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Let's talk about cystic fibrosis
real quick. 

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Cystic fibrosis obviously is 
very, very high yield. 

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It is a genetic disorder. 
And the genetic disorder here is

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actually with the fact that we 
cannot keep sodium chloride in 

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our body. 
And because of the fact that we 

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lose sodium chloride, sodium, we
lose it through the largest 

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organ of our body. 
And the largest organ of our 

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body is the skin. 
If we're losing sodium chloride 

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through the skin, guess what 
follows it? 

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Water, and as water follows that
sodium chloride out the skin, 

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our body becomes dehydrated. 
More than likely what they're 

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going to do is they're going to 
give you the diagnosis of cystic

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fibrosis and most of your 
questions, that's exactly how 

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it's going to present. 
They will give you the 

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diagnosis. 
Now, it's your job to understand

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that the problem in cystic 
fibrosis is the fact that I 

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cannot keep sodium chloride in 
my body and I lose it through my

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skin and water follows sodium, 
so I become severely dehydrated.

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Think about the amount of sodium
chloride that actually leaves 

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the skin and how much water is 
actually going to follow it. 

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So we must understand that the 
first sign and symptom that I 

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want to look at is the fact that
we're going to have severe 

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dehydration, which is going to 
lead to the complications that 

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we're going to get into. 
But that dehydration, let's talk

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about those. 
If we're talking about a 

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pediatric patient, signs and 
symptoms of dehydration include 

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how about decreased tears? 
How about dry mucous membranes? 

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How about poor skin turgor? 
Or another way to say that is 

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how about skin tenting, low 
blood pressure and they're going

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to be tachycardic signs and 
symptoms of that dehydration 

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that we talk about everywhere. 
Now think about this. 

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Where does dehydration show up? 
Literally in every system I can 

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think about. 
We're here in a genetic disorder

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that we're losing sodium 
chloride and water. 

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But what about a patient that's 
an athlete that's running around

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in the heat, maybe a heat 
stroke, maybe a patient that's 

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pregnant? 
How about something like morning

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sickness? 
How about a gastroenteritis or 

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pyloric stenosis? 
There's so many ways. 

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How about like a DKA where they 
become dehydrated? 

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In all these situations, 
dehydration is so prevalent that

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it's a board favorite. 
The second thing is we want to 

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identify how this is going to 
affect our serum, our blood, and

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our urine. 
Osmolality, the concentration of

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our serum and our urine is going
to be affected if we're 

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dehydrated. 
So let's think about this. 

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If I'm dehydrated and I'm losing
blood or sorry and I'm losing 

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water because of this genetic 
disorder, is my blood going to 

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start to look thicker? 
Yeah. 

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How do I know that? 
Well, if I don't have fluid or 

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water in my body, my blood 
becomes more concentrated, my 

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serum becomes more concentrated,
my serum osmolality increases. 

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How do I see that? 
How about my H&H, my hemoglobin,

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hematocrit? 
You think those relatively look 

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higher now? 
Absolutely. 

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How about my red blood cells? 
Do they look more dense and 

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compacted and concentrated? 
Yes. 

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So we see elevated red blood 
cell count as well too. 

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So again, a great way for us to 
see signs and symptoms of cystic

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fibrosis, dehydration, the 
losing that sodium chloride. 

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Look, what I'm doing right now 
is I'm building you a case 

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study. 
The first thing I did was how 

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about these signs and symptoms 
of dehydration? 

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How about a history of a genetic
disorder? 

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How about the fact that I have 
to diagnose this as cystic 

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fibrosis? 
This is a perfect case study in 

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the making. 
And can I do this with a 

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adolescent patient that has a 
gastroenteritis and I see 

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dehydration or a patient that 
has a pyloric stenosis or 

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interception or any other form 
of dehydration because they're 

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vomiting or they have diarrhea? 
Absolutely I can. 

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So this is your matrix or grid 
that I'm developing in that same

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case study. 
So you see, as you start to 

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think like this, you're actually
going to see how the boards can 

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ask a next Gen. question. 
It can also affect the urine. 

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Look, I tell you all the all the
time that what I was in MA, 

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right? 
For the longest time I was in 

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MA. 
So I used to do urine analysis. 

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And if you look at a patient 
who's dehydrated, look at their 

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urine, it's darker in color, tea
colored, it's foul smelling. 

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The amount is less when they do 
a urine sample. 

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We have a high urine osmolality,
which means that my urine 

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specific gravity, the the way 
that gravity specifically is 

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going to work on that urine is 
going to be elevated because 

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it's more dense, it's more 
concentrated. 

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My urine osmolality is also 
increased as well too. 

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That's just some signs and 
symptoms. 

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Now let's take it a step 
further. 

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If I'm dehydrated, not only does
everything in my body become 

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thick and dense and concentrated
and viscous, now I start to 

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develop this viscous, this 
sticky mucus plugs. 

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And those mucus plugs are going 
to affect the lungs and the 

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pancreas. 
So let's start with the 

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pancreas. 
With the pancreas, we're talking

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about pancreatic enzymes. 
The efficacy of the pancreas 

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decreases. 
And what are those enzymes? 

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Amylase and lipase. 
So if we're not making 

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pancreatic enzymes, are we going
to be able to digest or 

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breakdown food? 
No, But now it becomes this 

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viscosity that is increased 
because I'm dehydrated plugging 

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up my pancreas. 
And as it plugs up my pancreas, 

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I'm not making amylase and 
lipase the way that I should. 

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And now I'm not making or I 
don't have the ability to 

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breakdown food and digest food 
and, and vitamins and minerals 

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and all the different things 
that I need to be able to stay 

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nourished. 
So this becomes now a process of

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malnourishment and malnutrition.
So what do I do? 

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Go back to the concept I always 
teach you. 

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Give them what they don't have. 
What don't they have? 

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Well, they can't keep sodium in.
Give them what they don't have 

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two to three times sodium. 
They don't have water. 

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Give them fluids. 
Give them what they don't have. 

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They can't make pancreatic 
enzymes. 

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Give them what they don't have, 
but educate, educate. 

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So do you think that, and here 
goes another part of your case 

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study, how about anticipated and
not anticipated? 

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How about pancreatic enzymes? 
Anticipated. 

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Absolutely. 
What if I made it a little bit 

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different? 
What if I made it pancreatic 

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enzymes as scheduled, with or 
without food? 

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Is that anticipated? 
No. 

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Why? 
The only time we give pancreatic

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enzymes is with a snack or meal 
because those pancreatic enzymes

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don't know the difference 
between our intestines, our 

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internal organs versus the food 
that it's digested with, right. 

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So the ultimate goal is to be 
able to digest the food because 

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it became a situation of 
malnourishment, malnutrition. 

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So again, that anticipated, not 
anticipated. 

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So we give them what they don't 
have. 

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The second complication that 
arises from cystic fibrosis, 

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this is a part where most of our
cystic fibrosis patients 

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actually end up passing away 
from and it is respiratory 

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complications because those 
mucus plugs are now plugging the

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lungs and our ventilation 
perfusion. 

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Now look, I'm with cystic 
fibrosis now, but I just went 

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into respiratory. 
How about that ventilation, 

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perfusion mismatch? 
You think those plugs are going 

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to decrease ventilation and 
perfusion? 

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Absolutely. 
So in this situation, what do we

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do? 
Well, we educate them. 

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How about changing positions? 
How about percussion? 

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How about oxygen as needed? 
So educate, educate, educate on 

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how to reduce the risk. 
Look at that part of your CPR. 

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How about manage their care? 
How about their basic care and 

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comfort here position? 
Oh, how about percussion? 

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Yeah, how about that oscillator?
Yeah, absolutely. 

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When do we do it? 
Well, how about before bed, upon

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awakening, when they need it. 
Basic care and comfort, 

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management of care. 
All these different things that 

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are so important in taking care 
of this patient with cystic 

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fibrosis because we don't want 
them to become hypoxemic over 

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the course of time because they 
will develop frequent 

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respiratory infections. 
And now we're worried about this

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patient deteriorating rapidly. 
So that is a quick little review

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on cystic fibrosis and how the 
boards would ask it.