Cystic fibrosis (CF) is a severe, genetically-determined disease involving the lungs, the gastrointestinal tract, and other organs. With cystic fibrosis, the body produces abnormally thick, sticky mucus. This mucus clogs the lungs, causing life-threatening lung infections, and obstructs the pancreas, impairing the ability of the intestines to break down food.
Cystic fibrosis occurs in about 1 in 3,000 live births among white children and at a far lower rate in black and Asian children. Boys and girls get cystic fibrosis equally. At present, the predicted median age for survival for a person with CF is 37 years old. However, that age is rising as an increasing number of patients are only mildly affected and survive longer.
Children with cystic fibrosis typically have frequent pulmonary infections. Since cystic fibrosis affects the glands that secrete sweat, they may also suffer from salt depletion while sweating during hot weather.
Fever, cough, difficulty in breathing, fast respiration, flaring of the nostrils, poor appetite, and reduced activity are typical features of acute cystic fibrosis. Cystic fibrosis is diagnosed with a sweat test, which measures the amount of salt in perspiration. If that test is positive, it should be confirmed with a second test. Since a child's siblings run an increased risk of also having cystic fibrosis, they should be tested as well.
The Cystic Fibrosis Center at NewYork-Presbyterian/Columbia University Medical Center is fully accredited by the Cystic Fibrosis Foundation and provides care annually for over 175 individuals with CF, nearly 40% of whom are adults. Our multidisciplinary team consists of adult and pediatric pulmonary physicians, gastroenterologists, genetic counselors, nutritionists, physical therapists, a nurse clinician, and a social worker.