What is cystic fibrosis?
Cystic fibrosis is a disease that causes Reference mucus Opens New Window 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 Reference lungs and the pancreas Opens New Window Reference Opens New Window. People who have cystic fibrosis can have serious breathing problems and lung disease. They can also have problems with nutrition, digestion, growth, and development. 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 one of the most common Reference genetic disorders Opens New Window in white children in the United States. It's caused by a change, or mutation, in a Reference gene Opens New Window. The changed gene is passed down in families. To pass on this disease, both parents must be Reference carriers Opens New Window of the changed gene.
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.
- 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.
- Reference Rectal prolapse Opens New Window (when part of the rectum protrudes from the anus).
- Growths (Reference polyps Opens New Window) 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 Reference sweat test Opens New Window Reference Opens New Window to see how much salt is in your child's sweat. People with cystic fibrosis have sweat that is much saltier than normal. The doctor may also suggest a genetic test. Finding a high amount of salt in two sweat tests or finding certain changed genes 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.
Frequently Asked Questions
Learning about cystic fibrosis:
Living with cystic fibrosis:
|By:||Reference Healthwise Staff||Last Revised: Reference March 12, 2012|
|Medical Review:||Reference John Pope, MD - Pediatrics
Reference Susanna McColley, MD - Pediatric Pulmonology