What is it and how is it used?
Naglazyme is used to treat patients with MPS VI disease (Mucopolysaccharidosis VI). People with MPS VI disease have either a low level, or no level, of an enzyme called N-acetylgalactosamine 4-sulfatase, which breaks down specific substances (glycosaminoglycans) in the body. As a result, these substances do not get broken down and processed by the body as they should. They accumulate in many tissues in the body, which causes the symptoms of MPS VI.
Naglazyme contains an artificial enzyme called galsulfase. This can replace the natural enzyme which is lacking in MPS VI disease.
What do you have to consider before using it?
Do not use Naglazyme
Take special care with Naglazyme
Kidney and liver insufficiency
Naglazyme has not been tested in patients with kidney or liver problems. Talk to your doctor if you have kidney or liver insufficiency.
Using other medicines
Please tell your doctor if you are taking or have recently taken any other medicines, including medicines obtained without a prescription.
Pregnancy
Naglazyme should not be taken during pregnancy unless clearly necessary. Ask your doctor or pharmacist for advice before taking any medicine.
Breast-feeding
It is not known whether galsulfase is excreted in milk, therefore breast-feeding should be stopped during Naglazyme treatment. Ask your doctor or pharmacist for advice before taking any medicine.
Driving and using machines
No studies on the effects on the ability to drive and use machines have been performed
Important information about some of the ingredients of Naglazyme
Each 5 ml vial contains 0.8 mmol (18.4 mg) of sodium. To be taken into consideration by patients on a controlled sodium diet.
How is it used?
Your doctor or nurse will administer the product to you.
The concentrate for solution for infusion has to be diluted prior to administration by intravenous infusion.
Dose
The recommended dose regimen is 1 mg/kg body weight administered once every week through a drip into a vein (by intravenous infusion). Each infusion will take approximately 4 hours. For the first hour the infusion rate will be slow (approximately 2.5% of the total solution), with the remaining volume (approximately 97.5%) being taken over the next 3 hours.
If you are given more Naglazyme than you should
Naglazyme is administered under the supervision of a nurse or doctor, he or she will check that the correct dose has been given and act accordingly if necessary.
If you forget to take Naglazyme
If you have missed a Naglazyme infusion, please contact your doctor.
If you have any further questions on the use of this medicine, ask your doctor.
What are possible side effects?
Like all medicines, Naglazyme can cause side effects, although not everybody gets them.
The following side effects were reported as being related to Naglazyme:
Very common (affects more than 1 user in 10): Abdominal pain, ear pain, pain, reddened eye, shortness of breath, chills, chest pain, sore throat, stomach pain, poor reflexes, cloudy eyes, swollen face, gastroenteritis, high blood pressure, malaise, nasal congestion, bulging belly button, fever, headache, rash, nausea, vomiting and joint pain.
Common (affects 1 to 10 users in 100): Hives, swelling of the tongue and throat, low blood pressure, difficulty breathing and apnoea.
If any of the side effects gets serious, or if you notice any side effects not listed in this leaflet, please tell your doctor.
How should it be stored?
Keep out of the reach and sight of children.
Do not take Naglazyme after the expiry date which is stated on the vial after EXP. The expiry date refers to the last day of that month.
Unopened vials:
Store in a refrigerator (2°C - 8°C).
Do not freeze.
Diluted solutions:
Chemical and physical in-use stability has been demonstrated for up to 4 days at room temperature (23°C - 27°C).
From a microbiological safety point of view, the product is to be used immediately. If not used immediately, in-use storage times and conditions are the responsibility of the user and must normally not be longer than 24 hours at 2°C - 8°C followed by up to 24 hours at room temperature (23°C - 27°C) during administration.
Do not take Naglazyme if it contains visible particles.
Medicines should not be disposed of via wastewater or household waste. Ask your pharmacist how to dispose of medicines no longer required. These measures will help to protect the environment.
