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Life & Events > Disease of the Royals
 

Disease of the Royals


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".
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 either mother or father.
This is due to the fact that, 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.That's because fathers determine the sex of the child, either by passing them another "X" chromosome, in which case the child will be female or by passing them a "Y" chromosome in which case the child will be a boy. Now, the "X" chromosome from the mother will dominate the "Y" chromosome from the father. The mother has two "X" chromosomes and the father has an "X and "Y" chromosome.

If the mother has one defective chromosome and one good chromosome, the odds become 50-50 that the girls will be carriers and the boys will have the disease. Since the hemophilia chromosome is recessive, the "X" chromosome from the father will mask the disease, making the daughter a carrier but not ill. /p>
 
Females who inherit the gene for haemophilia 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. However, it is possible that such cases have gone undocumented because of the potentially higher mortality affected females would likely experience, especially upon onset of menarche.
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.[1]
Victoria appears to have been a spontaneous or de novo mutation, and is considered the source of the disease in all known modern cases of haemophilia among royalty.
 Her mother, Victoria, Duchess of Kent, was not known to have a family history of the disease. Victoria's maternal half-brother, Carl, 3rd Prince of Leiningen, and the descendants of her half-sister, Feodora, Princess of Hohenlohe-Langenberg, are not known to have suffered from the disease. Queen Victoria's father, Prince Edward, Duke of Kent, was not a haemophiliac.
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 eldest 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. However, her son Leopold was a sufferer of haemophilia and her daughters Alice and Beatrice were confirmed carriers of the gene.

 

The royal family's history of hemophilia.

Those who suffered from or carried the disease are enclosed in a box.



Princess Alice: Alice, Victoria's third child, passed it on to at least three of her children: Friedrich, Irene, and Alix.

Prince Friedrich of Hesse and by Rhine (known in the family as "Frittie"). Died before his third birthday of bleeding on the brain resulting from a fall from a window (which might have been fatal even if he had not had hemophilia).


Princess Irene of Hesse and by Rhine (later Princess Heinrich of Prussia), 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, along with his parents and all four of his sisters. Alexei's haemophilia was one of the factors contributing to the collapse of Imperial Russia during the Russian Revolution of 1917.[2]

It is not known whether 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.

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, 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 (his posthumous son being unaffected, as is usual when the gene is carried only by the father):

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, has living descendants none of whom has been known to have or to transmit hemophilia.

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, both have living descendants none of whom has been known to have or to transmit hemophilia.

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 haemophilia is disputed by various sources: It seems unlikely that a known haemophiliac would be allowed to serve in combat.

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.

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 two eldest daughters, Beatrice and Sibilla are both married with children, none of whom, in the case of their sons, appear to be haemophiliacs.

If Sibilla's descendants were to express or transmit the gene, however, another reigning dynasty of Europe would, in the 21st century, join the rest of the reigning families that inherited the disease from Queen Victoria.

Olimpia's youngest daughters are still unmarried, but there is still a chance they could be carriers.

Another daughter, Laura, died as a child, as did her only son, Paul, the latter of whom was apparently not a haemophiliac.

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, born 2005.

Xenia is a male-line descendant of Victoria, but the disease did not come from Xenia's maternal family, the Croÿs.

If the disease came from Xenia, there are two possibilities. The first possibility is that it would have had to be inherited from her father, Kraft, Prince of Hohenlohe-Langenberg, a descendant of Victoria through the female line.

Kraft had some clotting issues, which led the family to believe he may have been a mild haemophiliac. If Kraft was a haemophiliac, then Xenia and her sister Cécile, as the daughters of a haemophiliac, had a 100% chance of being carriers.

The second possibility is that Xenia was a spontaneous mutation, as Victoria herself apparently was.

Xenia is descended from Victoria in two different lines, 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 /a>


posted on Jan 15, 2011 2:01 PM ()

Comments:

I've studied this "line" before. Most interesting.
comment by solitaire on Jan 16, 2011 6:38 AM ()
It is. I too have previously studied it. It is one of the classic examples of how hemophilia manifests itself in the male while the female is the carrier. Because we have such good historical records of the royal family, it is quite easy to follow it down through the various generations of the royals.3
reply by redimpala on Jan 16, 2011 8:39 AM ()
And I wonder what the current royals have and/or are dealing with?
comment by greatmartin on Jan 15, 2011 6:00 PM ()
Well, who knows?
reply by redimpala on Jan 15, 2011 7:06 PM ()
I love reading medical related articles. This one was most interesting.
comment by nittineedles on Jan 15, 2011 5:29 PM ()
Thanks, Marg. I have also been fascinated by diseases, especially ones such as this that are passed down from the mother to her sons.
reply by redimpala on Jan 15, 2011 7:06 PM ()
almost forgot about the hemophilia.Have that got this cured so to speak.
Loads of good information there.Along with febreize.
Thank you for the history on this.I hope that more people read this.

comment by fredo on Jan 15, 2011 3:39 PM ()
Thanks, Fredo. It could yet crop up again in the Royal family.
reply by redimpala on Jan 15, 2011 7:05 PM ()
Victoria is renowned for having infected Royal Europe with the illness - including Czar Nicholas son Alexi (can't spell his name).
Victoria hated pregnancy including labor (although she loved, her children). I think it was when she was delivering her third child, that she decided to try the new fan-dangle 'chloroform' - much against the advice of the medical profession of the time! Once the people found out that she had used it, everyone did (at least those who could afford it).
comment by febreze on Jan 15, 2011 3:30 PM ()
Victoria is not alone in hating pregnancy and the birthing process. I like the reward at the end but the process is awful.
reply by elderjane on Jan 17, 2011 5:42 AM ()
that she did
reply by febreze on Jan 16, 2011 6:09 AM ()
She hated everything about having a child, the pregnancy, breast feeding, which she found repugnant, childbirth. Yet, she had nine children. Obviously, she liked ONE part of making babies.
reply by redimpala on Jan 15, 2011 7:00 PM ()
We don't hear much about it in our daily life, I guess maybe here in America it's not a high priority subject, aside from hearing about people getting AIDS as a result of the blood transfusions required to help with their hemophilia, and haven't heard much about that lately, either.
comment by troutbend on Jan 15, 2011 2:40 PM ()
Methods of testing the blood have greatly improved, so that the chance now of getting AIDS from blood transfusions is next to nil...if not nil. There's a wonderful book entitled Nicholas and Alexandra that I read a few years ago by a man whose son was a hemophiliac in which he tells the story simultaneously of his son and Alexi Nicolai, the son of the Russian tzar. It really makes one realize how people with hemophilia suffer and why Alexandra was desperate to find anything or anyone who could help her son.
reply by redimpala on Jan 15, 2011 7:04 PM ()
Fascinating synopsis!
comment by marta on Jan 15, 2011 2:22 PM ()
Thanks!
reply by redimpala on Jan 15, 2011 2:25 PM ()

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