Cystic fibrosis is a progressive inherited disorder most often associated with lung damage that limits breathing. It can also affect the pancreas and digestive tract. Most people with this disorder develop lung complications and frequent infections. Over time, this can lead to severe health consequences, like pneumonia, and even death.
This article discusses the link between cystic fibrosis and pneumonia as well as symptoms, causes, diagnosis, and treatment.
What’s the Link Between Cystic Fibrosis and Pneumonia?
Cystic fibrosis is an inherited disorder that affects many organ systems, but its damage to the lungs is the most concerning problem. People develop frequent episodes of pneumonia or bronchitis, which can lead to severe complications and early death.
The faulty gene causing cystic fibrosis disrupts the movement of salts and water in the body, which leads to a buildup of mucus in places that can damage organ systems. The buildup of thick mucus is most prominent in the lungs, making it difficult for a person to clear bacteria that enter the lungs. Thus, people with cystic fibrosis frequently develop bacterial pneumonia.
Over time, repeated episodes of pneumonia lead to chronic (long-term) lung damage. Additionally, the overuse of antibiotics to treat frequent infections leads to antibiotic resistance. Eventually, the lung damage is so severe and the treatments no longer effective that a person cannot survive.
How Many People Have Cystic Fibrosis?
Cystic fibrosis affects approximately 30,000 people in the United States.
Symptoms of Lung Infections
Cystic fibrosis affects many different organ systems. Often, the initial symptoms of the disease are due to problems in the pancreas and intestines. People with cystic fibrosis cannot digest food because of a lack of digestive enzymes from the pancreas, which leads to malnutrition.
Since cystic fibrosis is an inherited disorder, the symptoms can start when a person is a baby. The initial symptoms of lung disease are nonspecific and include:
Newborns in the United States are screened for cystic fibrosis so the illness can be detected and symptoms treated as early as possible. However, in places without neonatal screening for cystic fibrosis, these nonspecific lung symptoms are initially misdiagnosed as asthma, mild bronchitis, or pneumonia not related to an underlying lung problem.
Which Organs Are Affected by Cystic Fibrosis?
Causes of Pneumonia and Other Lung Infections
Bacteria and viruses constantly circulate in the environment, and they can be passed from one person to another through respiratory droplets, such as by coughing or sneezing. Most people can clear their lungs of these invaders by producing mucus that can be coughed up and expelled.
However, people with cystic fibrosis are unable to clear mucus from their lungs. When mucus mixed with bacteria sits for a long time in a part of the lung, the area becomes inflamed and the person develops pneumonia.
Early in life, the bacterial organisms that usually lead to pneumonia in people with cystic fibrosis are:
- Staphylococcus aureus
- Haemophilus influenzae
As a person’s lungs become repeatedly damaged from recurrent infections, other, more unusual and dangerous bacteria cause pneumonia, including:
- Pseudomonas aeruginosa
- Burkholderia cepacia
- Methicillin-resistant Staphylococcus aureus (MRSA)
- Stenotrophomonas maltophilia
- Achromobacter xylosoxidans
Once a person has been infected and has lived with Pseudomonas aeruginosa for a while, they develop severe lung infections more frequently, have a more rapid decline in lung function, and are at higher risk for death.
Most Common Causes of Bacterial Pneumonia
Staphylococcus aureus is the most common bacterial organism found in children and adolescents with cystic fibrosis. The most common bacterium found in adults with cystic fibrosis is Pseudomonas aeruginosa.
Like people without cystic fibrosis, people with cystic fibrosis can develop viral infections of the lungs, such as influenza and respiratory syncytial virus (RSV). People with cystic fibrosis are not more likely to develop a viral infection. Instead, they are sicker when a viral infection occurs compared with people who do not have cystic fibrosis.
People with cystic fibrosis can also develop pneumonia caused by a fungus instead of a bacteria. The most common fungal organism that leads to pneumonia in patients with cystic fibrosis is Aspergillus fumigatus.
In the United States, there is a nationwide program that screens newborn infants for cystic fibrosis. The screening is performed in the first few days of life from a few drops of blood. Different states use different screening tests to confirm the diagnosis.
Newborn screening tests are vital because they help parents and healthcare providers know when to begin specialized cystic fibrosis treatments. Initiating treatment early extends the time without complications.
If an infant or a person suspected of having cystic fibrosis tests positive during the initial screening test, they are given an additional test. This definitive diagnostic test is called a sweat test. It measures the amount of electrolytes in a person's sweat.
What Are Autosomal Recessive Carriers?
Testing for cystic fibrosis involves looking for the gene responsible for the disease. Some people are carriers of the gene but do not develop the disease. This is because the gene is autosomal recessive (passing from both parents to a child). If you are one of these people, speak with your healthcare provider about what this means.
When people are treated for pneumonia several times over several years, they eventually develop antibiotic resistance. When a particular bacteria is resistant to many different antibiotics, the organism is called a multidrug-resistant bacterium. Infections from these types of bacteria are complicated to treat and can lead to death from pneumonia in a person with cystic fibrosis.
Healthcare providers need to treat Pseudomonas aeruginosa colonization or pneumonia early to help clear the infection. People chronically infected with the specific bacteria are treated regularly with inhaled antibiotics, such as tobramycin, which helps prevent severe infection.
What to Do About Pseudomonas aeruginosa
People with cystic fibrosis do not develop Pseudomonas aeruginosa lung infections until they are older children, young adults, or adults. Research has shown that if a person is treated early for this bacterium and the organism is destroyed completely, people live longer.
Antibiotics and Antifungals
Treatment of acute lung infections requires antibiotics. The choice of antibiotic depends on the bacterial organism in the lungs and whether a person has developed resistance to antibiotics needed to kill it.
If a person develops fungal pneumonia, treatment involves an antifungal agent and corticosteroids.
Airway Clearance Techniques
Since people with cystic fibrosis cannot clear mucus out of their lungs, other treatments include airway clearance techniques. Examples include:
These techniques help move the mucus up and out of the airway. Airway clearance techniques are recommended daily for people with cystic fibrosis.
Regular aerobic exercise is also recommended for patients with cystic fibrosis because it stabilizes lung function.
People with cystic fibrosis are also encouraged to use inhalers that rehydrate and break up the mucus so that it can be coughed up and expelled. The inhaled medications are called mucolytics and options include:
- 7% hypertonic saline solution
- Dornase alfa
People with end-stage lung disease with cystic fibrosis are eligible for lung transplantation. People can often live an additional ten years after a lung transplant.
How to Prevent a Lung Infection
People with cystic fibrosis who are infected with Pseudomonas aeruginosa are started on chronic preventive therapy. Patients are treated with inhaled and oral antibiotics to prevent future bacterial pneumonia. The most commonly used antibiotics include:
Other general preventative measures recommended for anyone with respiratory symptoms include:
- Using masks when in healthcare facilities or close contact with someone with a respiratory illness
- Social distancing when needed
- Cleaning and disinfecting nebulizers and other tools used by the cystic fibrosis patient
Cystic fibrosis is a chronic, inherited disorder that leads to frequent lung infections. Symptoms of a lung infection include cough, wheezing, and shortness of breath. People with cystic fibrosis can develop various types of pneumonia, but bacterial pneumonia is the most common.
In the United States, treatment for cystic fibrosis usually starts early because of widespread neonatal screening programs used to detect it. Treatments aim to prevent infections, treat acute infections, and rid the lungs of mucus.
A Word From Verywell
Living with cystic fibrosis can be extremely difficult because of the number of lung infections people develop, even in childhood. However, scientists have come a long way in improving life expectancy for people with cystic fibrosis through new treatments and preventive strategies.
If you are expecting a baby, make sure you participate in cystic fibrosis screening programs for your child. If you have cystic fibrosis, follow your healthcare provider's recommendations in trying to prevent frequent lung infections.
Frequently Asked Questions
What is the most common cause of pneumonia in people with cystic fibrosis?
The most common cause of pneumonia in a child with cystic fibrosis is Staphylococcus aureus. The most common cause of pneumonia in an adult with cystic fibrosis is Pseudomonas aeruginosa.
What is the risk of getting pneumonia if you have cystic fibrosis?
Due to a buildup of mucus in the lungs, patients with cystic fibrosis are at risk of developing lung infections early in life. Sometimes the first episode develops as a baby.
Is pneumonia a symptom of cystic fibrosis?
A single episode of pneumonia is not a symptom of cystic fibrosis. A diagnosis of cystic fibrosis would not be suspected in a person unless they had recurrent episodes of pneumonia. Cystic fibrosis is an inherited disorder, so it usually presents early in life.