Cystic Fibrosis

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.

Surgical Treatment for Cystic Fibrosis

Although current medical management strategies can control the disease into adulthood, lung transplantation offers extended survival in patients with advancing pulmonary disease.

NYP/Columbia has taken a leadership role in perfecting techniques and patient selection criteria for lung transplantation in patients with cystic fibrosis. As a result we are home to the largest CF lung transplant program in the New York metropolitan area. We have expanded the program to include patients under the age of 18 years.

The CF group works closely with the Center for Lung Disease and Transplantation at Columbia University Medical Center, providing ready access for CF patients to be evaluated for transplantation. After a patient receives a lung transplant, we continue to work closely with the transplant specialists to ensure continued medical care of issues unique to CF patients.

To learn more about lung transplantation, please click here.

Medical Treatment for Cystic Fibrosis

Cystic fibrosis is often treated with antibiotics, as well as through special exercises for draining mucus, including physical therapy for the chest, physical exercise, and aerosols. Diet therapy, which emphasizes the replacement of deficient digestive enzymes, is also critically important.

Some of the tenacity of the mucus and secretions results from increased amounts of DNA. An inhaled medication, Pulmozyme® contains an enzyme that breaks down the DNA, thereby liquefying the secretions and making them easier to clear.

Cystic Fibrosis Research Studies

Physicians in the Cystic Fibrosis program regularly participate in clinical research trials studying new treatments for the management of cystic fibrosis. Milestone studies include the Pulmozyme trial, the Thairapy Vest trial, and the TOBI trial. For more information about cystic fibrosis research, click here.