BRONCHIECTASIS - a patient's guide
What is Bronchiectasis?
Air is carried in and out of the lungs by branching air passages. Normally these air passages (the medical term is bronchi) taper smoothly towards the periphery of the lung.
In bronchiectasis the airways have been irreversibly damaged and as a result are abnormally dilated, often irregular and do not taper normally.
Normally the air passages produce small amounts of clear mucus. This is swept along by small hair-like structures (cilia) which line the airways. This mucus is then swallowed - generally without us being aware. This mucus traps any particles or neutralises any toxic substances which have been inhaled. Excess amounts of mucus, such as may occur in an infection, are cleared by coughing.
In bronchiectasis these clearance mechanisms of the lung are impaired. The cilia are damaged and do not clear the mucus effectively. The cough is less effective at clearing dilated and damaged airways. Hence, physiotherapy is needed to help clear these secretions.
Normally the airways in the lung are sterile - they are not infected.
In bronchiectasis, because of the inability to clear secretions effectively, these secretions become infected. As a result of this infection the airways produce more secretions which in turn become infected. Thus, most people with bronchiectasis even have a chronic cough and produce sputum.
What causes Bronchiectasis?
- In many cases of bronchiectasis a clear-cut cause cannot be identified.
- Some respiratory infections in childhood can damage the airways and lead to bronchiectasis. These may be viral infections (such as cause some types of bronchiolitis, or measles) or bacterial infections (such as whooping cough).
- In most cases it is thought that the damage to the airways occurs in early childhood although the symptoms may only become apparent in early adulthood.
- There are specific conditions that may cause bronchiectasis. These include cystic fibrosis and defects of the immune system - such as a deficiency of immunoglobulins (which protect against infection). Your doctor may do tests for these conditions.
- Occasionally bronchiectasis can occur following inhalation of a foreign body - such as a peanut or other objects. If detected early these can be removed and any damage (including bronchiectasis) may be avoided.
What are the symptoms of Bronchiectasis?
- Most people with bronchiectasis have a chronic cough. In most cases, sputum (phlegm) is produced and this is often infected (yellow/green rather than white/clear). The volume of sputum is variable but tends to increase when there is an acute infection. Sometimes the sputum is foul smelling - this can be embarrassing and is a reason to see the doctor for antibiotics.
- Occasionally small amounts of blood may be coughed up (called haemoptysis). This is due to the breaking of small blood vessels in the lining of the airways, and generally indicates an infection and the need for antibiotics.
- Very occasionally larger amounts of blood may be coughed up. This can be frightening and is a reason to go to your doctor or the nearest Emergency Department. However, virtually all episodes stop spontaneously.
- Many persons with bronchiectasis feel generally tired and "below par". This usually improves as the chronic infection in the airways is brought under better control. Sometimes appetite is reduced and may even lead to loss of weight.
- The chronic infection in bronchiectasis may cause damage to the lungs and impair the function of the lungs. As a result the person may feel short of breath or have reduced ability to exercise.
- Persons with bronchiectasis sometimes have wheeze (whistling in the chest), like persons with asthma.
- Often there is also chronic infection in the nose and sinuses.
What tests are likely to be done?
- A chest x-ray. Sometimes the chest x-ray will confirm the presence of bronchiectasis. However, the chest x-ray may be normal or the abnormalities be non-specific. Further special x-ray tests are then required.
- CT scan of chest. This is now the best test for the diagnosis of bronchiectasis. It also provides information on the type and extent of bronchiectasis as well as other valuable information for the specialist. (A test called a bronchogram, in which dye is used to outline the airways, is no longer performed).
- Specimens of sputum (phlegm) will be collected to see what sort of organisms ("bugs") it contains.
- Breathing tests to measure your lung function will be done.
- Certain blood tests (and perhaps also tests of sweat) may also be done.
- Your doctor may also do tests to check out your nose and sinuses, and to check if you have heartburn (gastric-oesophageal reflux). Both of these conditions can adversely affect the bronchiectasis.
Is Bronchiectasis a common problem?
Although bronchiectasis is not as well known as some other respiratory problems, it is a common problem and one that is responsible for substantial disability in the community.
Bronchiectasis is also under-diagnosed in the community. Now that a non-invasive method of making the diagnosis is available (CT scanning), it is hoped that many more persons will be correctly identified and receive the appropriate treatment.
In recent years a number of advances in our understanding of lung disease have had direct relevance to the treatment of bronchiectasis. Thus, it is important that the correct diagnosis be made, the person be reviewed by a respiratory specialist, correct and appropriate treatment instituted, and medical follow-up arranged.
What can be done about Bronchiectasis?
The most important component of treatment is regular physiotherapy to clear secretions from the lungs. There are a number of different methods to do this and an individual programme will be developed by your respiratory physiotherapist. These clearance techniques should be undertaken regularly (usually daily) at home and more frequently when there is an acute infection. All persons with bronchiectasis should also maintain a regular exercise programme.
All patients with bronchiectasis are at greater risk of respiratory infections. These are indicated by increased volume and purulence (more yellow or green) of sputum. Coughing up of blood, fever (and/or shivers), pain in the chest, or feeling generally unwell may also occur. Under these circumstances, antibiotics are generally needed. This means a visit to your doctor - or starting the antibiotics that your doctor has given to be kept at home.
Patients who have wheeze (or narrowing of airways) may be given a reliever inhaler (a bronchodilator such as Airomir, Atrovent, Bricanyl, Respolin or Ventolin).
Sometimes asthma preventer inhalers are also used in bronchiectasis (e.g. Becotide, Becloforte, Flixotide, Pulmicort, Respocort). These need to be used regularly and long term. It is thought that these medicines may reduce the inflammation within the airways and prevent further damage.
A variety of other treatments may be used such as long-term oral antibiotics, nebulised antibiotics and other nebulised or inhaled treatments. The decision about these treatments will be made by the respiratory specialist.
Attention will also be paid to treatment of any nose or sinus problems, reflux, any (heartburn) or indigestion symptoms.
Occasionally the area of the lung affected by bronchiectasis can be removed surgically.
Can Bronchiectasis be prevented?
Because the damage to the airways is thought to occur as a result of childhood infections, any prevention needs to take place in childhood.
Children should be vaccinated against measles and whooping cough to prevent this damage to airways early in childhood. A cough (particularly a moist cough) that persists in a child, is a reason to take the child to the doctor.
Prompt removal of an inhaled foreign body can prevent bronchiectasis. (Thus, if any friend or family member is thought to have inhaled something they should see their doctor immediately).
What is the outlook for persons with Bronchiectasis?
Although bronchiectasis cannot be cured, almost all persons with bronchiectasis live long and productive lives.
Regular treatment with chest physiotherapy, exercise programmes and other therapies, mean that symptoms can be reduced, and many infective exacerbations prevented.
With such treatment, the majority of persons remain stable for many years. This is particularly the case for persons in whom correct treatment was started before substantial lung damage occurred.
Prompt antibiotic treatment of any infections also reduces the risk of further lung or airway damage.
All persons with bronchiectasis should be reviewed by a respiratory specialist; preferably in the context of a multidisciplinary team with a specific interest in this condition. For the majority of patients long-term follow-up by a respiratory specialist is not required but under these circumstances regular review by the family doctor is most important.