Haemophilia is a genetic blood disorder that impairs the body's ability to control blood clotting or coagulation. It occurs when abnormal clotting factors are inherited, making them unable to function normally. Clotting factors are useful in stopping bleeding from an injury and to prevent spontaneous bleeding.
The disease affects mostly men and women are usually only carriers of the disease. This is because the genes responsible for hemophilia are carried by chromosome X. In women, if one of the two chromosomes is defective, the other usually is able to take information. If there is a defect within the X chromosome responsible for blood proteins (factor VIII or IX), then a male child is born with hemophilia. The severity of the disease depends on the level of each factor in the blood. Based on these levels, haemophilia is classified as: mild, moderate (average) or severe.
There are two more common forms of Haemophilia (but be aware, there are other types of hemophilia, depending on the absence of other blood factors: X, XI, XII, etc.):
Haemophilia A is caused by a deficiency of clotting factor VIII activated. Approximately 80% of the hemophiliacs have haemophilia A, and most of these cases are severe. About 1 in 5,000 boys is born with hemophilia A.
Hemophilia B is caused by a lack of clotting factor IX. It occurs rarely, only in 1 of 30,000 boys. It is also known as Christmas disease after Stephen Christmas, the first patient described to have the disease in 1952.
The risk of hemophilia in a child depends on the parent’s genetic material. Sometimes a child is born with hemophilia because of mutations that occur in the chromosomes of the egg or sperm from one or both parents, but in some cases mutations appear. Researchers do not know the reasons why these mutations occur in some people.
Britain's Queen Victoria (24 May 1819 – 22 January 1901) passed the disease through her heirs (it is believed only 2 of her daughters passed the mutation: Princess Alice and Princess Beatrice) into royal houses across the continent. It is widely believed Queen Victoria carried a spontaneous mutation.
Queen Victoria had the longest reign of a British monarch. In Victorian times the British Empire’s development was impressive: it reached the height of the industrial revolution, and underwent through major social, economic and technological changes, becoming one of the great powers of Europe. The Queen tried to establish international domination and influence through advantageous marriages to other European royal house, but sadly she also passed a deadly disease:
The most famous case is that of Tsarevitch Alexei of Russia. One of Queen Victoria’s granddaughters married Nicholas II of Russia. She became Empress Alexandra Feodorovna. In the first 10 years of marriage, she gave birth to four girls: Olga, Tatiana, Marie and Anastasia. It is not known if they were carriers of the disease. In 1904, the long-awaited heir was born: Tsarevitch Alexei, but unfortunately he was a suffering from Hemophilia B. Feeling guilty for passing the painful disease to their child, Alexandra found her last hope in Rasputin: the monk that played a spectacular role in the fall Romanov dynasty. Rasputin claimed to be able to heal Alexei and the Queen trusted him to such an extent that everyone began to think that not only the Queen was having an affair with him, but that he also he also had an unnatural relationship with her daughters. Alexei was murdered alongside his family and three retainers during the Russian Civil War.
Victoria Eugenia, granddaughter of Queen Victoria married King Alfonso XIII of Spain. Since she was carrying the disease, she brought the haemophilia gene into the Spanish royal family. Two sons, Alfonso, heir to the throne, and Gonzalo have suffered from the disease. The disease weakened the influence that the monarchy had in Spain. Stories began circulating, according to which every day a young soldier was killed for fresh blood to keep the princes alive. Historians argue that the gene Eugenia Victoria passed helped discredit the royal family and was one of the factors that led to the Spanish Civil War.
Prince Leopold, Duke of Albany was Queen Victoria’s eight child. He had haemophilia, and the disease led to his death at age 30 due to a fall in which he injured his knee. He had trouble in finding a wife due to his haemophilia, but at his other suggestion he met and married Princess Helena of Waldeck and Pyrmont. It was a happy marriage and by the time he died, his wife was pregnant with their second child.