Lung problems in CF

Question

What causes cystic fibrosis lung disease?

Answer

CF or cystic fibrosis is a disease caused by an abnormality in the CFTR gene. This gene contains the code for the synthesis of the CFTR protein, a protein functioning as a salt channel. In cystic fibrosis the transport of salt at the mucosal level is disturbed, leading to dehydrated mucus. We also refer to the theme “cause”.
The mucus layer around the cilia is less liquid, leading to decreased movement of the cilia and thus less efficient clearing of secretions and bacteria from the airway. All these factors predisposes towards infection of the airways. Additionally the natural defense against airway infections is slightly disturbed in CF. Inefficient cleaning of the airways and decreased natural resistance in the lung leads to chronic infection. Recurrent and chronic infection damages the lung in the long term. Infection and lung damage leads to more mucus production and infection and in this way a vicious circle emerges. In the long term the normal functioning of the lung (uptake of oxygen in the blood and excretion of C02) is compromised. One should not forget that the CFTR protein is necessary for the normal functioning of different organs in our body. Many CF patients have digestive problems due to insufficient functioning of the pancreas, liver disease, nose and sinus problems and fertility problems.
With kind regards
K. De Boeck

23.03.2010