A Girl Ate Leftover Potato Salad for Lunch.
This is What Happened To Her Liver.
CG is a 17 year old girl, presenting to the emergency room with a progressive deterioration
in balance, gait and speech.
She tells the admitting nurse that she felt a burning in her stomach over the past couple
days.
She had several stomach movements from one particular end.
You see, CG was an American high school student.
On the weekends, she got to stay with her dad in the city.
Over the last couple of months, she started noticing that she had trouble with balance,
writing and speaking.
Sometimes she had something to say, but she’d have trouble getting the words out.
One weekend, CG was with her dad.
The only food he had in his fridge was homemade potato salad.
He was really proud of this one because he made all the ingredients from scratch, including
a new dill pickle recipe he found on the internet.
Before his daughter heated up some leftovers, he warned her that it was too delicious and
that she wouldn’t be able to eat just one serving.
But he also told her that he felt some discomfort from both ends after eating some a few days
earlier.
Ignoring both of these warnings, CG heated up a large bowl of leftover potato salad.
She was feeling great after eating it.
She went back for seconds.
And thirds.
As the day continued, CG became incredibly thirsty.
She quickly gulped down 2 whole bottles of water.
Must have had a lot of salt in that potato salad, she thought as she started doing her
homework.
Over the next few hours, CG felt a burning sensation brewing in her stomach.
It would come in waves.
She didn’t want to complain to her dad about it, because she didn’t want to hurt his
feelings about his cooking.
She kept quiet about everything.
She saw a rash growing on the dorsum, or backside of her hands, and decided to go to bed early.
The next morning, CG woke up.
There was no more rash on her hands, but her entire body was in a world of hurt.
The burning in her stomach was now accompanied by a sharp prodding feeling in her side.
Her joints all felt stiff.
When she told her dad about this, he said “welcome to old age,” as he left to go
run some errands.
As the day passed, CG found it increasingly difficult to move.
She started noticing that her skin was turning a greenish yellow.
She hiccuped.
And then huddled over the sink.
Something green came up.
This kept happening, every hour.
As the week continued, CG had to miss school because she felt so ill.
She couldn’t keep anything down.
Every time she had to hiccup, it was followed by another movement up and out.
As she finally couldn’t take it anymore, her mom called 911 and she’s brought to
the emergency room where we are now.
At examination, doctors noted that CG’s stomach was distended, or swollen.
She was clearly jaundiced as evidenced by the yellowing of her skin and the whites of
her eyes.
Both of these are indicators that whatever’s wrong with her, is coming from her liver.
This gives the medical team some clues as to what’s happening.
CG noted a metallic taste had come up every time she hiccuped, and as she was telling
this to the medical team, she hiccuped again.
And things came up again. And doctors saw it first hand.
This could be some kind of food poisoning from the potato salad she ate, except a blood
test revealed anemia.
An meaning without and emia meaning presence of blood.
There was something wrong with her red blood cells.
That’s not something that usually comes from food poisoning.
Lab abnormalities from that blood test confirmed liver dysfunction (students, can you give
3 examples of what these could be?), bringing us to the next clue.
The liver is important for several things in the body.
For starters, It makes proteins.
Some of these proteins are blood clotting factors, which stop you from bleeding when
you get a cut.
Other proteins known as albumin hold water in specific compartments in the body, so that
fluid isn’t flowing freely all throughout.
And because of how it’s connected to the small intestines, the liver processes all
the nutrients that you put in your body, from your mouth.
This sets the stage for defining CG’s liver problems because
Her blood clotting factors aren't working fast enough (students, how is INR defined?)
meaning the liver might be having trouble making them. And it might be having this trouble
because something is preventing it from making those proteins
But this measure was still in normal range, even if it was at the upper limit of normal.
This could be a problem, maybe not.
CG’s abdomen is swollen with fluid, something called ascites and it means that that albumin
isn’t being sufficiently produced by her liver now. When combined with jaundice and anemia, all of this points to some degree
of potential liver injury for CG.
But what could be causing this?
This brings us back to anemia.
CG reported metallic taste in her mouth, and a green emesis that followed her hiccups.
This could be a problem of B vitamins.
Both of these are generally greenish yellow liquids.
They’re stored in the liver.
And, their deficiencies can cause anemia (students- macro or microcytic anemia?), but CG didn’t
have vitamin B deficiency.
She was tested for iron and copper.
These are actual metals-- that could be contributing to her metallic taste
Iron Deficiency causes anemia.
This is because red blood cells hold on to oxygen using hemoglobin, a protein that contains
iron.
And for copper, too little and too much, both cause anemia.
And in CG’s case, she has too much copper
The medical team finds that she has hypercupremia.
Hyper meaning high
Cupro from latin Cuprum, which was borrowed from Greek Κύπρος which is Cyprus in
English.
The island where western ancients got their metals from.
And emia meaning presence in blood.
High copper presence in blood.
But where did this copper come from, she ate potatoes and why is it causing these problems?
At this realization, CG’s dad tells the medical team that he had gotten sick from
the potato salad, in a similar fashion, just not as severe.
He recalled that he used a copper pot and utensils that he bought from an antique store
to prepare the pickles he put in that potato salad.
But how does copper from a pot and tongs, get into pickles?
Well, there’s some basic chemistry to be known here.
Copper when exposed to oxygen and moisture in the air, reacts.
This can happen to pots if they’re not coated well.
If it’s antique, there’s a good chance, then there's a good chance it wasn't coated well (or at all).
The moisture from condensation in the pot, likely reacted with the copper and created
something called copper oxide.
This is exactly how old pennies got dirty, because they’re made of copper too.
When pickling vegetables, vinegar is used.
Vinegar is an acid.
Vinegar, reacts with copper oxide, picking it off the pot,
and putting it in the pickles, and then into the potato salad.
In the body, copper disrupts normal functions.
Because CG ate it in the potato salad, it traveled from her stomach, to her liver.
And just like how it reacted with oxygen when it was in the pot, it reacts with oxygen in
her liver, creating reactive oxidative species, that destroy everything.
For CG, her mitochondria cannot be the powerhouse of the cell because this reactive oxygen,
disturbs energy production.
Disrupting this metabolism, damages the cell.
Additional injury is delivered to the DNA, as CG’s liver cells all start to burst.
And as this process happens at a cellular level, the liver tissue becomes injured, causing problems with her blood clotting factors
causing problems with albumin production
causing impaired uptake of bilirubin, causing jaundice
everything that is happening to CG.
If too much copper is her problem, is there a way we could somehow remove that copper
from her body?
Maybe.
Because copper likes to react with oxygen, and it does so by pulling electrons away,
then we can ask a heavier element, similar to oxygen, to react with copper instead?
On the outside of this molecule, we want it to dissolve in water, so that it can be removed
by the kidneys, and put in the urine.
CG was given Penicillamine.
Not the antibiotic penicillin.
Penicillamine.
The sulfur and nitrogen capture the copper, and the entire complex is filtered by her
kidneys, and released in her urine.
After 8 days in the hospital, CG was discharged, as she seemed to make a recovery.
More visits with her dad prompted her to never again, eat any of his cooking, even if he
vowed to never use antiques to cook.
But as the weeks go by, CG doesn’t seem to be ok.
After discharge from the hospital, that deterioration in balance, gait and speech she had before
eating the potato salad, was getting worse.
Even though she never again ate her dad’s food, her mom started noticing her jaundice
again.
At school, rumors started going around that CG was going to have a baby.
Her stomach had a bump.
She’d be found before class in the bathroom, huddled over the toilet, with what looked
like morning sickness.
And during class, she had to leave the room, because she couldn’t stop sweating.
Back in the emergency room again, CG tells the doctors about a burning in her stomach.
She is found to have ataxic gait.
A meaning without and taxic meaning order.
Gait being her walk.
It was awkward, imbalanced, disorderly.
This was combined with dystaxic handwriting and slurred speech, all of which can point
to some potential neurodegeneration, not found when she ate that potato salad.
meaning something has been happening since then
Jaundice and ascites are found, again.
A blood test this time reveals that her clotting factors, are just not working properly now
(students- INR limit for acute liver failure?).
They were borderline last time, but it’s clear now that something’s wrong.
Usually, a liver problem on this scale like this would cause mental status change (hepatic encephalopathy)
This is because the liver processes ammonia that comes from a breakdown of proteins in
your GI tract.
But if the liver is failing, this ammonia spills into the body, and goes to the brain.
But now the question is, does CG have liver injury, or liver failure?
It’s important to make the distinction because failure is much more severe than the injury.
Jaundice, ascites, and the blood test confirm something’s wrong with her liver.
The clotting factor problem is an emergency because it can lead to something terrible,
if nothing is done about it.
In adults, it’s hard to qualify liver failure, without the mental status change.
But CG isn’t an adult.
She’s 17.
Her motor dysfunctions are not mental status change.
About half of pediatric patients can present without this, and still have acute liver failure.
And in CG, she has acute liver failure.
Ultrasound finds that her gallbladder is swollen.
Inside, there appears to be a sludge that has been building up over time.
Bile is a fluid made by the liver, and stored in the gallbladder, that helps the body digest
fats.
This sludge means that CG’s liver could be making something, that it shouldn’t be.
A calculation is made on her blood test results.
With 100% specificity and sensitivity, the calculation reveals that CG has Wilson’s
Disease, a genetic disease that prevents the liver from eliminating copper from the body.
(students, what is this Wilson’s disease test?).
She is immediately transferred to the intensive care unit, and evaluated for liver transplantation,
because what’s left of her current liver, is beyond repair.
This Wilson’s disease is something that had been brewing for sometime in CG, and was
made much worse when she ate that potato salad.
In humans, copper has to be shuttled around by proteins.
We’ve already shown how reactive copper is when alone and flowing freely in the body.
But in Wilson’s disease, an inherited genetic defect prevents the correct production of
this protein, meaning its ability to transport copper is impaired.
Causing it to build up.
Causing damage to the liver cells by creating reactive oxidative species.
This forms a sludge in the gallbladder as the acute liver failure in CG causes permanent
damage.
As the copper spills into the bloodstream, it enters the brain.
In normal function, the same protein as found in the liver also transports copper out.
But because of the genetic defect, that doesn’t happen.
Copper accumulates in the brain, and this metal toxicity begins to damage nerve cells, permanently.
Showing up as motor dysfunction in CG.
Copper in Wilson’s Disease often deposits in the eyes as a complex with sulfur, forming a brownish or gray-green ring.
This happens often in Wilson’s disease, but not all the time.
It can also happen when there’s problems of bile flow from the liver, in patients without Wilson's Disease
But in the intensive care unit, the medical team calculates a score to predict her survival,
given the results of her blood test.
(King’s criteria btw).
A score higher than 11 gives little chance of survival without liver transplant.
And in CG, it’s higher than 11.
She is immediately placed, on the liver transplantation list.
Because she was noted to have acute liver failure instead of injury, her placement is
high.
Because her life expectancy is less than 7 days because of the extent of her disease,
she was placed at the very top of the list.
Days pass while she’s in waiting.
A donor was finally located.
A 19 year old who sustained a head injury and passed because of shock.
CG undergoes transplantation and transferred to the transplant unit afterwards.
She received medicines that suppressed her immune system, so that it wouldn’t attack
her new liver.
10 days after the operation, she was sent home, and monitored.
Her new liver was from a donor who didn’t have Wilson’s disease.
Meaning, those liver cells do not have the genetic defect that she inherited, and they
can make the protein that transports copper, curing her from the disease.
But the DNA in her body, still has those genes.
When she has kids, they will at a minimum be a carrier for the defect, if they don’t
have Wilson’s disease themselves.
And if they’re carriers, there’s a chance that CG’s grandkids will be affected in
the same way, as she was when she had this critical illness.
But with the new liver, and support from her family.
With a father who learned to never pickle foods using copper containers, or really,
use anything copper at all for cooking, CG made a good recovery.
Thank you so much for watching.
Take care of yourself and be well.