What does MPS VI do to the body?

GAG is a key component of tissues throughout the body, so, in MPS VI, many major organs and systems can be harmed. This can lead to a wide range of symptoms as you can see from the table below.

MPS VI affects people differently. Not everyone shows all these symptoms. Some people have obvious symptoms almost from birth. Others may only notice problems over many years. Either way, as GAG builds up in the cells, MPS VI causes serious disabilities over time.

Common Symptoms of MPS VI

  • Enlarged head
  • Short stature
  • Poor endurance
  • Coarse facial features
Eyes and Ears
  • Vision problems
  • Cloudy corneas
  • Glaucoma
  • Optic nerve disease
  • Hearing problems
  • Ear infections
  • Sinus infections
  • Enlarged tongue
  • Abnormal teeth
    Throat and Lungs
    • Breathing problems
    • Sleep apnea
    • Lung infections
    • Valve disease
    • Irregular heartbeat
    • High blood pressure
    • Swollen belly (due to enlarged liver or spleen)
    Bones and Joints
    • Joint stiffness
    • Skeletal changes
    • Hip problems
    Brain and Nerves
    • Spinal cord compression
    • Water on the brain
    • Carpal tunnel syndrome and “trigger finger”
    Note: Unlike other lysosomal storage disorders, MPS VI does not have an effect on a patient’s intelligence.
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    Important Safety Information


    NAGLAZYME® (galsulfase) is indicated for patients with Mucopolysaccharidosis VI (MPS VI; Maroteaux-Lamy Syndrome). NAGLAZYME has been shown to improve walking and stair-climbing capacity.

    Important Safety Information

    Severe and life-threatening allergic reactions can occur during NAGLAZYME (galsulfase) infusions and up to 24 hours after infusion. Typical signs of an allergic reaction include shock, difficulty breathing, wheezing, swelling of the throat, and low blood pressure. If a severe allergic reaction occurs during infusion, the infusion should be stopped immediately and you should receive medical attention. Contact your doctor or get medical help right away if you develop any severe symptoms after infusion.

    In clinical trials, most patients developed antibodies to NAGLAZYME treatment. There was no clear relationship between antibody formation and the safety or effectiveness of NAGLAZYME.

    Serious and severe infusion reactions are associated with NAGLAZYME, including hives, chest pain, rash, abdominal pain, difficulty breathing, swelling, fever, and eye irritation. You should receive medication such as antihistamines before NAGLAZYME infusions to reduce the risk of infusion reactions. If an infusion reaction occurs, the infusion should be slowed or stopped and you may be given additional medication.

    The most common side effects of NAGLAZYME seen in clinical trials were rash, pain, hives, fever, itching, chills, headache, nausea, vomiting, abdominal pain and difficulty breathing. The most common side effects requiring medical attention are infusion-related effects.

    These are not all of the possible side effects with NAGLAZYME. Talk to your doctor if you have any symptoms that bother you or that do not go away.

    NAGLAZYME is a prescription medicine. Before treatment with NAGLAZYME, it is important to discuss your medical history with your doctor. Tell your doctor if you are taking any medication and if you are allergic to any medicines. Your doctor will decide if NAGLAZYME is right for you. If you have questions or would like more information about NAGLAZYME, contact your doctor.

    Spinal cord damage may occur due to the natural MPS VI disease process. Signs of spinal cord injury include back pain, loss of bladder and bowel control, numbness, and paralysis. Contact your doctor immediately if you develop any of these symptoms.

    Call your doctor for medical advice about side effects. You may report side effects to BioMarin Pharmaceutical Inc. at 1-866-906-6100, or FDA at 1-800-FDA-1088 or go to www.fda.gov/medwatch.

    For more information, call BioMarin RareConnections at 1-866-906-6100.

    Please see full Prescribing Information.