Information about Non-Cystic Fibrosis Bronchiectasis (NCFB)
Bronchiectasis is a long-term condition that affects the airways in your lungs.
The airways are scarred and inflamed with thick mucus, also called phlegm or sputum. The airways become widened and cannot clear themselves properly. This means mucus builds up and the airways can become infected by bacteria. Pockets in the airways mean that mucus gets trapped and is likely to get infected. All of this leads to difficulty in breathing. It is a chronic (i.e. long-term) condition.
Chronic infections lead to chronic inflammation, further damaging the airways and causing more dilatation and worsening bronchiectasis.
For many people with NCFB no cause can be identified. This is called “idiopathic”.
There are many other causes of NCFB, for example:
- having had a lung infection such as pneumonia, whooping cough or tuberculosis even in the distant past
- it can be associated with inflammatory conditions, (for example, such as inflammatory bowel disease, ulcerative colitis and Crohn’s disease or arthritis like rheumatoid arthritis)
- immune system deficiencies, which can make NCFB more common
- some genetic disorders in which the lungs are abnormal, such as primary ciliary dyskinesia
- a severe allergic response to fungus or moulds such as aspergillus
- gastric reflux where stomach acid can enter the lungs and cause damage
- a blockage of your airways by something inhaled (like a peanut!)
- sometimes, people who have lived with chronic obstructive pulmonary disease (COPD) or asthma for many years develop bronchiectasis.
Symptoms typically include a long-lasting wet/productive cough (with lots of mucus) or just a dry cough where little mucus is produced.
Other symptoms can include shortness of breath, wheezing, coughing up blood and chest pain. Patients often have recurrent chest infections. The severity of symptoms can vary widely. Some people have only a few symptoms that don't appear often, while others have wide-ranging daily symptoms. The symptoms tend to get worse if you develop an exacerbation (usually caused by a chest infection).
Non-cystic fibrosis bronchiectasis (NCFB) is usually diagnosed by a lung specialist rather than a family doctor. It is usually diagnosed by:
- The general clinical picture of someone with a long-term cough that produces large amounts of sputum.
- A CT scan of the lungs that shows dilatation of the airways and usually thickening of the wall of the airways.
- Lung function tests.
Pseudomonas aeruginosa, or Pseudomonas, is a bacterium (or 'bug') that can cause lung infection in people with bronchiectasis. It affects about 1 in 5 of those with bronchiectasis at some point.
It is found everywhere, in soil, water, on the skin of some people and in most man-made environments. It does not usually cause infection in healthy people, but it can infect damaged lungs such as those in bronchiectasis.
People with Pseudomonas can sometimes have more chest infections and more problems than other people with bronchiectasis because of its resistance to most antibiotics, and sometimes it can damage the lung further.
In most countries, current guidance recommends treating bronchiectasis patients, who are infected with Pseudomonas aeruginosa, with antibiotics (this is often by inhalation, i.e. administration of the medication in the form of a mist inhaled into the lungs). However, none of the recommended inhaled antibiotics are currently approved to be used for the treatment of this specific bacterial infection in bronchiectasis.
A pulmonary exacerbation is a temporary worsening of the lung function often due to an infection or inflammation. Many people with bronchiectasis know when an exacerbation is starting.
Possible signs include a day or more of:
- Coughing more than usual.
- More sputum (mucus) is coughed up or it is thicker or harder to cough up.
- The sputum is darker or has changed colour.
- More breathless than usual.
- Feeling more tired, or feeling symptoms of infection like fever and muscle aches.
- Coughing up blood.
- Chest discomfort or tightness.