Alexei and Hemophilia

[Louise]

With hemophilia playing such a pivotal role in the Royal Houses of Europe, I was wondering if there was any memoirs, diaries, correspondence between the families afflicted with this disability?

I have often wondered if Alix and Queen Ena commiserated about the affliction with their heirs? Did Alix seek advise from her sister Irene?

One would figure that each party would seek out advise and try to comfort each other.

Louise

[Thomas_A.]

As far as I know the disease was never mentioned in the family.
Queen Victoria used to say : "This disease does not exist in our family".

But in the letters Princess Alice of Hesse wrote to her mother she mentiones the suffering of her little Frittie - his bleedings and behaviour during these days...
It seems that it was a secret which everybody knew but nobody talked about it.
I read that Nikolaus II said the word "hemophilia" only once - when he was informed by the doctors after Alexej's first bleeding in 1904. Also Ernst Ludwig left no single word about the illness - neither in addition to his own brother, nor in Alexej's. He wrote " soon it became clear that the boy was ill.." But THE WORD never appears.

[rjt]

Does anyone know of the presence of the disease in the current reigning houses?

[ChristineM]

The haemophilia gene, passed through mother to daughter, only the symptoms of the disease are manifested in the son.   Males do not pass on the gene.   (I know this is an extremely simple explanation of a very complicated genetic disorder).    So far as the House of Windsor is concerned, it was only Queen Victoria's daughters who could carry the gene.   Therefore through Edward VII, George V, George VI.   It is likely therefore, the line has lost this genetic inheritance because none of their respective wives carried the haemophilia gene.   Suffice to say, neither Princes William nor Harry are haemophilics.

What appears to have been overlooked is that Alexandra Feodorovna's sister Irene's - Princess Henry of Prussia - youngest son, Henry, suffered from haemophilia.  He was born in 1900 and died in 1904.    Princess Henry was therefore only too aware of the agonies suffered  both by her young sister and her nephew who was born the same years as her own son's death.

tsaria

[Penny_Wilson]

The haemophilia gene, passed through mother to daughter, only the symptoms of the disease are manifested in the son.   Males do not pass on the gene. ...

tsaria

Males definitely do pass on the gene if they live long enough to have children.  

Each person has two "sex" chromosomes: a male has an X and a Y(XY); a female has an X and an X(XX).  Each parent passes one of these chromosomes on to each child:

XY
XX = XX, XY, XX, XY

As the hemophilia chromosome is carried on the X chromosome, every single daughter of a hemophiliac man will be a carrier courtesy of the X chromosome she received from her father(let's indicate an "infected" X chromosome as X(h):

X(h)Y
XX     = XX(h), XY, XX(h), XY


As you can see, no daughter is free of the hemophilia gene because her father had no other X chromosome to pass on to her. No son will have the condition, because they will receive their X chromosome from their mother, and an uneffected Y chromosome from their father.

A case in point is Queen Victoria's son, Prince Leopold.  He grew to adulthood, married a German princess and had two children before his death: an un-"infected" son who became the Duke of Coburg; and a daughter, Princess Alice of Athlone, who was a carrier.  She passed hemophilia on to her own son, who died at a young age.  Princess Alice's daughter was Lady May Abel Smith, and I'm not sure is she was a carrier -- perhaps someone else here knows...

[ChristineM]

Dear Penny

I stand corrected and bow to your scientific knowledge.   I am sure you will agree re the original question 'Alexei's haemophilia and the Royal Houses - the House of Windsor need no longer worry about falling prey to the curse of haemophilia.

I think the point that this dreadful disease, uniting two august sisters in fear and grief, underlines the frailty and vulnerability of all humankind.

tsaria

[jehan]

As far as I know the disease was never mentioned in the family.
Queen Victoria used to say : "This disease does not exist in our family".

In all fairness, while I have read this quote in QV bios, one has to put it in context.  The Queen was not in denial, but stated this after her son Leopold was born and she was told of his disease.  And she was right- at that point, there HAD been nobody else with haemophilia in her family, as far as anyone knew.

Certainly it was discussed frequently in letters to and from Alice, Vicky and others. usually as "this awful disease".  While it was not announced publicly, it was certainly something the family was aware of, and it was NOT ignored.

(My first post here, although I have been lurking for a while!)

[olga]

Is it fairly certain then that OTMA Nikolaevna were carriers?

[Penny_Wilson]

Is it fairly certain then that OTMA Nikolaevna were carriers?

No, I don't think it's at all certain that any of the girls were carriers, though statistically speaking, half of them should have been.  Alexandra had one X(h) chromosome(from Alice) and one X chromosome(from Ludwig as his family did not have this condition).  Nicholas had an uneffected XY set of chromosomes:

XY
XX(h)

So the girls could have been XX or XX(h).

If the family was extraordinarily lucky, then no daughter would have inherited the X(h) chromosome -- if they were extremely unlucky, then all of them would have had it.  

As far as I know, we just don't know if any of them was a carrier, though Marie's bleeding difficulties during her tonsillectomy have led some to believe that she could have been a "symptomatic carrier," meaning that while she herself didn't have hemophilia, she was a carrier who exhibited in herself some of the symptoms.

[Pravoslavnaya]

Poor Alexei, if he had lived to marry!    Imagine his pain if he lived long enough to see one of his grandsons suffer from what he'd known he'd pass on.

[hemphiliamom]

      Hello All, I am new to this but have been dealing with this "awful disease" as someone put it my entire life almost 28 years now. And it is not as awful now as it once was. I can only imagine how it was for them back then, but I wanted to say that PLEASE do not look at it as awful, because it is no longer that way.
Granted I think it is the worst thing to ever hit our family...I lost my father because of it and many many others have lost from it as well. BUT I do have two sons that do have it and they are no different than anyone else! You cannot look at them and tell.

I am sorry if I sound a little rude about it...I do not mean to come off as that way. I tend to be defensive though and if anyone out there has this touching their lives I would hope you know what I am talking about.

  Now that that is out of my system...I am just learning about the Russian Royals....it was told to my Grandmother when my father was born that she and my Grandfather BOTH had recessive genes and that is where it came from. NOW in all my research that I have done, and I have done a lot, I have never come across this explanation for it. I have heard of genetic mutation, and it just being in the chromosones that the good Lord gave you, but never this.

  IF there is anyone out there that can explain this paticular explanation to me I would be forever greatful.

  Thank You and I look forward to reading more about this family.
                                               Shan

[Pravoslavnaya]

Dear Shan --

I appreciate your writing in to this forum, as your letter serves as a reminder to all of us that no one who bears the cross of illness, genetic inheritance or disability would ever want to be defined by the cross they bear.

Certainly the Martyred Tsarevich lived far above his hemophilia, and I am glad you and your sons do, too.  Thank God that now there are treatments and may He bless your family!

Your remarks about two recessive genes contributing to the inheritance of hemophilia make me wonder if the considerable inbreeding common to the royal families of Europe may have had something to do with the emergence of the gene in Victoria in the first place.  I confess I do not know much either -- is there in fact much to that theory?

[grandduchessella]

With hemophilia playing such a pivotal role in the Royal Houses of Europe, I was wondering if there was any memoirs, diaries, correspondence between the families afflicted with this disability?

I have often wondered if Alix and Queen Ena commiserated about the affliction with their heirs? Did Alix seek advise from her sister Irene?

One would figure that each party would seek out advise and try to comfort each other.

Louise

I've never heard of any correspondence between Ena and Alix, but Alix & Irene did commiserate with each other. During one of Alexei's bouts, Nicholas wrote that Irene was there and it was a great blessing as she was so kind and sympathetic.  Regarding concerns within the family & intermarriage, QV wrote to Victoria Hesse that a marriage between Ernie & Maud wouldn't be wise because of the symptoms being shown in "dear Irene's" little boy (Waldemar). I don't think that enough was known about genetics to know that Maud wasn't a carrier and neither could Ernie be--it seemed to be believed on QV's part that the closeness of the relationship could contribute plus Maud's not "being strong".

[hemphiliamom]

Thank you for the kind words! As for a link between Royal imbreading...one would have to wonder! I know I have thought that many times....because they were all related in some sort of way. I am unsure of the facts on this paticular subject, and it is time consuming to sift though all the documentation to find the facts!

I have often wondered also why they named the disease what they did...look up the meaning of the two words that go into making the word....

Hemo=blood   Philia=love or lover

Isn't that a strange thing? I mean you can look at it in a lot of different ways but when it comes back to it that is just a strange word for this disease.

Anyway, I bought Nicholas and Alexandra last night and I intend on getting it read as soon as I can...I am sure there will be a lot more to say.

Thank you, Shan

[Janet_W.]

Does the concept of "inbreeding"--with particular regards to the European royal houses--offend anyone else on this website? Cousins marrying cousins was not such a strange thing 100 years ago and more; selection of a mate was much more limited, whether you lived in a village hamlet or belonged to a royal family.

One of the problems I face in explaining my interest in Queen Victoria, Empress Alexandra, etc., is that the general response is, "Oh yeah--they were really inbred!"  With the implication being that the majority of European royals were walking around with three eyeballs each and drool running down their faces.

I happen to have seen the negative results of an actual case of "inbreeding," and I can tell you that it does not resemble anything we've seen in the royal houses of Europe! Naturally, if one person was the carrier of a gene, it would be likely to passed on to that person's children . . . and cousins marrying cousins would reinforce the situation. But if you study the various descendants of Queen Victoria, you will see there was a wide variety of intelligence, looks, temperment, ambition and health situations among her children, grandchildren and great-grandchildren . . . as in any family.  

I hope I treat folks with hemophilia the same way I treat folks with any other type of condition, be it cancer, Alzheimer's disease, AIDS, diabetes, Downs Syndrome, loss of sight or hearing, or whatever--with consideration (as I would treat anyone) and the knowledge that is just one aspect of that person's life, and it doesn't mean the rest of us are licensed to be judgmental or patronizing, or that we need to run screaming in the other direction.