Alexandra, a Potential Hemophilia Carrier, Was Permitted to Marry a Future Tsar?

[Kalafrana]

The most likely scenario is that Queen Victoria's being a carrier resulted from a genetic mutation. Apparently, about a third of haemophilia cases appear in families with no previous history, so mutations are not all that uncommon.

There has been a suggestion - discussed elsewhere on the Forum - that Queen Victoria was not fathered by the Duke of Kent, but by an unknown haemophiliac. This was first postulated by writers who believed - erroneously - that there was no porphyria among Victoria's descendants, but there was haemophilia. However, if you read Purple Secret by John Rohl et al, it is clear that there was quite a lot of porphyria among Victoria's descendants.

It is also unlikely in any event that a haemophiliac could have fathered Queen Victoria for a number of reasons. First, and most obviously, it is unlikely in those days that a haemophiliac would have lived long enough and in sufficiently good health to father children (the only royal haemophiliac who did was Leopold of Albany). Second, there is no evidence whatever that Victoria's mother had an affair during her husband's lifetime (there were rumours involving her comptroller, Sir John Conroy, but these involve her during her widowhood).

It does seem that the haemophilia gene has now been bred out of the royal families of Europe. As far as I know, the last living royal haemophiliacs were the two haemophiliac sons of Alfonso XIII of Spain, who both died in the 1930s, and Waldemar of Prussia, son of Heinrich of Prussia and Irene of Hesse, who died in 1945.

Hope that answers your questions.

Ann

[sjazama]

Oh yes, there's been quite a bit of speculation on how the gene was introduced into Victoria's family.  Some people like to believe that her mother had and affair with a hemophiliac so Victoria's father was not of royal blood, but most medical specialist say that is highly unlikely.  It isn't likely that anyone afflicted with hemophilia in that day and age would have survived long enough to father a child.  So mutation is the most probable cause.  

Genes frequently spontaneously mutate, no one knows why, and either Victoria or her mother was probably the first carrier.  In other words her gene would have mutated to produce the first hemophilia gene in her family, and was not inherited from a relative.  From then on it was passed on to some of her children, Leopold, Alice and Beatrice.

I've done some research on which of Victoria's known descendants would still be affected and/or carriers (by scanning the WWW).  From what I've read, it has mostly died out.  Only two females in the Spanish royal family "might" still be carriers, even though it has not shown up for there several generations.  That doesn't mean they are not carriers--just that it hasn't shown up.  It could still pop up.  

I sometimes wonder why they aren't tested for it, but I don't think it is always possible to test for the hemophilia gene.  There has to be an afflicted family member to compare against.  I'm no authority, however.

 I don't know who the last person affected was, but it was probably someone in the Spanish royal family.  

[Alixz]

I thought that another thread had mentioned that the gene does not come from males and that males with Hemophilia will have children with normal clotting factors.  So Queen Victoria's mother having an affair with a man who had hemophilia would not matter.

The mutant gene would have to have come into being in a female of the line and as a carrier she would then have children would would either have the disease or carry it.

Victoria's son Leopold had it and at least two of her daughters, Alice and Beatrice, were carriers.


http://www.royalist.info/execute/biog?person=705  link to Royal List On Line

[Kalafrana]

'I thought that another thread had mentioned that the gene does not come from males and that males with Hemophilia will have children with normal clotting factors.'

Not quite. The haemophilia gene is carried on the X chromosome. A haemophiliac man will inevitably pass his defective X chromosome to his daughters, so they will all be carriers. He will pass his healthy Y chromosome to his sons, so they will be unaffected.

Nicely illustrated by Leopold of Albany and his two children. Alice had a haemophiliac son, plus a daughter who seems not to have been a carrier. Charles Edward was healthy.

Regards

Ann

[PAVLOV]

Thank you for the interesting answer Kalafrana. Yes it may have been bred "out", strange though how it just came, destroyed so many families and affected peoples lives, and then just dissapeared.
Of course today having the disease would be no big deal. One would just be put on a heamoglobin drip and the problem would be solved.

Hope it does not pop up again in the furure.

[rosieposie]

Thank you for the info and it has been a very fascinating insight.   I agree on the genetic mutatiion,  sometimes a healthy family tree will produce a person that has genes that can cause a mutation on the way. 
Such as musclar dystrophy,  downs syndrome and of course hemophilia.    There is always going to be xxxx% on weither a new generation will carry on the effective gene.   So with Alexandra and Nicholas they had no way of knowing if their children would have anything wrong.  Because they themselves were extemely healthy.
   
After four healthy girls,  I am sure they would have expected a healthy boy.   

Ann your correct some marriages waver when a child is born with a disability,  (OT: I just read in my crime book of a poor woman who was murdered because her first child with her husband turned out to have downs syndrome and the husband couldn't handle it) Nicholas and Alix were one of the lucky marriages who tried to cope best they could and with the skilled doctors they had on hand to handle the situation.

[Kalafrana]

Haemophilia is not really 'no big deal'. It is vastly easier to cope with nowadays than it used to be, but still imposes restrictions - would you let your haemophiliac son play rugby?

The real tragedy in recent years is that a large proportion of haemophiliacs in Britain and the US were infected with HIV through contaminated blood products in the 1980s, at a time when HIV led almost inevitably to full-blown AIDS. I got involved in the litigation over this when training as a solicitor, and my main role was in working my way through the medical records of haemophiliacs treated in Newcastle upon Tyne. Factor VIII, which is the clotting factor applicable to haemophilia A, had begun to be used in the 1970s and it was very noticeable that the younger haemophiliacs, who had been on Factor VIII and its precursor, cryoprecipitate, all their lives, were not too severely affected by the bleeds, so that they were able to get a normal education and normal jobs (one I remember was a physiotherapist). This was in sharp contrast to the ones in their 30s and older, who had spent long periods in hospital as boys, got very little education and were having hip and knee replacements because of the consequences of bleeds. And then they all got AIDS.

Donor blood for transfusions is now heat-treated to kill the virus, but you can't do that with Factor VIII because it kills the active element.

Ann

[PAVLOV]

Very interesting, I did not realise that it was still so much of a problem in present times, despite medical progress. Yes I am sure that it still causes as much stress for a parent as it did before it became treatable. And of course the transmission of aids and other diseases via any transfusion is always a possibility.

I wonder if the Tsarina recieved any support from her sister and Ena of Spain, or if any correspondence exists between them regarding the illness of their sons.   

[LisaDavidson]

Very interesting, I did not realise that it was still so much of a problem in present times, despite medical progress. Yes I am sure that it still causes as much stress for a parent as it did before it became treatable. And of course the transmission of aids and other diseases via any transfusion is always a possibility.

I wonder if the Tsarina recieved any support from her sister and Ena of Spain, or if any correspondence exists between them regarding the illness of their sons.   

I don't know about Ena, but I believe Alix did get support from her sister.

[PAVLOV]

I sometimes wonder if these deseases are not a result of inbreeding. The Royal families of Europe married 1st and 2nd cousins, which in my opinion is not really "normal"'. They seemed very tightly interbred, perhaps the porphyria and the haemophilia were a result of some weird gene mutation.
Something strange must happen when you marry your cousin, and then your children marry their cousins etc.

I remember reading somewhere that Queen Victoria was quite keen on getting some " new " blood into the family.
So perhaps she had the same idea. The extended family was becoming a bit too interbred, and things were going wrong.
Also there may have been other diseases and deformations that were hidden from the public and family, things we will never know.

[Kalafrana]

Inbreeding is not itself a cause of genetic diseases, but it does make them much more likely to be transmitted.

For example, there is a gene which causes cystic fibrosis. I forget what proportion of the general population has it, but in order for a person to have the disease, both parents must have the gene. If the parents are related, and the gene is in the family, then the chancers are much higher than in the general population that one or more of their children will have the disease.

My maternal grandparents were first cousins and my paternal grandparents were first cousins once removed. No obvious genetic disease in the family, though one of my maternal uncles was partially deaf from birth. My mother and her three brothers were also seriously brainy people. My father and his sister are less colourful individuals, but both very healthy at 83 and 81 respectively (my father has a private pilot's licence, for which he has to pass a stringent medical every year). I am in very rude health, apart from my teeth. My brother had asthma and eczema as an infant, but pretty much grew out of them.

That being said, any more intermarriage in our family would certainly not be a good idea!

Recently, it has been argued that there is an unusually high rate of genetic disorders among Asian Muslims in Britain because of successive cousin marriages.

Ann

[PAVLOV]

Yes very interesting. This could have been the cause perhaps, they have been intermarrying for hundreds of years.. I read recently that there is a very high incidence of Downs Syndrome in European Jews, I think mainly the Ashkenazi Jews, also possibly because of marriage within the same gene pool for more than 1000 years.
As you say, one can only take it so far. Perhaps after a second round within the same family, things may start to go wrong.

[rosieposie]

I am reading a pregnancy guide, and I thought if they had advance medical testiing back then do you think they would have picked up the fact Alexei had hemophilia by doing the genetic testing while he was in the womb??

Just a thought I had when I read the chapter on it.

[Kalafrana]

rosieposie

I'm not sure about that. I believe what they do nowadays when a woman is a known carrier is to go down the IVF route and do what I think is called pre-implantation diagnosis on the embryos to pick out only those which don't have the gene. Haemophilia is one of those medical conditions where UK law allows this.

Obviously that's no help with the cases that arise from spontaneous mutation.

Ann