Cystic Fibrosis - What Happens
cystic fibrosis generally follows certain patterns,
each person's symptoms depend on what is happening with his or her
mucus-producing cells. These kinds of cells are found throughout the body in
many different organs and systems, including the:
- Lungs and respiratory system. People
with cystic fibrosis have thick and sticky mucus that traps bacteria. This
causes lots of lung infections and often, permanent lung
- Pancreas and digestive system. The first sign of
pancreas and digestive system problems may be a
meconium plug that prevents the passing of a newborn's
first stool. Within the first year, a child may also have diarrhea that does
not go away or large, greasy, smelly stools. Mucus from cystic fibrosis can
interfere with how the
pancreas works, which then makes it hard for the child
to absorb nutrients from food. As a result, the child may not gain weight and
may even lose weight.
- Sweat glands. Parents of a newborn who
has cystic fibrosis may notice that their baby has unusually salty skin. Cystic
fibrosis can cause a person to become easily
dehydrated or to have very low salt levels. This makes
it very important for people with cystic fibrosis to drink lots of water and
fluids. People with cystic fibrosis need extra salt, even when they are
- Reproductive organs. Almost all men who have
cystic fibrosis are unable to father a child (infertile). Women who have cystic fibrosis can have successful
pregnancies. But they may have more difficulty getting pregnant than other
- Skeletal system. People who have cystic fibrosis may
have weaker bones than other people because their bones contain less minerals.
Weakened bones can lead to bone
osteoporosis. Cystic fibrosis can also cause swollen
or painful joints (arthropathy or
arthritis). These problems are more common in adults
than in children.
Regular medical care, home treatment such as
postural drainage, and attempts to reduce infection
can help people with cystic fibrosis lead relatively normal lives. If symptoms
of cystic fibrosis get worse, treatment in a hospital may be needed.
Lung transplant surgery may be an option for people
who have very severe lung disease.
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. People who have a mild form of cystic fibrosis may have a
normal life expectancy.
There is no cure for cystic fibrosis. But
experts are hopeful that the discovery of the cystic fibrosis gene defect may
soon lead to a cure.