The Habsburg Jaw and Other Royal Inbreeding Deformities
The Longterm Effects of Incest and Inbreeding
In some places in the United States, folks make jokes about inbreeding. My own mother was from West Virginia, and some of her friends would often make “pumpkin head” jokes about the inbred folks where she came from. (It is true that her parents, my grandparents, were second cousins; however, both my mother and her brother were adopted.)
For a long time, the dangers of inbreeding or having children with a close relative weren't completely understood. The biggest problem with inbreeding is that when close relatives choose to mate, it results in homozygosity, which can increase their offspring's chances of being affected by deleterious recessive traits for all kinds of physical and cognitive disabilities, including ailments like hemophilia and cystic fibrosis as well as deformities like the Habsburg jaw. These incestual pairings also run a greater risk of...
- Reduced fertility (both for the related parents and in their offspring)
- Lower birth rate and higher infant mortality
- Congenital birth defects (including facial asymmetry)
- Certain kinds of cancer
- Suppressed immune systems
- Smaller adult size (pedigree collapse)
These days, we have genetic testing and other valuable research tools to help us determine what may happen if we have kids with someone who is biologically related to us. But up until just a few centuries ago, it was common practice for people to marry their cousins and even their brothers and sisters (and in many remote rural regions, the practice continues today).
Historically, family line relations were often formed in royal houses to secure political alliances, strengthen the lines of succession, and ensure the noble purity of the bloodline. This practice caused a host of ailments and deformities that can still plague descendants of these royal houses to this day.
Charles II of Spain and the Habsburg Jaw
Also called the Habsburg Lip and the Austrian Lip, the Habsburg Jaw is a physical condition known by the modern term mandibular prognathism. It is characterized by a jutting lower jaw that is often accompanied by an abnormally thick lower lip and sometimes a tongue that is abnormally large.
It is believed that the Habsburg Jaw originated in a family of Polish royals, and the first person to have been known to have it was Maximilian I, a Holy Roman Emperor who ruled from 1486 to 1519. Many portraits of this monarch feature a pronounced underbite.
Through generations of royals marrying each other and therefore closing the ranks of their gene pools, the Habsburg Jaw manifested itself just about everywhere in Medieval Europe. The House of Habsburg, which is named after the Habsburg Castle in Switzerland, is associated with the Habsburg Jaw because so many of its members had it.
Charles II, the last Spanish Habsburg, was feeble-minded and physically deformed as a direct result of his limited gene pool. His ancestor, Joanna of Castile, actually appears in his family tree no less than fourteen times because of first and second cousins intermarrying. It is said that Charles II's genetic makeup was more muddled than it would have been if his parents had been brother and sister.
Charles II's mandibular prognathism was so pronounced that it was said that he could not chew his food and that the size of his tongue caused him to drool significantly. He also had mental issues and was considered mildly retarded. He didn't learn to talk until he was four and couldn't walk until the age of eight. As an adult, his speech was so poor that he was, for the most part, unable to be understood.
Charles II was also sterile, and when he died just shy of his 39th birthday, the country went to war to decide on an heir. The War of the Spanish Succession lasted for thirteen years, and Philip V began the House of Bourbon at the end of the war.
King Juan Carlos I, the current ruler of Spain, is a distant descendant of the House of Habsburg, although he does represent Philip V's House of Bourbon. He has the Habsburg Jaw, but only slightly.
Other Habsburgs with the pronounced jaw deformity included Charles V, Holy Roman Emperor, and Ferdinand I, Holy Roman Emperor, as well as countless others.
Hemophilia has hit the royal houses of Europe pretty hard. Hemophilia isn't necessarily the product of inbreeding, but because these different monarchies intermarried to secure territorial and familial alliances, and because many were carrying the gene for hemophilia, they spread the disease throughout Europe.
It all began with England's Queen Victoria, who reigned from 1837 to 1901. So many of her children and grandchildren married into royal families that she is sometimes referred to as "the grandmother of Modern Europe." It is believed that she inherited the gene that causes hemophilia (haemophilia is the British spelling) from her father, Prince Edward, and not her mother, Princess Victoria of Saxe-Coburg-Saalfeld. According to some historians, however, there is a possibility that hemophilia hadn't been introduced into the royal bloodlines before Victoria because Prince Edward may not have been her biological father.
What happened with Queen Victoria's descendants, however, is well documented. Victoria passed the hemophilia gene to her son, Leopold, and some of her daughters who, in turn, passed it to their sons and daughters. The effects of this hereditary disease caused disastrous consequences in the lives of Victoria's descendants:
- Prince Leopold, Duke of Albany - Queen Victoria's son, he died at the age of 31 after a brain hemorrhage caused by a fall.
- Prince Friedrich of Hesse and by Rhine - The son of Louis IV, Grand Duke of Hesse and Princess Alice of England, Queen Victoria's daughter, Friedrich also died of a cerebral hemorrhage from falling out of a window twenty feet to the ground below. His injuries were not severe enough to have killed him; he died because his body could not stop bleeding. He was two and a half years old.
- Prince Waldemar of Prussia - The son of Princess Irene of Hesse and by Rhine, who was the daughter of Princess Alice of England, Queen Victoria's daughter, died in 1945 at the age of 56. During World War II, he desperately needed a blood transfusion, which does help hemophiliacs. His doctor had been diverted to help the victims of a concentration camp, and Prince Waldemar died awaiting his return.
- Lord Leopold Mountbatten - His mother was Princess Henry of Battenberg, known before her marriage as Princess Beatrice, a daughter of Queen Victoria. He died on the operating table during surgery for his hip. He was 32.
- Prince Heinrich of Prussia - Great-grandson of Queen Victoria through his mother and father, the little prince died at the age of four after a fall. His brother was Prince Waldemar of Prussia. Their brother, Sigismund, did not have hemophilia.
- Infante Alfonso and Infante Gonzalo of Spain -These two guys, both princes of Spain, were descended from Queen Victoria by way of their mother, Princess Victoria Eugenie of Battenberg, who was the Queen's granddaughter. Both of them died after car accidents that they could have survived if they had not had hemophilia. Alfonso was 31, Gonzalo was 19.
- Tsarevich Alexei Nikolaevich of Russia - Alexei's story is a sad one. He inherited hemophilia from his mother, Empress Alexandra Feodorovna, a granddaughter of Queen Victoria. His father Nicholas was the last Tsar of Russia and the whole family—mom, dad, four daughters, and Alexei—were murdered during the Russian Revolution of 1918. Some say that this civil war came about in part because of Alexei's parents' patronage of a man named Rasputin, whom history still calls "the mad monk." Because he was able to control Alexei's hemophilia symptoms, Rasputin was also called upon for advice in other areas which, in turn, angered the Russian people.
Children of Egypt: Elongated Skulls of King Tut and Family, a Video by Brien Foerster
Egyptian Royal Inbreeding Disorders
Philadelphia may be the City of Brotherly Love, but the ancient Greek word philadelphoi was used to describe a whole different kind of thing. It was a nickname given to the brother and sister marriage of Ptolemy II and Arsinoe. In fact, Ptolemy II's name is often given as Ptolemy II Philadelphos.
The ancient Egyptian royal families were expected to marry their brothers and sisters and it happened in virtually every dynasty. There were not only brother and sister marriages, but there were what was called "double niece" marriages, where a man married a girl whose parents were his own brother and sister. This may have been done because of the ancient Egyptian belief that the god Osiris married his sister, Isis, to keep their bloodline pure.
King Tutankhamen, more widely known as King Tut or the Boy King, was the product of a marriage between brother and sister. It is also possible that Tut's wife, Ankhesenamun, was either his full or half sister or his niece. Tests on the mummies of two stillborn children found in Tut's tomb have confirmed that Tut was the father and that the mother and father were related.
Cleopatra, the last pharaoh, was at one time married to her own brother before she (may have) had him killed.
Because of close marriages like these, stillbirths were common in the royal families, as were birth defects and genetic disorders. By the same DNA testing that identified Tutankhamen's children, we also now know that Tut himself was plagued by illnesses and disorders caused by his limited gene pool. Tut had a cleft palate, a club foot (as well as missing bones in his feet), and scoliosis, all of which either occurred or were worsened due to his parentage.
Unfortunately, many other deformities were present in almost every dynasty because of inbreeding. All through the 18th dynasty, we see huge overbite problems as well as elongated skulls in almost all of the royals, evidence of the shallow gene pool.
Outbreeding Saves European Royals
Today, we understand the dangers and consequences of inbreeding. Most societies have attached taboos and stigmas to marrying inside one's family, and it is rarely done. In fact, today there are only a few places where marrying within the family is still condoned, mostly because of the remoteness of those locations (and their relatively small populations).
The term "outbreeding" is the exact opposite of inbreeding and has become the norm, even amongst royals. As people became aware of the damage they were causing their descendants, they began to reach further for potential mates and, in some cases, brought commoners into the royal bloodlines.
Morganatic marriages, which occur when a royal marries someone of a lesser status, are becoming increasingly common and necessary to inflate the gene pool. Prince Charles and Princess Diana were seventh cousins, once removed, but their marriage was considered morganatic because Diana was not royalty (she was nobility, but not royalty). Prince Charles and his second wife, Camilla Parker-Bowles, are ninth cousins, once removed. Their marriage is also morganatic.
When Prince William, son of Prince Charles and Princess Diana, married Catherine Middleton in 2011, many people spent days digging up ancestry charts to see if the two shared any relatives. If they had, then any children they had could have had serious issues with recessive genes or a myriad of diseases and disorders. Fortunately for these two, their closest possible connection would be as eleventh cousins.
Fortunately, royal inbreeding is now virtually non-existent. Although it is still practiced in a few societies, technology and awareness are catching up. The days of the Habsburg Jaw may be over.
© 2012 Georgianna Lowery