
In Amish village, a rural doctor sees the rarest diseases on Earth - onetimemanytime
https://www.usatoday.com/in-depth/news/nation/2019/11/29/wisconsin-rural-doctor-treats-amish-studies-rare-genetic-diseases/3995625002/
======
collsni
This is my father's family doctor and was my doctor throughout my childhood.

He actually birthed me in Viroqua Wisconsin where he spends a few days a week.
In the past few years (since they got a new Clinic in La Farge) his time has
been pretty exclusive to La Farge and the Amish community. However he still
sees my father multiple times a year, who is not Amish.

------
cstross
... Or the doctor in question could just have moved to Dewsbury, in West
Yorkshire.

(n the mid-1980s I made an early career mistake and qualified as a pharmacist.
I did my pre-registration work at the -- now closed -- district hospital in
Dewsbury, a Yorkshire town of roughly 60,000 people. Dewsbury is in a steep
valley and the working-class population tended not to travel: according to an
older relative who worked there all his life, he knew co-workers who had been
born, worked, lived, and retired in Dewsbury without ever travelling more than
ten miles away from their birthplace. (Automobiles didn't really become a
mass-cultural phenomenon there until the 1950s-1960s.) Cue lots of first-
cousin marriages. During the 1970s, a Pakistani immigrant community arrived
... also from an isolated mountain valley town that didn't have much time for
exogamy.

The hospital serving a city of 60,000 people shouldn't have a 30-bed cystic
fibrosis ward (CF is an inborn error of metabolism) ... but Dewsbury needed
one. (CF has a frequency of roughly 1 in 3000 in Northern Europe, so you'd
expect 20 living cases in a town the size of Dewsbury: but they wouldn't need
hospital beds simultaneously! The local frequency was much higher.) Nor should
it have exotic conditions like familial hypobetalipoproteinaemia showing up in
the population. Again, Dewsbury had that. In fact, Dewsbury was so notorious
for genetic disorders that house officers and registrars training in the
relevant fields got rotated there from the big teaching hospitals in Leeds
(the nearest big city).

Upshot: if you get geographically isolated communities with religious cultures
that discourage exogamy, you get _really weird_ genetic disorders cropping up
within surprisingly few generations.

~~~
growlist
Also in Surrey. A relative worked with visually impaired children, one of them
of Pakistani heritage and having albinism, and this kid said one day "it's
because we marry our cousins". This is one of those situations where the
authorities etc. being all cringey and PC about things _harms the very people
they are trying to protect_.

~~~
cstross
It's not specific to Pakistan: the same problem tends to be seen in many if
not all cultures with a strong dowry tradition. If substantial wealth is
transferred with a bride, there's a powerful incentive to marry within the
family.

(This used to happen in Europe, but during the middle ages the church banned
cousin marriages for political reasons: it stopped the nobility consolidating
too much power.)

~~~
iguy
It was certainly common for a long time in many places, and still is across
what's now the muslim world (although I think it long predates Islam). But I'm
not sure dowry tradition is quite the right marker, IIRC it's much less common
in Hindu tradition (where the pattern was larger caste groups instead).

But it's certainly a way of concentrating power in the family. And presumably,
for a very long time, having lots of grandsons of undivided loyalty was
important in conflicts with the neighbouring family/tribe... worth the
individual fitness cost (and the extra miscarriages etc -- many of these
strange illnesses would of course have killed the child).

~~~
masklinn
> still is across what's now the muslim world (although I think it long
> predates Islam).

It can trivially be seen to have little to do with islam in examples like the
House of Habsburg, and the famously ignominous end of the Spanish line. Or
orthodox jews (the endogamous set is wider than just a family, but you still
see lots of rare genetic conditions crop up, and some jewish communities don't
even stray outside of it e.g. Mashhadi jews).

Though it does look related to _Arabization_.

While first-degree cousin marriage has gotten quite a bit rarer used to be
extremely common across the globe, and aside from the middle-east it also
remains legal throughout eurasia (except for a few eastern european countries
and notably china where it's entirely banned and india where it's dependent on
culture and religion) and south america

> IIRC it's much less common in Hindu tradition

It's less common but hardly non-existent: according to Shrikant Kuntla,
Srinivas Goli, T.V. Sekher, Riddhi Doshi via wikipedia[0] rate of
consanguinous marriage is 25% of indian muslims, 15% for indian hindu, and
under 10% for other indians. State / culture presents a much wider spread,
from 1.2% in Himachal Pradesh to 38% in Tamil Nadu.

[0]
[https://en.wikipedia.org/wiki/Cousin_marriage#India](https://en.wikipedia.org/wiki/Cousin_marriage#India)

~~~
iguy
Well, royalty are special and weird, and a drop in the bucket, and prove
little. My point was that it was a widespread pre-islamic custom, not one
which arrived with the book; the geographical correlation with islam is
because (as cstross says) it was largely stamped out in Europe a thousand
years ago.

It's my understanding that cousin-marriage was rare among european Jews, but a
bit more common elsewhere. (Not sure if as high as the surrounding peoples,
not sure.) There are indeed rare and interesting genetic conditions in some
Jewish groups, but that's another story.

(Am surprised by Tamil Nadu's 38%, thanks. But certainly north-indians take
dowry pretty seriously, while avoiding cousins.)

~~~
masklinn
> (Am surprised by Tamil Nadu's 38%, thanks. But certainly north-indians take
> dowry pretty seriously, while avoiding cousins.)

Yes, India seems extremely split between the north and the south, northern
cultures have disapproved of it or forbidden it forever while southern states
much less so.

~~~
iguy
I wonder how this happened... do you know if it's known? Could it be one of
the relics of Buddhism adopted by the ~ Gupta revival, and never made it down
south?

------
runeb
Link that works in Europe

[https://eu.jsonline.com/story/news/special-
reports/2019/10/1...](https://eu.jsonline.com/story/news/special-
reports/2019/10/10/amish-mennonite-health-wisconsin-doctor-has-found-rare-
genetic-diseases-founder-effect/2119965001/)

~~~
smallbigfish
GDPR brought too much hate and it solved too little.

------
jstewartmobile
Similar thing going on with the Navajo and _xeroderma pigmentosum_ :

[http://archive.pov.org/blog/pressroom/2012/06/sun-kissed-
pre...](http://archive.pov.org/blog/pressroom/2012/06/sun-kissed-premieres-on-
pov/)

Attributed to gene-pool shrinkage and/or ancestry mix-ups arising from the "
_trail of tears_."

~~~
protomyth
Don't you mean the _Long Walk_?

~~~
jstewartmobile
Why y'all downvoting this man? He's right.

~~~
protomyth
The full reference is
[https://en.m.wikipedia.org/wiki/Long_Walk_of_the_Navajo](https://en.m.wikipedia.org/wiki/Long_Walk_of_the_Navajo)

The _Trail of Tears_
[https://en.m.wikipedia.org/wiki/Trail_of_Tears](https://en.m.wikipedia.org/wiki/Trail_of_Tears)
was other tribes such as the Cherokee, Muscogee (Creek), Seminole, Chickasaw,
and Choctaw nations.

It still amazes me that my most down-voted posts are about Native American
facts.

~~~
gamegoblin
I assume most downvoters (not having heard of the Navajo event) thought you
were making an inappropriate joke referencing the Stephen King novel of the
same name:
[https://en.wikipedia.org/wiki/The_Long_Walk](https://en.wikipedia.org/wiki/The_Long_Walk)

~~~
Nasrudith
Also out of context it sort of sounds like a denial of the suffering and
conditions - even if that is the name thet used.

In my experience there was no denial about the travesties but the Trail of
Tears and maybe the Black Hills got name checked as events perhaps because of
the precedent and disregard for even the Constitution and the Supreme Court in
the first and the egregious "kick them off of their own reserved lands for a
short term gold rush".

~~~
protomyth
Perhaps folks might like to do a search before making such damning judgements
about other HN users.

------
beardedwizard
This was a good story. I enjoyed the rural vibe and the simple mechanics: do
it because you care, it's right, and it will still be hard.

------
onetimemanytime
I remember seeing a documentary--too lazy to look it up now--but it was about
the Inuit (or a portion of them) that let a guest sleep with the woman /women
in their igloo. At first look, this goes against Darwin, who wants to raise
someone else's "bastard" but without it they'd be doomed. So the lesser of
evils.

~~~
Iv
Evolution focuses on populations, not individuals. A lot of things make more
sense if you consider that.

~~~
marton78
On the contrary. Evolution focuses on individuals.

~~~
dredmorbius
_Darwin himself argued for group selection. He postulated that moral men might
not do any better than immoral men but that tribes of moral men would
certainly “have an immense advantage” over fractious bands of pirates. "_

[https://www.scientificamerican.com/article/whats-good-for-
th...](https://www.scientificamerican.com/article/whats-good-for-the-group/)

Though the pendulum swings. The case isn't clear-cut, and the argument for
group-level selection (or even symbiotic or more general multi-species co-
evolution) is strong.

~~~
im3w1l
Group level selection is irrefutably a thing (Multicellular organisms are
selected as a group. Eusocial insects are too). The only question is how
important it is in various contexts, in some it may be negligible.

~~~
toasterlovin
I guess you should define what exact mechanism you’re describing when you use
the term ‘group selection’. Richard Dawkins would describe the phenomena you
mention (multi-cellular organisms and eusocial insects) as cases of the genes
which create those complex phenotypes being selected.

~~~
hyperpallium
In a group without exogamy, selection for the group is selection for those
genes responsible for the complex phenotype that is the group, in a similar
but less strict way. This is true even with sufficiently minor exogamy.

~~~
toasterlovin
Right, but then you don’t need a mechanism other than genetic selection.
Because that’s what is typically meant by group selection: that there is an
_additional_ selective mechanism that exists aside and apart from genetic
selection.

~~~
im3w1l
Maybe we are talking past each other then. Because to me group selection is a
type of genetic selection.

~~~
toasterlovin
Then we are in agreement. Typically, group selection is not meant in that way,
though. Just an FYI.

------
TazeTSchnitzel
The link just redirects to eu.usatoday.com's homepage for me :(

~~~
adventured
This may work:

[https://outline.com/4wdE3t](https://outline.com/4wdE3t)

------
neonate
[https://web.archive.org/web/20191130183259/https://www.usato...](https://web.archive.org/web/20191130183259/https://www.usatoday.com/in-
depth/news/nation/2019/11/29/wisconsin-rural-doctor-treats-amish-studies-rare-
genetic-diseases/3995625002/)

------
NorthOf33rd
Colorado City, AZ Is Another great example. It seems if you want to See some
weird stuff, closed off religious communities are the place to be.

[https://www.bbc.com/future/article/20170726-the-
polygamous-t...](https://www.bbc.com/future/article/20170726-the-polygamous-
town-facing-genetic-disaster)

