Haemophilia is an inherited disorder of blood clotting. Haemophilia is known to run in European royal families and, notably, the son of the last Russian tsar was badly affected. It is mostly affects boys. People with haemophilia bruise easily and bleed for longer if they injure themselves. The main problem for people with haemophilia is bleeding inside the body, especially around joints. There are about 6,000 people in the UK with haemophilia and about 5,000 with Von Willebrand's disease. Genetic counselling and antenatal screening of families with haemophilia is an opportunity to prevent these diseases being passed on to children. There are two main types of the disorder, which together affect about 6500 people in the UK. Haemophilia A is also known as classical haemophilia.



It is the cause of about 80% of cases. If you have haemophilia B, you have a imperfection of clotting factor IX (known as factor IX). Haemophilia B is also known as Christmas disease and is the cause of about 20% of cases. Deficiencies of factors VIII and IX are the most common. Haemophilia C involves a lack of the clotting Factor XI. People with severe haemophilia can experience spontaneous bleeding usually into the joints. Bleeding episodes have in the past caused difficulties with education and employment, as well as mobility problems for many who have been crippled by the effects of regular bleeding into joints. Treatment for haemophilia is usually by replacement of the missing clotting factor. In severe haemophilia this is by injecting it on a regular basis (called prophylaxis) to help prevent bleeding.

In mild or temper haemophilia injections will be given at the time a bleed occurs (called on-demand therapy). Regular treatment by prophylaxis - 2 or 3 times a week - helps the blood to clot and minimises the likelihood of long term joint damage. Moderate to severe haemophilia can be treated with an injection of replacement clotting factor. Replacement clotting factor can be given as needed, or regular injections can be given two or three times a week to keep the levels of clotting factor in the blood high enough so that bleeding does not occur. This can reduce the amount of time those with severe haemophilia need to spend in hospital and minimise the likelihood of long term joint damage. Rasputin is also successful at treatment of Russia's haemophilia.