A starving mom suddenly ate 40 cookies,
this is what happened to her heart.
MJ is a 35 year old woman,
presenting to the emergency room delirious,
the paramedics found her on the street
lying on the ground, shaking with several empty boxes of cookies and her children.
You see,
MJ was a homeless woman who had been starving for more than a year,
she had no legal spouse, didn't eat very often, and when she did have food,
she'd give it to her kids.
A few months ago, she made it to a major city,
around this time, she had become accustomed to not eating much.
In fact, the few times she did eat,
she would feel dizzy and thirsty afterwards, her heart rate would increase,
she would feel uncomfortable, so she opted to not eat much; if at all on most days.
During this time, MJ had lost a significant portion of her body weight,
at 5' 5" tall, she weighed a total of 75 pounds,
that's nearly half of her ideal body weight.
Finally, one hot summer day, MJ felt exhausted,
she received a few boxes of cookies late at night from a local bakery,
they had past their sell-by date, so it was typical for the bakery to give the food away.
In a strange deviation from her recent behavior of not eating much,
she had a taste of one of the cookies, she enjoyed it,
then she had a taste of another, and another, and another.
She looked away, kept eating and without paying attention in 30 minutes,
she had finished a total of 30 cookies by herself.
It's an hour later,
her kids are asleep and MJ feeling lethargic, her vision is blurred,
she thinks she feels a little dizzy, but is unsure,
she feels that she's just tired and now that she's eating well for the first time in years,
she can finally have a good sleep.
It's morning now,
MJ wakes up, she didn't sleep well,
she eats 10 more cookies for breakfast.
As she walks on the street throughout the day,
she feels her heart fluttered,
she has a headache, her field of vision starts to blur and warp, she's dizzy,
collapses on the street and starts seizing.
Her kids cry out for help and an ambulance is called
where she's brought to the emergency room where we are now.
Given this past medical history,
there's a few glaring clues as to what's happening:
most immediately, MJ is suffering from Refeeding Syndrome.
The name is intuitive, there's 40 cookies that she ate,
and they're a form of refeeding,
this is the first time, that she's had a major oral intake of calories in over a year.
"Syndrome" refers to the grouping of symptoms
that she's having related to this refeeding.
But how could this be?
Even if it was just cookies, eating something should be better than nothing, right?
Well, not quite,
and it's less the fact that cookies are unhealthy for you
and more so the changes in MJ's physiology since she is gone in starvation.
Let's look at this clinically,
first:
her body has been malnourished for a long period of time
and this is physically evident.
At 5' 5" tall, her ideal body weight is 125 pounds,
that number is calculated and standardized in humans
and we used this in clinic to assess status and to dose drugs.
She weighs 75 pounds
60% of her ideal and this is a problem, because under 70% is the threshold,
where someone would be at risk for Refeeding Syndrome,
and she's there.
On presentation to the emergency room, she's hypothermic,
meaning her body temperature is low as she was found shivering,
her blood indicates that her liver enzymes are elevated
and her kidney function is abnormal,
these are typical symptoms for presentation of a chronically starving patient.
She has to some extent compensated for these losses over time;
her baseline heart rate before refeeding was 35 beats per minute,
that could be normal for a well-trained athlete, but MJ certainly isn't one at the moment.
She should be somewhere between 60 to 100 beats per minute,
like most people and at presentation, she has 70 beats per minute,
so she should be okay, right?
Actually, no.
For patients in starvation, whether it's rooted in eating disorder,
"dysphagia," known as an inability to eat due to difficulty swallowing,
or rooted in a different etiology,
bradycardia is very typical in patients like MJ.
"Brady" meaning slow and "cardia" referring to heart rate,
slow heart rate typically defined as under 60 beats per minute,
in extreme starvation, it can dip to below 30 beats per minute,
meaning one beat every two seconds or longer.
Baseline heart rate of 35 now up to 70 with a sudden refeeding
is an extreme change in a short amount of time,
educating a major cardiovascular complication,
her heart EKG shows that her heart is beating irregularly.
But why is this happening?
We can start to explain it through the delirium and the seizure
that she suffered when she arrived to the emergency room,
Because she's been in starvation,
she has vitamin deficiencies, the important one here being thiamine or vitamin B1.
Thiamine isn't produced endogenously,
meaning that your body doesn't produce it by itself,
we need to source it from your dietary intake.
It's important in cells, it helps promote the breakdown of sugars
and plays a central role in cerebral energy utilization
Its deficiency initiates the injury of brain cells by inhibiting metabolism,
causing MJ's delirium and her seizures.
But thiamine isn't active by itself,
it's usable in your cells in a form known as thiamine pyrophosphate.
Phosphate,
that is the root of the problem here,
MJ is suffering from hypophosphatemia.
"Hypo" meaning low,
"phosphate" referring to the important anion that exists mainly inside of your cells,
and "emia" meaning presence in blood.
Low phosphate presence in blood.
This has severe consequences in multiple aspects of MJ's biology,
one major contribution to her physical decay from malnutrition is muscle atrophy.
"A" meaning without, and "trophy" referring to nourishment,
her muscles have broken down over time because she hasn't been eating.
But this isn't just limited to the muscles we can see just by looking at her though,
what we would call skeletal muscle,
the smooth muscle of her stomach and intestines have atrophied and most importantly,
her heart muscle has atrophied.
What does this have to do with phosphate?
Well, muscle tissue is the prime utilizer of it,
in the blood, there's a few important electrolytes involved in muscle contraction:
sodium and potassium signal for a contraction,
calcium commits to a contraction,
and phosphate provides the energy for the contraction.
You may have heard of it, it's called ATP,
adenosine triphosphate.
Adenosine being an adenine molecule, that's the a in your genetic information
attached to a ribose sugar and triphosphate,
three phosphates.
That energy provided by ATP is responsible for almost all movement in your body,
whether it's food through your stomach,
blood pumped to your brain, or you walking on the street.
But have you noticed when you walk faster on the street
that you start to breathe heavier and deeper?
In a state of activity,
your muscles need more oxygen to produce more ATP to meet energy demand
and in order to provide that oxygen,
the body uses another phosphate-containing compound
known as 2,3-Diphosphoglycerate,
the name tells you exactly what it is.
Glycerate, with two phosphates on carbons number two and three.
2,3-DPG, in red blood cells potentiates oxygen dissociation,
increasing availability of oxygen to tissues like the muscle from the blood,
that oxygen is used to create more ATP.
So all kinds of muscle are very dependent on phosphate
to provide energy for contraction.
But let's go back to the name of MJ's condition: hypophosphatemia
low phosphate presence in blood.
Why is the phosphate low?
She just ate 40 cookies,
so everything in terms of electrolytes and nutrients should be high in her blood, right?
Actually, no.
The cookies MJ ate were high in sugar
sugar mainly glucose strongly stimulates the pancreas
to release a hormone known as insulin,
that decreases blood glucose by shifting it into the body cells from the blood.
In a healthy person, insulin release is highly regulated in very precise,
you get exactly what you need.
But MJ isn't healthy, she's malnourished,
40 cookies totaling 1200 grams of sugar within 12 hours
is a huge shock to her system,
her body is sensitive to insulin,
meaning small amounts of it
will have a greater impact on her body than if she were healthy.
This happened over a long period of time, as MJ ate less and less,
she felt dizzy after eating those cookies,
because her blood sugar became lower than before she ate them,
due to this massive insulin response.
But it's not just glucose shifting into her cells,
the electrolytes in her blood:
sodium, potassium, calcium, magnesium, and phosphate,
they're all shifting out of the blood and into her cells.
Most of these nutrients are literally getting sucked out of her blood
and shoved into her cells,
this signals the beginning of MJ's heart problems.
Because the time scale is short,
the movement of sodium
into all cells of the body, not just the muscle gets detected by the kidney,
they respond by reabsorbing the sodium that would have shown up in the urine,
a constant of this is that waterfall of sodium and is retained in the body.
This begins a process of volume overload inside MJ,
as more and more fluid begins to accumulate in her body.
Fluid that should have been urinated out,
her heart is starting to have difficulty handling it,
she exhibited signs of this when her heart was fluttering in the morning,
there's congestion in her heart.
That extra fluid accumulating in her body
is more than her heart can handle,
and she's starting to show signs of acute decompensated heart failure.
"Decompensated" means that the heart hasn't had enough time
to adapt to the changes in her body
and "acute" meaning an underlying physiologic change was recent due to an event,
in this case, the eating of 40 cookies in a person who was previously starving.
This isn't a one-dimensional problem, because her heart muscle has atrophied,
it's not as strong as it would be if she were well fed,
because phosphorous is shifting intracellularly due to high insulin response,
the heart muscle has less phosphate available to it from the blood,
meaning less production of ATP for energy
and less 2,3-DPG for oxygen dissociation to produce that ATP.
Her heart muscle has a higher workload through an overload in fluid
and less energy to provide work to that load,
this still isn't the end of the story.
the muscle that draws air into the lungs the diaphragm
in MJ's case, it too has atrophied in her starvation
and with less phosphorous available to it means she has dyspnea.
"Dys" meaning abnormal and "pnea" meaning breath,
it's not only harder for her to provide energy to her muscles,
but she also can't breathe in enough oxygen with those muscles.
This could have been avoided,
if MJ ate slowly and didn't succumb to her appetite.
Refeeding Syndrome isn't new to humans, in 100 A.D.,
the Jewish Roman scholar, Flavius Josephus,
described an epidemic of deaths among Jews
who had been entrapped and starved by the Romans,
he wrote that those who engorge themselves died,
but the ones who restrained their appetite
in the face of abundant food were able to survive.
In 600 A.D. China,
Sun Si Miao, the most influential physician in East Asia for a thousand years,
wrote about Refeeding Syndrome in context of beriberi.
The symptoms caused by thiamine deficiency,
it was a problem in rural China at the time
as peasants were often only allowed to eat white rice
and the removal of its husk depleted its primary source thiamine.
And when there wasn't enough food due to natural disaster,
people starved and faced Refeeding Syndrome when food was available again.
In World War II, prisoners of war were liberated in 1945 and those who were fed
paradoxically died after eating.
And this was the first modern description of Refeeding Syndrome,
even today, Refeeding Syndrome isn't well known outside
of nutritional support circles in sight clinic.
Cancer patients commonly have dysphagia
or difficulty swallowing due to their treatments,
or stomatitis, a mouth sore and common side effect of treatment.
They typically don't eat much and in effect they become malnourished,
when hospitalized, they're typically fed entirely through a tube
or parenterally through their veins and they get a sudden bolus of nutrients
that their body isn't used to,
causing insulin response, the intracellular shift of electrolytes and glucose,
causing Refeeding Syndrome.
If a person who is previously starving, refeeds too suddenly,
they're put at an incredible risk
of dying inside a decayed form of their own body.
For MJ, we treat her underlying conditions namely her hypophosphatemia,
this is easier said than done.
Since her levels are extremely low due to the nature of her Refeeding,
we give her intravenous phosphate, but only up to a limit,
because she's delirious, oral intake is likely unreliable for her,
but IV administration is only for a short amount of time.
The reason being is that IV phosphate is very dangerous,
as it can form solid salts with the calcium in the blood,
which can damage the kidneys causing renal failure and hypocalcemia,
or low calcium presence in blood.
Since muscle cells use calcium to commit to a contraction,
when it's low in blood that too can cause problems in MJ's heart,
we switch to oral repletion of phosphate when the levels begin to increase.
For potassium and magnesium,
we replace those intravenously as well,
but we do it in a saline solution without dextrose,
in some cases, students and residents will want to replete the electrolytes
in a 5% dextrose solution,
but it's contraindicated in this case.
Dextrose is the D-isomer of glucose,
presence of dextrose will stimulate insulin release,
which got MJ into the trouble that she's in right now,
by treating the underlying electrolyte abnormalities and later, thiamine deficiency,
MJ was able to resolve her feeding syndrome
and subsequent cardiac dysfunction and encephalopathy.
What happened to her and her kids afterwards?
Well, that's a part of the story that you can help write.
Thanks so much for watching.
Take care of yourself and be well.
English CC by Charles Baluyot