health

CF is a disease that affects many organ systems in the body. The defective
gene involved in cystic fibrosis results in lower levels of chloride, a
chemical compound that is important in the production of sweat, digestive
juices, and mucus of normal consistency. Chloride regulates the flow
of water in and out of cells, which is needed to produce mucus that is
thin enough to act as a lubricant and sweat that contains normal amounts
of salt. When there is not enough chloride, the mucus that the tissues
produce is thicker and stickier; it interferes with the proper functioning
of the lungs, pancreas, sweat glands, and digestive tract. The thick mucus
in the lungs provides a breeding ground for bacteria and fungi, which
cause inflammation and gradual destruction of lung tissue.
In the digestive system, the mucus blocks the ducts that convey
digestive enzymes from the pancreas, a large gland located just below the
stomach. Without these enzymes, the patient’s body cannot absorb
nutrients from food as it passes through the digestive tract, a condition
known as malabsorption. The thick mucus may also cause irreversible
damage to the pancreas itself and cause inflammation of the liver as well.
In men, it may block the tubes that carry sperm from the testicles, thus
leading to infertility.