Cystic fibrosis (CF) is an inherited disease characterized by an abnormality in the body's salt, water- and mucus-making cells. It is chronic, progressive, and is usually fatal. In general, children with CF live into their 30s.
Children with CF have an abnormality in the function of a cell protein called the cystic fibrosis transmembrane regulator (CFTR). CFTR controls the flow of water and certain salts in and out of the body's cells. As the movement of salt and water in and out of cells is altered, mucus becomes thickened. The thickened mucus can affect many organs and body systems including:
- respiratory - sinuses and lungs
- digestive - pancreas, liver, gallbladder, intestines
- reproductive - more so in the male, where sperm-carrying ducts become clogged
- sweat glands
Many children with cystic fibrosis develop liver problems later in life.