In a person who has CF, the CFTR protein may be missing, or if present it does not function properly. When there is too little water in the mucus, Cl- cannot be secreted across the apical membrane, and there is no blockage of the epithelial sodium ion channels. Since the Na+ channels are always open, there is continual Na+ absorption by the epithelial cells. The raised levels of Na+ draw chloride ions and water out of the mucus and into the cells. This makes the mucus more viscous, which makes it harder for cilia to move it, so the mucus is not effectively cleared up and out of the lungs. Sticky mucus builds up in the airways. This mucus frequently becomes infected with bacteria, causing a downward spiral of airway inflammation and damage.
The effect of CF on the digestive system
In a person with CF, the pancreatic duct becomes blocked by sticky mucus, impairing the release of digestive enzymes. The lower the concentration of enzymes within the small intestine reduces the rate of digestion. Food is not fully digested, so not all of the nutrients can be absorbed. A higher proportion of partially digested and undigested food means energy is lost in the faeces. This is called malabsorption syndrome. An additional complication occurs when the pancreatic enzymes become trapped behind the mucus blocking the pancreatic duct. These enzymes damage the pancreas itself.
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