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Haemophilia

Haemophilia is a bleeding disorder. The blood of a person with haemophilia does not clot normally because they lack one or more of the plasma proteins needed to form a clot, and stop the bleeding.

A person with haemophilia does not bleed faster than other people; however, bleeding will last for longer and in many cases will not stop without adequate treatment with factor replacement therapy.

The most common type of haemophilia is factor VIII deficiency, known as haemophilia A. Recent studies indicate that around 87% of haemophilia cases globally are haemophilia A*. The second most common type is factor IX deficiency or haemophilia B (sometimes known as Christmas Disease).

Both haemophilia A and B are very rare disorders. Estimates indicate that approximately one in 10,000 males born in New Zealand has haemophilia. Recent studies suggest that over 430 New Zealand residents have haemophilia.

Small cuts on the skin are usually easily treated with first aid, but bleeding deeper into muscles or joints can cause major pain and permanent damage. Although superficial bruising can look dramatic, the much bigger problems are often unseen. Internal bleeding causes the most damage and can lead to complications for a person with haemophilia. Some bleeding episodes occur as a result of injury (trauma), but many occur seemingly without cause. If untreated, bleeds can be life threatening, and immediate treatment is often necessary for bleeds in the head, throat, gut, or iliopsoas (groin). Thankfully, most bleeds can now be managed and treated effectively.

Although physical fitness and activity is encouraged to build muscles and prevent bleeding, in order to minimise the risk of injury, people with haemophilia are advised to avoid contact sports and activities with a potential element of contact injury. Advice on individual activities varies according to the severity of haemophilia. 

*World Federation of Hemophilia Global Survey 2004

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