Cystic Fibrosis
Introduction:
Cystic fibrosis (CF) also known as mucoviscidosis is an inherited disease that causes the body to produce mucus that's extremely thick and sticky. The mucus in people with CF is thicker than normal because CF affects cells in the epithelium, the layer of cells that lines the passages in the body's organs. In a person who does not have CF, the epithelial cells produce a thin, watery mucus that acts like a lubricant and helps protect the body's tissues. In a person with CF, however, the thicker mucus doesn't move as easily. This thick, sticky mucus clogs passages in many of the body's organs and infection sets in.
The two organs that are most affected are the lungs and pancreas, where the thick mucus causes breathing and digestive problems (Warrell,2003). The thicker mucus has trouble moving out of the lungs, so bacteria can remain and cause infections. The thick mucus can also be found in the pancreas — an organ that produces proteins called enzymes that flow into the intestine to support the body's digestion process. Because the mucus can block the path between the pancreas and the intestines, people with CF have trouble digesting food and getting the vitamins and nutrients they need from it (Warrell,2003).
CF can also affect the liver, the sweat glands, and the reproductive organs (McPherson & Pincus,2006).
Cystic fibrosis is caused by mutations (changes) in a gene on chromosome 7, one of the 23 pairs of chromosomes that children inherit from their parents. Cystic fibrosis occurs because of mutations in the gene that makes a protein called CFTR (cystic fibrosis transmembrane regulator) (Warrell,2003). A person with CF produces abnormal CFTR protein — or no CFTR protein at all, which causes the body to make thick, sticky mucus instead of the thin, watery kind.
People who are born with CF have two copies of the CF gene. In almost all people born with CF, one gene is received from each parent. This means that the parents of kids with CF are
Cystic fibrosis (CF) also known as mucoviscidosis is an inherited disease that causes the body to produce mucus that's extremely thick and sticky. The mucus in people with CF is thicker than normal because CF affects cells in the epithelium, the layer of cells that lines the passages in the body's organs. In a person who does not have CF, the epithelial cells produce a thin, watery mucus that acts like a lubricant and helps protect the body's tissues. In a person with CF, however, the thicker mucus doesn't move as easily. This thick, sticky mucus clogs passages in many of the body's organs and infection sets in.
The two organs that are most affected are the lungs and pancreas, where the thick mucus causes breathing and digestive problems (Warrell,2003). The thicker mucus has trouble moving out of the lungs, so bacteria can remain and cause infections. The thick mucus can also be found in the pancreas — an organ that produces proteins called enzymes that flow into the intestine to support the body's digestion process. Because the mucus can block the path between the pancreas and the intestines, people with CF have trouble digesting food and getting the vitamins and nutrients they need from it (Warrell,2003).
CF can also affect the liver, the sweat glands, and the reproductive organs (McPherson & Pincus,2006).
Cystic fibrosis is caused by mutations (changes) in a gene on chromosome 7, one of the 23 pairs of chromosomes that children inherit from their parents. Cystic fibrosis occurs because of mutations in the gene that makes a protein called CFTR (cystic fibrosis transmembrane regulator) (Warrell,2003). A person with CF produces abnormal CFTR protein — or no CFTR protein at all, which causes the body to make thick, sticky mucus instead of the thin, watery kind.
People who are born with CF have two copies of the CF gene. In almost all people born with CF, one gene is received from each parent. This means that the parents of kids with CF are