Med-Surg Respiratory System, part 6: Cystic Fibrosis

by Cathy Parkes August 23, 2021 Updated: December 29, 2021 4 min read 1 Comment

In this article, we'll cover the key facts you need to know about cystic fibrosis for your Med-Surg exams and nursing practice, including signs and symptoms, labs and diagnostics, treatment, and nursing care.

The Med-Surg Nursing video series follows along with our Medical-Surgical Nursing Flashcards, which are intended to help RN and PN nursing students study for nursing school exams, including the ATI, HESI, and NCLEX.

Cystic fibrosis is a genetic disorder that severely impairs lung function and causes dysfunction in other organs and tissues that make mucus or sweat.

In cystic fibrosis, autosomal recessive disorder causes obstruction of sodium transport within cell membranes, producing secretions with low water content. This results in abnormally thick, sticky mucus that plugs organ ducts of the pancreas, lungs, liver, small intestine, and reproductive organs and can lead to failure of those organs.

Signs and symptoms

The signs and symptoms of cystic fibrosis include respiratory, gastrointestinal, and integumentary effects.

Respiratory

The respiratory signs and symptoms of cystic fibrosis are wheezing, coughing, dyspnea (difficulty breathing), mucus plugs, cyanosis (blue skin), barrel-shaped chest, clubbing, and chronic respiratory infections.

Gastrointestinal

The gastrointestinal signs and symptoms of cystic fibrosis include steatorrhea, delayed growth, and fat-soluble vitamin deficiency (deficiencies in vitamins A, D, E and K).

Steatorrhea

Steatorrhea is fatty, malodorous stool. Cystic fibrosis can lead to pancreatic insufficiency, and if the pancreas cannot digest fat properly, inordinate amounts of it will remain in the stool.

Integumentary

The integumentary, or skin-related, signs and symptoms of cystic fibrosis include high sodium content in the sweat, saliva, and tears.

Labs and diagnostic tests

Labs and diagnostic tests that are available for cystic fibrosis include the sweat chloride test, DNA testing, pulmonary function tests, and a stool analysis.

Treatment

There are both pharmacological and procedural treatments for cystic fibrosis.

Medications

The medications that are available for cystic fibrosis include bronchodilators like albuterol and salmeterol, anticholinergics like ipratropium, dornase alfa (which helps to thin the secretions in the respiratory tract), antibiotics for pulmonary infections, mucolytics like acetylcysteine, and pancreatic enzymes like pancrelipase to take with meals and snacks.

These important medications are covered in our Pharmacology Flashcards for Nursing Students.

Procedure: Chest physiotherapy

Chest physiotherapy is another method available for treating cystic fibrosis. Chest physiotherapy uses percussion, vibration, postural drainage, and breathing exercises to loosen respiratory secretions. Chest physiotherapy sessions should be scheduled before meals, or 1-2 hours after meals, once food has been digested, to avoid the possibility of vomiting.

Patients should use a bronchodilator 30 minutes to 1 hour before treatment.

Nursing care

For nursing care of a patient with cystic fibrosis:

  • Administer oxygen
  • Encourage increased fluid intake
  • Encourage a higher protein and calorie diet.
  • Provide supplements, including fat-soluble vitamins A, D, E, and K.

Full Transcript

Hi, I'm Cathy with Level Up RN. In in this video, we are going to talk about cystic fibrosis. And at the end of the video, I'm going to give you guys a little quiz to test your understanding of some of the information I'll be presenting here in the video. So definitely stay tuned for that.

So cystic fibrosis is a genetic disorder. It's an autosomal recessive disorder that severely impairs lung function. And it also affects the function of other organs as well. With cystic fibrosis, we have abnormally thick sticky mucus that really plugs the organ ducts in the lungs, in the pancreas, in the liver, in the small intestine, and in the reproductive organs. And this can lead to organ failure.

In terms of symptoms, we have a lot of respiratory symptoms, which include wheezing, coughing, dyspnea, mucus plugs, cyanosis, a barrel-shaped chest, clubbing, and chronic respiratory infections. And then we will have a lot of gastrointestinal symptoms as well, which can include steatorrhea, which is like fatty malodorous stool, delayed growth, and a deficiency in fat-soluble vitamins, which include vitamins A, D, E, and K. And then in terms of the skin, a patient with cystic fibrosis will have abnormally high sodium chloride content in their sweat, saliva, and tears. Diagnosis of cystic fibrosis can include a sweat chloride test, DNA testing, pulmonary function test, as well as a stool analysis.

In terms of treatment, there are a variety of medications that can be helpful with cystic fibrosis. This includes bronchodilators, anticholinergics, dornase alpha, which helps to thin the secretions in the respiratory tract, mucolytics, as well as antibiotics for pulmonary infections. And then we would also give the patient pancreatic enzymes with their meals and snacks to help break down and digest their food because pancreatic function is impaired with that thick sticky mucus.

In terms of procedures, chest physiotherapy is definitely going to be helpful for your patient with cystic fibrosis. Chest physiotherapy involves the use of percussion, vibration, postural drainage, and breathing exercises to help loosen up those respiratory secretions. So you want to make sure you schedule your patient's chest physiotherapy sessions before meals or several hours after meals so that they don't vomit while they're doing these different exercises. And then you also want to give them a bronchodilator treatment 30 minutes to one hour before they're going to have their chest physiotherapy. In terms of nursing care of cystic fibrosis, you're going to administer oxygen as ordered. You're going to encourage the patient to increase their fluid intake as well as their protein and calorie intake. And then you want to provide fat-soluble vitamin supplements as well. So this is vitamins A, D, E, and K.

Okay, time for a quick quiz. I have three straightforward questions for you guys. No select all that apply or case studies. Just trying to test your understanding of the key facts I discussed in the video.

Question number one: fatty malodorous stools are an expected finding in a patient with cystic fibrosis. True or false?

The answer is true.

Second question: when should your patient take their pancreatic enzymes?

The answer is with their meals and snacks.

And then question number three: chest physiotherapy should be scheduled directly after meals. True or false?

The answer is false because the patient may vomit [laughter] with their getting chest physiotherapy right after they eat. So you want to schedule it before meals or several hours after meals.

Okay. That's it for cystic fibrosis. Hope you enjoyed this video. Be sure to like the video and leave me a comment and let me know what was most helpful for you. Thank you so much for watching.


1 Response

Thelia
Thelia

September 07, 2021

I love the way you explain the condition that you’re highlighting in your videos. It is easy to understand because you don’t complicate the topic, but go about explaining it in a very logical manner. Although I have already graduated nursing school, I am using your videos and lectures to review many nursing topics and it really helps me to take notes about the topic as you are explaining it. Thank you for being so thorough!

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