History of royal diseases

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Royal Maladies: Inherited Diseases in the Royal Houses of Europe (Book cover)

The British royal family was once known for certain inherited disorders like hemophilia and porphyria. Anchor Marco Werman talks with medicine geneticist Alan Rushton about the history of Royal diseases. Rushton is the author of “Royal Maladies: Inherited Diseases in the Royal Houses of Europe.” Download MP3

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Marco Werman:  The royal wedding’s over and some say the big job now for William and Kate is to produce heirs.  Kate Middleton is a commoner; she doesn’t come from British royal or noble stock.  I guess that means an expansion of the gene pool now.  In the past, royal families have tended to be, and there’s no other way of saying this, they’ve tended to be inbred.  With all the hazards associated with inbreeding.  Dr. Alan Rushton specializes in medical genetics and has written several books on the topic, including Royal Maladies: Inherited Diseases in the Royal House of Europe.  He joins us from his offices in Flemington, New Jersey.  Dr. Rushton, fact check my statement for me.  Has inbreeding affected the British royal family in the past, leading to, as it does, to diseases most notably hemophilia?  Or is that notion of royal inbreeding and disease a myth?

Dr. Alan Rushton:  It certainly is true, there’s evidence of several different hereditary diseases that have been transmitted through successive generations of the British royals.  And certainly inbreeding has contributed to that.

Werman:  And was hemophilia a result of inbreeding?

Dr. Rushton:  The best evidence we have is that hemophilia resulted from a mutation, a sudden change in a gene, affecting clotting factors.  And Edward Duke of Kent, who’s the father of Queen Victoria, she inherited the mutated gene but had no symptoms herself, as only males are affected.  Then she went on to have eight children.  And one son, Prince Leopold, actually had the bleeding disorder hemophilia.  And two daughters were unaffected carriers and then passed it on to male children in the subsequent generations.

Werman:  Queen Victoria was apparently eager to get fresh blood into her family and some of her descendants married into other royal families of Europe.  Wouldn’t that though just spread hemophilia further across Europe?

Dr. Rushton:  That is correct.  Prince Albert and Queen Victoria encouraged their own children to marry into the royal houses of Europe, for political purposes.  To stabilize the relations between Britian and Russia and Germany and Spain.  But unfortunately, that did pass the hemophilia gene into the royal houses in Russia, several German states, and eventually Spain as well.

Werman:  Now hemophilia wasn’t the only royal disease in Britain.  There is porphyria, a disease that’s thought to have led to King George the Third’s madness and portrayed some time ago in the film The Madness of King George.  Tell us what porphyria is and how it entered the royal family.

Dr. Rushton:  Porphyria is an interesting disease; it’s a neurologic and skin disease.  There are about seven different kinds of inherited porphyria.  The type that seems to be present in the British royal family is called variegate porphyria and produces symptoms of sudden attacks of severe abdominal pain, skin rashes on the arms and the face, palpitations in the chest, fevers, thought disorders, seizures, and even coma.  And it can go on for weeks at a time before gradually improving.  In between these episodes, individuals are perfectly healthy.  And they seem to be brought on by certain environmental factors like excessive alcohol use.   Heavy metals are another factor too, like lead and arsenic. In historical times, lead was used in a lot of foods and medicines and arsenic, for example, is used to whiten the wigs that were worn in royal households.  And individuals who had porphyria would have these unfortunate attacks, which could put a person out of commission for weeks at a time.

Werman:  Are hemophilia and porphyria still present in the British royal family today?

Dr. Rushton:  The best evidence we have is no.  The most recent individual in the royal family with porphyria, as far as we know, was Elizabeth the Second’s cousin named Prince William.  He died in a plane crash over 30 years ago, he had no children.  We assume that all the current royals have been tested.  None of this health information has been made public, but they appear to be reasonably healthy people and don’t have any evidence of porphyria as far as we know.

Werman:  Dr Alan Rushton, author of Royal Maladies, joining us from his offices at the Hunterdon Medical Center in Flemington, New Jersey.  Thank you very much indeed.

Dr. Rushton: Thank you.

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