When the CFTR gene is not working like it should, there will be problems with chloride-transport across the surface of cells (specifically mucosal cells). This decreased secretion of chloride leads to an increased reabsorption of sodium and water, which leaves the mucus much more sticky than normal. This sticky mucus can attract bacteria which makes the person prone to infection and inflammation. This stickiness of the mucus is also what makes it so difficult to clear from the body.
The two most common complications experienced by cystic fibrosis patients are lung disease and intestinal disease. Typically, it is the severity of these two diseases that determine how long an individual can live with CF. Typically, with lung disease it will progress from bronchitis, to bronchiolitis, to bronchiectasis. This will eventually lead to cor pulmonale (abnormal enlargement of the right side of the heart) and end-stage lung disease. The cause of death is usually respiratory failure or cor pulmonale in an individual with cystic fibrosis.
References:
Sharma, G. (2014). Cystic Fibrosis. Medscape.
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