Lung problems in A-T

A serious problem

Lung problems are one of the most serious aspects of A-T. The great majority of deaths in people with A-T, when not caused by cancer, involve lung disease of one kind or another.

It is only recently that we have come to realise the importance of the regular monitoring and proactive management of lung health. Major contributions to this have been made by the experts at the Nottingham and Papworth specialist A-T centres.

There is no simple or complete answer to how or why A-T causes lung disease, but we are building up a picture of the different factors involved. Three principle ones seem to be:

Immune problems

Many people with A-T suffer from repeated infections of the respiratory tract. These may take the form of repeated colds and ‘runny noses’ or of bronchial infections. Over time the effects of these can build up and where there have been repeated infections of the lower airways, these can become damaged. This can give rise to a condition known as bronchiectasis. This in turn leads to more infections and loss of lung function.

Decline in respiratory muscle function

The difficulties of co-ordination caused by A-T can lead to an impaired ability to cough. This can make it difficult to clear the airways and result in more infections. It can also affect breathing, leading to shallower breaths which again make it more difficult to keep airways open and clear.

Impaired swallowing and aspiration

Swallowing is a complex process requiring the co-ordination of different muscles. People with A-T often develop problems with swallowing and this can lead to particles of food or drink getting into the lungs, where they can become a source of infection. This process is known as aspiration.

Other factors

Nevertheless, this is not the whole story. There is a form of lung disease called Interstitial Lung Disease which sometimes develops in people with A-T. Its origin is uncertain but no link has yet been established with prior infections, with aspiration or with particular abnormalities of the immune system.

Compared to neurological symptoms which are usually very obvious, lung problems are not always easy to spot. It is therefore particularly important to have regular check-ups with a respiratory expert.

 

 

 

Picture of a boy having his lungs tested at the Nottingham A-T clinic

A boy with A-T has his lungs tested at the Nottigham A-T clinic