• 1: Home
• 2: MPS Diseases
  • 2.1: Overview of symptoms
  • 2.2: Overview of treatment
  • 2.3: Which are the MPS Diseases
    • 2.3.1: MPS I
    • 2.3.2: MPS II
    • 2.3.3: MPS III
    • 2.3.4: MPS IVA
    • 2.3.5: MPS VI
    • 2.3.6: MPS VII
    • 2.3.7: ML I
    • 2.3.8: ML II and III
    • 2.3.9: ML IV
    • 2.3.10: Fucosidosis
    • 2.3.11: Mannosidosis
    • 2.3.12: Sialic Acid Storage Disease
    • 2.3.13: MSD
    • 2.3.14: AGU
    • 2.3.15: Winchester
  • 2.4: Personal stories
• 3: Fabry Disease
• 4: Advocacy Support
• 6: Fundraising
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• 8: Research
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• 30: Terms and Conditions

MPS II Hunter Disease

Hunter disease is one of the mucopolysaccharide diseases and is also known as MPS Type II. Hunter disease takes its name from Charles Hunter, the professor of Medicine in Manitoba, Canada, who first described two brothers with the disorder in 1917.

It is devastating news for parents to learn that their child has Hunter disease and some time may pass before it is clear how severely affected the child will be. It is important to remember that the disease is very varied in its effects and that your child will not necessarily have all the symptoms described.

What causes MPS II?

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 sulphate sulphatase which is essential in cutting up the mucopolysaccharides called dermatan and heparan sulphate. The incompletely 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 is MPS II?

The MPS Society which co-ordinates the Registry for Mucopolysaccharide and Related Diseases has shown that this is a rare condition affecting one in 150,000 live births. Over a ten year period 39 babies with Hunter Disease were born in the United Kingdom.

How is MPS II inherited?

Hunter disease has a different form of inheritance from all the other MPS diseases as it is 'sex linked' like haemophilia. Girls may be carriers of the disease but, except in very rare cases, only boys will be sufferers. The few girls who have been found to have the disease have an associated chromosomal abnormality.

All families of children with Hunter disease should seek further information from their doctor or from a genetic counsellor before planning to have more children. If the mother is a carrier there is a 1 in 2 risk that any boy born to her will have the disease and that any girl will be a carrier.

The sisters and maternal aunts of an individual with MPS II may be carriers of the disease who would also have a 1 in 2 chance of passing the disease to a son.

In many families it is possible to detect female carriers by direct analysis of the genetic material. The doctor may wish to take a sample of blood from your affected child so that the exact genetic abnormality can be detected.

All families of affected children should seek further information from their doctor or from a genetic counsellor before planning to have more children. There is a more detailed explanation of this complex subject in a booklet available from the MPS Society on the Pattern of Inheritance in MPS diseases. Please visit our Information Resource section.

Prenatal diagnosis

If you already have a child with Hunter disease, if you know you are a carrier, or if you have not been carrier tested but are related on the mother's side to a boy with Hunter's Disease, it will be possible to have tests during a subsequent pregnancy to find out whether the baby you are carrying is affected. It is important to contact your doctor as soon as you suspect that you may be pregnant if you wish tests to be arranged. If you have any difficulty please contact the Society without delay.

Further information

There is a detailed booklet on MPS II produced by the MPS Society in collaboration with parents and doctors drawing on their experience and with reference to medical literature. The booklet includes detailed information on the presentation and clinical management of the disease and treatment options available. Please visit our Information Resource section.