Family, duty, honor: looking at historical parallels to the inbreeding in Europe and Game of Thrones

With the premiere of the final season of Game of Thrones (GoT) fast approaching, we take a look at some of the more hazardous GoT relationships and their parallels to the history of royal inbreeding in Europe and the disastrous results often associated with the practice.

GoT touches on many dicey topics, though, as the seasons have progressed; torture, murder and war have become widely accepted by the public as just good TV. However, one aspect of the show that still manages to disgust viewers is the incest that occurs within the ruling families.

That being said, the ‘twincest’ in the Lannister family and the common practice of sibling marriage between Targaryens is simply reflecting the practice of royal inbreeding as it occurred within royal families throughout history, and the usual result of ending the dynasty it was trying to protect.

Inter-marriage within royal families has been demonstrated throughout history. Mirroring the incest demonstrated by the mythical gods they worshiped, royalty often held the opinion that as they were akin to gods on earth, they should have close family marriages too. This concept backfired regularly and due to madness, genetic disorders and sibling rivalry, many dynasties built on the practice collapsed.

In ancient Egypt, the famous pharaoh Tutankhamen was the product of sibling incest, born with severe genetic disorders including a club foot and bone deficiencies. He married his half-sister who had two miscarriages. His exact cause of death is unknown, though some believe it to be due to malaria that had been worsened by his genetic bone disorders. Aged 18, his death resulted in the end of the family’s reign.

Later in Egypt, Cleopatra VII was married to both of her brothers and 12 of her ancestors had done the same. Though it kept the wealth and royal status in the family, rivalries meant murder was rife and Cleopatra killed both of her brothers/husbands and her sister in an attempt to maintain power.

The practice of dynastic marriage rarely ended well, though it is the cases of genetically inherited disease that can be more drastic.

Hemophilia B

Having married her own first cousin, Queen Victoria was known to have arranged the inter-family marriages of her nine children, hoping that the close family ties would promote peace between the countries of Europe. Despite good intentions, her plan failed and family grudges between her grandsons, Kaiser Wilhelm of Germany, King George V of the United Kingdom and Tsar Nicolas II of Russia, played a key role in World War I.

As the genetic lineage spanned across Europe, so did the genetic mutations and the resultant spread of the hemophilia gene led to the downfall of many royal families, significantly contributing to the end of the Imperial Age.

Known as the ‘royal disease’, hemophilia B is a recessive, X-linked, genetic blood disorder where the body loses its ability to clot blood. A mutation in the F9 gene of the X chromosome alters RNA splicing, resulting in the production of a shortened form of the clotting factor IX. Individuals with hemophilia B find that wounds take longer to heal, they may have frequent spontaneous bleeding episodes and they may have deep internal bleeding which, in some cases, can be fatal. Though rare in the general population, the frequency of the mutated allele and the incidence of the disorder was greater among the royal families of Europe due to the high levels of royal inbreeding.

A case in which the presence of hemophilia B had a particularly significant effect was that of the Romanovs of Russia.

The fall of the Romanovs is often linked to the disenchantment of the public with the royal family, with the public believing that the family hid from the public eye in their palace and ignored the views of the people. The marriage of Tsar Nicolas II to Victoria’s granddaughter Alexandra Feodorovna was seen as the start of this unrest. Unknown to the Russian people at the time, this union brought the hemophilia B gene into the Romanov family. Heir to the throne Tsarevich Alexei inherited the disease and, not wanting the public to know of his illness, this led the family to distance themselves further from the public.

In an attempt to alleviate the symptoms of the prince, the Tsarina relied heavily on the Russian mystic Rasputin, a man hated by the people who disapproved of him having such close ties and influence over the royal family. As the family increased their reliance on a man few trusted and hid themselves away to protect their youngest member, they lost the support of their people, leading to the start of the Russian revolution and ultimately, their deaths.

Porphyria and the madness of King George

Another instance of an inherited disease in the British royal family is the congenital disease porphyria. The most famous example and the case that first sparked an interest in this disease with relation to the royal family is that of King George III. His struggle with the disease and its effects throughout his reign in the late 1700s gave him the moniker “The Mad King George.”

Porphyria is the resulting condition of a defective heme biosynthesis pathway. When one of seven key enzymes in the pathway is disrupted due to an inherited defect in their corresponding gene, the hemoglobin-associated porphyrin and its precursors build up in the patients’ blood.  These build-ups can lead to a series of severely distressing symptoms that can occur in outbursts or acute attacks.

During these attacks patients can experience: a discoloration in their urine to a red-brown color, often referred to historically as port-wine urine; severe abdominal pain; pain in the chest, legs and back; changes to mental state such as bouts of anxiety, confusion, hallucinations and paranoia; alongside a host of other symptoms.

The theory that George III had porphyria, specifically the variant variegata porphyria, was first put together by Ida Macalpine and Richard Hunter in 1966 and was presented in a paper titled “The ‘insanity’ of King George III: a classic case of porphyria.” While the paper received a great deal of critical analysis, this theory is largely accepted today.

To support the theory, a great number of George’s ancestors and descendants have presented with symptoms indicative of the condition, a perhaps unsurprising occurrence due to the close genetic pool of the royal families of the time. Mary Queen of Scots, George’s fifth great grandmother, was known to experience the fits of mental disturbance and the bouts of abdominal pain commonly associated with porphyria. Mary’s son, James I of England, was reported to have urine as “purple as Alicante wine” according to his physician.

Meanwhile some of George’s descendants present with much more definitive cases. George’s second great-granddaughter Princess Charlotte of Prussia and her daughter Princess Feodora of Saxe-Meiningen, who died as a result of suicide in 1945, were both suspected of having porphyria. DNA From both mother and daughter were examined in 1996 by geneticists Martin J. Warren and David Hunt, which diagnosed both as sufferers of variegata porphyria. A more recent descendant, Prince William of Gloucester, was diagnosed with the disease in 1968, a diagnosis that was further confirmed by two separate doctors.

The hapless Hapsburgs

Perhaps the most prominent and confronting example of the issues of incest were displayed by Charles II, the final representative of the Hapsburg dynasty. Numerous portraits of family members depict the Hapsburgs with hugely distended chins and thick, heavy set jaws, referred to as “The Hapsburg Jaw.”  This dynasty eventually culminated in King Charles the II of Spain, the product of a union of uncle and niece and the only surviving offspring of the pair who was beset by an impressive medley of maladies.

Charles’ health was fragile throughout his life, but the postmortem report produced on his death by his physician makes for some astonishing reading. The report states that the Kings body “did not contain a single drop of blood; his heart was the size of a peppercorn; his lungs corroded; his intestines rotten and gangrenous; he had a single testicle, black as coal, and his head was full of water.” While this report cannot be fully verified and was produced in an age where exaggeration was common, it is clear that the combined effects of years of inbreeding had resulted in deformities to the King so severe that make it astounding that he made it to the age of 39.

While depictions of incest in GoT do mirror the real historical occurrences in the royal families, the results are often glossed over and whitewashed. The three offspring of Cersei and Jaime Lannister all grow to be healthy and handsome (if occasionally sociopathic) young adults, a far cry from the often unfortunate and malformed progeny of incest such as King Charles II.