Christmas Disease

Unfortunately not a disease in which you receive socks, get cross and eat too much but a type of haemophilia. Haemophilia is an X linked disorder that causes an inability for the blood to clot. Because it is X linked males are more likely to have the disease needing only a mother that has the gene. For a female to have haemophilia a mother and father who have the gene would be needed. Haemophilia was especially prominent in European Royalty, which is why it’s known as the “royals disease”. Queen Victoria passed on the gene to her son Leopold and then to her daughters who spread the disease across the various royal families of Europe and Russia.

 Haemophilia stops clotting by lower the levels of clotting factors in the blood plasma. As a result when the vessel is cut a clot does not form and the wound may bleed for day or even weeks even for a small injury. If bleeding occurs inside the joints or in the brain it can easily be fatal. The condition can be solved by transfusing blood containing the clotting factor i.e. from a healthy individual to some one with haemophilia. Unfortunately in the day before propter testing this led people with haemophilia open to pathogens in the blood such as hepatitis and HIV. In 1994 researches discovered that if you exchanged blood between certain but not all people with Haemophilia both could clot. In 1952 British researches discovered that this was because there were two different types of the disease, each resulting in the loss of one clotting factor. The most common type of the disease they called Haemophilia A (roughly 80% of cases) and the less common type, which was first, found in a ten year old boy called Stephen Christmas, Haemophilia B or Christmas Disease. In a joke too far the disease was also first published in the Christmas edition of the British medical Journal.