MSU's Dr Bruce Uhal explains...


CF on Wikipedia

Cystic fibrosis (also known as CF, mucovoidosis, or mucoviscidosis) is agenetic disorder known to be an inherited disease of the secretory glands, including the glands that make mucus and sweat.

 

The hallmarks of cystic fibrosis are salty tasting skin, normal appetite but poor growth and poor weight gain, excess mucus production, frequent chest infections and coughing/shortness of breath. Males can be infertile due to congenital absence of the vas deferens. Often, symptoms of CF appear in infancy and childhood. Meconium ileus is a typical finding in newborn babies with CF. As the child grows older, he or she will have to exercise to release mucus stuck to the alveoli. Cilial epithilial cells in the patient have a mutated protein that instead of creating the right resin that is used to prevent the alveoli from collapsing, it makes a thicker resin, mucus. This makes the oxygen extraction in the alveoli harder as the molecules must travel through the mucus leading to breathlessness. Since the mucus stays there most of the time bacteria will thrive in it, causing multiple chest infections.

 

Although technically arare disease, cystic fibrosis is ranked as one of the most widespread life-shortening genetic diseases. It is most common among nations in the Western world; one in twenty-two people of Mediterranean descent isa cancer of one gene for CF, making it the most common genetic disease in these populations. An exception is Finland, where only one in 80 people carry a CF mutation. In the United States, 1 in 4,000 children are born with CF. In 1997, about 1 in 3,300 caucasian children in the United States was born with cystic fibrosis. In contrast, only 1 in 15,000 African American children suffered from cystic fibrosis, and in Asian Americans the rate was even lower at 1 in 32,000.