Cystic Fibrosis - Topic Overview
Cystic fibrosis is a genetic
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,
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, and growth. The disease generally gets worse over time.
The life expectancy
for people with cystic fibrosis has been steadily increasing.
Cystic fibrosis is one of the most common genetic disorders in white children in the United States and Canada. It's caused by a change, or
mutation, in a
gene. The changed gene is passed down in families. To
pass on this disease, both parents must be
carriers of the changed gene.
Cystic fibrosis is usually
diagnosed at an early age. The symptoms aren't the same for everyone. But
some common symptoms in a baby who has cystic fibrosis include:
- A blocked small intestine at birth. This
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
- Clubbing (rounding and flattening) of the
- Rectal prolapse (when part of the
rectum protrudes from the anus).
- Growths (polyps) in the
nose or sinuses.
The doctor will likely notice the signs of cystic fibrosis during a routine exam. Some states routinely screen newborn babies for cystic fibrosis.
Screening tests look for a certain health problem before any symptoms
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. 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.
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.
Doctors usually recommend a combination of medicines, home treatment methods (including respiratory and nutritional
therapies), and other specialized care to manage the disease.