Cystic Fibrosis Fact Sheet
Cystic fibrosis treatment at UK HealthCare
The team at the Cystic Fibrosis Clinic works with more than 175 patients and is dedicated to working with the most advanced therapies to improve the life of every patient.
Understanding cystic fibrosis
Cystic fibrosis (CF) is a genetic disease affecting approximately 30,000 children and adults in the United States. A defective gene causes the body to produce abnormally thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. These thick secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines to help break down and absorb food. More than 80 percent of cystic fibrosis patients are diagnosed by age three; however, nearly 10 percent of newly diagnosed cases are age 18 or older.
Symptoms of cystic fibrosis
Patients with cystic fibrosis have a variety of symptoms:
- Very salty-tasting skin
- Persistent coughing
- Wheezing or shortness of breath
- Excessive appetite but poor weight gain
- Greasy, bulky stools
Symptoms may vary from person to person due to more than 1,000 mutations of the CF gene.
Diagnosing cystic fibrosis
The sweat test is the standard diagnostic test for cystic fibrosis. In the first part of the test, a colorless, odorless chemical, known to cause sweating, is applied to a small area on an arm or leg. An electrode is then attached to the arm or leg, which allows the technician to apply a weak electrical current to the area to stimulate sweating. The second part of the test consists of cleaning the stimulated area and collecting the sweat on a piece of filter paper, gauze or plastic coil. The collected sweat is sent to a hospital laboratory for analysis.
Treatment for cystic fibrosis
The treatment of cystic fibrosis depends upon the stage of the disease and the organs involved. Clearing mucus from the lungs is an important part of the daily CF treatment regimen. Chest physical therapy is a form of airway clearance done by clapping on the back and chest to dislodge the thick mucus from the lungs. Various antibiotics are also used to treat lung infections. When CF affects the pancreas, as it does in the majority of patients, the body does not absorb sufficient nutrients needed. Most people with cystic fibrosis take daily vitamin supplements, eat a high-calorie diet and take pancreatic enzyme replacements with every meal to help them digest their food.
Kentucky Children's Hospital
Cystic Fibrosis Foundation