Cystic Fibrosis - Topic Overview

Topic Overview

What is cystic fibrosis?

Cystic fibrosis is a disease that causes mucus in the body to become thick and sticky. This glue-like mucus builds up and causes problems in many of the body's organs, especially the lungs and the pancreas. People who have cystic fibrosis can have serious breathing problems and lung disease. They can also have problems with nutrition, digestion, growth, and development. There is no cure for cystic fibrosis and the disease generally gets worse over time.

The life expectancy for people with cystic fibrosis has been steadily increasing over the past 40 years. On average, people who have cystic fibrosis live into their mid-to-late 30s, although new treatments are making it possible for some people to live into their 40s and longer.

What causes cystic fibrosis?

Cystic fibrosis is a genetic disorder. A child must inherit a specific gene from both parents to get cystic fibrosis.

What are the symptoms?

Cystic fibrosis is usually diagnosed at an early age. Although the symptoms are not the same for everyone, some common symptoms of a baby who has cystic fibrosis include:

• A blocked small intestine at birth, which prevents the baby from passing his or her first stool.
• Very salty sweat or skin.
• Diarrhea.
• Not growing or gaining weight the way that other children do.
• Breathing problems, lung infections, a cough that does not go away, and wheezing.

Other symptoms may also develop in childhood such as:

• Clubbing (rounding and flattening) of the fingers.
• Rectal prolapse (when part of the rectum protrudes from the anus).
• Growths (polyps) in the nose or sinuses.

How is cystic fibrosis diagnosed?

Most people who have cystic fibrosis have signs of it when they are children. In the United States, some states routinely screen newborn babies for cystic fibrosis. Screening tests look for a certain health problem before any symptoms appear. If your child has a positive newborn screening test or symptoms of cystic fibrosis, your doctor will order a sweat test to see how much salt is in your child's sweat. People with cystic fibrosis have sweat that is much saltier than normal. Most of the time, two abnormal sweat tests will confirm a diagnosis.

How is it treated?

Experts have not yet found a cure for cystic fibrosis, but new and improved treatments help people who have cystic fibrosis live longer. The types of treatment your child receives depends on what kinds of health problems the cystic fibrosis is causing and how your child's body responds to different types of treatment. Most people combine medicines, home treatment methods (including respiratory and nutritional therapies), and other specialized care to manage the disease.

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