Cystic fibrosis 

Cystic fibrosis affects the internal organs, especially the lungs and digestive system, which become clogged with thick, sticky mucus. It is caused by a faulty gene that controls the movement of salt and water in and out of cells in the body. Dr Alan Day explains how the disorder is treated.

Find out how cystic fibrosis is diagnosed

Transcript of Cystic fibrosis

I'm Alan Day, consultant paediatrician in Cheltenham General Hospital.

I have a special interest in cystic fibrosis.

If I get a family and parents that come with a newly diagnosed child,

the kind of things they're going to ask me,

first is, "Is my child going to survive?"

And I can say that it is a condition

that, over the last 30 years, has quadrupled its life expectancy

from 14, when I started looking after patients, now to at least 54.

Cystic fibrosis occurs in roughly one in 2,000 births in the UK,

which means that there would be roughly one new patient a day,

about 300 a year.

The number of cystic fibrosis patients in the UK

is approximately 8,000.

The numbers are increasing.

The classic triad, as we call it, of symptoms for a young child

would be that they're not putting on adequate weight,

they will be coughing a lot, and they would probably have loose stools.

There are complications elsewhere in the gut, particularly.

The pancreatic gland doesn't work properly

in 98 per cent of cystic fibrosis patients.

But the main cause of death in the past,

and still sadly now, even in young adults,

can be the long complications and the chronic chest infections,

which... We're constantly battling to treat them

both by antibiotics and also by other more specialist treatments.

From the time of diagnosis of cystic fibrosis in a child,

we want to minimise the complications.

We want to clear the chest,

and a major way of doing that is physiotherapy.

Parents will be taught techniques appropriate to the age of the child.

We also need to minimise any infection,

so the children are usually started on an antibiotic

for every day to minimise the number of germs that get there.

The gut is affected in most patients.

So they normally have pancreatic supplements

given as capsules, or granules in babies, and vitamins,

because the pancreas doesn't work properly

and can't absorb the number of vitamins in the body.

We would aim in cystic fibrosis to have no limitations to what the person does.

We like them to take all their treatment,

but, in fact, vigorous sports are a very good alternative to physiotherapy.

In cystic fibrosis in paediatrics in 2007,

we can be very upbeat that we're getting earlier diagnosis

through neonatal screening,

that the treatments are improving,

that there is a lot of research still going on

run normally by the Cystic Fibrosis Trust in the UK.

We can be very optimistic to families

that we will minimise the complications.


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