How Genetic Disease Makes and Unmakes Kings, Queens and Empires

In life, sometimes it's the little things that count, and by little, I mean that which must be seen by microscope. Tiny breaks in a few key chemical bonds, transcription errors in the genes lie at the root of two revolutions-The American Revolution and the second Russian Revolution.

Our story begins with science where something totally beyond our control can make and unmake our destiny. How many of us have heard recriminations when Junior turned out to be a bad seed--he must have gotten it from YOUR side of the family. It happens even in the best families, and by best, I mean the families whose foibles shook the lives of millions-the ruling houses of Great Britain and of Russia.

The tale of the American Revolution has been told many times: No taxation without representation and all that. But it was a mental illness born of in-breeding and genetic error which helped unhinge the judgment of England's King George the III during the crucial moments of the American Revolution and heap the further indignity of being ruled by a mad king to the rest of the colony's woes.

His disease was known as porphryia and it is also associated with vampires as those who suffer this dread ailment religiously avoid sun exposure since exposure causes acute pain and blistered skin. His illness is one of 8 forms of porphryia that interfere with hemoglobin, the iron-rich pigment responsible for fixing and releasing oxygen for transport by red blood cells to every other cell in the body. Although there are several possible ways that one can contract this, in George's case it was genetic.

George suffered at least 5 distinct episodes of serious mental illness-all while presiding over the proverbial empire on which, "the sun never set." Although probably exacerbated by arsenic, a severely toxic heavy metal found in the fashionable wigs of the time and an arrow in the quiver of all the best poisoners, there is no doubt that genetic illness was present and culpable.

The royal houses of Europe had punk luck with royal blood. Propaganda against marrying the riff-raff, would cost at least one king of England, Edward the VIII the throne. In his own words he " found it impossible to carry the heavy burden of responsibility and to discharge my duties as King as I would wish to do without the help and support of the woman I love." More of the rulers should have gotten out of the palace one in a while, but since they did not, snobbery and bad blood would claim another victim. This time, the throne of Russia.

For centuries, the powerful liked to keep it all in the family. The pharaohs of Egypt went so far as to arrange marriages between brothers and sisters. But nature, is if nothing else, conservative. When cells divide scores of times throughout their lives inevitably mistakes will occur. When it happens to the cells of the body, special scavenger processes will quietly put an end to such goofs. In the rare case when this does not occur, cancer results.

But things are different in the sex cells responsible for producing the next generation. When things go wrong, the results are often tragic. Color blindness, sickle cell anemia, Huntington's Chorea and so many other sorrows are the direct result of mistakes here. On rare occasion, such genetic mutations can advance the species and incrementally improve it or produce new species, but usually not. Most mutations are either neutral or actively harmful, so usually two copies of a defect are required to create the actual disease. If only one copy exists, then only a potentiality known as a "trait" will be formed.

Most of us avoid marrying close blood relatives. In fact, there are some religious traditions including Roman Catholic cannon law that expressly forbid such unions. The reason? Genetic disease. For centuries mountain men in Appalachia and Spain and inhabitants of Ireland's isolated Aran Islands, more commonly known for its elaborate and prized fisherman's sweaters, have suffered disproportionately from genetic illnesses such as extra fingers or neurological ailments that shorten lives.

But these are the unfortunate side effects of living in isolated areas where marital choices are few. In the case of the ruling houses of Europe, complex alliances between countries were sealed at the altar. In the service of concentrating wealth and power, illness was concentrated and preserved as well.

Just as in the case of George III, an illness in the blood would tear Russia asunder and forever alter the direction of world history. Nicholas II, the last Czar of Russia loved his darling family and would do anything for them. Alas his son, Alexei, suffered from hemophilia.

Hemophilia is a defect in the chemicals that make the platlets in blood clot. Besides the real danger of bleeding to death, it causes terrible pain. Hemophilia is carried on the "X" chromosome and males who inherit a defective "X" chromosome from mom develop this illness. It does not appear in women because women carry two "X" chromosomes while hemophiliac males carry only the defective "X" and the male-only "Y" chromosome. In the rare instance where a woman inherits two defective "X" chromosomes, she does not live long enough to reproduce.

Enter Grigory Rasputin. Rasputin is often seen as the mad genius of the last days of the czar. This monk was poisoned, stabbed, shot, beaten and drowned, all at the same time by determined assassins. The invulnerable monk was his own best proof of the health regimen that he pushed on the Czar for the treatment of his young son.

Because his cure did successfully help the child (he began by taking the child off the newly-invented blood thinning aspirin that the family was using to ease his pain) the controversial holy-man achieved disproportionate power during the last days of the Russian Throne. He became a magnet for the increasingly distressed Russian populace as their completely out-of-touch Czar refused all entreaties to end World War I. As the workers and peasantry bled and starved, the Czar ordered up a few more of his glorious Faberge' eggs and the Russian Revolution was on.

Although the monk was probably not responsible for the Czar's bad decisions-some authorities credit him with begging the Czar not to start the war-he was a complete weirdo with no obvious reason to hang around the court; not a healthy place to be when scapegoats are elected.

It all ended very badly with the execution of the entire family and their secret burial under railroad tracks (the better to keep would-be monarchists from using the relics to rally a counter-revolution.) So you see, little things do mean a lot.

Sources:
http://digestive.niddk.nih.gov/ddiseases/pubs/porphyria/
http://www.porphyriafoundation.com/about-porphyria
http://www.independent.co.uk/news/uk/this-britain/madness-of-king-george-was-caused-by-arsenic-499698.html
http://www.nhlbi.nih.gov/health/dci/Diseases/hemophilia/hemophilia_what.html
Aspirin,: The Remarkable Story of A Wonder Drug, Diarmuid Jeffereys, Copyright 2004, Bloomsbury, USA