problems in their blood, bone, liver and spleen that are caused by Gaucher's disease. Problems in the blood, liver and spleen generally improve within the first year of treatment with imiglucerase, whereas a person's bones may not receive the full benefit for two to three years of treatment.

Imiglucerase is injected directly into a vein for administration into the bloodstream. It is injected slowly over one or two hours as an infusion. Your infusion may be done at home or at your doctor's office or another location. Imiglucerase is a protein and, therefore, cannot be administered orally without being destroyed by the digestive system.

Imiglucerase can cause some side effects. These side effects are usually not severe and generally do not require medical treatment. However, if you are experiencing severe side effects or if your side effects persist or worsen, talk to your doctor about it. These side effects may be problematic or your doctor may be able to help you with ways of reducing or preventing some side effects.

It is rare for a patient to experience a severe allergic reaction to imiglucerase. If you experience any signs of a serious allergic reaction, get medical help immediately.

Condition Treated

  • Gaucher's disease

Type Of Medicine

  • Enzyme replacement

Side Effects

All medicines can cause unintended side effects as well as the intended effects on the body. You may experience the following common side effects:

  • Pain, itching, or swelling at the injection site

If these side effects do not go away or get worse over time, talk to your doctor or pharmacist about it. Your doctor may be able to recommend ways to reduce the severity or discomfort associated with some of these side effects. You may also report side effects to the FDA at 1-800-FDA-1088.

Make sure that your doctor or healthcare professional knows about all of your allergies. If you are allergic to imiglucerase, you may not be able to receive this treatment. If you have any of the symptoms of a serious allergic reaction to your infusion, get medical help immediately. These symptoms include:

  • Rash

These symptoms may indicate a dangerous allergic reaction. You should contact a doctor or get help right away.

Dosage

Your dosage will be determined by your doctor. Dosage will be different for different patients, depending on the patient's weight and the severity of their Gaucher's disease. Do not change your dosage for any reason without your doctor's instructions to do so. If you are receiving your infusion at home, make sure to follow your doctor's instructions carefully.

Imiglucerase is usually injected every two weeks. When you first begin treatment with imiglucerase, your doctor may administer it to you more often.

Tell your doctor if your weight changes, as this could affect your dosage of imiglucerase.

Interactions

Talk to your doctor about your medical history. Make sure your doctor knows about any medications you are taking. Some medications can interact with each other and reduce their effectiveness or put you at a higher risk of adverse reactions. Your doctor will tell you if you need to adjust the way you take other medications.

The medications miglustat and Zavesca are known to interact with imiglucerase. Tell your doctor or medical professional if you are taking or have taken these medications, or if you are planning to begin taking them.

Talk to your doctor about your diet and about your use of alcohol and tobacco. Some foods, as well as alcohol and tobacco use, can interact with some medications and alter the effectiveness of the medication. Your doctor will tell you if you need to maintain a particular diet during your treatment or if there are foods you should avoid. Your doctor may tell you not to use tobacco or alcohol around the time of your treatment.

Warnings

Talk to your doctor about any allergies that you have. Talk to your doctor about your medical history, especially if you are aware of the presence of antibodies to imiglucerase. It is possible for your body to develop antibodies to imiglucerase during treatment. This triggers an immune response to treatment with imiglucerase, and it can cause an allergic reaction or hypersensitivity. Your doctor may test you for antibodies periodically, especially during your first year of treatment.

Imiglucerase has not been tested to determine whether it is safe for pregnant women. Because of this, it is possible that it may not be safe if you are pregnant. If you are pregnant or plan to become pregnant, make sure you talk to your doctor about it. Your doctor will help you weigh the risks and benefits and determine if you should receive treatment.

Imiglucerase has not been tested on breastfeeding mothers. It is not known whether imiglucerase can pass into a mother's breast milk and affect her baby. If you are breastfeeding, discuss this risk with your doctor in order to decide if you should receive treatment.

Imiglucerase has been shown to be safe and effective for use in children who are between two and 16 years old. It has not been shown to be safe and effective for treatment of children under two years old.

You should always discuss the risks and benefits of a medication with your doctor in order to determine if it is right for you.

Storage

Imiglucerase should be stored at temperatures between 2 and 8C (36-46F). After reconstituting, examine imiglucerase carefully before using, and do not use if it is discolored or opaque. Do not store any imiglucerase that has been reconstituted - it should be diluted and administered promptly.

Do not use imiglucerase after it has reached its expiration date.

If you take imiglucerase at home, follow your doctor's instructions exactly. If you do not understand the instructions your doctor gave you, contact your doctor or a pharmacist before taking imiglucerase. Do not change your dosage or administration procedure without talking to your doctor about it first.

Summary

Imiglucerase has been determined to be a safe and effective treatment of the symptoms of Gaucher's disease. Gaucher's disease is a rare genetic condition whereby a patient's body does not produce the enzyme glucocerebrosidase, which breaks down fats in the body. The lack of this enzyme can cause fats to build up in the body, leading to joint and bone pain, excessive bruising or bleeding, weakness, anemia, enlargement of the liver or spleen and weakened bones. Imiglucerase is not a cure for Gaucher's disease, but it does help to reduce the symptoms and improve the quality of life of patients with Gaucher's disease.

Imiglucerase is administered by an injection. It is slowly released into a vein over the course of one or two hours. Infusion may be administered at home, at your doctor's office or at a treatment center or another facility.

Imiglucerase has not been tested for safe treatment of pregnant women or nursing mothers. If you are pregnant or breastfeeding, do not take imiglucerase without first talking to your doctor about the potential risks. Your doctor will help you weigh the risks and benefits to decide if you should receive treatment.

Imiglucerase has not been tested for safe and effective treatment of children under two years of age.

All medications can cause side effects. There are a number of side effects that are considered common in those who receive imiglucerase. If you have any severe side effects or if your initial side effects persist or worsen, talk to your doctor about them.

Severe allergic reactions to imiglucerase are rare. If you experience any of the symptoms of a severe allergic reaction, seek medical help immediately.

While you are receiving treatment with imiglucerase, your doctor should monitor your condition and how you are responding to treatment. If your weight changes, your doctor needs to know so that they can change your dosage or the frequency of administration, which is partially based on weight.