Cystic Fibrosis (CF)

Hey guys! Welcome to your lesson on Cystic Fibrosis or CF as it’s most commonly called.

Cystic fibrosis is a really important diagnosis to be familiar with. You're going to see quite a bit it in real life and it is very testable content!

Cystic fibrosis is an autosomal recessive disorder. It's a life shortening disease with most patients living into the late thirties.

Inn cystic fibrosis there is an amino acid called CFTR, (which stands for cystic fibrosis transmembrane conductance regulator) that is defective. CFTR is responsible for regulating chloride and sodium and when this isn't regulated properly the patient ends up with thick, sticky mucus. This mucus then causes obstruction in various locations in the body- primarily affecting the lungs, intestines, pancreas, liver and sex organs.

We are going to focus on the impact on the lungs and intestines because that’s where most of our nursing care is focused on.

Most newborns are screened for cystic fibrosis. The blood is analysed looking for elevated levels of trypsinogen and further DNA testing can be done looking for the mutated Gene, Δ508, that is responsible for the disease.

If it is not diagnosed with these tests, there are a few symptoms to look out for. The first is that the newborn may have a meconium ileus. This means that they don’t pass their first stool within 24 hours of being born.

The second clinical indicator is parents reporting that their baby tastes salty when they kiss them.

This leads me to the next diagnostic test that you may see you used, it’s called the sweat chloride test or pilocarpine iontophoresis. No one ever calls it that, but I wanted you to at least see it written out here in case you come across it on a really mean test question or something! You can see in the photos here a little device that’s used. It collects sweat and analyzes it for chloride.

For your nursing assessment, I want you to remember that everything we are talking about is happening because of the thick, sticky mucus.

In the respiratory system, the mucus can cause obstruction in the lungs and ultimately lead to frequent lung infection, leading to symptoms like fever, cough, congestion and increased work of breathing.

The more often patients have infections the more problems they will have with long-term damage in their lungs. Over time, they may end up being chronically hypoxic and then you would see signs like clubbing and a barrel chest.

In the GI system the mucus can also cause an obstruction and constipation

The mucus also affects their ability to absorb nutrients so the are at risk for being malnourished. Stools will be large, bulky, frothy and foul smelling. The frothiness of the stool is from undigested fat and the foul smell is from undigested protein.

They also can’t absorb fat soluble vitamins so they will be deficient in vitamins A, D, E and K.

All of those issues together create the potential for failure to thrive. Failure to thrive is a descriptive term used to when babies and kids are losing weight and aren’t meeting growth and developmental milestones.

Therapeutic management focuses on preventing pulmonary complications, like infection, and also preventing malnutrition, weight loss and intestinal obstruction.

We are going to dive into each of those goals a bit more in the next two slides, but I want to point out two other important parts of management. The extra thick mucus also affects the pancreas making these kids prone to developing CF Related Diabetes. So throughout this kids life we have to be on the lookout for those cardinal signs of diabetes, like excessive urination, excessive thirst, extreme hunger and weight loss.

And lastly, CF is a really tough diagnosis for kids to cope with. It really impacts day to day life. Even on the best day, it can take hours to complete the respiratory treatments. Because of this, depression and non-compliance are common issues.

So always be willing to look for ways to modify nursing care to promote quality of life. This is especially important for adolescents coping with this disease. Work with them to schedule treatments around things that are important to them and always include them in the care so they can prepare for adulthood and managing it on their own!

Pulmonary treatments are all about breaking up the thick mucus so patients can get it out of their lungs. This is done through chest physiotherapy. Sometimes patients may use a high frequency chest compression vest. These vibrate the patient’s chest and loosen the mucus. Then, patients cough the mucus out using a flutter device or something called a huff cough to help them expectorate.

We linked to a video in the resources that actually shows an adolescent boy going through his entire chest physiotherapy routine. It shows the vest and also the huff cough if you haven’t seen those in clinical practice.

Medications commonly used are bronchodilators, IV antibiotics and nebulised antibiotics. One thing to know about the bronchodilators is that they should be given right before starting the chest physiotherapy. The reason for this is that it helps open up the lungs so that the mucus can actually break free and loosen during the percussion.

And as you can imagine for a patients with CF it’s very important to always monitor for signs of a respiratory infection and to keep a close eye on their work of breathing as well.

The primary goals for our gastrointestinal treatments are to promote adequate nutrition and prevent weight loss.

This starts with administering pancreatic enzymes. Remember the pancreas is affected as well, and these patients are lacking enzymes that are needed to digest food properly. The enzymes need to be given within 30 minutes of eating and are provided in the form of a capsule. These capsules can be opened up and the medicine can be sprinkled on top of food for younger kids who can’t swallow a capsule.

They also need a supplement of vitamins ADEK because they aren’t able to absorb these fat soluble vitamins.

To help prevent weight loss these patients to be on a high calorie high protein diet.

And of course we need to be monitoring their weight very very closely. It's not uncommon for patients with CF to end up needing a g-tube because they're not able to maintain the caloric intake that they need.

Your priority nursing Concepts for a paediatric patient with cystic fibrosis are oxygenation, gastrointestinal and liver metabolism and gas exchange.
So, there is a lot going on with our patients who have cystic fibrosis so let’s recap the key points for this topic.

CF is an inherited, life-shortening, disease and it causes the body to produce very thick and sticky mucus.

This thick and sticky mucus causes obstruction in the body and the places that it affects the most are the lungs intestines and pancreas.

Treatment is focused on minimising pulmonary complications and preventing GI problems.

For the lungs we are working to help patients loosen and get rid of that thick mucus in order to prevent infection. And this is done through chest physiotherapy, bronchodilator, flutter devices and antibiotics.

To prevent GI problems, we are giving pancreatic enzymes and fat soluble vitamins ADEK and the patients needs to eat a high fat high protein diet to prevent weight loss.

Keep in mind that cystic fibrosis really challenges quality of life. Always assess for signs of depression and poor coping because again this is a really difficult disease to live with.

That's it for our lesson on Cystic Fibrosis. Make sure you check out all the resources attached to this lesson. Now, go out and be your best self today. Happy Nursing!