What is Cystic Fibrosis?
Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:
- clogs the lungs and leads to life-threatening lung infections; and
- obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.
Symptoms of Cystic Fibrosis
People with CF can have a variety of symptoms, including:
- very salty-tasting skin;
- persistent coughing, at times with phlegm;
- frequent lung infections;
- wheezing or shortness of breath;
- poor growth/weight gain in spite of a good appetite; and
- frequent greasy, bulky stools or difficulty in bowel movements.
- About 1,000 new cases of cystic fibrosis are diagnosed each year.
- More than 70% of patients are diagnosed by age two.
- More than 40% of the CF patient population is age 18 or older.
- The predicted median age of survival for a person with CF is more than 37 years.
Since 1955, the Cystic Fibrosis Foundation has been the driving force behind the pursuit of a cure. Thanks to the dedication and financial backing of our supporters–patients, families and friends, clinicians, researchers, volunteers, individual donors, corporations and staff, we are making a difference.
- Learn more about Testing for Cystic Fibrosis.
- Explore all aspects of Living with CF at every stage of life.
- See what Treatments are available and what’s in development.
What is Pseudomonas Aeruginosa?
About 60% of people with cystic fibrosis have a chronic respiratory infection caused by a bacteria called Pseudomonas aeruginosa that settles into the thick mucus trapped in the airways. Once it sets up house in the respiratory tract, Pseudomonas aeruginosa is hard to get rid of. Respiratory failure caused by the infection is often the ultimate cause of death in many people with CF.
How is Pseudomonas Aeruginosa Treated?
Years ago, chronic Pseudomonas aeruginosa infection in people with CF was treated rather unsuccessfully with oral antibiotics or inhaled injectable formulations of antibiotics. In the late 1990s an inhaled form of the antibiotic tobramycin, or TOBI®, was developed specifically for the treatment of chronic respiratory Pseudomonas aeruginosa infections. TOBI® is now the standard treatment for this infection, and is a commonly prescribed medication in people with cystic fibrosis.