The Unofficial British Royal Family Pages

Last month I discussed a book called Purple Secret:  Genes, �Madness,� and the Royal Houses of Europe by John C.G. Rohl, Martin Warren and David Hunt, about the genetic disease porphryia.  This month I will discuss another book about genetic disease in the royal family, Queen Victoria�s Gene:  Haemophilia and the Royal Family, by D.M. Potts and W.T.W. Potts.   

Hemophilia is a much simpler disease to explain than porphyria.  Hemophilia is the inability of the blood to clot.  Not only can a hemophiliac bleed to death easily from a cut, but any bump can cause fatal internal bleeding.  During the early twentieth century, before seat belts, several hemophiliacs died in minor car accidents.  Hemophilia can be cured now by injections of the substance that causes clotting, which is extracted from donated blood.  In part because of this cure, it is unclear if there are still living hemophiliac descendants of Queen Victoria. 

Hemophilia is located on the X chromosome, so it must be inherited in certain sex-specific ways.  Generally, women carry the gene but do not suffer from the disease, while men have the disease but cannot pass it on to their sons, while their daughters can only be carriers.  Women can only have the disease if they inherit it from both parents, most often through a marriage between cousins.  While Queen Victoria�s descendants do tend to marry each other, apparently no female carrier ever married a male hemophiliac.   

Queen Victoria�s Gene is largely a rehash of facts that are already well known, with some additional scientific evidence and far more biographical material than is necessary for the topic.  Perhaps in an attempt to find something original to say, the authors claim that Queen Victoria was not the Duke of Kent�s daughter, but was fathered by a hypothetical man who carried the hemophilia gene.  It is true that there is no evidence of hemophilia in the royal family (or Victoria�s mother�s family) before Victoria�s son Prince Leopold.  Most likely Victoria�s hemophilia gene was a mutation that occurred at her conception, and there is no evidence whatsoever that her mother might have committed adultery with a hemophiliac.  The authors of Purple Secret attempt to debunk this theory by showing that Victoria inherited the gene for porphyria from the Duke of Kent and passed it on to the royal houses of Europe.  I would not say that I am completely convinced that Victoria inherited porphyria, but I do believe that she was the Duke of Kent�s daughter. 

Queen Victoria had five daughters and four sons.  Only one of her sons, Leopold, was a hemophiliac.  Prince Leopold�s daughter, Princess Alice, Countess of Athlone, was a carrier.  (Her husband, who was Queen Mary�s brother, may have inherited porphyria from George III.)  Their son Viscount Trematon died of hemophilia.  Their daughter Lady May Abel Smith apparently was not a carrier, as the disease has not appeared in her children or grandchildren.  So Leopold�s gene is probably extinct. 

Two of Victoria�s daughters, Alice and Beatrice, were carriers.  Alice�s three-year-old son died of hemophilia after falling out of a ground-floor window.  Two of Alice�s daughters, Irene and Alexandra, were carriers.  Irene married her cousin Prince Henry of Prussia and two of their sons were childless hemophiliacs (though one lived to be 56); they had no daughters.  Alexandra�s son the Czarevitch notoriously was a hemophiliac.  The Czarina turned in desperation to the eccentric monk Rasputin, whose hold over the imperial family caused a scandal that contributed to the overthrow of the monarchy.  The massacre at Ekaterinburg probably eliminated all of the carriers of Alice�s hemophilia gene.