The Unofficial British Royal Family Pages

I recently read two books on the generic inheritance of the British royal family:  Purple Secret:  Genes, �Madness,� and the Royal Houses of Europe by John C.G. Rohl, Martin Warren and David Hunt, and Queen Victoria�s Gene:  Haemophilia and the Royal Family by D.M. Potts and W.T.W. Potts.  Reading them together is helpful in understanding how these two diseases, hemophilia (spelled without an �a� in America) and porphyria, were inherited and passed down by Queen Victoria.  This column will discuss porphyria, and we will move on to hemophilia next month. 

Porphyria is a complex disease that is still not widely understood.  It is genetic, but not linked to a sex chromosome like hemophilia, so either parent can pass (or not pass) porphyria on to any child.  Its most definite symptom is purple urine, but it can also cause skin sensitivity, abdominal pain, mood swings, delirium, sensitivity to sunlight, weakness, insomnia, and breathing problems.  These are such disparate and vague symptoms that it can be difficult to diagnose the disease without knowing about the urine color.  It also comes and goes in �attacks� and does not appear until adulthood.  There is still no cure, but some triggers for attacks are now recognized, including sunshine, barbiturates, and an uneven intake of carbohydrates. 

The presence of porphyria in the royal family was first suggested as an explanation for the �madness� of King George III.  There is enough archival evidence of his symptoms, including dark urine, to diagnose him with porphyria.  The theory was first proposed by British psychiatrists Ida Macalpine and her son Richard Hunter in 1966.  However, some skeptics pointed out that he has so many descendants that there should be many other cases of porphyria among them, and there was little such evidence at that time. 

Purple Secret is largely concerned with identifying other possible cases of porphyria in the royal family.  Among George III�s ancestors they identify Mary, Queen of Scots and her son James VI and I as likely porphyria sufferers, but the authors ignore the fact that there is as yet no evidence of porphyria in the intervening generations of Elizabeth, Queen of Bohemia; Sophia, Electress of Hanover; George I; George II; and Frederick, Prince of Wales.  They do claim that Queen Anne�s constant ill health might have been porphyria.  They also identify some of George III�s Prussian relatives as probable cases of porphyria. 

Interestingly, the authors present evidence that all of George III�s thirteen adult children had porphyria.  Because genetic conditions typically are passed to 50% of a parent�s children, I would strongly suspect that Queen Charlotte also carried the porphyria gene, but the authors do not give this possibility any consideration.  George IV�s daughter Princess Charlotte also probably had it. 

Purple Secret suggests that Queen Victoria had porphyria.  The authors cannot make a truly convincing case, but it may be that Victoria�s complaints of unspecific illness and her intense emotions were caused by porphyria.  It is now known that a constant intake of carbohydrates wards off porphyria attacks - perhaps that is why the plump Victoria had fewer symptoms than other family members!  The book goes on to make a stronger case that Victoria�s eldest daughter Vicky, the Empress Frederick, had porphyria.  The authors then definitely identify Vicky�s daughter Charlotte and Charlotte�s daughter Feodora as having had porphyria.  However, Charlotte and Feodora could have inherited it from the Prussian royal family.