Characteristics of MPS II

Although babies with MPS II usually appear normal at birth, over time characteristic symptoms begin to appear such as:

• Coarse face and hair
• Loss of skills such as speech and learning
• Sight and hearing difficulties
• Frequent respiratory infections
• Behavioural problems
• Mental retardation in the severe forms
• Bone deformities and joint stiffness
• Heart disease
• Enlarged organs such as liver and spleen

All individuals with MPS II tend to look very alike and when several are together they can look like carbon copies of each other. Their faces are chubby with rosy cheeks and their heads rather large with prominent foreheads. The neck is short and the nose is broad with a flattened bridge. The lips are often thickened and the tongue enlarged. The hair tends to be thick and luxuriant, the eyebrows bushy and there may be more hair than usual on the body. Individuals have prominent bellies due to an enlarged liver and spleen and a characteristic way of walking and holding their arms.

Hunter boys are very much loved by those who know them.  However, there will be times when parents will not feel so indulgent towards their son and his antics. For a few years young Hunter boys may be overactive, strong, usually cheerful but hard work to look after. Some have limited powers of understanding and their mental age will be lower than their physical capabilities; they could for example bolt the bathroom door but be unable to understand how to get it open again even when told. They enjoy rough and tumble play, making a lot of noise and throwing toys rather than playing with them.

The change from the overactive noisy period is likely to be gradual. Parents will realise that their son no longer runs everywhere and is happier sitting than standing. Many will be easily pleased, perhaps by looking through the same little book of photographs or by having stories read.

Individuals with mild Hunter syndrome should be encouraged to be as independent as possible since many Hunter adults can lead full and enjoyable lives. Some mildly affected Hunter adults have completed tertiary studies and found satisfying work. A few adults with Hunter disease have married and had children. The children would not have the disease unless the mother happened to be a carrier. Daughters of a Hunter father will all be carriers of the disorder.

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