At one time, few sufferers of cystic fibrosis lived beyond adolescence. Now many, if not most, make it to early middle age and the outlook is constantly getting better.
It is a hereditary disease, transmitted by recessive genes carried by both parents. The incidence varies from country to country and race to race. In most peoples of Northern European extraction, the gene is carried by about 3 per cent of the population.
The basic problem is that cystic fibrosis induces mucus by many glands, including those of the pancreas, in bronchial tubes and in parts of the intestines and reproductive organs.
The mucus produced in the bronchial tubes is so sticky and excessive that it blocks the airways and these become prone to infection, including pneumonia and severe bronchitis.
If the problem hasn’t been picked up at birth it is usually diagnosed within a year because of recurrent chest infections. Diagnosis is by a blood test, sweat analysis to measure sodium, and X-rays. Treatment is with postural drainage, to ensure that the lungs are draining properly, and antibiotics to treat infection.
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Improved prognosis is largely because of better antibiotics and methods of delivery. Mucolytics — drugs that make the mucus less sticky — also help.