About CYstic Fibrosis

The name "cystic fibrosis" refers to the characteristic fibrosis and cysts that form within the pancreas

Cystic Fibrosis is an autosomal recessive disease i.e. People with CF have inherited two copies of the defective CF gene -- one copy from each parent. Both parents must have at least one copy of the defective gene.

People with only one copy of the defective CF gene are called carriers, but they do not have the disease. Each time two CF carriers have a child, the chances are:

25 percent (1 in 4) the child will have CF

50 percent (1 in 2) the child will be a carrier but will not have CF

25 percent (1 in 4) the child will not be a carrier and will not have CF

 The disease is expressed in the exocrine system of the human body, i.e. lungs, pancreas, liver and other organs.

In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. For this reason, avoiding germs is a top concern for people with CF.

In the pancreas, the build up of mucus prevents the release of digestive enzymes that help the body absorb food and key nutrients, resulting in malnutrition and poor growth. In the liver, the thick mucus can block the bile duct, causing liver disease. In men, CF can affect their ability to have children.

There is no known cure for cystic fibrosis

History

The first modern description of the disease was made in 1938 by pathologist Dorothy Andersen. Based on autopsies of children who had died of malnutrition, she termed the disease “cystic fibrosis of the pancreas.” It had previously been known as “mucoviscidosis,” referring to the thickening of mucus

There are descriptions that fit the condition occurring at least as far back as 1595

There exists an urban legend regarding literature from Germany and Switzerland in the 18th century that warned "Wehe dem Kind, das beim Kuß auf die Stirn salzig schmeckt, es ist verhext und muss bald sterben" ("Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon must die"), recognizing the association between the salt loss in CF and illness