Cystic fibrosis is often thought of as a ‘lung disease’ owing to a collection of respiratory symptoms that manifest in people with the condition. In reality, CF affects many parts of the body. The faulty gene that causes CF is responsible for transporting water into and out of cells, including lung cells, so in people with the condition this transport does not happen effectively, resulting in many of the symptoms described below.
In healthy lungs, there is a thin layer of mucus that helps your body move dirt and bacteria out of the lungs. In people with CF, this mucus is much thicker and clogs the lungs, creating the perfect environment for harmful bacteria.
This means that people with the condition experience persistent coughing or wheezing, and produce thicker, stickier mucus (sputum). There is also a lack of airway surface liquid (ASL), so clearance of these sticky secretions is less effective. As lung disease progresses and chronic airway infection becomes established, there is an increase in the volume of sputum produced.
People with CF may also have restricted lung function due to the thickening of the walls of the airways, narrowing of the lumens (the space inside the airways), damage to the airways and obstruction of the airways with mucus. They also suffer from frequent lung infections, resulting in shortness of breath.
These infections are so serious that it is common for people with CF to spend weeks in hospital several times a year for intravenous antibiotic treatment and monitoring. The lung function of someone with CF can drop even lower than 30% of that expected in someone with fully functioning lungs, which is the level at which a lung transplant may be considered.
A combination of physiotherapy and medication can help control lung infections and prevent the build-up of mucus that causes the damage.
Cystic fibrosis and lung infection
The symptoms of a lung infection can include increased coughing and wheezing, getting out of breath more easily and the production of more sputum. If a bad lung infection occurs, which can happen often in some individuals, intravenous antibiotics are required, which may have to be administered in hospital.
The rise in antibiotic-resistant bacteria, such as MRSA, and ongoing challenges presented by bacteria such as Burkholderia cepacia complex, Pseudomonas aeruginosa and Mycobacterium abscessus, means that more and better antibiotics are needed.
Long-term or severe lung infections can also cause scarring in the lungs, which can result in a lower lung function.
Research we are funding
Advances in medical science mean that people with cystic fibrosis are managing their symptoms more effectively and living longer and healthier lives than ever before, and we will keep funding research until the day that everyone with the condition can live a life unlimited.
For many people with cystic fibrosis, a double-lung transplant becomes the only option. While this tends to improve quality of life, the operation comes with risks.
After a transplant, certain drugs are required to prevent the donor organs from being rejected.
Cystic fibrosis cross-infection
Owing to the increased risk posed by infections, people with CF should not meet each other face to face. Although the infection-causing bacteria do not pose a risk to people without the condition, people with CF may have different types of bacteria that would prove dangerous to each other.
Find out more about cross-infection and what it means for people with cystic fibrosis. You can also watch our video by CF nurse Jacqui Cowlard explaining more about cross-infection.
Other respiratory complications
It is common for someone with CF to have other respiratory conditions, such as asthma, gastro-oesophageal reflux disease (GORD), aspiration and allergies. It is 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 are not due to infection. Occasionally, people with CF may get other respiratory complications such a collapsed lobe or a pneumothorax. If there is a sudden change in breathing it is important to inform your centre so these can be recognised and treated as soon as possible.
Antibiotic resistance is the process by which bacteria become immune to an antibiotic treatment that was previously effective against them.
Overuse of antibiotics in the past, as well as people using them for things that they are not effective for (such as the common cold), and stopping taking them before they finish the whole course, have all added to the problem, which is now a global concern. Chronic bacterial infections are common in people with cystic fibrosis, and increasing resistance to common antibiotics is leaving clinicians with no option but to prescribe older, less-effective antibiotics that can come with a host of unwanted side effects.
The Trust is funding research into alternatives to antibiotics and the development of new ones. To find out more, read a statement made by Dr Keith Brownlee, Director of Impact at the Trust, for World Antibiotic Awareness week 2015.
You can also watch a video by the World Health Organisation, explaining to individuals why it's important to only take prescribed antibiotics and to keep taking them until the end of the course – regardless of how quickly their symptoms clear up.