Haemophilia – The Most Royal of Diseases

In days of old, European royalty married only into other members of European royalty. This led to everyone being somewhat related, which is still true to this day.

In the Victorian and Edwardian era’s this become even more apparent, and it was all down to these two:

English: Queen Victoria and Prince Albert 1861

Queen Victoria of The United Kingdom and Prince Albert of Saxe-Coburg and Gotha. This couple were first cousins themselves and went on to have a total of nine children!

These children, including the future King Edward VI (who would be nicknamed the Uncle of Europe), all had to be married off to well respected spouses. Victoria was from the House of Hanover, a German royal house, and she married into the House of Saxe-Coburg and Gotha, a name that was used until 1917 when it was changed to the less German sounding Windsor by George V.  German royalty were particularly eligible to the British royalty due to their dynastic connections and Protestant religion, as even nowadays, a Catholic is barred from the succession to the British throne.

So off the children went, out to Germany and beyond, not knowing that they could all be afflicted or carrying a disease that would have ramifications in several royal houses, including Russia.

It would later be found out that Victoria was carrying a genetic disorder called haemophilia, a bleeding disorder where sufferers fail to form blood clots,leading to bleeding for long periods of time. This can be dangerous, especially when serious injuries or internal bleeding occur. There are two types of haemophilia, A and B. A is the more common form, and sufferers lack the blood clotting factor VIII, whilst haemophilia B is due to a lack of factor IX, it was the descendants of Victoria that would have the mutation for factor IX.

Luckily for women, they tend not to be affected by haemophilia. The reason behind this is because of our genetics, every human has 46 chromosomes which arrange into 23 pairs. One of these pairs defines what sex we are, if you’re female, you have two X chromosomes (XX) and if you’re male, you have one X chromosome and one Y (XY). The mutated gene that leads to haemophilia is found on X chromosomes, so if one set of genes is a bit faulty and you’re female, you have a second X chromosome to fall back on (to put it simply). Sadly for men, they don’t have a second X chromosome, and only carry the much smaller Y; therefore, if they carry a mutation that leads to a disease (the genotype), then it is likely that the disease (the phenotype) will occur. Haemophilia is therefore an X-linked recessive inheritance disorder:

X-linked recessive inheritance: Affected boys ...
X-linked recessive inheritance (Photo credit: Wikipedia)

Of the nine children of Victoria and Albert, one son, Prince Leopold was affected by the disease and died an early death after falling over and haemorrhaging. He passed the affected gene onto his daughter Alice, who then passed it onto her son Rupert.

Two of Victoria and Alberts daughters became carriers for the disease, Beatrice, the beloved youngest daughter, had three children and at least one of her two sons, also named Leopold, died at 32 during an operation. Her daughter, Victoria Eugenie, married into Spanish royalty and passed on the disorder to her two sons who both died young.

It was Princess Alice and her descendants that would be greatly affected by haemophilia though. Alice definitely passed it onto three of her six children. Prince Friedrich died as a toddler after falling from a window, Princess Irene passed it onto two of her three sons and Princess Alix, who later married the Russian emperor Nicholas II and became Empress Alexandra Feodorovna, passed it onto her only son and heir to the Russian throne, Alexei Nikolaevich. Sadly for the Russian Imperial Family, haemophilia would play it’s part in the revolution that overthrew the dynasty and led to their murders by the Bolsheviks.

Nowadays, haemophilia cases within European royalty have dwindled, though there is still the chance of it resurfacing, it just depends on who marries who, as all royal houses are now related, sharing the ancestors that were Victoria and Albert.

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2 Comments

  1. Excellent essay. Like most biology instructors, I use this case every year for my introductory biology class – I’ll be sure to steer my students to this page next month when we start genetics.

    1. Thank you! I got my inspiration for this from a genetics lecture I had recently, covering single gene disorders. I think that the history of this particular disease makes is very memorable.

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