Pronunciation:

eye-dur-SUL-fase

Brand Names:

• Elaprase

Dosage Forms:

• Solution

Warnings:

Life-threatening anaphylactic reactions, presenting as respiratory distress, hypoxia, hypotension, urticaria, and/or angioedema of throat or tongue have occurred in some patients during and up to 24 hours after idursulfase infusions, regardless of duration of the course of treatment. Closely observe patients during and after idursulfase administration and be prepared to manage anaphylaxis. Inform patients of the signs and symptoms of anaphylaxis and have them seek immediate medical care should symptoms occur. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to hypersensitivity reactions, and require additional monitoring .

Classifications:

Therapeutic—

Enzyme Replacement

Pharmacologic—

Enzyme

Uses of This Medicine:

Idursulfase injection is used to treat Hunter syndrome (Mucopolysaccharidosis II, MPS II), a rare condition that is most often seen in males. Hunter syndrome is an inherited disease in which the breakdown of a certain chemical in the body (mucopolysaccharide) is defective due to the lack or absence of an enzyme called iduronate-2-sulfatase.

Idursulfase improves signs and symptoms in patients, especially walking capacity, by replacing the missing enzyme in Hunter syndrome.

This medicine is available only with your doctor's prescription.

Before Using This Medicine:

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:

Allergies—

Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

Children—

Appropriate studies performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of idursulfase injection in children. However, safety and efficacy have not been established in children younger than 16 months of age.

Older adults—

Appropriate studies have not been performed on the relationship of age to the effects of idursulfase injection in the geriatric population. Safety and efficacy have not been established.

Breast-feeding—

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

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

Other interactions—

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.

Other medical problems—

The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:

• Fluid overload (too much fluid in the blood) or
• Heart disease or
• Illness with fever or
• Lung disease or breathing problems—Use with caution. May increase risk of developing life-threatening complications.

Proper Use of This Medicine:

A nurse or other trained health professional will give you or your child this medicine in a hospital. This medicine is given through a needle placed in one of your veins.

This medicine is usually given one time each week. The medicine is given slowly, so the needle will remain in place for a few hours. You or your child may also receive medicines to help prevent possible allergic reactions to the injection.

Precautions While Using This Medicine:

It is very important that your doctor check your or your child's progress at regular visits. This will allow your doctor to see if the medicine is working properly and check you for unwanted effects that may be caused by this medicine.

This medicine may cause serious allergic reactions, including anaphylaxis. Anaphylaxis can be life-threatening and requires immediate medical attention. Tell your doctor or nurse right away if you or your child have a rash, itching, trouble breathing, trouble swallowing, or any swelling of your hands, face, or mouth after you or your child get the injection.

Side Effects of This Medicine:

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 or nurse immediately if any of the following side effects occur:

More common

Bone or muscle pain

chest pain

chills

cough

fast, pounding, or irregular heartbeat or pulse

feeling of warmth

fever

headache

hives or welts

itching

rash

redness of the face, neck, arms, and occasionally, upper chest

redness of the skin

sneezing

sore throat

tightness in the chest

unusual tiredness or weakness

Less common

Blurred vision

confusion

dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position

sweating

Incidence not known

Difficulty with swallowing

dilated neck veins

extreme fatigue

heart stops

irregular breathing

no breathing

no pulse or blood pressure

puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue

unconscious

weight gain

Some 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. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More common

Change in hearing

diarrhea

ear drainage

earache or pain in the ear

Less common

Nausea

vomiting

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

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.