Let's talk about cystic fibrosis real quick.
Cystic fibrosis obviously is very, very high yield.
It is a genetic disorder. And the genetic disorder here is
actually with the fact that we cannot keep sodium chloride in
our body. And because of the fact that we
lose sodium chloride, sodium, we lose it through the largest
organ of our body. And the largest organ of our
body is the skin. If we're losing sodium chloride
through the skin, guess what follows it?
Water, and as water follows that sodium chloride out the skin,
our body becomes dehydrated. More than likely what they're
going to do is they're going to give you the diagnosis of cystic
fibrosis and most of your questions, that's exactly how
it's going to present. They will give you the
diagnosis. Now, it's your job to understand
that the problem in cystic fibrosis is the fact that I
cannot keep sodium chloride in my body and I lose it through my
skin and water follows sodium, so I become severely dehydrated.
Think about the amount of sodium chloride that actually leaves
the skin and how much water is actually going to follow it.
So we must understand that the first sign and symptom that I
want to look at is the fact that we're going to have severe
dehydration, which is going to lead to the complications that
we're going to get into. But that dehydration, let's talk
about those. If we're talking about a
pediatric patient, signs and symptoms of dehydration include
how about decreased tears? How about dry mucous membranes?
How about poor skin turgor? Or another way to say that is
how about skin tenting, low blood pressure and they're going
to be tachycardic signs and symptoms of that dehydration
that we talk about everywhere. Now think about this.
Where does dehydration show up? Literally in every system I can
think about. We're here in a genetic disorder
that we're losing sodium chloride and water.
But what about a patient that's an athlete that's running around
in the heat, maybe a heat stroke, maybe a patient that's
pregnant? How about something like morning
sickness? How about a gastroenteritis or
pyloric stenosis? There's so many ways.
How about like a DKA where they become dehydrated?
In all these situations, dehydration is so prevalent that
it's a board favorite. The second thing is we want to
identify how this is going to affect our serum, our blood, and
our urine. Osmolality, the concentration of
our serum and our urine is going to be affected if we're
dehydrated. So let's think about this.
If I'm dehydrated and I'm losing blood or sorry and I'm losing
water because of this genetic disorder, is my blood going to
start to look thicker? Yeah.
How do I know that? Well, if I don't have fluid or
water in my body, my blood becomes more concentrated, my
serum becomes more concentrated, my serum osmolality increases.
How do I see that? How about my H&H, my hemoglobin,
hematocrit? You think those relatively look
higher now? Absolutely.
How about my red blood cells? Do they look more dense and
compacted and concentrated? Yes.
So we see elevated red blood cell count as well too.
So again, a great way for us to see signs and symptoms of cystic
fibrosis, dehydration, the losing that sodium chloride.
Look, what I'm doing right now is I'm building you a case
study. The first thing I did was how
about these signs and symptoms of dehydration?
How about a history of a genetic disorder?
How about the fact that I have to diagnose this as cystic
fibrosis? This is a perfect case study in
the making. And can I do this with a
adolescent patient that has a gastroenteritis and I see
dehydration or a patient that has a pyloric stenosis or
interception or any other form of dehydration because they're
vomiting or they have diarrhea? Absolutely I can.
So this is your matrix or grid that I'm developing in that same
case study. So you see, as you start to
think like this, you're actually going to see how the boards can
ask a next Gen. question. It can also affect the urine.
Look, I tell you all the all the time that what I was in MA,
right? For the longest time I was in
MA. So I used to do urine analysis.
And if you look at a patient who's dehydrated, look at their
urine, it's darker in color, tea colored, it's foul smelling.
The amount is less when they do a urine sample.
We have a high urine osmolality, which means that my urine
specific gravity, the the way that gravity specifically is
going to work on that urine is going to be elevated because
it's more dense, it's more concentrated.
My urine osmolality is also increased as well too.
That's just some signs and symptoms.
Now let's take it a step further.
If I'm dehydrated, not only does everything in my body become
thick and dense and concentrated and viscous, now I start to
develop this viscous, this sticky mucus plugs.
And those mucus plugs are going to affect the lungs and the
pancreas. So let's start with the
pancreas. With the pancreas, we're talking
about pancreatic enzymes. The efficacy of the pancreas
decreases. And what are those enzymes?
Amylase and lipase. So if we're not making
pancreatic enzymes, are we going to be able to digest or
breakdown food? No, But now it becomes this
viscosity that is increased because I'm dehydrated plugging
up my pancreas. And as it plugs up my pancreas,
I'm not making amylase and lipase the way that I should.
And now I'm not making or I don't have the ability to
breakdown food and digest food and, and vitamins and minerals
and all the different things that I need to be able to stay
nourished. So this becomes now a process of
malnourishment and malnutrition. So what do I do?
Go back to the concept I always teach you.
Give them what they don't have. What don't they have?
Well, they can't keep sodium in. Give them what they don't have
two to three times sodium. They don't have water.
Give them fluids. Give them what they don't have.
They can't make pancreatic enzymes.
Give them what they don't have, but educate, educate.
So do you think that, and here goes another part of your case
study, how about anticipated and not anticipated?
How about pancreatic enzymes? Anticipated.
Absolutely. What if I made it a little bit
different? What if I made it pancreatic
enzymes as scheduled, with or without food?
Is that anticipated? No.
Why? The only time we give pancreatic
enzymes is with a snack or meal because those pancreatic enzymes
don't know the difference between our intestines, our
internal organs versus the food that it's digested with, right.
So the ultimate goal is to be able to digest the food because
it became a situation of malnourishment, malnutrition.
So again, that anticipated, not anticipated.
So we give them what they don't have.
The second complication that arises from cystic fibrosis,
this is a part where most of our cystic fibrosis patients
actually end up passing away from and it is respiratory
complications because those mucus plugs are now plugging the
lungs and our ventilation perfusion.
Now look, I'm with cystic fibrosis now, but I just went
into respiratory. How about that ventilation,
perfusion mismatch? You think those plugs are going
to decrease ventilation and perfusion?
Absolutely. So in this situation, what do we
do? Well, we educate them.
How about changing positions? How about percussion?
How about oxygen as needed? So educate, educate, educate on
how to reduce the risk. Look at that part of your CPR.
How about manage their care? How about their basic care and
comfort here position? Oh, how about percussion?
Yeah, how about that oscillator? Yeah, absolutely.
When do we do it? Well, how about before bed, upon
awakening, when they need it. Basic care and comfort,
management of care. All these different things that
are so important in taking care of this patient with cystic
fibrosis because we don't want them to become hypoxemic over
the course of time because they will develop frequent
respiratory infections. And now we're worried about this
patient deteriorating rapidly. So that is a quick little review
on cystic fibrosis and how the boards would ask it.