Children's Health Children's Diseases and Conditions

Understanding Cystic Fibrosis

Cystic fibrosis (CF) is a genetic disorder that causes severe lung damage and nutritional problems in children. Years ago children with the disease were not expected to survive beyond their teens, but today they can expect to live a lot longer thanks to a new understanding of the disease and the development of effective treatments.

Early diagnosis and starting the right treatment early is key to getting children with cystic fibrosis on track for a longer, more comfortable life. Dr. Bruce Marshall, vice president of clinical affairs at the Cystic Fibrosis Foundation, helps parents understand CF and all of the issues that go along with it.

What is cystic fibrosis?
CF is a genetic disease affecting approximately 30,000 children and adults in the United States. CF obstructs the movement of sodium and chloride (salt) from the inside of the cells that line certain organs (such as the lungs and pancreas) to their outer surfaces. This results in the body producing abnormally thick, sticky mucus in these organs. All of the trapped mucus causes various respiratory problems and makes patients prone to infection. When the thick CF mucus impacts the pancreas, it prevents enzymes from reaching the intestines to help break down and digest food.

Who most commonly gets the disease?
CF occurs in approximately one of every 3,500 live births in America. According to the 2004 Patient Registry, 95.3 percent of CF cases were in Caucasians, 6.7 percent were in Hispanics and 4 percent were in African Americans. CF is uncommon in Asians and most Native Americans. CF occurs equally in males and females.

More than 80 percent of patients are diagnosed by the age of three. However, nearly 10 percent of all newly-diagnosed cases are age 18 or older.

How is it transmitted?
CF is a genetic disease. You cannot "catch" it; you are born with it. In order for a child to have CF, both parents must be carriers of the CF gene. [There are genetic tests available to determine if you are a carrier]. But even if both parents are carriers of the gene, their children will not automatically have CF—there is a 25 percent chance that their child will have the disease.

What are the symptoms of cystic fibrosis?
The symptoms of CF vary from person to person. Some of the most common symptoms can include persistent coughing or wheezing—sometimes with phlegm—shortness of breath, respiratory infections, such as pneumonia, a failure to gain weight at the normal rate and difficulty in passing stools or frequent, abnormal stools. CF does affect the respiratory system, including the lungs and sinuses. It can also cause the person to have very salty-tasting skin. Note that CF does not cause a person to sweat more, it is just that they have more salt in their sweat.

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