Hi and welcome to Nursing School Explained.
Today's topic is cystic fibrosis. Cystic fibrosis is a genetic disorder that is
autosomal recessive and it is a disorder of the exocrine glands. So exocrine
glands or glands that secrete something to help the body perform some sort of
function. And in this particular case these exocrine glands of the
bronchioles, small intestine, pancreas and bile ducts are affected. And what
cystic fibrosis does there's an abnormal, a secretion of abnormally thick mucus in
all of these organs. So now when we think about mucus production we always have to
think that something is get getting clogged. So when we have abnormal
secretions, abnormally thick mucus in the lungs, then we have to think about
shortness of breath and then consequences that kind of continue
because this is a chronic genetic disorder that there is no cure
for currently. And so because it affects the bronchioles and then most of the
digestive tract let's look at the signs and symptoms that we will be seeing in
these body systems. So in the respiratory system that will be wheezing because we
have that mucous production. And initially there will be a dry and
non-productive cough and the patient is prone to frequent respiratory infections
because it's a chronic condition and there's all this mucus that's trapping
the bacteria, the viruses that the patient might be encountering and then
it is difficult for the patient to get rid of that mucus so they're at higher
risk for infections and complications from those. In later stages the cough
will turn wet with thick sputum production because we know this these
exocrine glands produce this abnormally thick mucus. There might be crackles or
rhonchi and rhonchi are typically there when there's a lot of sputum or
mucus in the lungs. That's something that we can hear. The
patient will have this dyspnea or tachypnea. And overtime because again this is a
chronic condition it can lead to emphysema and atelectasis as well as cor
pulmonale. And cor pulmonale is an expression or another term for
right-sided heart failure. So this is basically over years when their lungs
have been chronically congested, all this mucus accumulating and the heart is
trying to pump blood to the lungs. So because of all this resistance in the
lungs it's congested so the right heart has to pump harder to get the
fluids pumped into the lungs that are congested and eventually it'll lead to
right ventricular hypertrophy - enlargement of that right ventricle -
because it's just pumping so hard trying to get the blood to flow to these
congested lungs. And over over time it'll lead to that right-sided heart failure.
And then as with any chronic lung conditions over time
signs and symptoms will also include clubbing of the fingers and the barrel
chest. And this is basically something that we also see in patients with
emphysema, where typically a emphysema is caused by smoking but in this case
cystic fibrosis is the culprit. So for the digestive system because there is all
this thick mucus that's blocking the bile ducts and the pancreatic enzymes
from being secreted as well as the small intestine to be sluggish, the patient
will not be able to break down fats. So there's all this congestion in the bile
ducts they can't really get the enzymes out and therefore the patient
will have steatorrhea or fatty stools. Now the patient, because the digestive
system is just not functioning appropriately, will also be at risk for
malnutrition and delayed growth.They will be deficient in fat soluble
vitamins because we just cannot absorb the fat and those are vitamins A, D,
E and K. And the most significant ones here are A which leads to eye tissue
thickening and then vitamin K which can lead to problems with clotting or
bleeding disorders. Now the patient will also have a protuberant abdomen and
thin extremities that's a pretty typical sign of a chronically ill cystic
fibrosis patient. They will have this kind of protuberant abdomen with very
thin extremities and that has to do with all this malnutrition over years that
has accumulated. And they'll also be thin and underweight because they just cannot
absorb the nutrients that a normal child would be able to absorb. And then for
exocrine glands, because exocrine glands are also in the sweat, so there will be
increased sodium and chloride in the sweat because they are just over producing
that, increasing the sweat and therefore the patient is at risk for electrolyte
imbalances. And if the patient has not been diagnosed through a routine newborn
screening, which we'll get to in a moment, then mothers in infants with cystic
fibrosis they will say, they'll kiss their infant and they have this abnormally
salty taste.And that is because of this increased sweat production that these
children have. Now for diagnostic tests, again this is a routine newborn
screening that is done for all children nowadays. And then there is a special
test called the pilocarpine iontophoresis test. I know it's a
mouthful. But it basically measures the sodium and chloride content of the sweat.
Then there is a 72-hour fecal fat determination test to see how bad is the
fat malabsorption that the patient has. We also want to look at liver function
because we have all these digestive organs affected so small intestine, pancreas,
and bile ducts and whenever we think about those three we can think of
something and damage happening to the liver because the bile ducts are not secreting,
they're not functioning appropriately. Now certainly if the patient has
complications or even just symptoms of respiratory, any kind of respiratory
symptoms then they will need a chest x-ray and a sputum culture to see if
there is a bacterial or viral pathogen that's right now residing in the
patient's lungs. As well as pulmonary function tests to measure the ability of
the lungs to expand, the lung volumes, all those things. Now for treatment because
current because it is a genetic disorder and there's currently no treatment to
fix these endocrine endocrine exocrine excuse me exocrine glands, the treatment
goal is to prevent and treat respiratory infections because certainly that's the
most concerning goal if there is a lot of respiratory infections or congestion
that the patient eventually will get a bad respiratory infection that they
might die from. Right so we want to prevent and treat those. We want to
promote optimal nutrition and we'll get into that here in a moment. And then
certainly their psychological adjustment. Not only to the patient but also the
family because this is a lifelong disease and the life expectancy of
children with cystic fibrosis is much shorter than for anybody who doesn't
have cystic fibrosis. It used to be that life expectancy was about in their 20s
and now with the different treatment options this has extended to maybe the
40's but again cases are different depending on the severity and their
ability to manage symptoms. Now how do we manage these patients? So we want to
manage their respiratory signs and symptoms because we have all this thick
mucus production, we want to get rid of it. And the way we do that to mobilize
the secretions, we do chest physiotherapy, postural drainage
and percussion. And if you're not familiar with chest physiotherapy I
would look that up. There's a variety of different options and how that can be
accomplished. Percussion is just as simple as cupping the hand and kind of
drumming on the patient's chest to mobilize these secretions. Postural
drainage means maybe having the patient lay on their stomach maybe with the head
angled down because we want to percuss and mobilize these secretions and
get them up into the upper airway so hopefully the patients can spit them out.
And then regular aerobic exercise is important for any child but particularly
for patients with cystic fibrosis because it's going to help expand the
lungs, prevent them the atelectasis, help on delaying the onset of the emphysema
and all those good things. Now they will certainly need antibiotics and steroids
as needed if they suffer from a complication or even just a very mild
upper respiratory infection. They might need supplemental oxygen as the disease
progresses. And then genetic counseling will be important not only for the
patient as they might come to reproductive age but also to the parents
of this child because we know it's a genetic disorder so we might want to
screen them to see what's the risk of them having another child with this
chronic cystic fibrosis disease. Now for the digestive system because we know
that they are at risk for malnutrition, delayed growth and they have the
deficiency of these fat soluble vitamins. So treatment will be high calorie, high
protein diet to promote their growth and promote their nutritional status. They
will need pancreatic enzymes because where it's affecting the pancreas and
the pancreas and bile ducts are not going to be able to secrete those
digestive enzymes that we need to break down proteins, carbohydrates and fat. So
patients will need supplements of those as well as fat soluble vitamin
supplements because we know that they are deficient in these
vitamins because they have these fatty stools so their body is not really able
to absorb fats which will put them at risk for these fat soluble vitamin
deficiencies. Thank you for watching this video on cystic fibrosis. Please also
watch the other videos in the pediatric playlist that discuss more of the more
acute pediatric respiratory disorders and I will see you next time. Thanks for
watching!