Cystic Fibrosis

Although 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:

• Reference 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 damage.
• Reference Pancreas and digestive system. The first sign of pancreas and digestive system problems may be a Reference 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 Reference pancreas Opens New Window 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.
• Reference 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 Reference dehydrated Opens New Window 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 babies.
• Reference 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 women.
• Reference 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 Reference fractures Opens New Window, Reference osteopenia Opens New Window, or Reference osteoporosis Opens New Window. Cystic fibrosis can also cause swollen or painful joints (arthropathy or Reference arthritis Opens New Window). These problems are more common in adults than in children.

Regular medical care, home treatment such as Reference postural drainage Opens New Window, 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. Reference Lung transplant surgery may be an option for people who have very severe lung disease.

Life expectancy

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.

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