How much do you know about haemophilia?

Written by: Top Doctors®
Edited by: Cal Murphy

You will have heard of haemophilia. Perhaps you know it as that disease where if you get a cut you bleed to death. Maybe someone once told you that it is called “the royal disease” because it runs in the royal family. And haven’t you read somewhere that it only affects men? The question is, how many of these “facts” about haemophilia are true? Haemophilia: true or false? How much do you know? Let’s find out…

Haemophiliacs can bleed to death from a cut


False. A haemophiliac’s blood takes longer to clot than the average person, but this doesn’t mean they bleed at a faster rate; they just bleed for longer. A small cut or scrape will still clot and heal. They are only at risk of bleeding to death if the injury is severe.


Haemophilia is genetic


True. Human beings have 23 pairs of chromosomes (46 in total), and we get one of each pair from each parent. The 23rd pair are the sex chromosomes (XY in men, XX in women). The genes that code for blood clotting proteins are located on the X chromosome, and mutations in these genes can cause haemophilia. These mutated genes can be passed on from parent to child, meaning haemophilia runs in families.

It is also possible for the gene to mutate spontaneously, without any previous family history.


Only men can get haemophilia


False. While it is true that it is far more common in men and boys, it is possible for girls to be born with haemophilia too. The thing is that girls have two X chromosomes, whereas boys only have one, and as the mutant clotting gene is recessive, it means that girls have a “back-up” – if a female child inherits the haemophilia gene from one parent, but receives a normal X chromosome from the other, the normal chromosome will take control and her blood will clot properly. However, she will be a carrier, and there is a 50-50 chance that she will pass the mutant X chromosome to her children.

If a male child inherits the haemophilia gene, the disease will manifest, as he only has one X chromosome from his mother, and a Y chromosome from his father – there is no “back-up” X chromosome, so the haemophilia gene comes into play.

In the unlikely event that a baby girl receives two mutant X chromosomes (if her father was a haemophiliac and her mother was a carrier, for example), the disease would manifest, thus it is possible that women can have haemophilia.


Haemophilia runs in the royal family


False, but this one used to be true. The disease is thought to have originated with Queen Victoria, as two of her daughters were confirmed carriers of haemophilia, and her youngest son Prince Leopold, Duke of Albany was a haemophiliac (in fact, the condition may have played a part in his death from a cerebral haemorrhage after a fall). All of them married into other European royal families, passing the haemophilia gene onto their descendants and into the royal families of Spain, Russia and Germany, giving rise to the nickname “the royal disease”.

However, all known descendants of Victoria who had haemophilia or carried the condition are deceased, and as far as anyone knows none of the living royal family are haemophiliacs.

Fun fact: it was only in 2009 that it was confirmed that “the royal disease” was haemophilia B after genetic testing was done on the Romanov family remains.


You shouldn’t touch a haemophiliac, because they are easily hurt


False. Although many haemophiliacs avoid rough activities or sports in order to avoid causing any internal or external bleeding (which would take longer than normal to clot), they are no more easily hurt than the average person, and non-violent physical contact, such as hugging or handshakes would do them no harm. In fact, with management, such as taking a dose of clotting factor beforehand, many haemophiliacs can engage in limited-contact sports such as football (soccer) and basketball.


Haemophiliacs have a lower life expectancy than the average person


True, but not as much as you might think. The condition is manageable with the help of modern medicine and the average life expectancy for someone with haemophilia is only about ten years less that for someone without it, meaning haemophiliacs can live long and full lives.

If you are worried that you or your children might have haemophilia, you should contact your GP or a specialist to diagnose and manage the condition.


By Topdoctors

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