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Haemophilia (and the Royals)

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Arcane
591532.  Wed Jul 29, 2009 8:29 am Reply with quote

Haemophilia (hemophilia in North America) is a disorder of blood clotting, where a "factor" that causes clotting is deficient. The most common sites for bleeding (internal) are in weight bearing joints, such as knees, ankles and elbows, muscles and mucous membranes. Damage to joints and muscles can occur over time from these bleeds, which can result in joint damage, arthritis and chronic pain. It is a common misconception that a haemophiliac will bleed to death from so much as a small cut; it will stop but just take longer than most other people. Haemophilia can be mild, moderate or severe.

Haemophilia comes from the Greek words haima αἷμα "blood", and philia φιλος "friend". Haemophilia A occurs in about 1 in 5,00010,000 male births, while Haemophilia B occurs in about 1 in about 20,00034,000 male births (North American figures).

Dr John Conrad Otto was the first to be aware of haemophilia in 1803 stating that "a hemorrhagic disposition existing in certain families." He recognized that the disorder was hereditary and that it affected males and rarely females, and traced the disease back to a woman who settled near Plymouth in 1720.

There are two main types of haemophilia: Haemophilia A or "Classical" haemophilia, where Factor VIII is deficient, and Haemophilia B or "Christmas Disease", where Factor IX is deficient. There is also Haemophilia C, which involves a lack of functional clotting Factor XI.

Males are almost always sufferers of haemophilia; haemophilia in females is very rare. Haemophilia is not curable, but managed by either prophylactic or on demand treatment via infusion. Contamination issues with HIV and Hepatitis C emerged in the 1980's, initially the factor was heat treated, then monoclonal factor concentrates were used to inactivate any viral agents. It is estimated that over 50% of haemophiliacs in the US alone contracted HIV via contaminated blood. The treatment for haemophilia in the time of the Tsarevich Alexei was aspirin; there was a claim that Rasputin "cured" him of it, it is likely he simply advised it not be given it as it would obviously have made his haemophilia worse.

Haemophilia is inherited, but can spontaneously appear where it has not occured before in up to 1/3 of cases (there is a story about Queen Victoria not being genetically related to the royal family because of haemophilia occurring in her offspring where it had not occurred before, so that fact shows it is possible).

As far as transmission goes:

A father with haemophilia and a mother with no haemophilia gene will not pass haemophilia to their sons, but will pass the gene to their daughters.
When the mother has the haemophilia gene, and the father is unaffected, there is a 50% chance at each birth that the son will have haemophilia, and a 50% chance at each birth that the daughter will carry the gene.

Famous haemophiliacs or those who carried the gene:

Tsarevich Alexei Nikolaevich (descendant of Queen Victoria, see below)
Ryan White
Queen Victoria > her son Leopold was a haemophiliac and his daughter Alice went on to have two haemophiliac sons. Alice and Beatrice, two of her daughters, were carriers. Alice had a son named Friedrich, who was haemophiliac. Another daughter Alix married Tsar Nicholas of Russia and was the mother of Alexei. A daughter of Alice's named Irene (grand daughter of Queen Victoria) married Henry of Prussia, her son Henry died in childhood, Waldemar had a difficult life. Beatrice married Prince Henry of Battenberg, and had two haemophiliac sons, Leopold and Maurice. A daughter of Beatrice named Victoria married King Alfonso of Spain. Two of their sons, Alfonso and Gonzalo had haemophilia.

World Haemophilia Day is celebrated on the 17th of April, which is the birthdate of Frank Schnabel, the World Federation of Haemophilia founder.

Sources: Wiki article on haemophilia, haemophilia.org, englishmonarchs.co, chronicillness.org, healthinsite.gov.au


Last edited by Arcane on Wed Oct 21, 2009 5:56 am; edited 1 time in total

 
Arcane
597582.  Sun Aug 09, 2009 6:38 am Reply with quote

I also saw articles stating that Mother Teresa, Abraham Lincoln and Richard Burton suffered from haemophilia but I couldn't find definitive proof. They were oft repeated, many times in the same order. Does anyone know more about this?

 
austinallegro
597647.  Sun Aug 09, 2009 8:17 am Reply with quote

http://blog.kelleycom.com/2008/03/most-famous-celebrity-with-hemophilia.html
Apparently a Richard Burton Haemophilia Fund was set up.

 
CB27
597784.  Sun Aug 09, 2009 4:05 pm Reply with quote

Does anyone still speak Green these days?

 
Ian Dunn
624392.  Sun Oct 11, 2009 10:49 am Reply with quote

Scientists have now identified the type of the kind of haemophilia that affected the royals - haemophilia B. The scientists learned about this following a study on Crown Prince Alexei.

I've also found a quite interesting coincidence. You may remember that the question about "Victoria's Secret" that came up in the Series E Christmas special. Coincidently, haemophilia B is also known as "Christmas disease". It was named after Stephen Christmas, the first patient described with this disease. The first report of Christmas disease appeared in a Christmas edition of the British Medical Journal.

Source: BBC
Source: Who Named It

 
AlmondFacialBar
624395.  Sun Oct 11, 2009 10:57 am Reply with quote

mother theresa had haemophilia? that must have made her literally one in a million in a wholly unexpected way...

:-)

AlmondFacialBar

 
Peregrine Arkwright
624500.  Sun Oct 11, 2009 5:16 pm Reply with quote

.

Someone correct me, but I believe the last member of the royal family to be haemophiliac was King George VI. If either of his daughters Elizabeth and Margaret had been carriers, then one might have expected the disease to have shown itself in at least one male of the next generation - Princes Charles, Andrew or Edward, or Viscount Linley. Had Princess Anne been a carrier, then Peter Phillips would be at risk, as would Princes William and maybe Harry also in the next generation. So far as I am aware - no inside knowledge - none of them have been affected.

Does this mean the royal family's haemophilia gene has actually been stamped out, possibly by those quite remarkable genes of Elizabeth Bowes-Lyon?

Peregrine Arkwright

 
96aelw
624632.  Mon Oct 12, 2009 6:27 am Reply with quote

Peregrine Arkwright wrote:
Someone correct me


Well, all right then. I'm afraid he wasn't, for the simple reason that George VI wasn't a haemophiliac at all. Of Queen Victoria's 9 children, only one (Prince Leopold) was a haemophiliac, and only two (Princesses Alice and Beatrice) were definitely carriers. As to her other daughters, Princess Louise had no children, so there's really nothing to go on to work out if she was a carrier or not, Princess Victoria seems not to have been a carrier, as it doesn't turn up in her various descendents, and Princess Helena may or may not have been (her two surviving sons weren't sufferers, which needn't mean she wasn't a carrier, and her two daughters had no children). Her other sons, Princes Alfred, Arthur, and, crucially for our purposes, the future Edward VII, did not suffer from haemophilia. Essentially, haemophilia was never really a major feature of the British royal family; it emerged briefly, and we largely managed to pass it on to other royal families, notably the Russians.

 
96aelw
624635.  Mon Oct 12, 2009 6:29 am Reply with quote

In fact, while we're here, I may as well note in passing that wiki reckons the last member of any European royal family known to have been a haemophiliac was Infante Gonzalo of Spain, who died in 1934 at the age of 19 after a car accident. He had acquired his haemophilia through his mother, Queen Victoria Eugenie, and thus ultimately owed it, via Princess Beatrice, to our own dear Queen Vicky.

 
Peregrine Arkwright
624692.  Mon Oct 12, 2009 9:09 am Reply with quote

.

Many thanks, aelw. Another myth laid to rest.

PA

 
suze
624743.  Mon Oct 12, 2009 11:20 am Reply with quote

The one that absolutely is true is that porphyria runs in the British royal family.

It's by now generally accepted that it's what George III had, and some suggest that James V and his daughter Mary Queen of Scots had it as well.

More recently, the late Princess Margaret always denied that she was a sufferer, but it didn't stop the allegation being made. She used to say that it wasn't her who had it, but Prince William of Gloucester, who died in an airplane crash in 1972.

 
Iamtheelephant616
627781.  Tue Oct 20, 2009 11:51 am Reply with quote

Seen as this is about Haemophilia and the royals, and not simply Haemophilia on its own:

http://www.watchdoctorwhoepisodes.com/2-tooth-and-claw/ go to about the 41st minute. :P

 
Arcane
628150.  Wed Oct 21, 2009 5:56 am Reply with quote

CB27 wrote:
Does anyone still speak Green these days?


Yup, that'd be the one typographical in a very long article.

Will fix pronto.

And the article is on haemophilia, but with some side information about the Royals. It has been quite difficult to obtain information on "famous" haemophiliacs, without as you saw, people like Richard Burton and Mother Theresa being stated in many articles as being haemophiliacs.

 
Curious Danny
628519.  Thu Oct 22, 2009 4:02 am Reply with quote

suze wrote:
It's by now generally accepted that it's what George III had, and some suggest that James V and his daughter Mary Queen of Scots had it as well.


Not necessarily, i have read articles saying that poryphia (sic?) is quite a bad fit - some see it as more likely to be manic depression bought on by severe stress, such as that bought about by the debacle of the American war of independence.

 
Arcane
629057.  Fri Oct 23, 2009 11:13 pm Reply with quote

I have come across the same thing CD, I will have a look to see if I can find any articles that are more definitive.

 

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