Inter-Marriage and In-Breeding

[basilforever]

The Hemophilia spread more throughout the families, and was helped to continue on through first cousins marrying each other, but the probably initial case of Hemophilia (in QV as a carrier) was due to a spontaneous genetic mutation at her conception, which may have had something to do with the advanced age of her father.

[Rudolf_II]

I thought haemophilia was recessive X-chromosome condition.  Inbreeding wouldn't really come into it, as affected males only needed one copy of the gene to have the disease (not having another X chromosome to dominate over the one with the haemophilia gene).  Female carriers (Alice, Beatrice, Irene, Alix) also had one affected X chromosome, but their non-affected X had a working copy of the gene so were not haemophiliacs.  Or so the theory goes.

[basilforever]

I thought haemophilia was recessive X-chromosome condition.  Inbreeding wouldn't really come into it, as affected males only needed one copy of the gene to have the disease (not having another X chromosome to dominate over the one with the haemophilia gene).  Female carriers (Alice, Beatrice, Irene, Alix) also had one affected X chromosome, but their non-affected X had a working copy of the gene so were not haemophiliacs.  Or so the theory goes.

The existence/cause of the disease itself has nothing to do with inbreeding, but the disease spread from QV to other royal families because her grandchildren married other royals and spread it to those royal houses. So if they had married commoners I suppose, other royal families would not have been affected, but obviously that wasn't an option in those days.

That family tree is too complicated, I don't even want to try to understand it completely, let alone draw one. But you did a great job of drawing the family tree I think.

[basilforever]

Since there was a fifty/fifty chance of each of Queen Victoria's sons being hemophiliac, it was very lucky that only one out of her four sons ended up being a sufferer.

[eejm]

I wonder if more of her daughters besides Alice and Beatrice were carriers.  The only one that almost certainly was not a carrier was Vicky.  Helena had two healthy sons, but who knows about the two who died as infants?  Also, neither of her daughters had no children.  Louise had no children either, so one will never know if she was a carrier - same with Ella and Marie of Hesse.  There were some reports that the Romanov girls bled more than normal and could have been asymptomatic carriers (Maria in particular), but no one can be sure. 

In the grand scheme of things I suppose none of this matters, but it does make one wonder.

[basilforever]

There were some reports that the Romanov girls bled more than normal and could have been asymptomatic carriers (Maria in particular), but no one can be sure.

What does that mean? I didn't think carrier women bled more than usual? Queen Victoria didn't bleed more than usual, did she?

[eejm]

There were some reports that the Romanov girls bled more than normal and could have been asymptomatic carriers (Maria in particular), but no one can be sure.

What does that mean? I didn't think carrier women bled more than usual? Queen Victoria didn't bleed more than usual, did she?

I don't know about Queen Victoria, but some carrier women do bleed slightly more than non-carriers and non-hemophiliacs.  These women are called symptomatic carriers, and they usually have lower levels of clotting factor in their blood, but not as low as hemophiliacs.  They do not generally bleed so much that it endangers their health, like hemophiliacs, however. 

[Eric_Lowe]

We are getting clinical here now...

[lori_c]

I have always felt that the hemophilia mutation was related to inter marrying, another disease that appears every so often in QV's family is porphyria - evidences by King George III.  It appeared as far back as the time of Mary Stuart.

[Duke of New Jersey]

We are getting clinical here now...

What is wrong with that?

-Duke of NJ

[Eric_Lowe]

Nothing wrong of course, but somebody may not be interested if you get too scientific (like arguing the merits and fallicies (yes DNA testing is not 100 % accurate. only about 94%). 

[eejm]

Well, no one will probably ever know for certain if four Grand Duchesses were carriers of hemophilia or not.  Or, rather, not with today's technology.  The fact that their aunt Olga said that the Grand Duchesses bled more than usual, and that Maria bled a lot during her tonsillectomy is merely an indication that they may have been carriers like their mothers.  Each did have a 50-50 chance of carrying the gene.  As has been said in many threads here, it may not have any impact on history, but it is fun and interesting to speculate on the hows, what ifs, and whys of people and events.  *shrug*

[basilforever]

I have always felt that the hemophilia mutation was related to inter marrying, another disease that appears every so often in QV's family is porphyria - evidences by King George III.  It appeared as far back as the time of Mary Stuart.

Well the hemophilia mutation orginally occurring has nothing to do with inbreeding and intermarriage. It can happen in anyone. However as I said before the spreading of it to other royal families was faciliated by intermarrying, although not necessarily to anyone closely related to them at all - like Ena and Alfonso - not related.

Eric, I found the explanation by eejm interesting in the extreme, as I had not heard before about some carrier women sometimes bleeding more than usual, so there is nothing wrong with getting a little clinical/scientific!

At least one of the grandduchesses would have been a carrier of hemophilia, since all four of them had a fifty fifty chance of getting it.

[Eric_Lowe]

You are right. I have read "Queen Victoria Gene" and "Purple Secret". Both good books, but wetted my appetite for more...

[lori_c]

It always seemed to me that the inbreeding caused it to sponaneousely mutate in QV as her half sister Feodoria was not a carrier.  But it doesn't seem to rule out something that Charlotte Zeepvat raised in QV Family, that perhaps this gene went back generations through the Duchess of Kent.