Author Topic: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?  (Read 164637 times)

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Offline Janet_W.

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I'm wondering how well Alix understood the basics of hemophillia--its genetic probability in particular--and especially before she married?  Uncle Leopold had been a hemophilliac, Aunt Beatrice was a carrier, and little brother "Frittie" had also been so afflicted, but did Alix and her sisters understand anything about it?

And by the time Alix and Nicky had established their own family and the girls were becoming marriagable, do you suppose that OTMA understood that they, too, might be carriers?

Offline bookworm8571

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #1 on: April 22, 2004, 11:02:07 PM »
I doubt they understood exactly how it is inherited. She certainly knew enough to understand that the disease came from her. It was in her family, not Nicholas's. The guilt must have been terrible since she obviously loved her children and suffered with Alexei.

By the time Alexei was born, there were numerous other hemophiliacs in the family that she'd have had direct knowledge of, unless it was hushed up somehow. Her sister Irene had two hemophiliac sons. Her Aunt Beatrice had at least one and possibly two hemophiliac sons. I remember reading that when Beatrice's daughter Ena became engaged to the Spanish heir, the relationship was discouraged because they knew about the hemophilia in her family. Doctors of the time apparently knew something about the inheritance of hemophilia, even if the Royal Family was foggy on the details.

I think either Alix's sister Victoria or her aunt Victoria, the Empress Frederick (they were both bright women) was concerned about their brother Ernest marrying his first cousin Victoria Melita because of fear of the hemophilia. From that, I gather that they understood it ran in the family but didn't know exactly how it was passed down.

Cousin Princess Alice of Athlone, daughter of the hemophiliac Leopold, was younger than Alix. Her children would have been born a few years after Alexei, but Alix would probably have known that her two sons were hemophiliacs. But I don't remember reading anything that suggested concern over Princess Alice passing that disease down to her children, even though we know today that the daughter of a hemophiliac will ALWAYS be a carrier.

One thing that has intrigued me is that a female carrier of hemophilia is HERSELF abnormal. She produces a lower than normal blood clotting factor because she carries one defective gene. That often means that she might bleed more than is normal after an injury, have unusually heavy menses, maybe heal slower after a wound, etc. Alix had a lot of health problems in her later years. Is there a chance that her being a hemophilia carrier had something to do with it?

Very sad, in any event. Chances are good that two or more of the Grand Duchesses were hemophilia carriers. Of Alice's five daughters, Irene and Alix were both carriers and it's unclear whether Ella or May were.

Offline nerdycool

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #2 on: April 23, 2004, 12:06:44 AM »
After reading the above posts, one question came to me and I was hoping someone would know, because I certainly don't know much about testing and the such. Anyway, I am wondering if there is any way to test remains for hemolphelia. Would you need real blood, or would it be sufficient to use bone marrow, or whatever sample would be availabe on old bones? If it were possible, I think it would be interesting to find out if any of the grand duchesses carried the gene. I'm not saying that they should be dug back up to find out... just that it would be interesting.

Offline Sarai

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #3 on: April 23, 2004, 08:17:00 AM »
Quote
One thing that has intrigued me is that a female carrier of hemophilia is HERSELF abnormal. She produces a lower than normal blood clotting factor because she carries one defective gene. That often means that she might bleed more than is normal after an injury, have unusually heavy menses, maybe heal slower after a wound, etc.


This reminds me of something that I read, I seem to recall it was in Vorres biography of GD Olga A., although I am not exactly sure. It was of an incident where the GD Maria was having her tonsils extracted and she was bleeding a lot more than was expected. Upon seeing this, however, Alix was calm and insisted that the doctor continue what he was doing. What stuck out about this passage was the fact that Maria was bleeding an unusual amount, which may or may not have been due to the possibility that she was a carrier.

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #4 on: April 23, 2004, 01:06:00 PM »
Great information! Helps put things in better perspective. And I, too, remember reading that passage in Olga A's biography re: Marie bleeding profusely.  By that time Alix had no doubt learned to become very, very stoic in a crisis . . . though afterwards she understandably displayed symptoms of what we would call delayed stress syndrome.

Offline bookworm8571

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #5 on: April 24, 2004, 01:47:49 PM »
I had never read that story about Marie's excessive bleeding following surgery. Did the book make the connection between her bleeding and the possibility of her being a hemophilia carrier?

It's rather a morbid subject, but it would be interesting to know which of the four girls were carriers. The "what ifs" of history are fascinating. What if Nicholas had married someone who wasn't a hemophila carrier? What if his daughter Olga had married Carol of Romania and produced a hemophiliac son, or if Tatiana had married the heir to the British throne and done likewise?

Offline Lisa

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #6 on: April 24, 2004, 03:24:15 PM »
Hi!
"I had never read that story about Marie's excessive bleeding following surgery. Did the book make the connection between her bleeding and the possibility of her being a hemophilia carrier?  "

My mother's cousine have a hemophiliac son ,but she have a rate of blood-platelet too hight.As you can see,there no connection between a "mother-carrier" and a "son-ill". The only way to know if the disease is in a family,is to see if there is some hemophiliacs,or to refer a whole family (aunts,cousines,sisters,etc,etc)for tests.not very easy... :-/

I think that the "hemophiliac problem" in the Romanov family (and in the queen Victoria's family) will always be an unsoluble problem or at least an unlimited source of discussions... ;D

Offline bookworm8571

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #7 on: April 24, 2004, 03:28:09 PM »
I think it can vary depending on the woman. SOME women who are hemophilia carriers have some of the same symptoms as a hemophiliac. Other carriers have no such symptoms at all. I think there are also different types of hemophilia and similar blood disorders that women can have as well as men.

Offline Lisa

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #8 on: April 25, 2004, 04:31:54 AM »
Excuse me to insist. It's true that there are several types of hemophilia, more or less serious. I know something of it, since there is in my family (I 'm perhaps myself carrying).My father did his medicine thesis on the repercussions of the spontaneous saignements in the inferior members with the hemophiles.En reading it, I learned a lot of things:The women, normally cannot have the symptoms of the disease. nevertheless, some can be hemophiliac.  The gene carrier the gene X'S, transmitted by the mother. When it meets a gene THERE, the gene X'S is dominating and that "created" a hemophiliac boy.  When it meets another gene X healthy, it is "recessive"(not dominant) and therefore the girl will not be sick, "only" carrier.  Nevertheless when a hemophiliac one and a woman carrier have children, it can himself that they generate a girl hémophile:quand the two genes X'S are carriers of the disease...  The girl has one luck on two to be hemophiliac since this is the gene that it is transmitted for him by his mother that decides. The hemophiliac girls are therefore olus that rare and one can say unlucky...  :'(

Here, I hope that my terrible English :-[ will not have harmed this that I wanted to say you and that that will have you a little enlightened...  

Offline bookworm8571

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #9 on: April 25, 2004, 09:57:28 AM »
Sorry, but there ARE women who are "symptomatic carriers" of the hemophilia gene and have lower than normal blood factor levels themselves. There are also a large number of women who are carriers and have no symptoms at all. I did some research on this a few years ago because I was interested in the Romanovs.

  Here's an address to an informational site for carriers of hemophilia you might find interesting:
  www.wfh.org/Content_Documents/TOH_Monographs/TOH8_Carriers.pdf

  Here's a passage from that site on symptomatic carriers:
  "A word about girls and women who are symptomatic carriers; they have additional needs which should be understood. These women usually need treatment for menorrhagia (protracted severe bleeding during menstrual periods), teeth extraction, surgery, and trauma, but can in rare instances, also have spontaneous bleeding episodes. Many respond to DDAVP, but some may also need treatment in concentrates.
  The greatest issue for symptomatic carriers is usually not the treatment they require but the fact that no one believes they have a bleeding problem, especially not one called haemophilia!"
 
  Here is another medical site with similar information:
  www.pediatriconcall.com/FORDOCTOR/DiseasesandCondition/hemophilia.asp

  And a relevant passage from that site:
  "Most carriers have a factor VIII level between 30-70 U/dl. Factor VIII levels should be assayed in all females who might be carriers to identify those with levels below 30 U/dl. These women should be treated the same as way as male hemophiliacs with similar factor levels.
  Assays of factor VIII activity and factor VWF: Ag can be used to predict whether a given woman is a carrier. If the Factor VIII level is below normal, then she can be designated as a true carrier. If her factor VIII is in the normal range, but her VWF: Ag is higher, then she is a true carrier."
 
  I'm no doctor and don't understand most of the medical terminology, but it certainly appears to me that female carriers can be both symptomatic and asymptomatic. Maybe it depends on the type of hemophilia and its severity?

Offline Candice

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #10 on: April 25, 2004, 02:51:19 PM »
"Haemophilia. Human disease in which blood-clotting is defective.  Known only in males.  Transmitted from mother to son. Determined by sex-linked recessive gene.  Women homozygous for this gene are unknown. " Homozygous - having identical genes.

No disrespect intended here.  Could the Tsar and the Tsarina have had the same genes?

Offline bookworm8571

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #11 on: April 25, 2004, 03:00:25 PM »
No, the Tsar could not have been a hemophiliac or a carrier of hemophilia.

The defect for hemophilia is carried on an X gene. A male hemophiliac -- like Alexei -- inherits the defective gene from his carrier mother. A carrier mother like Alix has one healthy X gene and one defective X gene. She can pass either a healthy X or a defective X onto her offspring, so not all the children will have hemophilia or be carriers.

Because a carrier does have one healthy X, she produces enough blood factor to avoid serious problems for herself. In some cases, apparently a carrier female might have some of the symptoms of hemophilia and produce a lower than normal blood factor, but those symptoms vary. Usually carriers are not symptomatic.

If Alexei had lived to have children, all of his daughters would have been carriers because he could only pass down a defective X to them. If he had married a cousin who happened to be a carrier of the hemophilia gene, there IS a chance he would have had a hemophiliac daughter who inherited two defective X's. A true hemophiliac female is extremely rare. However, all of Alexei's sons would have been healthy UNLESS he happened to marry a cousin who carried the hemophilia gene herself, because the boys would have inherited their healthy X from their mother.

Offline Joanna

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #12 on: April 25, 2004, 09:49:55 PM »
Further to bookworm's reference to Empress Frederick, in reading different books on the Romanovs I have come across the story of Kaiser Wilhelm's love for Ella and his wish to marry her but that she spurned him. The authors would then comment negatively on Wilhelm's pomposity etc. But then recently I have read (sorry I cannot remember the book) another theory that it was Wilhelm who stepped aside from further pursuing of Ella due to the fear that she was a carrier and his determination to continue his family line with healthy children. This is not to say that his love for her was not genuine however we wish to interpret the question of his character.

Joanna

Offline Louise

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #13 on: April 25, 2004, 09:56:05 PM »
Joanna, would the same not apply to Prince Henry who eventually married Princess Irene? Would Henry not be warned about the possibility of hemophillia? Or would it not really matter seeing he was not going to inherit the throne? Was Henry forwarned and not heed the warning? Obviously he didn't heed it or he ignored it, seeing two of his sons were hemophilliacs.

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Offline Joanna

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Re: Alexei had Hemophilia - Why Didn't Alix and Nicholas try Again?
« Reply #14 on: April 25, 2004, 10:25:07 PM »
Hi Louise !

Your comments are intriguing. I had not thought of Prince Henry and have not read very much about him. I wonder if the theory of whether it was Wilhelm or Ella who decided to decline further pursuing is in letters in the archives that have been overlooked and the same with Prince Henry's. Although I tend to believe that for the Kaiser his life has been searched diligently but for Prince Henry there must be a great deal as he is like Prince Leopold, the forgotten Prussian Prince.

Joanna