Key Points
Cystic fibrosis (CF) is an inherited disease of your mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs.
In CF, an abnormal gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene causes mucus to become thick and sticky. The mucus builds up in the lungs and blocks the airways, creating an environment that makes it easy for bacteria to grow. This leads to repeated serious lung infections that can damage your lungs.
The mucus can also block tubes, or ducts, in your pancreas so that the digestive enzymes it produces cannot reach the intestines where they are needed to break down food.
You have extremely salty sweat. When you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood, which can cause a heat emergency.
The most common symptoms of CF are frequent coughing with phlegm, frequent bouts of bronchitis and pneumonia, salty-tasting skin, dehydration, poor growth, and infertility, mostly in men.
The sweat test is the most common diagnostic test for CF. It measures the amount of salt in your sweat.
Other tests that can be used to help diagnose CF include a chest x ray, sinus
x ray, lung function tests, analysis of sputum cultures and/or stool samples, and genetic testing of a blood sample.
Prenatal genetic testing can help you find out if your baby is likely to have CF.
Antibiotics are the primary treatment for lung problems in CF. They treat airway infections. Other treatments include chest physical therapy, exercise, mucus-thinning drugs, and other medications to reduce inflammation in your airways and help open them up.
Lung transplantation is an option for some people with CF.
The digestive problems in people with CF can be managed with nutritional therapy, enemas, mucus-thinning drugs, and medications to reduce stomach acid.
Ongoing medical care from a team of health care providers who specialize in CF is important. Good self-management includes eating a healthy diet, avoiding tobacco smoke, exercising frequently, doing chest physical therapy every day, drinking lots of fluids, and washing your hands often to reduce your chances of infection.
