These words are from a letter written by Yevgeny Botkin, the family doctor of the Russian Tsar Nicholas II and his wife Alexandra (Steinberg and Khrustalëv, 1995). The suffering boy is Alexei, the son of the couple, born in 1904 and the successor to the throne of all Russians. He suffered from a congenital bleeding disease known as haemophilia, in which the defect is a missing or malfunctioning blood-clotting factor. The particular haemophilia that affected Alexei is a genetic disease linked to the X chromosome, and for this reason it is more prevalent in males. Females rarely show symptoms of the disease but may carry the gene on to the next generation.

Royal disease

Alexei inherited his disease gene from his great grandmother, Queen Victoria of England (Fig. 12.1). In fact, the queen transmitted her gene to a number of members of European royalty and thus decimated the thrones of Britain, Germany, Russia and Spain. Typically the affected males are exceptionally sensitive to injuries – for example, both Infante Gonzalo of Spain and Prince Rupert of Teck died young as a result of bleeding following car accidents. The last carrier of the disease in the royal family was Prince Waldemar of Prussia, who died in 1945.