Haemophilia

Haemophilia was the first bleeding disorder to be identified and it is also the most famous.  A lot of people know that Queen Victoria carried the gene for Haemophilia and that Alexei Nikolaevich, Tsarevich of Russia, famously had the condition.

As the video (above) explains it has usually been seen as a condition that affects men.  However, these days it is widely recognised that many women who carry the affected gene have low clotting factor levels and can experience bleeding issues. So, women can have Haemophilia too.


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Throughout the world, haemophilia A affects one in 5,000 males, and haemophilia B one in 25,000 males.
If Hampden Park were filled to near capacity with 50,000 men and boys, then on average 10 would have haemophilia A, and 2 would have haemophilia B.

 


Haemophilia A or B occurs when you have a shortage of a clotting factor VIII (8) or IX (9) in your blood, or when the clotting factor doesn’t work properly. This means that whenever a blood vessel is damaged it is difficult for your blood to clot properly – it is slow to make the clot, and when a clot is formed it might not hold together very well.

If you have haemophilia A or B you tend to bleed more often than people without a bleeding disorder. The bleeding might be visible – such as to a cut or a nose or gum bleed – or may be internal, such as painful bleeds inside joints such as ankles and knees (which are the joints that bear your weight), or dangerous bleeds in the head or stomach.

Bleeds are usually treated by an injection of the relevant clotting factor concentrate – Factor VIII or IX. This is best given as soon as possible, and is known as on demand treatment. In addition, people with severe haemophilia A or B treat themselves every 1 to 14 days with clotting factor to help prevent bleeds occurring. This is known as prophylaxis.

It is not possible to tell the difference between haemophilia A and B without doing a blood test, but you need to know which type you have in order to use the right treatment.

The way your haemophilia is managed is very dependent on how severe it is. The amount of clotting factor VIII or IX in your blood will be measured and expressed as a percentage of the amount of an average person without haemophilia (they are said to have 50%-100% clotting factor).

People who don’t have enough Factor XI are sometime described as having Haemophilia C as well.

In this section

Haemophilia A
Haemophilia B
Spot the difference
Inheriting Haemophilia

NB Haemophilia C will be added soon.

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