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Haemophilia figured prominently in the history of European royalty in the 19th and 20th centuries. Britain's Queen Victoria, through two of her five daughters (Princess Alice and Princess Beatrice), passed the mutation to various royal houses across the continent, including the royal families of Spain, Germany and Russia. Victoria's son Prince Leopold, Duke of Albany suffered from the disease. For this reason, haemophilia was once popularly called "the royal disease". Tests of the remains of the Romanov imperial family show that the specific form of haemophilia passed down by Queen Victoria was probably the relatively rare Haemophilia B.
Contents
- Queen Victoria family tree
- Princess Alice
- Prince Leopold
- Princess Beatrice
- Today
- Chronological order
- Type of haemophilia discovered
- References
The sex-linked X chromosome disorder manifests almost entirely in males, although the gene for the disorder is located on the X chromosome and may be inherited from the mother for male children or from either mother or father for female children. Expression of the disorder is much more common in males than in females. This is because, although the trait is recessive, males only inherit one X chromosome, from their mothers. Thus if the haemophilia gene is transmitted on it, there is no possibility for the male to inherit a haemophilia-free gene from his father to mask or dilute the symptoms. By contrast, a female who inherits a gene for haemophilia on one of her X chromosomes will also have inherited a second X chromosome from the other parent which is likely to carry a haemophilia-free gene that would prevent full expression of symptoms.
Females who inherit the gene for Haemophilia A or B from both parents would be expected to manifest full symptoms, similar to those seen in affected males, but this is extremely rare. Despite frequent inter-marriage among royalty, no case of such double inheritance is known among Queen Victoria's descendants. This is largely because only one of the individuals with Hemophaelia had any children.
Although an individual's haemophilia can usually be traced in the ancestry, in about 30% of cases there is no family history of the disorder and the condition is speculated to be the result of spontaneous mutation in an ancestor.
Victoria appears to have been a spontaneous or de novo mutation and is usually considered the source of the disease in modern cases of haemophilia among royalty. Queen Victoria's father, Prince Edward, Duke of Kent, was not a haemophiliac, and the probability of her mother having had a lover who suffered from haemophilia is minuscule given the low life expectancy of 19th-century haemophiliacs. Her mother, Victoria, Duchess of Kent, was not known to have a family history of the disease, although it is possible that the mutation began at her conception and was passed down only to Victoria and not to her two other children. In the same way, had Queen Victoria herself only had seven children, the mutation would probably be assumed today to have occurred at the conception of Princess Alice, as she was the only known carrier among Victoria and Albert's first seven children.
Queen Victoria's eldest daughter, Victoria, Princess Royal, apparently escaped the haemophilia gene as it did not appear in any of her matrilineal 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 Edward, Alfred, and Arthur were not haemophiliacs. However, her daughters Alice and Beatrice were confirmed carriers of the gene, and Victoria's son Leopold was a sufferer of haemophilia, making his daughter Princess Alice, Countess of Athlone a carrier as well.
Queen Victoria family tree
Children
Victoria, German Empress (1840 – 1901) Issue: Wilhelm II of Germany, Charlotte, Duchess of Saxe-Meiningen, Prince Henry of Prussia, Prince Sigismund of Prussia, Viktoria, Princess Adolf of Schaumburg-Lippe, Prince Waldemar of Prussia, Sophia, Queen of the Hellenes,Margaret, Landgravine of Hesse-Kassel
Edward VII (1841 – 1910) Issue: Prince Albert Victor, Duke of Clarence and Avondale, George V, Louise, Princess Royal, Princess Victoria, Maud, Queen of Norway
Princess Alice, Grand Duchess of Hesse and by Rhine (1843 – 1878) Issue: Victoria Mountbatten, Marchioness of Milford Haven, Grand Duchess Elizabeth Feodorovna of Russia, Irene, Princess Henry of Prussia, Ernest Louis, Grand Duke of Hesse and by Rhine, Prince Friedrich, Alexandra Feodorovna, Empress of Russia, Princess Marie of Hesse and by Rhine
Prince Alfred, Duke of Saxe-Coburg and Gotha (1844 – 1900) Issue: Alfred, Hereditary Prince of Saxe-Coburg and Gotha, Marie, Queen of Romania, Victoria Melita, Grand Duchess of Russia, Alexandra, Princess of Hohenlohe-Langenburg, Princess Beatrice, Duchess of Galliera
Helena, Princess Christian of Connaught and Strathearn (1846 – 1923) Issue: Prince Christian Victor, Albert, Duke of Schleswig-Holstein, Princess Helena Victoria, Princess Marie Louise, Prince Harald
Princess Louise, Duchess of Argyll (1848 – 1939) No issue
Prince Arthur, Duke of Connaught and Strathearn (1850 – 1942) Issue: Margaret, Crown Princess of Sweden, Prince Arthur of Connaught, Princess Patricia, Lady Ramsay
Prince Leopold, Duke of Albany (1853 – 1884) Issue: Princess Alice, Countess of Athlone,Charles Edward, Duke of Saxe-Coburg and Gotha
Beatrice, Princess Henry of Battenberg (1857 – 1944) Issue: Alexander Mountbatten, 1st Marquess of Carisbrooke, Victoria Eugenie, Queen of Spain, Lord Leopold Mountbatten,Prince Maurice of Battenberg
Princess Alice
Alice (1843-1878), Victoria's third child, and wife of the future Grand Duke Louis IV of Hesse and by Rhine (1837-1892), passed it on to at least three of her children: Irene, Friedrich, and Alix.
Prince Leopold
Leopold (1853-1884), Victoria's eighth child, was the first member of the family to manifest haemophilia; he died at age 30 from bleeding after a minor fall, only two years after marrying Princess Helena of Waldeck and Pyrmont. (1861-1922)
He passed the gene on to his only daughter, as all of the daughters of a haemophiliac father would inherit the gene:
Alice's younger son Prince Maurice of Teck died in infancy, so it is not known if he was a sufferer. Her daughter Lady May Abel Smith (1906-1994), Leopold's granddaughter, has living descendants none of whom has been known to have or to transmit haemophilia.
Leopold's posthumous son, Charles Edward (1884-1954), was unaffected, as fathers cannot pass the gene to a son.
Princess Beatrice
Princess Beatrice (1857-1944), Victoria's ninth and last child, and wife of Prince Henry of Battenberg (1858-1896) passed it on to at least two, if not three, of her four children:
Today
No living member of the present or past reigning dynasties of Europe is known to have symptoms of haemophilia or is believed to carry the gene for it. The last descendant of Victoria known to suffer from the disease was Infante Don Gonzalo, born in 1914, although dozens of descendants of Queen Victoria's (including males descended only through females) have been born since 1914. However, because the haemophilia gene usually remains hidden in females who only inherit the gene from one parent, and female descendants of Victoria have left many descendants in royal and noble families, there remains a small chance that the disease could appear again, especially among the female-line Spanish descendants of Princess Beatrice.
Chronological order
Queen Victoria died in 1901 so she lived to see her youngest son and a grandson die from the disease. A great-grandson was diagnosed with the disease as well. The gene can be passed down the female line without a haemophiliac son being born, but as the family line continues and no haemophiliac sons are born, it becomes less likely that a certain ancestor had the gene and passed it on through the female line.
Type of haemophilia discovered
Because the last known descendant of Queen Victoria with haemophilia died in the 1940s, the exact type of haemophilia found in this family remained unknown until 2009. Using genetic analysis of the remains of the assassinated Romanov dynasty, and specifically Tsarevich Alexei, Rogaev et al. were able to determine that the "Royal Disease" is actually haemophilia B. Specifically, they found a single-nucleotide change in the gene for clotting Factor IX that causes incorrect RNA splicing and produces a truncated, nonfunctional protein.