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Haemophilia: An affliction of the royalty!
The news item about the celebration of World Haemophilia Day on April 17, reminds me about the very strange genetic lineage of this dreaded disease.

This bleeding disorder is a genetic predisposition, which can bleed an afflicted person to death, even by a very minor shaving cut! Those who inherit this disease, do not have enough of blood clotting ability, to seal the wound soon enough. There is mention of this extreme bleeding and deaths even in the New Testament of the Bible. There are also reports of an Arab physician of 10th century, mentioning this fatal disorder.

I first came to know about this disease, when I picked up a book on genetics, 'Heredity and Environment' by Dunn and Dobszansky. The carriers of haemophilia are exclusively women, but they never suffer from it! Even among their progeny, the female offspring never suffer from this disease. But, only the male offspring do!

One of my relatives had haemophilia. Everyone in the family, among friends and colleagues were informed, to take care that nothing sharp should be in the vicinity. He was naturally extra careful about his routine actions. Earlier, frequent blood transfusions were used to prolong lives. Now advanced plasma infusions are more effective. If any family has a record, then before marriage medical screening of the proposed couple is advisable.

The most intriguing aspect of haemophilia is that many male members of European royalties suffered from it. And from where has the European royalty inherited this dreaded affliction? Well, from Empress of the Realm Queen Victoria!

According to a report:

'Hemophilia is sometimes referred to as "the royal disease," because it affected the royal families of England, Germany, Russia and Spain in the 19th and 20th centuries. Queen Victoria of England, who ruled from 1837-1901, is believed to have been the carrier of hemophilia B, or factor IX deficiency. She passed the trait on to three of her nine children. Her son Leopold died of a hemorrhage after a fall when he was 30. Her daughters Alice and Beatrice passed it on to several of their children.

Alice's daughter Alix married Tsar Nicholas of Russia, whose son Alexei had hemophilia. Their family's entanglement with Rasputin, the Russian mystic, and their deaths during the Bolshevik Revolution have been chronicled in several books and films. Hemophilia was carried through various royal family members for three generations after Victoria, and then this trait mysteriously disappeared.' Just as well!

Hope, genetic engineering will eradicate hemophilia in the near future.

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