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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.
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Living With Links
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