Quick Facts
What is MPS II (Hunter Syndrome)?
MPS II has also been called Hunter syndrome. It mainly affects boys. The disorder can be recognised by specific facial features, and sometimes the hands become stiff with curled fingers.
Not all people with Hunter syndrome are affected by the disease in exactly the same way, and the rate of symptom progression varies widely. However, Hunter syndrome is progressive and life-limiting.
There is no cure for MPS diseases, but there are ways of managing and treating the problems they cause.
What causes this disease?
Mucopolysaccharides are long chains of sugar molecule used in the building of connective tissues in the body.
- “saccharide” is a general term for a sugar molecule (think of saccharin)
- “poly” means many
- “muco” refers to the thick jelly-like consistency of the molecules
There is a continuous process in the body of replacing used materials and breaking them down for disposal. Children with MPS II are missing an enzyme called iduronate sulfatase, which is essential in cutting up the mucopolysaccharides called dermatan and heparan sulphate. The incomplete broken down mucopolysaccharides remain stored in cells in the body causing progressive damage. Babies may show little sign of the disease, but as more and more cells become damaged, symptoms start to appear.
How common are these diseases?
This is a rare condition affecting 1 in 100,000 to 1 in 150,000 males. There is an estimate in the United States that 1 in 25,000 births will result in some form of MPS.
How is the disease inherited?
This is a rare condition affecting 1 in 100,000 to 1 in 150,000 males. There is an estimate in the United States that 1 in 25,000 births will result in some form of MPS.
Is there a cure for MPS II?
There is no cure but treatments such as enzyme replacement therapies can help make the disease more manageable.
For more information on the treatment, visit http://www.elaprase.com/.
All families of affected children should seek further information from their doctor or from a Genetic Counselor.
What are the treatments for MPS II?
There is no cure but treatments such as enzyme replacement therapies can help make the disease more manageable.
Elaprase™ (idursulfase) is a long-term enzyme replacement therapy for patients with a confirmed diagnosis of MPS II and was approved for use by Health Canada on June 15, 2007. It has also been approved in the U.S., and many countries in Europe. Elaprase was developed and is produced by Shire Pharmaceuticals and is given as a weekly infusion.
For more information, visit http://www.elaprase.com/ or www.shire.com or contact OnePathSM at 866-888-0660. OnePath in the US provides information on product access, treatment centers and education about Elaprase and MPS II.
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