Lungs and cystic fibrosis
People with cystic fibrosis (CF) are susceptible to a range of infections and reduced lung function, which can have a huge impact on health and wellbeing.
Cystic fibrosis is often thought of as a lung disease, because it causes a lot of lung symptoms. However, in reality, CF affects many parts of the body. The faulty gene that causes CF prevents the body from effectively moving salt and water in and out of cells. This leads to a build-up of thick mucus, particularly in the lungs and digestive system.
How do the lungs work?
The lungs are naturally good at keeping clean. Lungs contain a thin layer of mucus that helps to trap the dirt and bacteria that we breathe in. Tiny hair-like structures, called cilia, then move this mucus up the airways and into the throat, where it can be coughed out or swallowed.
How does CF affect the lungs?
In people with CF, the mucus in the lungs is thicker and stickier than normal. This makes it difficult to clear and easier for bacteria to get stuck and cause an infection. Infection causes the lining of the airways to become swollen and produce extra sticky mucus. Repeated lung infections can lead to lung damage.
It is important to clear the mucus in your lungs to try and prevent infections and stop lung damage from happening. This is usually done through airway clearance, which are techniques used to help loosen and remove mucus that builds up in the lungs. You can read about the different types of airway clearance in our CF physiotherapy resources.
There are many different types of bugs that can cause lung infections, such as bacteria, viruses, or fungi. These bugs can be a serious problem for people with CF, even though they are often harmless to people who don’t have CF. This is because of how CF affects the lungs. The symptoms of a lung infection can include:
- increased coughing and wheezing
- producing more mucus
- a change in the colour of mucus
- getting out of breath more easily
- tiredness and lack of energy
- loss of appetite or weight loss
- chest pain
- a drop in lung function.
These infections can be really serious. It's common for people with CF to spend weeks in hospital several times a year for intravenous antibiotic treatment and monitoring. Infection can cause the lung function of someone with CF to drop to far lower than that expected in someone with fully functioning lungs.
You can learn more about some of the most well-known bugs like Pseudomonas aeruginosa, Mycobacterium abscessus, Burkholderia cepacian complex and Aspergillus fumigatus. You can also read about some of the research we are funding to better understand treat these infections.
For some people with CF, a double-lung transplant becomes the only option when existing treatments are no longer working as well as they should.
This is a major operation that comes with a number of risks.Whether someone decides to have a lung transplant is a very personal decision.
We've worked with people with CF, their families, and CF and transplant clinicians to create lots of information on transplants.
Different bugs spread in different ways. Bugs might be picked up from the environment or they might pass from one person with an infection to another (cross-infection). People with CF can carry different bugs in their lungs, which can easily pass from one person with CF to another.
Avoiding cross-infection is important to reduce the risk of spreading or getting bugs. These bugs might not be harmful to people without CF, but people with CF may carry bugs that are dangerous to each other. This is why people with CF should not meet each other face to face.
Find out more about cross-infection and what it means for people with cystic fibrosis.
We've also created a video with CF nurse Jacqui Cowlard explaining more about cross-infection.
Other respiratory complications
It's common for someone with CF to have other problems that affect their breathing and lungs. This can be due to asthma, gastro-oesophageal reflux disease (GORD), aspiration (when something is accidentally breathed into the lungs, such as food or liquid) and allergies.
It's important that these other conditions are recognised and treated to help maintain normal lung function and minimise any symptoms, such as cough or wheeze, that aren't caused by a lung infection.
Occasionally, people with CF may get other more serious complications, such as a collapsed lung or area of the lung. If you notice a sudden change in breathing, it is important to inform your centre so this can be investigated and treated as soon as possible.
Research we fund
We fund research to tackle some of the most pressing issues in CF today. Find out how your donations are making a difference.
We are here to shout loud for better care and services for people with cystic fibrosis, but to really be heard, we need you to join us!
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