Porphyria in European Royalty

[juliamontague]

Porphyria was another genetical illness in the british royal Family and it spread to Prussia through QV's oldest daughter Victoria who had that illness and passed it to her daughter Charlotte, who herself passed it to her daughter Feodora of Saxe-Meiningen.

 

[Furienna]

Well, I watched a documentary once, and it was about how a disease, that was carried down through generations of English monarchs. It was, for example, the very disease, that George III suffered from in the end of his life. But I don't think it was haemophilia. Was it called porphyria?

 

[SusanE]

Well, I watched a documentary once, and it was about how a disease, that was carried down through generations of English monarchs. It was, for example, the very disease, that George III suffered from in the end of his life. But I don't think it was haemophilia. Was it called porphyria?

It was porphyria which is very different than hemophilia.

Porphyria is a metabolic disease with many symptoms and most sufferers do not exhibit the severe behavioral symptoms that George III exhibited. If you have seen the file "The Madness of King George," you will be aware of the symptoms. Modern medicine provides treatment for porphyria that was not available to George III. See http://health.allrefer.com/health/porphyria-treatment.html

Porphyria is "a group of inherited disorders involving abnormalities in the production of heme pigments (the base material responsible for hemoglobin (red blood cell pigment), myoglobin (reddish muscle cell pigment) and another group of materials called cytochromes." (From http://health.allrefer.com/health/porphyria-info.html#definition)

From http://health.allrefer.com/health/porphyria-symptoms.html

Porphyria symptoms and signs include:

Red urine (sometimes called purple)
Sensitivity to sunlight
Blister formation on exposure to sunlight
Skin swelling on exposure to sunlight
Photodermatitis
Crampy abdominal pain (may be severe)
Constipation
Vomiting
Pain in the limbs
Personality change
Numbness or tingling
Muscle pain
Muscle weakness or paralysis


Prince William of Gloucester, who died in a plane crash in 1972, was diagnosed with porphyria. He was the eldest son of Prince Henry, Duke of Gloucester (son of George V). I do not know the type or extent of his symptoms. According to http://www.sussex.ac.uk/press_office/bulletin/25jun99/article1.html , "There is a one-in-two chance of any member of the Royal family with the faulty gene passing it on to each offspring. Of that number, around 10% will suffer symptoms."

 

[Hereditary Thane]

It is even more amazing to think how successful Victoria's and Albert's genes have been given the fact that her grandaughters gave haemophilia to two crown princes, and several other more distantly descended offspring. It has also been mooted that Victoria also passed on the genetic disposition towards porphyria to some of her German descendants.

 

[Leslie2006]

...It has also been mooted that Victoria also passed on the genetic disposition towards porphyria to some of her German descendants.

Very true. It started with King George III, Victoria's grandfather, and later showed up in her own great-great granddaughter Feodora (through Vicky) as it did with her mother Charlotte (Vicky's daughter). Those are the only two QV descendants I can think of, but are there any more?

 

[Mariel]

I'm surprised you have not heard of the link of Porphyria with the royal families. It can be traced through the Stuarts, Tudors, Hanovers, and Katherine of Valois probably brought it from France, where he father Charles VI was a "typical porph" according to some. Anyway, my Scots families were followers of the Stuarts before they were Stewards, went from Brittany to England to Scotland with them, and somehow picked up the porphyria gene.
So I am very aware who had it, although it is concealed by many. Q Elizabeth's cousin Duke of Gloucester had it but died without children. Many Stewart monarchs had it, and Elizabeth, daughter of James I/VI probably took it to Germany. It came back with the Hanovers and really got going. Victoria or Albert had it, or both of them, because DNA has been done on bones of Victoria's daughter Vicki and Vicki's Charlotte (see The Purple Secret book, forget author). It is a dominant gene and affects 50% of children, but is recessive in very rare forms. So there are no "carriers" except that some men are able to not "show" it--because not triggered, and thus could pass it on.
I am most admiring of Guillome and Stephanie of Luxemburg and pray they are not closely enough related to be threatened.

 

[Mariel]

I am hoping Prince Guillome and Countess Stephanie are not closely enough related to get the dominant gene Porphyria. Some who had it are all the Stuart monarchs (at least from Mary queen of Scots) Tudors (especially those who married Stuarts such Lord Darnley) Probably Ernst August of Hanover (this is rumored because of his reaction to alcohol but I am not sure of this),
QE II's cousin The Duke of Gloucester (not the current one), Victoria and/or Albert of England, because their daughter Vicki had it and her daughter Charlotte (which was proved by DNA on bones). The Hanovers had it (George III and probably his wife, some of their children, maybe Duke of Kent, Victoria's father, who was George III's son. Many more. Katherine of Valois probably, who married Henry V and the Owen Tudor. It is very strong in royals of these lines but I'm unfamiliar with it in the more Alpine stock. Vlad and Elizabeth of Hungary are thought to have had it, though, as well as some Spanish royalty who were so badly inbred that they could barely function....some time ago, not at present. 50% of children get it. It is a dominant gene. This is a concern and I've been relieved to see all the commoner marriages, which I am certain must be inspired by the need to rid these families of this gene--and other genes I don't know about?
One can usually live with this disease pretty well if you know the "triggers" and the right diet and how to avoid certain prescription drugs and alcohol, and environmental toxins, so it is not a death sentence. But if two lines cross which are both affected it seems to worsen the disease, but I'm not sure how it does that. Little is known, unfortunately. But it has a diagnosis code, it is real.

 

[Iluvbertie]

QE II's cousin The Duke of Gloucester (not the current one),

The Queen has only one cousin who has held the title Duke of Gloucester who inherited the title from his father - who was The Queen's uncle.

However, I do think you mean the present Duke's older brother who died before his father and thus never inherited the title. Yes there are rumours that he had the disease along with rumours about maybe Princess Margaret.

 

[Lumutqueen]

QEII only has one cousin who is the current Duke of Gloucester, who has children and is still alive.

 

[Furienna]

It seems like Mariel meant the current duke's older brother, who died before they father did. Someone just said so in another thread.

 

[Mariel]

My posts were moved here

I posted these replies elsewhere, and they must have been moved here. Very efficient! I am new on this forum so have not been finding my way efficiently around the forum.
Red urine is not always found in porphyria, nor even purple. It was a strong feature of James I/VI, one reason for fairly easy diagnosis of him, even though he lived before we had the modern "tests". But porphyria is generally dx'd as much from family history and ruling out other causes as it is from tests, as the tests are not very good and not very well done except under the best of circumstances.
As for the earlier Gloucester heir, I had not read that he had not succeeded to the title when he was killed. He was dx'd by an English doctor at the behest of his mother, who noticed lesions on his face, a feature of some kinds, but not of all, porphyria. Then he was re-diagnosed in South America where he was a business representative. The fact that his brother married a non-royal, and their children have married non-royals, offers hope that this family will escape the disease, which is "dominant", and affects 50% of children...but maybe the present Duke of Gloucester was one of the blessed 50% who did not get it--and in that case, it is dead as a doornail... it is not recessive in the type this family has had, although there is a rare form of porphyria which is recessive.

The disease affected the royals before the Hanover arrival. As I said, James I/VI is thought to have gotten it from Mary Queen of Scots and/or Lord Darnley. Lord Darnley had not only the Stewart genes but also the Tudor genes, and the Tudors, in my opinion, became active with porphyria through Katherine of Valois, who married first Henry V and then Owen Tudor. Katherine is not written up as "mad", but her father Charles VI of France was actually more often "mad" than George III. He did terrible things such as kill his aides, and then fall into sorrow over what he'd done when he recovered his right mind. He thought he was made of glass and could break. He came from a very inbred line, and his wife Isabeau may have had it as well...thus Katherine had a very high chance of having it. However, in these old cases, there are no "lab tests" as there are now since the 20th century.

My type of porphyria, HCP, is not the same as the Hanover type dx'd through DNA on the bones of Vicki and Charlotte, Queen V's child and grandchild. They had VP. Both types do involve skin lesions, so I am well covered when outside and have tinted windows. I have wondered if somehow I got HCP from the Stuarts, since my ancestor Robert de Pollock came from a Jacobite family in the 11th and 12th centuries in Brittany, England (shropshire) and Scotland (Renfrewshire). Robert de Pollock had land adjacent to that of Walter Fitzallen and contributed to the same monasteries that Walter did (Paisley and Melrose). there are Stuart marriages in the lineage of Robert de Pollock, but I have no knowledge of a direct line to me. In limbo.

 

[Mariel]

Porphyria facts about QV's descendants: Her daughter Vicki, who married the German prince, and Vicki's Charlotte both have been DNA tested (their bones) and proven to have porphyria, the form being Variegate Porphyria. Porphyria of this type is not recessive, that is, once it is not in a line, it is gone. It is dominant, so that 50% of children get it on one parent's line, and if two parents have it, 75% (on average, of course). The only way a person can be thought to be a carrier (unlike hemophilia) is if that person has the gene but it is not triggered into disease, which happens frequently.

QV probably got the disease from her father, the Duke of Kent, whose father George III had it. She could have gotten it from her mother, who was a first cousin of the Duke of Kent, if that princess had it, but no one has researched that issue, to my knowledge.

Her descendant of Gloucester (brother of the present Duke) was definitely dx'd with it.
The book stating that QV had no descendants with porphyria has been proven false. One source, The Purple Secret, a book by a scientist associated with those who disinterred the graves of Vicki and Charlotte, on whose bones the DNA was tested. One point should be made concerning relatives of Vicki and Charlotte: they were courageous and servants of humanity to allow this, as porphyria needs to be treated (it can be) rather than hidden. Avoidance of triggers is the main way to go to achieve remission.

.

 

[Unclebilly]

What Royal inherited diseases do you have porphyria is the main disease of the Royal Bloodlines from

 

[Mariel]

Unclebilly, I have porphyria, and this is what made me try to see if I was related to any royals. I did find a Stewart connection in the Middle Ages, but actually the one I found definitely is an ancestor of Stewart kings, not descended from the first Stewart king. That was the wife of Walter Fitzallen, whose first marriage (prior to Walter) produced my ancestor Isabel Croce Pollock. The people in that period married within the landowning class, and thus are often related to "royals", and they married to preserve land ownership or increase it, thus inbreeding and encouraging the continuation of porphyria, if it started that early in the population, which it probably did.

However, Porphyria also came into England later than the early Stewarts, probably with the Plantagenets but more certainly with the Valois bride of Henry V, who gave her genes to the Tudors and the Stewarts, reinforcing the Stewarts' problems. I don't pretend to have any genealogies intact, other than those few generations in the 11th and 12th centuries. After that, the vast unknowing.

The curiosity is that, although my early ancestors produced thousands of descendants,
somehow that "porph gene" came down all the way to me.

 

[Unclebilly]

Porphyria is the inherited disease from the Royal Bloodlines, check the Kings and Royal family members with this diseases. Acute and Hereditary Porphyria are the main two, other inherited diseases come from the Porphyria's, check out King Henry's Bloodline and Hereditary Porphyria Royal Bloodlines. I have both and so do the members of my family, with other inherited diseases, Our leaves the doctors never seen before and we were born in America. My Cousins

 

[Mariel]

In modern times we know more about how to control the symptoms of porphyria. Most important is to look at a "safe/unsafe" drug list. Then, there are certain foods which incline to trigger an attack, and these are easily avoided if known. In the present times, though, there is increasing prevalence of porphyogenic substances in the environment, many of them man-made. This list is too long to put down here, but some of these, at random, would be composite shelving or cabinets which are not sealed, new rugs which have stain resistance added, the glue under rugs, the chemicals which come from auto exhaust such as benzine and arsenic, formaldehyde which is widely used to make clothing look smooth, formaldehyde in other products such as manufactured homes and trailers, products used to make cars smell "new", cleaning products for rugs (a bad one here), other household cleaning products--many other things. These triggers can get a porphyria attack started. The primary treatment is "glucose IV" which is usually dextrose in solution, but the main thing is prevention of exposures to toxins and the eating of a diet with sufficient carbohydrate.

By the time of Prince William of Gloucester, some of these things were known, and therefore he probably had some treatments which helped him and allowed him to live a normal life, although a life with restrictions.