The Dark Urines of 1776
Paper to be delivered at the exercises of the
Armando Susmano, M.D.
On July 4th, 1776, a total of fifty-five distinguished and prominent members and delegates of the newly constituted society in the territory of the thirteen North American colonies were assembled for the unique and historical purpose of declaring their independence from the rigid grip of the British Crown.
As they were endowed with the sincere, idealistic, and forceful purpose of becoming an independent nation and free from the tyrannical and despotic rule of the King and from the civic, legal, commercial, military abuses, and the continuous high taxation imposed by the Crown, the atmosphere in the assembly room was one of great tension and uneasiness. Rumors were rampant, and the uncertainty of the Crown’s reaction to this outrageous act of insubordination was visible and could be felt under the skin of everyone present at the meeting.
At
the moment that the delegates of the thirteen colonies were ready to start
their deliberations, an unimaginable event took place, and a sudden tremor
thundered through the room. A delegation
from
“Let it be known that Your Majesty George III, King of England, has resolved and declared that any resolution taken at this meeting will be considered null and void, and that a maximum penalty by a military tribunal, will be imposed to each and all who will attempt to disobey this order.”
Signed: George III, King of
A situation of virtual pandemonium fell upon the room. The unexpected set of events threw away, at that precise moment, the hopes and aspirations of the delegates who were planning to make the new independent country, the beacon of the world and a paradigm for other nations to emulate. None of the great events that were predicted by so many intelligent minds would come to fruition.
The development of a democracy based on freedoms in all aspects of human endeavors, liberty, and the pursuit of happiness, was suddenly dissipated. None of the great achievements that history would have witnessed from 1776 to 2006 would materialize.
The Spanish and Mexican-American wars, the Civil War, the participation of the country in World War I, the liberation of Europe during World War II from the tyrannical grip of an obsessed, psychopathic criminal would never happen.
The
confrontation to contain the spread of communism embraced by several countries,
the Korean and
The many Nobel Prizes obtained for great achievements in the United States of America in the diverse areas of science, economics, or in industry, business, the arts, and human rights, the People-to-People Project or the effects of the Peace Corps to establish better relations for a peaceful world, would only be present in the mind of Nostradamus, but not in the confines of historical reality.
I
was suddenly awakened by a strange noise in the house. I found myself sitting in my library with a
book turned over in my lap. I felt
sweaty and my heart was racing. As I
looked at the clock, it was only 9 p.m., and I realized that I had fallen
asleep for about an hour and had been the victim of a horrendous dream. The TV was still on, and some lovely Schubert
music could be heard through the
I
tried to collect my thoughts despite being slightly drowsy, and asked myself
what kind of a crazy dream was it, that I had, about the non-existence of the
On my lap, I had a slightly yellowish, discolored copy of my medical school thesis1, and gradually many recollections from my younger years flooded my mind and, with them, I began to make sense out of that illogical and unpleasant dream.
In
the early winter of 1957, as a very young physician, I was making rounds at a
municipal hospital in
As I reviewed her chart, the blood labs and urinalysis, I couldn’t figure out what was wrong and what the possible diagnosis could be. None of my older attendings and professors had any clue of what her ailment was.
I spent the rest of the day and night thinking about this young girl with an undiagnosed disease, until I arrived at the ward the following morning and noticed that her urine had a slightly darker color, such as one could frequently see in dehydrated persons, except that, when I tried to look at the urine sediment by exposing it to the bright sunlight projected through a window, the urine became markedly darker, almost bloody, but there was no blood in it. She had sustained an abortion two weeks before.
I was fortunate in that, the night before, I had read in a medical journal about a disease that fit the case, and when I saw that urine the diagnosis in this young woman rapidly became obvious to me.
I went and met the rest of my colleagues and, while all excited, I mentioned to them what I thought the diagnosis could be, only to see the consternation in my colleagues’ faces, since this was a disease that they themselves, despite their many years of practice and experience, had never seen before.
It
was a case of acute intermittent porphyria, a disease that immediately became
the subject of my medical school thesis.
I never expected that my life and this patient’s illness would connect
me to the medical problems of the royal family of
Of course, this disease was unknown then and therefore unrecognized during the lives of the English monarchy, many of whom, for centuries, were affected by this disease, as was uncovered by the magnificent detective work of Macalpine and Hunter2 and described in their paper published in the British Medical Journal in 1966.
The history of this disease in the royal family dates back to the famous Mary, Queen of Scots (1542-1587)3 who started, just like my patient, with bouts of abdominal pain, vomiting, and mental disturbance, when she was a teenager. She had several severe attacks in later years to the point of being speechless, unable to move her limbs, and lapsing into unconsciousness. She was thought to be close to death, only to be up and about and feeling well a few days later. It was on that basis that it was thought that she had been “hysterical” or poisoned, as was commonly thought in the conspiracy theories of the time. This conspiracy theory of “poison” was entertained in later years, when other royal members died of this disease, which is quite the opposite of what happened to Mr. Yushchenko, the Ukrainian leader just recently poisoned with dioxin.
In another medical paper, published in 19854, it was thought, however, that the Queen’s symptoms were more compatible with anorexia nervosa during her younger years, and that porphyria might have appeared during her adulthood.
Porphyria started in the House of Stuarts, was later passed on to the House of Hanover and to the House of Prussia later on. It had a significantly damaging influence on the health of the monarchs and other relatives, as well as on the political influence of the British Crown.
But what is porphyria?
Porphyria is a disease produced by an inborn error in the metabolism of the porphyrins, which are pigments that are widely distributed in the plant and animal worlds, in chlorophyll, in the hemoglobin of our red blood cells, and in a number of enzymes (cytochrome and peroxydases), which are vital to normal cellular functions. They are normally excreted in the urine and feces.5
Basically, there are two types of porphyria: the blood-related or erythropoietic and the hepatic porphyria.6, 7 The latter can be subdivided into the acute intermittent, the porphyria cutanea tarda, the porphyria variegata (South African variety), the mixed porphyria, etc.
Those with erythropoietic porphyria show, from an early age, a marked sensitivity to light, and this photosensitivity produces marked skin lesions, blisters, and deformities over the years and reddish discoloration of the teeth, as well as characteristic changes in the color of the urine, which can change from pink at times to red when it is left standing for a few hours and exposed to light. This type of urine shows an intense fluorescence when exposed to ultraviolet light.
Of the hepatic type of porphyria, the acute and intermittent form is the most common and the one that has affected most of the British royal families for four centuries. “The disease is characterized clinically by (1) periodic attacks of intense abdominal pains and colic, accompanied by nausea and vomiting, (2) constipation, (3) neurotic or psychotic behaviour and (4) neuromuscular abnormalities.” In addition, fever, rapid heart rate, high blood pressure, hair overgrowth, loss of vision, sensory changes, convulsions, delirium, pains in the extremities, and limb paralysis may be present.
Cutaneous changes are usually not a manifestation in this type of porphyria.
“All this constellation of symptoms may simulate a wide variety of diseases, such as polio, encephalitis, arsenic or lead poisoning.”
“Even during remissions, at a time when patients seemed to be asymptomatic, carriers of these genetic defects may experience mood swings, vague “neurotic” complaints and bodily pains, that in the absence of physical findings, fail to suggest the diagnosis of porphyria.”6
“At other times they can easily get mental confusion or even become psychotic under different set of circumstances.”5 However, “the mental abnormalities that occur in acute attacks are neither progressive nor persistent.”6
“An attack can be provoked by sedatives, several drugs, infections, alcohol, menstrual periods and pregnancy.”
This
is what actually happened to Princess Charlotte
Augusta of
Queen Anne had seventeen pregnancies, the majority of which resulted in miscarriages, or dead children a few hours after delivery, or eleven years later in the case of her son William Henry, Duke of Gloucester. The lack of descendants created a vacuum in the heir to the throne and in the Protestant succession, which was then transferred from the House of Stuart to the Royal House of Hanover, by bringing in a German-speaking cousin who ascended to the throne as George I.3, 5
“Although distant in the line of succession (but descendant from Mary Queen of Scots) George I, and then his son George II carried the defective gene, but it was in George III that porphyria expressed itself in its most virulent form.”7
George
III, the last American King, who was born in 1738, had an unhappy childhood and
a very poor education. He was eleven
years old before he was able to read.8 (Although this has been refuted by Cannon and
Griffiths.10) “He was then
educated privately and became very fond of Shakespeare. He had a large collection of books totaling
65,000, that he offered to the
He became the King of England in 1760, following the death of his grandfather, George II (his father, Frederick, had died a few years earlier). He was twenty-two years old and single.
Since an heir to the throne was necessary, matrimony became an urgent subject matter. A list of potential lady suitors was considered and Charlotte of Mecklenburg-Strelitz, aged seventeen, was selected. On September 8, 1761, she was introduced to the King that afternoon, and on the same evening they were married.15
During
his reign, the
In 1762 (two years after his coronation), he began to display the first manifestations of his disease, followed by a major episode three years later, in 1765 (at age 27). He had a minor episode in 1766 and his next major ones in 1788, 1801, 1804, and 1810, when his mental faculties were severely affected, to the point that the internal and foreign affairs of England were so damaged as to have a profound effect on the nation and its institutions. It was at that time that the concept of “royal insanity” was entertained.
“His final episode in 1810 began like the others with mental symptoms, hasty and incoherent conversation turning into violence.”15
Although during 1770 to 1780 he did not have evidence for a major episode, he had the underlying metabolic abnormality of porphyria nonetheless, and his personality, mental make-up, reasoning and judgment might have easily been influenced by it.
During remission, patients may still experience mood swings, vague neurotic complaints, body pains, and even transient confusion, due to the high concentration of porphyrins in the blood. He was known to have a manic-depressive personality or psychosis, and this could have been the manifestation and influence of high concentration of porphyrins in the brain.
A large constellation of mental symptoms were reported for many years during the life-time of the King, such as:
Irritability, excitement, agitation, prolonged insomnia (he was awake for fifty-eight and seventy-two hours at times), restlessness, incessant talking (during major attacks), self-blame, indecisiveness, violence, neuroticism, emotional lability, vasomotor disturbances, uninhibited behavior, indecent conversation, nocturnal confusion, gross errors of judgment, delusions, hallucinations or delirium during major attacks.2, 15
One can imagine the extraordinary difficulties and frustrations, and how puzzled all the various and most respected and famous physicians might have been (and four were fired as incompetent by the King’s sons) at the time when they had no knowledge of this disease, had no stethoscopes, nor laboratories facilities for blood or urinalysis, that would have permitted them to render a better quality of medical care, as compared with today.
Despite this disease, he ruled for fifty-one years, and died in 1820, totally deaf, blind, and insane, at age 82.
He ruled longer than any other monarch of his time. He had fifteen children, four of whom inherited the disease and had significant manifestations of it.
The mental disorder that affected King George III was the reason for an article suggesting, ironically, that he should be given more credit for the founding of the United States.7 “At that time the world’s weakest nation defeated the mightiest.”
King George III himself said: “I shall never rest my head on my last pillow as long as I remember the loss of my American colonies.”8
“The
French Revolution was soon coming, King George himself was the victim of
several assassination attempts and big anti-monarchic sentiments swept through
It was his obstinacy, his refusal to face facts, and the challenge to the authority of parliament, that led to the disastrous war against the American colonies, “my colonies,” as he used to say.
All of this based on a personality and character that were shaped, changed, modified, and altered due to a genetic and inborn error of metabolism of the porphyrins that intermittently manifests itself with severe physical and mental abnormalities, including rapid or sudden death, just like in my patient.
These types of deaths have been the subject of much speculation regarding foul play, conspiracy theories of poisoning with the suspicion always falling on close relatives of the patient.
“Henry, Prince of Wales, was rumored to have been poisoned by his father, James I, who himself would die a few years later from the same disease: acute porphyria. He left a detailed chronicle of his symptoms and of the abnormal reddish or port wine color of his urine. Henrietta Anne, Duchess of Orleans, thought to be poisoned by agents of her jealous husband. Caroline Matilda, Queen of Denmark, was thought to be killed at the instigation of her mother-in-law.”11
The reality is that “the royal houses of Europe trace their descent to James I of England, such as: the house of Hanover; the exile family of the Stuarts; the House of Brandenburg of Prussia (emperors of Germany); the House of Orleans; the House of Savoy (kings of Italy); the House of Orange (kings of Holland) and any number of German princes.”11
Porphyria
can therefore be called a royal malady, a concept that has been challenged by
Geoffrey Dean, a physician in
The
entire history of
Currently, Drs. Macalpine and Hunter3 have encountered in their investigations two living members of the royal families that trace their ancestry to George II (a descendant of James I, son of Mary, Queen of Scots), who were found to have symptoms of porphyria variegata. Two additional family members have by now been identified with porphyria,13 providing further evidence and proof of this disease as a royal malady.
As I turn now my attention to the dreadful and horrendous dream I had in my library, I can vividly remember that unfortunate young patient lying in bed, curled up in a fetal position, unkempt, thin, wasted, weak, with overgrown hair, and I can think and imagine an identical patient lying down thousands of miles away, in the royal bed of England: that being King George III....
As I can hear the moaning, the groaning, and the effects of severe abdominal pains on my patient, I can imagine King George III under similar conditions...
And as my patient lies in bed hallucinating, unable to move, with prolonged insomnia and passing a dark, reddish, port-wine discolored urine, I could see in front of my eyes today a similar bloody-looking urine of King George III and the bewilderment of his relatives, the prime minister, and his servants, who witness the apparent insanity and most pitiful physical condition of their beloved King.
As we know now that alcohol can be a significant precipitating factor of an acute attack, is it fair to ask or assume that wine and spirits that the King might have consumed during his official duties could have triggered some of his most severe and devastating bouts of porphyria? Even if consumed in small quantities, in the presence of unusually high concentrations of porphyrins in the blood, it could have been enough to tip the balance of his metabolic abnormality and produce deleterious effects upon h is personality and brain functions. It has been documented that he suffered from frequent bouts of insomnia, lasting up to fifty-eight and seventy-two hours without sleep.
Similarly, historians must now ask the question of what function, if any, the King’s disease played in the inept handling of the American colonies and of the revolution that ensued, and how many times, if any, the dark-colored urines of the King during 1776, due to high concentration of porphyrins, could have influenced his state of mind, his reasoning and judgment in dealing with the many problems presented to him by the nascent American colonies and their revolution.
“The health of world leaders is a factor of vast importance in history and international politics” wrote Blaine Taylor in a medical journal in 19768 during the bicentennial celebrations.
In retrospect, as was ironically suggested in a previous paper, George III should be given more credit for the creation of this country, because had he been a completely mentally sane and a healthy leader of the powerful nation that England was, the absurd reality of my dream could have resulted in an absolute tragedy, the United States of America might not have existed, mankind would have been deprived of what has been considered the beacon of freedom and liberty under the rule of law and, as David Horowitz states in the last paragraph of his book entitled “Unholy Alliance,”16 “The Communist Empire would still be standing, the Taliban would rule Afghanistan, Saddam Hussein would still be in power, and the world would be a place of infinitely greater cruelty, injustice and tragedy, than the world that confronts us today.”
5/06
REFERENCES
1) Susmano, Armando. Porfiria (porphyria). Actualization, fisiopatológica, clínica y
terapéutica (Update of the physiopathology, clinical and therapeutic aspects of
porphyria). Thesis to opt for the Degree
of Doctor of Medicine, 1961,
2) Macalpine,
3) Macalpine,
4) McSherry, I.A. Was Mary, Queen of Scots, anorexic? Scottish Medical Journal, 1985, Vol. 30, pp. 243-245.
5)
6)
7) Cecil (Wyngarden and Smith), Textbook of Medicine. W. B. Saunders Co., 1988, 18th edition, pp. 1182-1187.
8) Batley, Frank. King George III’s insanity. Porphyria: A royal malady.
9) Witts, L. J. Porphyria and George III. British Medical Journal, 1972, Vol. 4, pp. 479-480.
10) Miller, J. M.
Vignette of medical history: Porphyria in royalty.
11) Brooke, John. Historical implications of porphyria. British Medical Journal, 1968, Vol. I, pp. 109-111.
12)
The maddening business of King George III and porphyria. Tibs-Elsevier Science Ltd., 1996 (June), pp. 229-234.
13) Hurst, Lindsay. Royal porphyria.
14) Dean, Geoffrey. “The royal malady.” Letter to the Editor. Journal of the Irish Medical Association, 1969, Vol. 62, No. 388, p. 380.
15) Cannon, J. and Griffiths, R. The
16) Horowitz, David. Unholy