Haemophilia in European royalty

Haemophilia figured prominently in the history of European royalty in the 19th and 20th centuries. Queen Victoria, through two of her five daughters (Princess Alice of the United Kingdom and Princess Beatrice of the United Kingdom), passed the mutation to various royal houses across the continent, including the royal families of Spain, Germany and Russia. Victoria's son Leopold suffered from the disease. For this reason, haemophilia was once popularly called "the royal disease".

The sex-linked, X chromosome disorder manifests almost entirely in males, although the gene for the disorder is inherited from the mother. However females carrying the faulty X chromosome can pass the chromosome to their descendents. Expression of the disorder is more common in males due to the fact that females have two X chromosomes while the male only has one. If a male's X chromosome is defective, there is not another to mask the disorder. In about 30% of cases, however, there is no family history of the disorder and the condition is the result of a spontaneous gene mutation. [cite web |url=http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=181&contentid=46&rptname=bleeding |title=Hemophilia B |accessdate=2007-11-21 |format= |work=]

Victoria appears to have been a spontaneous or "de novo mutation", and is considered the source of this line of the disease. Her mother, Victoria, was not known to have a family history of the disease. Descendants of Victoria's maternal half-sister, Feodora, are not known to have suffered from the disease. Queen Victoria's father, Edward, was not a haemophiliac and the probability of her mother having had a lover who suffered from haemophilia is minuscule, primarily due to the low life expectancy of 19th century haemophiliacs.

Queen Victoria's daughter Victoria apparently escaped the haemophilia gene as it did not appear in any of her descendants. Victoria's fifth child, Helena may or may not have been a carrier; two healthy sons survived to adulthood but two other sons died in infancy and her two daughters did not have issue. Victoria's sixth child, Louise, died without issue. Her sons King Edward VII, Alfred, and Arthur were not haemophiliacs.

Three of Victoria's children were unlucky. The disease passed through her daughters Alice and Beatrice and to her son Leopold.

Princess Alice

Alice, Victoria's third child, passed it on to at least three of her children:
* Prince Friedrich of Hesse and by Rhine. Died before his third birthday of bleeding on the brain resulting from a fall.
* Princess Irene of Hesse and by Rhine (later Princess Heinrich of Prussia), who passed it on to two of her three sons:
** Prince Waldemar of Prussia. Survived to age 56; had no issue.
** Prince Heinrich of Prussia. Died at age 4.
* Princess Alix of Hesse and by Rhine. Alix had a marriage proposal from Prince Albert Victor, eldest son of the future King Edward VII; had she accepted, haemophilia could have returned to the direct line of succession in Britain. Instead she married Tsar Nicholas II of Russia, and passed it on to her only son:
** Tsarevitch Alexei. Murdered by the Bolsheviks at the age of 13. Alexei's haemophilia was one of the factors contributing to the collapse of Imperial Russia during the Russian Revolution of 1917 [Massey, "Nicholas and Alexandra," 1967] . It is not known if any of Alexei's sisters were carriers, as all four were executed with him before any of them had issue. One, Grand Duchess Maria, is thought by some to have been a symptomatic carrier, because she haemorrhaged during a tonsillectomy [Ian Vorres, "The Last Grand Duchess," 1965 p. 115.] .

Princess Victoria of Hesse and by Rhine (later Victoria, Marchioness of Milford Haven), Alice's oldest child and maternal grandmother to Prince Philip, Duke of Edinburgh, might have inherited the mutation, though the gene remained hidden for several generations before reappearing in the descendants of her eldest granddaughter, Princess Margarita of Greece and Denmark. Princess Elizabeth of Hesse and by Rhine (later Grand Duchess Elizabeth Feodorovna of Russia), may or may not have been a carrier. She was childless when killed by the Bolsheviks in 1918. Princess Marie of Hesse and by Rhine, Alice's seventh and last child, may or may not have been a carrier. She died of diphtheria at the age of four.

Prince Leopold

Leopold, Victoria's eighth child, was a haemophiliac who died from bleeding after a fall. He lived to the age of 30, long enough to pass the gene on to his only daughter:
* Princess Alice of Albany (later Countess of Athlone), who in turn passed it on to her oldest son:
** Prince Rupert of Teck (died at the age of 20, bleeding to death after a car accident)

Alice of Albany's youngest son Prince Maurice of Teck, died in infancy, so it is not known if he was a sufferer. Her daughter Lady May Abel Smith, Leopold's granddaughter, apparently was not a carrier, as the disease has not appeared in her descendants.

Princess Beatrice

Princess Beatrice (later Princess Henry of Battenberg), Victoria's ninth and last child, passed it on to at least two, if not three, of her children:
* Princess Victoria Eugenie of Battenberg (later Queen Victoria Eugenia of Spain), who passed it on to
** Infante Alfonso of Spain, Prince of Asturias. Died at age 31, bleeding to death after a car accident.
**Infante Gonzalo. Died at age 19, bleeding to death after a car accident.
**Victoria Eugenie's two daughters, Infantas Beatriz and Maria Cristina of Spain, apparently were not carriers, as none of their descendants have had the disease.
*Prince Leopold of Battenberg. Later Lord Leopold Mountbatten. Died at age 32 during a knee operation.
*Prince Maurice of Battenberg. Killed in action in World War I in 1914 at the age of 23. Maurice's haemophila is disputed by various sources. It seems unlikely that a known haemophiliac would be allowed to serve in combat.

Today

As of today, hemophilia is extinct in the reigning royal houses of Europe. The last royal descendant of Victoria known to suffer from the disease was Infante Gonzalo (born 1914). Many sons have been born to European royalty since and none is known to have had hemophilia. However, since the hemophilia gene remains hidden in females with only one bad gene, and female descendants of Victoria exist in several royal houses today, there remains a small chance that the disease could appear again, especially among the female-line Spanish descendants of Princess Beatrice.

*Infanta Beatríz's two sons were not affected by the disease. Beatriz's eldest daughter, Sandra, has two children, a son and daughter. Her son is not affected, and her daughter has two sons, who are apparently unaffected. Beatríz's youngest daughter, Olimpia, had six children; her only son, Paul, died shortly after his fourth birthday. He could have been a possible sufferer. Another daughter, Laura, also died as a child. Her two eldest daughters, Beatrice and Sibilla are both married with children, none of whom, in the case of their sons, appear to be haemophiliacs. Olimpia's youngest daughters are still unmarried, but there is still a chance they could be carriers.

*Infanta Maria Cristina had four daughters, all potential carriers. Her eldest daughter, Vittoria Eugenie, had a daughter and three sons, the latter all apparently unaffected. The Infanta's second daughter, Giovanna, had only one child, an unaffected son. Her two youngest daughters, Donna Maria Teresa and Donna Anna Sandra, also have only daughters. Of these, only one, Maria Teresa's second daughter, Isabel, is married, but she also has only a daughter. There is a chance the disease may remain in this branch of Princess Beatrice's descendants.

Ferdinand Soltmann

At least one modern descendant of Queen Victoria has been diagnosed with haemophilia: Ferdinand Soltmann, the son of Princess Xenia of Hohenlohe-Langenberg. Xenia is a male-line descendant of Victoria, but the disease did not come from Xenia's maternal family, the Croÿs. Xenia's father, Kraft, Prince of Hohenlohe-Langenberg, had some clotting issues, which led the family to believe he may have been a mild haemophiliac. [ [http://www.royalsportal.de/forum/index.php?&showtopic=29531 Baby Girl Soltmann (Desc. Hohenlohe-Langenburg)] ] As the daughter of a haemophiliac, Xenia had a 100% chance of being a carrier.

Xenia is descended from Victoria twice paternally, through Victoria's children, Princess Alice of the United Kingdom and Alfred, Duke of Saxe-Coburg and Gotha. Alfred was not a haemophiliac, but Alice was a carrier, and her line of descent to Xenia, and subsequently, Ferdinand, is as follows.

Notes

References and external links

*Potts, D. M. "Queen Victoria's Gene". Sutton Publishing, 1999. ISBN 0-7509-1199-9.
* [http://www.sciencecases.org/hemo/hemo.asp "Hemophilia: The Royal Disease"]
* [http://ftp.cac.psu.edu/~saw/royal/r01.html#I1 Family tree] of Queen Victoria and her descendants
*Another [http://www.btinternet.com/~allan_raymond/QV_Descendants_Haemophilia.htm family tree]