Cystic Fibrosis - What Happens

What Happens

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:

• 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.
• 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 babies.
• Reproductive organs. More than 95% of men who have cystic fibrosis are unable to father a child (infertile).¹ Women who have cystic fibrosis can have successful pregnancies, but they may have more difficulty getting pregnant than other women.
• 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 fractures, osteopenia or 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.

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.