
Rose Marie Bentley lived for 99 years with organs in all the wrong places - nradov
https://www-m.cnn.com/2019/04/08/health/99-year-old-backward-organs-medical-oddity/index.html
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LilBytes
This is fascinating! I was diagnosed with dextrocardia w/ situs inversus at
19.

I didn't make it to 99 years old to find out I had it, but several physicians
and doctors in my childhood missed the diagnosis too. Story time!

This is written on mobile so I apologise for grammar and spelling errors.

I had awful asthma as a child and would frequently visit hospital till my
teenage years because of asthma attacks and similar. I couldn't estimate how
many X-rays and Ultrasounds I had growing up. No one picked up the Dextro
Cardia till I got my medical exam for Permanent Residency in Australia. You
can imagine how surprised I was when I found out all my major internal organs
are a mirror of where they should be. The consensus so far is that because my
organs are all mirrored, most doctors look straight past the condition and
consider it to be an X-ray Technician error.

Similar to Rose I experience chronic heart burn and I'm at heightened risk of
heart disease, but little more than that. Unlike the majority mentioned in the
article, but like Rose, I'm quite healthy and I'm now at the age of 30 with no
signs of stopping.

One funny story within a story. I have to wear a medical alert to describe my
condition in case of an accident.

When I was in my mid twenties I was in a car accident and my (car hits
bicycle, I was on the bicycle) and didn't wear my medical alert. The one time
in my life I definitely needed it. In vehicular accidents it's common for your
organs to be moved around your chest cavity and abdomen as a consequence of
the trauma and inertia of your body travelling and the inevitable sudden stop
at the end. I got taken straight to triage in ER at the hospital and was
whisked away to get stabilised, x-rays taken and my visible wounds cleaned.

I wasn't quite with it at the time but vividly recall the doctors looking
distressed at my X-rays, the doctors came over to mention there's some
'anomalies' in my chest and require an ultrasound.

I have a brief moment of lucidity and mention to the doctor, 'Oh, I usually
have a medical alert for this. I have Dextro Cardia w/ Situs Inversus. Thought
you should know.'

The doctors lost their minds with laughter and took particular attention to
finding the apex of my heart beat (it's not where it should be), prodding and
poking my body to find out how it ticks.

To think if I wasn't with it at that time, I may have ended up with corrective
surgery which is quite common when DC w/ SI is found to minimise risks
associated with the condition.

I was quite fortunate, I had no broken bones, but I did have a concussion and
so much road rash and other open wounds as a consequence of the accident.

Still to this day I now wear my medical alert daily, and when ever meeting a
doctor they show visible delight in getting to play around with me and my rare
genetic disorder. It's all quite fascinating. Including my girlfriend who's a
doctor, as are most of her friends. :)

Thanks for reading!

~~~
shages
I also have Situs Inversus Totalis. Thankfully I haven't had any major health
incidents to date, and my experiences with doctors and nurses has been
similar. EKGs in particular can be quite interesting. :)

I'm also diagnosed with Primary Ciliary Dyskinesia, and your mention of asthma
issues as a child had me wondering if it's something you're aware of? About
50% of people with PCD also have Situs Inversus Totalis. The primary symptoms
are a chronic cough and congestion.

[https://en.wikipedia.org/wiki/Primary_ciliary_dyskinesia](https://en.wikipedia.org/wiki/Primary_ciliary_dyskinesia)

~~~
LilBytes
Holy shit! Yeah perhaps, I'm due for a medical examination, I'll bring this
up.

Do you often get chest infections too? Apparently that's very common as it is
with me and those who have DC or Situs Inversus.

~~~
shages
Yes, it's common, and I have regular mucus build up because, well, my cilia
don't clear it well for me. :)

As rscho mentioned, you should definitely bring it up and see if you can get a
referral to a specialist. I see a pulmonologist regularly to keep an eye on
it. The term Kartagener syndrome (SI + PCD) may ring a bell to them more than
PCD will.

I was misdiagnosed as a kid as having asthma (or something like that, I don't
remember this well), and doctors prescribed an inhaler but it had no effect.
My parents had me seen by a pulmonologist which then led to a diagnosis.

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joe_the_user
Biochemical Individuality by Roger Williams [1] is an amazing read. It
discusses a wide variety of "unusual" human physiological characteristics,
including bones that only occur sometimes.

One hears the refrain "everyone is unique". The thing is, technically you can
say "every factory made part is unique" at some level. But humans are much
more unique on many more different levels, than any human-made-machine (things
like bone density or blood saltiness vary widely. Indeed, very few measures
are uniform for everyone).

The remarkable thing is how the different parts of a living thing can still
function well with that capacity to vary.

[1] [https://www.amazon.com/Biochemical-Individuality-Roger-
Willi...](https://www.amazon.com/Biochemical-Individuality-Roger-
Williams/dp/0879838930)

~~~
Gibbon1
My favorite was a statistician in the late 40's early 50's who was tasked by
the air force to find the 'average pilot'. The reason was new fangled jet
fighters were crashing due to pilot error. At higher rates than could be
anything but problems with the man machine interface.

He and his helpers took 10's of thousands of measurements of thousands of air
force pilots and determined that, there is no such thing as the 'average
pilot'.

Well there was. About two dozen of them. Turns out that all the measurements
were uncorrelated in the already highly selected group that is air force
pilots.

Aerospace manufacturers were forced to redesign cockpits with adjustable seats
and controls. And the rate of accidents declined.

> The remarkable thing is how the different parts of a living thing can still
> function well with that capacity to vary

What's really whack when you consider it is the same neuromuscular system that
allows us to walk can also be repurposed to drive a car, fly a plane and
operate other machinery.

~~~
booleandilemma
_What 's really whack when you consider it is the same neuromuscular system
that allows us to walk can also be repurposed to drive a car, fly a plane and
operate other machinery._

Isn’t this because we purposely design all those things around our anatomy
though?

I’d imagine alien octopuses’ vehicles would be designed around their anatomy
and they would have no trouble using them.

~~~
kosievdmerwe
Not OP, but I always thought it’s amazing how it becomes second nature.

You think you want to turn left and slow down, not I have to use my left foot
to slowly press the brake and slowly move my hands in a circle while grasping.

~~~
fosstax
Breaking with the left foot sounds really dangerous. What is the right foot
doing?

...though I guess you were just reinforcing your point: driving becomes so
second nature that you forget the implementation details.

~~~
monort
Actually, two feet driving is superior to traditional method in every way,
especially on a slippery road. The only problem with it is that when you try
it, you are a newbie again, so of course you can mix up pedals just like when
you were learning driving. So train it on roads without traffic.

[https://www.vox.com/2015/7/1/8877583/two-foot-driving-
pedal-...](https://www.vox.com/2015/7/1/8877583/two-foot-driving-pedal-error)

~~~
Shorel
If you are used to a stick shift car, the issue is that the left foot is too
'heavy' and totally lacks the sensitivity to apply the brake.

At least that was my experience. I drove an automatic for the first time some
years ago, and that was the only issue I detected.

The fact that you never have to move your feet from the corresponding pedals
actually helps to avoid mixing up pedals.

It would be an issue in a rally when downshifts require a bit of throttle and
you have to use two feet for three pedals.

~~~
monort
Yes, that's a problem, but I'm used to a stick, and that issue was quickly
resolved after an hour or two of practicing. One hint - use your ankle
muscles, not calf to apply pressure with a left leg. If you don't have a low
traffic road to practice, you can use go-kart for that too.

~~~
kosievdmerwe
I'm not sure what you mean, but I noticed back when I was driving stick one of
the things I do with my left foot (and I hate how I misspoke and derailed
stuff) was lifted my heel from the floor of the car.

With my right foot on the gas and on the brakes I can just pivot my ankle to
control the pedal with my heel resting on the floor, but with the clutch you
typically have to quickly press it in completely, but then slowly and smoothly
release it. Which is easier to do with your heel in the air.

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ecmascript
Very interesting. I have "Intestinal Malrotation" and have had surgery for it
(cutting ladds bands). Still experiencing several of the issues that I had
before the surgery. I am 28 and was diagnosed when I was about 25-26. It is a
very rare condition.

One thing I've learned since I was diagnosed is how little we seem to know
about the gut and the surrounding area. I hope more research will shed light
for future generations.

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chrstphrhrt
One of my cats was born with a diaphragmatic hernia and we didn't know until
she was like five years old. Organs were all over the place but things were
fine. Stomach was in the upper chest, and the lungs had grown into the lower
abdomen.

She ended up showing symptoms of not being able to keep food down (although
eating slower seemed okay) and so we got it fixed for a pretty penny. It
really seems like depending on where the organs end up, there might not be any
issue. I was really impressed with how mechanical the problem is and how
reliably successful surgery is. Presumably there could be complications if
there's a real tangle.

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changoplatanero
How does the body figure out which side is left and which side is right? I can
think of an easy way to break symmetry but it seems harder to consistently
know which side is which.

~~~
dnautics
Short answer: at the single cell stage there's a cilium that, spins in a
single direction and establishes chirality. People with immotile cilia thus
have a 50/50 chance of getting SI, because the developing body chooses
randomly.

~~~
twic
I thought it was multiple cilia, wafting fluid in a current around a cavity, a
bit later than the single cell stage but still very early?

Either way, the reason a cilium spins in one direction rather than another is
that the motor is a molecular machine built out of amino acids, and amino
acids are themselves chiral. If you made a person out of amino acids with the
opposite chirality, their cilia would go the other way, and their body would
be flipped. It's a lovely example of magnifying something up from the
molecular to the macroscopic.

~~~
dnautics
The motion of the cilia is not directly coupled to the chirality of the amino
acids, you could probably make a cilium that obligatorily moved in the other
direction with a handful of mutations.

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krilly
The article states that situs inversus sufferers "invariably" have severe
heart defects, and generally gives a pretty bleak outlook for the condition.
And yet this woman made it to 99, without diagnosis, despite several
surgeries.

I wonder if anyone has done a statistical analysis to find the true number of
undiagnosed cases, because it would seem to be very high.

~~~
heinrichf
They explain the likely reason:

> But Bentley was an anomaly, one of the few born with the condition that
> didn't have heart defects, Walker said. "That is almost certainly the factor
> that contributed most to her long life," he said.

~~~
oska
Which means that they shouldn't have used the word 'invariably'.

I'm also not keen on their use of the word 'survivor' in the same paragraph.

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fallingfrog
My uncle has a condition where all his organs are flipped left to right too, I
think it's a slightly different condition though.

~~~
riahi
There’s multiple variants of heterotaxy syndromes (left and right variants) as
well as many on a wide spectrum. We see a couple a cases a year pass through
the radiology department.

The working understanding is that these are ultimately ciliary dysfunctions in
utero which cause aberrant gradients for body patterning.

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classichasclass
I've already planned to do willed body when I pass on. There's nothing like it
in anatomy class. Ours had a pneumonectomy, which was interesting to discover
(why was the tissue different on both sides?).

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bayareanative
My very elderly paternal grandfather has a similar condition. He had a minor
abdominal surgery (spleen or gallbladder) in the 1960's, and it took an
unusually long time to find the correct surgical site.

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j7ake
The molecular mechanism that determines left-right asymmetry in the body is
extremely interesting. For mice, it comes down to Nodal flow from ciliary
movement that occurs around E8. The cilia rotate clockwise to generate
leftward flow to establish a gradient to generate left-right asymmetry.

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crimsonalucard
Why does a prefect mirror image of organs in your body result in things not
working? One would assume most things would work...

The only explanation I can think of as to why this would cause an issue is
that not everything is mirrored.

~~~
DoreenMichele
In many cases, it doesn't cause problems.

 _In the absence of congenital heart defects, individuals with situs inversus
are phenotypically normal, and can live normal healthy lives, without any
complications related to their medical condition. There is a 5–10% prevalence
of congenital heart disease in individuals with situs inversus totalis_

[https://en.m.wikipedia.org/wiki/Situs_inversus](https://en.m.wikipedia.org/wiki/Situs_inversus)

~~~
crimsonalucard
I understand this. My question is what is the mechanism that causes the 5-10%
prevalence? Was something not mirrored?

I only ask because it wasn't explained on wikipedia.

~~~
DoreenMichele
The rest of that paragraph: _most commonly transposition of the great
vessels_.

From my read, yes, sometimes not everything is mirrored. But, also, a problem
with the cilia can be a cause of the organ reversal. This will cause
additional health problems.

Just trying to be helpful. Hope that answers your question.

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gatherhunterer
Here is the Associated Press News story on this.

[https://www.apnews.com/456c51d0a69b4b86bde1a52e9a5e9a60](https://www.apnews.com/456c51d0a69b4b86bde1a52e9a5e9a60)

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vagab0nd
> To honor and respect the privacy of those who offer their bodies to science,
> no further details are given medical students about the person who had once
> inhabited the body lying on the silvery slab before them.

Unless you are special I guess?

~~~
hello_asdf
It sounds like they talked with her surviving family and got permission. The
second half of the article is from them.

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qwerty456127
Why is this so rare?

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stevekemp2
Given the number of people who commented here with their own stories I wonder
if it actually is rare.

I imagine you only find out if you're undergoing x-rays, operations and
similar.

If there are no obvious side-effects there could be a lot more flipped-people
out there.

~~~
qwerty456127
Doesn't almost everybody receive x-ray, ultrasonography, abdominal palpation,
stethoscopy, ECG or surgery today? Does it happen often that a person grows up
never ever examined by a doctor closely?

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golergka
Autoplaying video that starts after I already scrolled down and switched to HN
tab with the comments? Thanks, no.

