By Jon Obert

• It is an genetic disease that is caused by a deletion of three base pairs on chromosome 7.
• This deletion causes a mutation in the cystic fibrosis transmembrane conductance regulator protein (CFTR), which is responsible for transporting chloride into and out of cells.
• This causes it to not function properly or to be totally absent from the cell

• Since the epithelial cells do not take in chloride, the salt balance of the skin gets messed up.
• This causes all the mucus in the body to become extremely thick.

• The Pancreas is an organ in the body that secretes enzymes into the intestines to digest foods.
• Some of the enzymes it secretes helps digest fats.

What does cystic fibrosis do to a
Pancreas?
• The pancreas normally has a thin mucosal coating around it.
• The mutated protein that causes cystic fibrosis causes the mucus to become very thick.
• This blocks the pathway the enzymes take to the intestines so no enzymes can reach their destination.

• Since no enzymes reach the intestines and these enzymes help digest and absorb fat along with a few other nutrients, Not much fat is absorbed in the body, and other nutrients are not absorbed properly.
• That is why people with cystic fibrosis are normally skinny, even with strong healthy diets, they still have trouble gaining weight.

What happens to all the fat that is undigested?
• It is removed with the feces and can cause severe diarrhea and weight loss.

• The mutation of this one protein affects so many different things in the body which makes cystic fibrosis a very difficult disease to deal with and life threatening.

• http://www.ygyh.org/cf/cause.htm
• http://kidshealth.org/parent/medical/lungs
/cf.html
• http://kidshealth.org/kid/health_problems/ heart/cystic_fibrosis.html