Naglazyme contains an enzyme that occurs naturally in the body in healthy people. Some people lack this enzyme because of a genetic disorder. Galsulfase helps replace this missing enzyme in such people.

Naglazyme is used to treat some of the symptoms of a genetic condition called mucopolysaccharidosis VI (MYOO-koe-pol-ee-SAK-a-rye-DOE-sis type 6), or MPS VI, also called Maroteaux-Lamy syndrome.

MPS VI is a metabolic disorder in which the body lacks the enzyme needed to break down certain natural substances. These substances can build up in the body, causing enlarged organs, abnormal bone structure, changes in facial features, breathing problems, heart problems, vision or hearing loss, and changes in mental or physical abilities.

Naglazyme may improve walking and stair-climbing ability in people with this condition. However, this medication is not a cure for MPS VI.

An allergic reaction may occur during or shortly after the Naglazyme infusion. Tell your caregivers or get emergency medical help right away if you have any signs of a severe allergic reaction, such as chest pain, trouble breathing, vomiting, skin rash or redness, and feeling like you might pass out.

Before taking this medicine

Your Naglazyme infusion may be delayed if you have a fever or cold symptoms.

To make sure Naglazyme is safe for you, tell your doctor if you have ever had:

• breathing problems; or

• sleep apnea, and you use a continuous positive airway pressure (CPAP) machine.

Tell your doctor if you are pregnant or breastfeeding.

Your name may need to be listed on an MPS IV Registry while you are using Naglazyme. The purpose of this registry is to track the progression of this disorder and the effects that galsulfase has on long-term treatment of MPS IV. The registry also tracks the effects of galsulfase on a baby if you are pregnant or breastfeeding.

Naglazyme is given as an infusion into a vein. A healthcare provider will give you this injection.

Naglazyme is usually given once per week.

Tell your doctor if you have been sick with a fever or cold. You may need to wait until you get better before receiving your dose of Naglazyme.

Naglazyme must be given slowly, and the infusion can take up to 4 hours to complete.

About 30 to 60 minutes before each injection, you will be given other medications to help prevent a serious allergic reaction.

Your doctor will need to check your progress on a regular basis.

Usual Adult Dose for Mucopolysaccharidosis Type VI:

1 mg/kg IV once a week

Comments:
-Clinical studies did not include patients older than 29 years and it is therefore unknown if they respond differently than younger patients.

Use: For the treatment of patients with Mucopolysaccharidosis VI (MPS VI [Maroteaux-Lamy syndrome])

Usual Pediatric Dose for Mucopolysaccharidosis Type VI:

5 years or older: 1 mg/kg IV once a week

Use: For the treatment of patients with MPS VI (Maroteaux-Lamy syndrome)

Your Naglazyme infusion may be delayed if you have a fever or cold symptoms.

To make sure Naglazyme is safe for you, tell your doctor if you have ever had:

• breathing problems; or

• sleep apnea, and you use a continuous positive airway pressure (CPAP) machine.

Tell your doctor if you are pregnant or breastfeeding.

Your name may need to be listed on an MPS IV Registry while you are using Naglazyme. The purpose of this registry is to track the progression of this disorder and the effects that galsulfase has on long-term treatment of MPS IV. The registry also tracks the effects of galsulfase on a baby if you are pregnant or breastfeeding.

Call your doctor for instructions if you miss an appointment for your Naglazyme injection.

Since this medication 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 signs of an allergic reaction to Naglazyme: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

An allergic reaction may occur during or shortly after infusion of this medicine (up to 24 hours later). Tell your caregivers or get emergency medical help right away if you have any signs of a severe allergic reaction, such as:

• rapid breathing, or feeling short of breath;

• chest pain;

• feeling like you might pass out, even while lying down;

• fever, chills;

• skin rash or redness, itching;

• eye redness;

• headache; or

• vomiting, stomach pain.

Even though it may not be a side effect of galsulfase, increased pressure on the spinal cord is a complication of MPS VI that may occur while you are using Naglazyme. Tell your doctor right away if you have any symptoms of spinal cord compression: back pain, loss of movement in any part of your body, loss of bowel or bladder control.

Call your doctor at once if you have:

• snoring or sleep apnea, trouble breathing;

• swelling, rapid weight gain; or

• shortness of breath (even with mild exertion).

Common Naglazyme side effects may include:

• fever, chills;

• trouble breathing;

• pain;

• rash or itching;

• headache; or

• nausea, vomiting, stomach pain.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.