CF affects many organs, primarily the lungs and those responsible for digestion. In CF there is a problem with the protein that controls the movement of salt and water in and out of the cells. Salt plays an important part in thinning out mucus in the body. Due to a lack of salt in the cells of someone with CF, mucus becomes thick and sticky. It then builds up in organs, including the lungs and pancreas. This mucus clogs the tiny air passages in the lungs and traps bacteria. Repeated infections and blockages can cause irreversible lung damage and death.
The life expectancy for someone with the disease today is 37. Outcomes vary considerably between patients but tragically, more than 50% of those with CF do not live to the age of 30. Medical advances are likely to result in a major increase to these figures but the researchers need funding to make that happen.
For more information on what Cystic Fibrosis is click here.