What is Cystic Fibrosis?

Cystic fibrosis is an inherited disease that causes unusually thick mucus to build up in the lungs and other organs. This makes it difficult to breathe. Bacteria can grow in the mucus and people with cystic fibrosis are prone to lung infections. When mucus collects in the pancreas, people with cystic fibrosis aren't able to produce the enzymes needed to digest food properly, and can be prone to malnutrition or diabetes. The median age of survival for someone with cystic fibrosis is only 37, but people are surviving longer than ever before thanks to medical advances; it is not unheard of for people with cystic fibrosis to live into their 40's or even older.

Symptoms of cystic fibrosis most commonly begin in childhood, although in milder cases the symptoms may not appear until teenage years or even adulthood. Symptoms can include salty skin, a persistent cough, repeated lung infections, wheezing and shortness of breath, failure to thrive and poor weight gain despite eating adequate amounts, and unusual bowel movements.

Treatment for most cystic fibrosis patients involves procedures known as airway clearance techniques. These are changes in position or methods of tapping or pounding that shake mucus loose from the lungs and allow it to be coughed out. Inhalers are also commonly used to apply medication to thin out the mucus in the lungs. Antibiotics can be taken orally, by inhaler, or through an IV to combat infections. Nutritional support is extremely important with cystic fibrosis because digestion is impaired and diabetes is such a common complication. Many patients take pancreatic enzymes to support their digestion. Finally, for a certain type of cystic fibrosis, there is a new medication called Kalydeco that works to help the defective protein in the body work better and clear mucus from the body.
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