Cystic Fibrosis (CF)
What is cystic fibrosis?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
Approximately one in 31 people in the US are carriers of the cystic fibrosis gene. These people are not affected by the disease, and usually do not know that they are carriers.
Cystic fibrosis is a complicated illness that requires clinical care by a physician or other healthcare professional. Listed in the directory below you will find additional information about cystic fibrosis, for which we have provided a brief overview. Multidisciplinary teams organized into CF centers are recommended for care of CF and have been shown to result in less illness and longer lives for CF patients.
The Cystic Fibrosis Center at Lucile Packard Children's Hospital at Stanford is the largest CF program in northern California. It is the only CF Foundation-designated Therapeutic Development center in California, Nevada and Oregon (only one of two West of the Rockies). Our program participates as a national leader in clinical trials and the development of novel therapies.
The CF Center is an integrated disease management program that follows patients from diagnosis through adulthood. With the current longer life expectancy for patients with cystic fibrosis, our clinic population includes patients of all ages. More than half the patients followed by the CF Center are adults aged 18 or older.
If you cannot find the information for which you are interested, please visit the Respiratory Disorders Online Resources page in the Web site for an Internet/World Wide Web address that may contain additional information on that topic.
Genetics of Cystic Fibrosis
Cystic Fibrosis and the Respiratory System
Cystic Fibrosis and the Digestive System
Cystic Fibrosis and the Reproductive System
Symptoms of Cystic Fibrosis
Diagnosis of Cystic Fibrosis
Treatment of Cystic Fibrosis
Feeding Your Child With Cystic Fibrosis
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