Johanna Margaretha van Deventer

a1 Gerrit Janse van Deventer van Veldcamp. Burger Stellenbosch. getroud: 29 Oct 1688 Ariaantje Jacobs, VARIEGATE PORPHYRIA - weesmeisie, van Rotterdam.

b5 Johanna Margaretha VARIEGATE PORPHYRIA getroud: Philip Snyman; hertroud: 7 Apr 1735 Johann Heinrich Debes of Delits

PORPHYRIC LINE OF DESCENT VARIEGATE PORPHYRIA ( R59W) - Dr. Geoffrey Dean - Ariaantje Jacobs VP

Jacobus Snyman 25 May 1732 VP

Anna Magrieta Snyman 1773 VP - Anna Sophia van der Merwe 1781 VP - Elsie van der Merwe 1817 VP

Jacoba van der Merwe 1785 VP - Pieter Johannes Schalk Willem van de Sandt 1806 VP

PORPHYRIA LINE OF DESCENT - VARIEGATE PORPHYRIA (R59W) XX Jan Hendrik Debes

Johanna Dorothea Debes c 1743 VP - Hendrik Christoffel v.d. Vyver 1765 VP - Johanna Christine v.d. Vyver VP - Godfried Willem Heinen (Heynen) VP

Willem v.d. Vyver 1762 VP - Johannes Hendrik v.d. Vyver 1792 VP - Maria Johanna v,d, Vyver VP

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ARIAANTJE JACOBS VAN DEVENTER my 7th Great Grandmother

WE DO NOT KNOW IF ARIAANTJE (ADRIAANTJIE ARIENS) JACOBS OR HER HUSBAND GERRIT JANSZ VAN DEVENTER CARRIED THIS PORPHYRIA DISORDER/MUTATION, BUT SEVERAL (FOUR) OF THEIR CHILDREN INHERITED IT, AND WITH EACH GENERATION IS HAS BECOME MORE AND MORE COMMON.

The name PORPHYRIA comes from the GREEK ‘porphuros’ meaning reddish-purple.

I have Variegate Porphyria (South African Porphyria) - It’s been in our family for generations (van Niekerks). My grandmother died at the age of 26 – giving birth to her fourth child. She died of kidney failure, due to medication complications…

My DNA test confirmed a positive South African R59W gene mutation – the common defect responsible for Variegate porphyria in South Africa.

That means PORPHYRIC DRUG PRECAUTIONS need to be exercised.

It was done in the PORPHYRIA LABORATORY MRC/UCT LIVER RESEARCH CENTRE (University of Cape Town) by Prof Peter Meissner and Prof Richard Hift.

Prof Richard Hift is now the HOD of the School of Clinical Medicine at UKZN Medical School. They are doing brilliant work in this respect!

A little bit of Historical background……………..

The South African mutation imported to the Cape in 1685/1688 from Holland and is now widespread in the South African population.

It is also identified in the Netherlands, where it is rare, and shown by haplotype analysis to be genetically related to the South African population. Also the mutation found on one allele of all four South African patients with compound heterozygous ("homozygous") VP.

The South African variegate porphyria gene PPOX mutation R59W could be traced back to Gerrit Jansz (the son of Jan) van Deventer, born in Veldkamp in the Netherlands, and to his wife Ariaentje (daughter of Jacob) van Rotterdam (who was born in Rotterdam).

In the 17th century most people did not have surnames but were described as the son of, or the daughter of, the father’s first name.

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 van Rotterdam, (or Ariaantje Adriaansse or Ariaantje van den Berg) father died when she was 5 months and her mother, when she was eight.

The spelling of Ariaentje varies in different documents.
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. It is not known whether porphyria was brought to South Africa by Gerrit Jansz or his wife Ariaantje Jacobs.

Mother of; Jacomina Gerritsz van Deventer, b2 SM; Aletta Gerritsz van Deventer, b4; Johanna Margaretha van Deventer; Jacob Gerrits van Deventer; FOUR OF THE CHILDREN WITH VARIEGATE PORPHYRIA - Dr. Geoffrey Dean - The Porphyrias

Based on the fact that Hendrik, the son of Willemijntje, the halfsister of Ariaantje, also had the porphyria mutation - the carrier was assumed to be Ariaentje.

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.

A cluster of porphyria was also identified in a community southeast of Portland, Oregon (Robert Vlietinck, unpublished results).

These people were descended from seven founders who all emigrated to the United States in the middle of the 19th century.

They were endogamous to keep the farming land in the families.

Their ancestry could be traced back to the province of North-Brabant, not far away from the village Veldkamp, where Gerrit Jansz van Deventer was born.

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 and if it wasn’t for modern medicine, porphyria would have done little harm…

Modern medicine, BARBITURATES, SULPHONAMIDES especially and PENTOTHAL are to be absolutely AVOIDED at all costs!!!

In 1939 two medical students at the University of Cape Town; Lennox Eales (Prof Eales) and Jack Chait published the first description on Porphyria in the l’nyanga journal.

The link with this founder family was identified first by Geoffrey Dean, a British physician who settled in South Africa in 1947.

Added by: Cecilia Jacoba Strauss - South African mutation R59W - VARIEGATE PORPHYRIA 4 September 2016

• Reference: MyHeritage Family Trees - SmartCopy: Mar 12 2019, 10:53:16 UTC

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• Reference: MyHeritage Family Trees - SmartCopy: Mar 12 2019, 10:54:25 UTC