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 DCI Home: Lung Diseases: Cystic Fibrosis: Key Points

      Cystic Fibrosis
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Key Points

  • Cystic fibrosis (CF) is an inherited disease of your secretory glands, including your mucus and sweat glands. CF mostly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs. It doesn't affect the brain.
  • If you have CF, your mucus becomes thick and sticky. It builds up in your lungs and blocks your airways. This leads to repeated, serious lung infections that can damage your lungs.
  • Lung function often starts to decline in early childhood in people who have CF. Over time, permanent damage to the lungs can cause severe breathing problems.
  • The thick, sticky mucus also can block tubes, or ducts, in your pancreas. As a result, the digestive enzymes that your pancreas makes can't reach your small intestine. Without these enzymes, your body can't absorb fats and proteins. This can cause vitamin deficiency and malnutrition.
  • CF also causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can upset the balance of minerals in your body and cause a number of health problems. Examples include dehydration, increased heart rate, tiredness, weakness, decreased blood pressure, heat stroke, and, rarely, death.
  • A defect in the CFTR gene causes CF. This gene makes a protein that controls the movement of salt and water in and out of your body's cells. Every person inherits two CFTR genes—one from each parent. Children who inherit a faulty gene from each parent will have CF.
  • Children who inherit one faulty gene and one normal gene will be "CF carriers." CF carriers usually have no symptoms of CF, but they can pass the faulty gene on to their children.
  • About 30,000 people in the United States have CF. It is one of the most common inherited diseases among Caucasians. About 1,000 new cases of CF are diagnosed each year.
  • The symptoms of CF vary from person to person and over time. Sometimes, you will have few symptoms. Other times, your symptoms may become more severe.
  • Doctors diagnose CF based on the results from various tests. Most States screen newborns for CF.
  • CF has no cure. However, treatments have greatly improved in recent years. Treatment may include nutritional and respiratory therapies, medicines, exercise, and more. Early treatment for CF can improve both your quality of life and your lifespan.
  • If you or your child has CF, you should learn as much as you can about the disease. Ongoing care and lifestyle measures can help you manage the disease.
  • As treatments for CF continue to improve, so does life expectancy for those who have the disease. Today, some people who have CF are living into their forties, fifties, or older.

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