What is haemophilia?

Haemophilia describes a group of bleeding disorders. They are quite rare and cause problems with blood clotting. In other words, someone with haemophilia may continue to bleed for longer than normal if they are injured, or start bleeding for another reason.

What stops blood from clotting?

When most people cut themselves, a complex natural reaction starts in the body to heal the wound. Within seconds of the injury, cells in the blood (known as platelets) clump together around the wound.

These platelets, along with blood proteins, calcium and other clotting factors react together and form what is known as a clot. This acts like a plug over the wound preventing further bleeding.Over the next several days to weeks, the clot strengthens and then dissolves when the wound is healed. 

Adapted from US National Hemophilia Foundation website.

In people with haemophilia, clotting factors are missing or don’t work as they should. This causes them to bleed for a longer time than those whose clotting factor levels are normal. Bleeding disorders can also be caused by platelet deficiency or dysfunction

How common is haemophilia?

Haemophilia generally only affects males. Although severe haemophilia is rare in females, women can still have mild symptoms or just be carriers of the gene responsible for the condition.

The most common type of haemophilia is known as Haemophilia A and affects around 1 in 5000 males worldwide. It occurs when there is not enough factor VIII (8) for normal clotting.

The second most common type of haemophilia is known as Haemophilia B and affects around 1 in 30,000 males worldwide. It occurs when there is not enough factor IX (9) for normal clotting.

Although you or your loved one has a rare condition, you are not alone. In Australia, there are currently around 2,600 people living with haemophilia.

Most people with haemophilia are diagnosed with the condition when they are babies or in childhood. However, in some cases of mild haemophilia, diagnosis may not occur until later in life.

Haemophilia can be mild, moderate or severe

Haemophilia is classified as mild, moderate or severe depending on how much clotting factor the body makes. People with mild haemophilia usually bleed excessively only after injury or surgery. In contrast, those with severe haemophilia may have around 20 to 30 episodes of excessive bleeding after minor trauma or spontaneous bleeding (i.e. not caused by an injury of some sort) every year if left untreated. Bleeding is particularly common in the joints and muscles.

Adapted from White et al, 2001.

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