The South African mutation imported to the Cape in 1688, from Holland and is now widespread in the South African population.It is also identified in the Netherlands, where it is rare.
The South African variegate porphyria gene PPOX mutation R59W could be traced back to Gerrit Jansz van Deventer, born in Veldkamp in the Netherlands, and to his wife Ariaentje Jacobs, who was born in Rotterdam.
Gerrit Jansz (the son of Jan) came from Deventer, or rather a suburb of Deventer called Veldkamp.
He was one of the free burghers and came to the Cape in 1685. He was given a grant of land in the Stellenbosch district but he did not have a wife. He must have come from a good family because his grandfather wrote a history of the Dutch-Spanish war.
Ariaentje Jacobs (or Ariaantje Adriaansse or Ariaantje van den Berg)
*The spelling of Ariaentje varies in different documents.
Her father died when she was 5 months and her mother, when she was eight. She was admitted to the orphanage ‘Gereformeerd Burgersweeshuis’ in Rotterdam, in 1687.
The director minister Sewentien decided to send eight of his female orphans (including Ariaentje and her half-sister Willemijntje) to the Cape to become wives of the Dutch settlers.
They were sent out on the ship China and arrived in the Cape in 1688.
Four of the female orphans were married within months of their arrival and their names are together in the Cape Marriage Register.
One of the four was Ariaantje. Ariaantje (the daughter of Jacob) married Gerrit Jansz van Deventer in 1688, and they and had eight children, of whom four had porphyria. They must have inherited porphyria either from Gerrit or from Ariaantje.
Based on the fact that Hendrik, the son of the halfsister, Willemijntje, also had the porphyria mutation - the carrier was assumed to be Ariaentje.
It is not known whether porphyria was brought to South Africa by Gerrit Jansz or by his wife Ariaantje Jacobs..........................
The details about the parents and grandparents of Gerrit Jansz have been found from the archives in Holland and it would have been possible to trace the ancestry of Ariaantje, from the orphanage in Rotterdam, if only the orphanage records wasn’t destroyed by fire during the bombing of Rotterdam in 1940.
Porphyria Variegate is so ‘common’ in South Africa because one of the early settlers happened by chance to have brought the porphyric gene from Holland and descendants multiplied rapidly.
It appears that the thousands who have inherited porphyria variegate in South Africa are members of this one huge family.
Those who have inherited Variegate Porphyria seem to be more emotional than average……………
Modern medicine, BARBITURATES, SULPHONAMIDES especially and PENTOTHAL are to be absolutely AVOIDED at all costs!!!
The link with this founder family was identified first by Dr. Geoffrey Dean, a British physician who settled in South Africa in 1947. In 2003 he was honored by Queen Elizabeth II with a CBE (Commander of the British Empire) award
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