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Porphyria
Contents
Foreword
The idea to write started a research project. The investigation followed two TV programs
concerning "Georges III madness" (shown on Channel 4) and "Kings and Queens of England"
(shown on UK TV history channel). The first one showed the link between Georges III and
the porphyry illness. It also showed how far the illness could be traced. The second TV
programme explained that since Henry VI, madness has always been connected in some sort to
the English crown. From these two programmes, I wondered if the madness of Georges III
could be connected to the one of Henry VI.
The investigation has not been enjoyable. Most of the information was missing or hidden in
a few books. Most of the contemporary biographies were written in view of flattering
current monarch. For example, Henry VIII biography was written when James I had just been
crown. As he was a heavy sponsor of the arts, most artists did not intend to disappoint him.
The other source of misinformation was the misdiagnosed of the illness. For example, the
illness could be either diagnosed as madness as for Georges III or as evidence of poisoning.
It is until recently that porphyry has been diagnosed for Georges III.
The research went on for a while with mixing with a day job. The idea to turn the research
into a book came from a mate. He suggested it when trying to write his on Signal Processing.
However, as life is too short, I have decided to put only a brief summary on line and see
what happens next.
This page subject is a bit controversial as it has never been developed. The origin of
porphyry could also have impact on the current English royal family. If the origin is up to
Henry VI, his mother, who married the Tudor dynasty founder, could have been the carrier.
However, if the origin is somewhere else or earlier, the importance could even be greater.
The longer the illness has been rooted in the family, bigger the risk is.
The conclusions could be debated for a while. Many of you may disagree with them. However,
I invited you to read my research update and draw your own conclusions from the finding.
This page is primarily (but not exclusively) intended for future researcher as if its
conclusions are correct, the illness origin can be traced up to the Medieval.
Contents
Forword
1. Georges III of England
1.1 Introduction
1.2 Porphyria definitions
1.3 Illness' origin
2. Charles VI of France
2.1 Introduction
2.2 Illness definition
3. The Search
3.1 Introduction
3.2 The Stuart of Scotland
3.3 The Tudor
3.4 Controversy
4. Further research
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1. Georges III of England
1.1 Introduction
For many, George III was simply mad and there is nothing else to say not to write about it.
For other, he suffered from late stage of syphilis. The truth is far from there. His
illness has been recently diagnosed as porphyry.
Contrary to general believe, Georges was not affected by his illness all his life. He was born
in 1738 and became king of England and Ireland in 1760. At the time of his nervous break
down in 1765, Great Britain has entered a turbulent period: wars against France,
independence of USA, etc. His mental state was so affected in 1788-9 that, at this point,
political discussion took place for the Regency. Georges III recovered the following year.
However, contrary to what might be expected, he was loved by his contemporary people.
He was also keen on knowledge. He had a large library that he donated to the country.
His illness resulted to various side effects. The most notorious one was hallucination:
primarily sexual one. In one occasion, he attacked a pregnant Queen-lady-in-waiting. He
also thought that he was poisonednote 1.
After his relapsed in 1810, He was declared
permanently insane and he did not recognise his own family. He died in 1820 without
knowing or realising that his wife has died note 1. Another consequence of his
illness was his urine colour that was described as pink.
His life is well documented. See note 1 for further readings as it is not the
purpose of this page.
From his doctor's diary note 2, it is widely accepted that he inherited
porphyria or porphyry. Usually, this type of illness is much minor than on George's
case.
1.2 Porphyria definitions
From the internet, there are multiple references. I took one from the medicine libray.
Porphyria is a group of at least eight diseases caused by abnormalities in the chemical
steps that lead to heme production. Found mostly in the blood, bone marrow, and liver,
heme is a vital molecule for all of the body's organs. Heme is a component of
hemoglobin, the molecule that carries oxygen in the blood.
Genetic changes are related to the following types of porphyria.
»acute intermittent porphyria
»ALAD deficiency porphyria
»congenital erythropoietic porphyria
»erythropoietic protoporphyria
»hepatoerythropoietic porphyria
»hereditary coproporphyria
»porphyria cutanea tarda
»variegate porphyria
The signs and symptoms of porphyria vary among types. Some types of porphyria
(called cutaneous porphyrias) cause the skin to become overly sensitive to sunlight.
Areas that are exposed to the sun develop redness, blistering, and often scarring.
The clinical features of other types of porphyria (called acute porphyrias) affect the
nervous system. Appearing quickly and lasting from days to weeks, these signs and
symptoms include chest and abdominal pain, emotional and mental disorders, seizures,
and muscle weakness. Some porphyrias have a combination of acute symptoms and symptoms
that affect the skin.
Environmental factors can strongly influence the occurrence and severity of signs and
symptoms in some types of porphyria. Alcohol, smoking, certain drugs, hormones, exposure
to sunlight, stress, and dieting or fasting can all trigger the signs and symptoms of
the disorder.
There are plenty of references regarding this illness see notes 3 and
4 for futher informations.
1.3 Illness' origin
Inheritance patterns depend on the type of porphyria. Some forms of the condition are
inherited in an autosomal dominant pattern, which means one copy of the altered gene is
sufficient to cause the disorder. Other porphyrias are inherited in an autosomal
recessive pattern, which means two copies of the gene must be altered for a person to be
affected by the condition.
It is general that Georges suffered from the acute form. However, the form is suffered
was more acute than it has been reported regarding for example alucination. His illness
origin is believed to come from mariage between first degree cousins.
Back to the contents
2. Charles VI of France
2.1 Introduction
Charles VI was King of France from 1374 to 1422 during the 100 years war.
He became King at the age of 6. Until
1488, his uncles governed France. As Georges III, he was loved by his people as most
of the power was with his uncles. His first madness attact occured in 1392 when he
killed four of his bodyguards. A year later, he had another attack after been saved from
a fire. He oscillated between periods of madness and recoveries. During the recovery
periods, he had as normal as possible relationship with his wife. He had a total of 11
children but only survived to the adult age.
His life has always been under stress due to his position. His life has well documented
by several authors although most of them in French. However, most of them were more
orientated in the civil war due to his illness than on his health.
At the end of his life, his dementia was quite severe to the point that he believed
that he was made in glass. To protect the queen, Charles VI had a mistress. Further,
details of his life can be found in his biography but it is not the purpose of this
page.
2.2 Illness definition
Some believed that Charles VI suffered from Bipolar disorder, also known as
manic-depressive illness.
Scientists do nott really know what causes bipolar disorder. They do believe that
genetics plays a role. For instance, more than two thirds of people with bipolar
disorder also have a relative with the same condition or some other mood disorder.
But genetics alone does not cause someone to develop bipolar disorder. We do not yet
know what those other things are. In the case of Charles VI, his mother was the carrier
as she was also believed to suffer from this illness.
In any case, a certain percentage of this illness is due to marriage between cousins.
For Charles VI and a lot of time during Medieval times, mariage between cousins was not
seen as such as an issue than nowdays.
The above figure showed the Bourbon and Valois families were heavily inter-connected.
This could explain that Charles VI illness. Alternatively, it is possible that he
suffered from a similar illness such as porphyria.
3. The Search
3.1 Introduction
The question of this page is: Are the two illnesses connected? The illness of
Georges is well documented. The one of Charles VI could be similar to the one of
Georges. The two persons are connected genetically. Georges, also an Hanovarian,
descends from the Tudor through James I. James I himself decends from Charles VI through
Catherine Valois who married Owen Tudor. However, what is the point of the research?
The answer is pure curiosity and principle. We know that the son of the Duke of
Gloucester i.e. first cousin of Charles Prince of Wales, suffered from Porphyria and
had his first attack resulting to a plane crash. The longer the gene is in the family
the longer it remains. The picture below showed how many members of the royal family
have been affected during the year.
The picture showed that the gene has been present over the years. However, its origin
has not been identified.
3.2 The Stuart of Scotland
We do know that note 6James I
and his son, Henri, suffered from Porpyria. Henri's first attack was fatal whereas
James I suffered continuously from the illness. What is less known is that
Lady Arbellanote 7
suffered from Porphyria too. Directly descended from Henry VII through his eldest
daughter Margaret Tudor, she was first cousin to James I, niece to Mary Queen of Scots
and Lord Darnley and daughter of Darnley's younger brother Charles Stuart and
Elizabeth Cavendish, daughter of Bess of Hardwick. Henry Stuart, Lord Darnley, born
7th December 1545 at Temple Newsham in Yorkshire, was the second husband of Mary,
Queen of Scots. The second son of Matthew, 4th Earl of Lennox and Lady Margaret
Douglas, who was the daughter of Margaret Tudor (sister of Henry VIII) with her second
husband, Archibald, 6th Earl of Angus.
The above picture showed that two options are available for the illness origin:
the Tudor or the Stuart as Angus was a Stuart through Robert III. To eliminate the
Stuart and hence the Valois, you need to find no evidence of porphyria in the
Tudor through Henri VII and his descendants.
3.3 The Tudor
The problem with the Tudor is that the last Tudors were plagued with illnesses. Edward
VI died young as his older brother the Dukes of Richmond and
Somersetnote10. Edward VI
was not healthy note9. His sisters health were again
not the best in the world even for the period
note12 and note9.
However, there are no evidence that they suffered from porphyria. Their illnesses
are well documented in the various biographies. In addition, there are few evidence
of mood changes, skin problems or other well known aspect of porphyria. In
addition, Henri VII note11
and Henri VIII note8
, his son, never showed any signs of the illness
either. However, although Arthur, eldest son of Henri VII, died misteriously, the
most likely cause of his death seems cancer in lieu of porphyria
note8.
If it was not the Tudor, then it must be the Stewarts
3.4 Controversy
I named this paragraph contreversy because I did not want to give the game away.
One piece of the puzzle is James IV of Scotland. His mood was swinging. To be fair,
it has been assumed that he killed his father and therefore, he could not reconciliate
with that factnote13.
I was going to try obtaining information about the Kings of Scotland up to James IV.
However, I found in my local library "Robert the Bruce" by Caroline Bingham. In page
317, there is a reference of a potential stroke and more intriguing is a mention of
disfiguration. Furthermore, in page 176, there is a mention that Robert suffered from
an unknown illness but recovered a year later. Again those two facts could mean
nothing. However, when looking at Robert's genealogy, there are very intriguing facts.
The number of mariage between first degree cousins is alarmly high.
The above diagram showed a simplified version of Robert's genealogy. In addition,
it is likely that Cecilia was the daughter of 7th Earl of Dunbar not
the 5th contrary to most records: the time lag would be too great.
From the above figure, it is possible that Porphyria comes from one the branches.
4. Further research
Further researches could investigate this possibility of Medieval time for the illness
origin. It is my belief that the truth is not far from there. However, I do not
have the time to do further research.
Note 1: Georges III by Christopher Hibbert
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Note 2: Georges III's doctor diary to be identified.
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Note 3:
Medline plus information on Porphyria
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Note 4:
Porphyria foundation
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Note 5:
Bipolar.com
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Note 6:
Charles I by James Hibbert: pages 23 and 35.
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Note 7:
Lady Arbella Stuart by Ruth Norrington.
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Note 8:
Henry VIII by Jasper Ridley
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Note 9:
Children of England, the heirs of Henry VIII by Alison Weir
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Note 10:
Bastard Prince Henri VIII's lost son by Beverly Murphy
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Note 11:
Henry VII by Roger Turvey and Caroline Steinsberg
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Note 12:
Marriage with my kingdom by Alison Plowden
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Note 13:
The sisters of Henri VIII by Maria Perry
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