The Princess and the Pea

I think we are all familiar with the Hans Christian Andersen fairy-tale titled "The Princess and the Pea". In the story, a prince is seeking a true-princess for a bride and is besieged by pretenders. Struggling to separate the rare, true-pricesses from the commoners, his mother, the Queen, tells him to place a single pea beneath the mattress in the guest-room where the princess will sleep.

Women who slept soundly were rejected and the family rejoiced when one woman complained that she had tossed-and-turned all night on the lumpy mattress.

The darker side of the story

Hemophilia is a clotting disorder that is carried by a gene on the X chromosome. Females carry two X chromosomes, one from their mother and one from their father. The chances of both chromosomes being defective is remote.

Males carry only one X chromosome which they receive from their mother. If that X chromosome carries the defect, then they have the clotting disorder. The odds of boy having a defective X chromosome if their mother is a carrier is 50%. The odds of his sister carrying the defective X chromosome is also 50% while the odds of her exhibiting symptoms is almost zero.

Hemophilia entered general awareness with European royalty, most notably the offspring of England's Queen Victoria. There is evidence that the mutation existed in European royalty before Queen Victoria based on the early death of her mother's half-brother due to internal bleeding...but we really have no solid information about HOW long it had been lingering in the blood-lines.

Hemophilia is not the only congenital clotting disorder, just the most famous. Von Willebrant disease is the most common disorder and it can affect both males and females.

Death due to hemorrhaging (via infectious diarrhea, wounds, nasal and dental infections and so on) was common before modern medicine. Somebody bleeding to death was not particularly news-worthy or memorable.

One symptom of any clotting disorder is easy and extensive bruising

If a royal with a clotting disorder traveled any distance in a carriage or stage-coach over rutted roads, it is likely that their upper arms would be covered by bruises the next morning.

That kind of bruising might have been the tickle that initiated the story in Hans Christian Andersen's creative genius. Combine easy bruising with the foppish aspects of pre-Revolution, French courts and Viola! The true test of "Royalty" became easy bruising, fragility and insatiable demands for luxury and accommodations.

And to this very day, some people equate displays of insufferable behavior with being a special little princess.

It wasn't always like that.

Early kings were war-chieftans with names like William the Bastard and Richard the Lionhearted and some of them died in battle.