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BRONCHIECTASIS
What is it?
Air is carried into the lungs through a series of branching
tubes called bronchi. The bronchi contain tiny glands that produce
a small amount of mucus, which helps keep the tubes moist and
trap dust and germs that are breathed in. The mucus is then normally
wafted away by the beating of tiny hairs, called cilia, which
line the tubes.
When the bronchial tubes get damaged, they can no longer clean
themselves, and the mucus accumulates in the tubes, spilling
over to adjacent tubes.
These tubes are then prone to infection by bacteria, causing
inflammation which
leads to damage called bronchiectasis.
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Who
gets it?
There are several known causes including:
- underlying genetic disease such as cystic fibrosis (where
the mucus in the bronchial tubes is too thick), and primary
ciliary dyskinesia (where the cilia lining the bronchial tubes
do not beat properly)
- mechanical obstruction of the bronchial tubes by inhaled
foreign bodies (e.g. peanuts)
- healing of the tubes resulting in puckering and scarring,
causing obstruction
- inhaling stomach acid which has been regurgitated back into
the gullet
- too little immunity to infection (for example after infantile
pneumonia from whooping cough or measles, or lack of antibodies
which occasionally occurs after a virus infection in adult
life)
However, over half the patients with bronchiectasis in the UK
have no obvious cause for it.
What are the symptoms?
The most common symptom is coughing up nasty phlegm, often in
large quantities, every day, which is socially embarrassing and
very tiring. Even taking this into account, there is often excessive
tiredness with lack of concentration. These symptoms frequently
result in the patient being accused of smoking. In fact, 80 per
cent of patients have never smoked and most of the remainder
have stopped. Eighty per cent of patients also have wheezy shortness
of breath and a runny nose, and one third suffer from chronic
sinusitis.
Less common symptoms are coughing blood (haemoptysis), chest
pain, and joint pain. Very rarely, there may be additional symptoms
of associated conditions, for example bloody diarrhoea from ulcerative
colitis, rheumatoid arthritis, and infertility (mainly in men).
What tests will the Doctor want to do?
When a doctor sees a patient with a persistent cough, producing
infected sputum, there are three categories of tests which should
be carried out:
- a test to determine whether the symptoms are due to bronchiectasis
and, if so, its distribution and severity; this is done by
high-resolution computerised tomography (CT) scanning, which
is painless
- tests to see if it has affected lung function, to determine
what bacteria are present by sputum culture, and to determine
whether the inflammation is active by white cell scanning
- tests to detect known causes of bronchiectasis (blood tests,
and a simple test of mucus clearance in the nose, measuring
the speed of beating of the cilia and how much salt is present
in sweat); a fibre-optic bronchoscopy may be necessary to exclude
a mechanical obstruction; in men, tests of the number of sperm
and their motility may be required
What is the treatment?
There are seven major components of treatment. The efficiency
of treatment is monitored, to detect early progression of disease
and to enable treatment to be rapidly modified:
- if there is no underlying cause which might cause bronchiectasis
to recur, and the bronchiectasis is localised to a single area
of the lung which could be removed without impairing breathing,
then removal by operation is a cure
- the cause, if determined, must be treated (for example, antibody
replacement for deficiency)
- utilising gravity to drain the infected tubes
- improvement of airflow through the bronchial tubes by anti-asthma
treatment
- treatment of nose or sinus infection, and runny nose, using
nasal drops and sprays
- antibiotics to treat infections, administered at regular
intervals or continuously, by intravenous or inhaled routes
- treatment of any associated disease
In addition, a number of treatments to assist in mucus clearance
and to reduce inflammation are being tested for the future. Prevention
will rely on future identification of people who are susceptible
to the disease.
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