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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 genesone 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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Living With Links
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