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Cystic Fibrosis
“Woe to the child that when kissed on the forehead tastes salty
...
” – Old Northern European quote on Cystic
fibrosis
Cystic fibrosis is a autosomal recessive disease that affects the transport of
electrolytes in the body
...

1 in 2500 live births is a cystic fibrosis (CF) sufferer
...
50 years ago, it was rare for
a CF patient to survive beyond their first birthday
...

CF affects almost all the parts of the body
...
A
small number of women are also affected
...
This is one of the first signs of CF that
medics can look for
...

Illness can be identified through screening tests
...
The
mucous is the ‘first line of defence’ against pathogens
...
In CF patients, the cilia cannot move the
mucous up towards the mouth and the pathogens can thrive
...

This localisation of pathogens triggers an immune response and since the
immune system is enhanced, it triggers chronic inflammation
...
Over the course of 40 years, CF patient’s lungs
are degenerated as lung tissue cannot grow back once killed
...
Sodium
chloride is critical to mucous composition
...
Movement of chloride ions outside of the cell causes
diffusion of sodium ions and water from the interstitial fluid to the lumen of
the respiratory tract
...
The diffusion of the water decreases the viscosity and allows the
movement of mucous by the cilia
...
There is a
theory that being a carrier of CF gave slight immunity to the cholera outbreaks
in Europe
...
In the colon, the more
chloride ions move the more water moves
...
Having an infection (e
...
cholera) causes mass efflux of
chloride ions into the colon
...
However, carriers of CF only have about half the number of chloride
channels and so would only lose about half as much water
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