 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 are 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 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 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 we that there's 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, there will be wheezing because we have that mucus production. And initially, there will be a dry and nonproductive 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 Ronchi and Ronchi 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 dysmia or tachypnea. And over time, because again, this is a chronic condition, it can lead to emphysema and adelektosis, as well as corpominal. And corpominal is an expression or another term for right sided heart failure. So this is basically over years when the lungs have been chronically congested, all this mucus is 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 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 emphysema is caused by smoking, but in this case, cystic fibrosis is the culprit. So for 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 that's therefore the patient will have statoria 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. There 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're just overproducing 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 still, they'll kiss their infant and they'll 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 pylocarpine ionotrophoresis tests. I know it's a mouthful, but it basically measures the sodium and chloride content of the sweat. Then there's a 72 hour FICO 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, some 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 it is a genetic disorder and there's currently no treatment to fix these endocrine 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. 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 the 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 40s 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 the stick 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 on 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 arrow because 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 the 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 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're 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.