GALSULFASE (Systemic)?

Some commonly used brand names are:

In the U.S.?

• Naglazyme

? Not commercially available in Canada.

Category

• Enzyme replenisher

Description

Galsulfase (gal-SUL-face ) is used totreat mucopolysaccharidosis (MPS VI) disease caused by the lack of a certainenzyme called N-acetylgalactosamine 4-sulfatase in the body.

Galsulfase is available only with your doctor's prescription, in the followingdosage forms:

Parenteral
• Injection (U.S.)

Before Using This Medicine

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

Allergies?Tell your doctor if you have ever had any unusualor allergic reaction to galsulfase. Also tell your doctor and pharmacist ifyou are allergic to any other substances, such as foods, preservatives, ordyes.

Pregnancy?Galsulfase has not been studied in pregnant women.However, galsulfase has not been shown to cause birth defects or other problemsin animal studies.

Breast-feeding?It is not known whether galsulfase passes intobreast milk. Although most medicines pass into breast milk in small amounts,many of them may be used safely while breast-feeding. Mothers who are takingthis medicine and who wish to breast-feed should discuss this with their doctor.

Children?This medicine has been tested in children 5 yearsof age and older and has not been shown to cause different side effects orproblems than is does in adults. It is not known if children under5 respond differently from older children.

Older adults?Many medicines have not been studied specificallyin older people. Therefore, it may not be known whether they work exactlythe same way they do in younger adults or if they cause different side effectsor problems in older people. There is no specific information comparing useof galsulfase in the elderly with use in other age groups.

Other medicines?Although certain medicines should not be usedtogether at all, in other cases two different medicines may be used togethereven if an interaction might occur. In these cases, your doctor may want tochange the dose, or other precautions may be necessary. Tell your health careprofessional if you are taking any other prescription or nonprescription (over-the-counter[OTC]) medicines.

Proper Use of This Medicine

Dosing?The dose of galsulfase will be different for different patients. Follow your doctor's orders or the directions on thelabel. The following information includes only the average doses ofgalsulfase.

• For Mycopolysaccharidosis VI:
  • For injection dosage form:
    • Adults and children?The dose is based on body weight and mustbe determined by your doctor. The usual dose is 1 milligrams (mg) per kilogram(kg), given once weekly. It is injected slowly into a vein over at least fourhours.

Precautions While Using This Medicine

Regularvisits: If you will be taking this medicine for a long time, it is very important that your doctor check you at regular visits

Side Effects of This Medicine

Along with its needed effects, a medicinemay cause some unwanted effects. Although not all of these side effects mayoccur, if they do occur they may need medical attention.

Check with your doctorimmediately if any of the following side effects occur:

Less common

Blindness; blurred vision; chest pain; decreased vision; difficult or labored breathing; dizziness; headache; hernia of thenaval; nervousness; poundingin the ears; shortness of breath; slow or fast heartbeat; swelling of the face; tightness in chest; wheezing

Frequency unknown

Bluish lips or skin; confusion; cough; dizziness, faintness, or lightheadednesswhen getting up from a lying or sitting position suddenly; facial swelling; fever or chills; hives or welts; itching; joint pain; large, hive-like swelling on face, eyelids,lips, tongue, throat, hands, legs, feet, sex organs; nausea; noisy breathing; not breathing; pain behind the sternum; redness of skin; skin rash;; stomach pain; sweating; troubled breathing; vomiting

Otherside effects may occur that usually do not need medical attention. These sideeffects may go away during treatment as your body adjusts to the medicine.However, check with your doctor if any of the following side effects continueor are bothersome.

More common

Body produces substance that can bind to drug making it lesseffective or cause side effects; ear pain; stomach pain ; diarrhea; loss of appetite; pain

Less common

Body aches or pain; burning, dry oritching eyes; congestion; discharge; dryness or soreness of throat; excessive tearing; general feeling ofdiscomfort or illness; hoarseness; loss of or increase in reflexes; redness, pain, swellingof eye, eyelid, or inner lining of eyelid; runnynose; stuffy nose; tender,swollen glands in neck ; trouble in swallowing; unusual tiredness or weakness; voicechanges

Observed during clinical trials

Difficulty in moving; ear congestion; loss of voice; musclepain or stiffness; nasal congestion; redness or swelling in ear; sneezingor sore throat

Other side effects not listed above may also occur in some patients. Ifyou notice any other effects, check with your doctor.