What is Cystic Fibrosis?
- Cystic Fibrosis (CF) is one of the most common life-threatening inherited diseases.
- It is caused by a defective gene that controls the movement of salt and water in and out of the cells within the body.
- Cystic Fibrosis affects the internal organs, especially the lungs and digestive system, by blocking them with thick sticky mucus. This makes it hard to breathe and digest food.
- Cystic fibrosis (CF) is a genetic disorder that particularly affects the lungs and digestive system and makes kids who have it more susceptible to recurring lung infections.
- Due to high-tech medical advances in drug therapy and genetics, children born with CF can live longer and more comfortable lives. In the last 10 years, research into all aspects of Cystic Fibrosis has helped doctors to understand the illness better and to develop new therapies. Ongoing research may lead to a cure in near future.
- The inherited CF gene directs the body’s epithelial cells to produce a defective form of a protein called CFTR (or cystic fibrosis transmembrane conductance regulator) found in cells that form the lining of the lungs, digestive tract, sweat glands, and genitourinary system. When the CFTR protein is defective, epithelial cells can’t regulate the way chloride to pass across cell membranes. This disrupts the essential balance of salt and water needed to maintain a normal thin coating of fluid and mucus inside the lungs, pancreas, and passageways in other organs. The mucus becomes thick, sticky, and hard to move.
- Normally, mucus in the lungs traps germs, which are then cleared out of the lungs. But in CF, the thick, sticky mucus and the germs it has trapped remain in the lungs, which become infected.
- In the pancreas, thick mucus blocks the pathways that would normally carry important enzymes to the intestines to digest foods. When this happens, the body can’t process or absorb nutrients properly, especially fats. Kids with CF have problems gaining weight, even with a normal diet and a good appetite.
- For a baby to be born with Cystic Fibrosis, both parents must be carriers of the defective CF gene. The diagram shows how CF is inherited. Where both parents carry the faulty gene, each child has a one in four chance of having CF, a two in four chance of being a carrier and a one in four chance of not having any CF genes.
- Cystic Fibrosis affects more than 30,000 kids and young adults in the United States.
- Each week, five babies are born with Cystic Fibrosis.
- Each week, two young lives are lost to Cystic Fibrosis.
- Presently half of the CF population will live past 41 years of age, and improvements in treatments mean a baby born today is expected to live even longer.
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