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Clinical Focus: Cystic Fibrosis

Cystic fibrosis (CF) is a genetic disease. Genetic can mean that a trait or disease is inherited from one parent, but a child with CF inherits the disease from both parents. Cystic fibrosis is also a chronic disease. This means the person will have it all of his or her life. We have no cure for CF yet, but we do have treatments to control the symptoms and to slow the progression of damage that CF causes. Parents usually find out that their child has CF in one of four ways:

  1. A mother has genetic screening during pregnancy that leads to the child being tested;

  2. A newborn infant is tested because a state has a newborn screening program;

  3. A child is tested because of a family history of CF;

  4. A child is tested because of symptoms of CF.

Airway clearance vest

The two most common problems in Cystic Fibrosis are partly caused by abnormal mucus, causing difficulty digesting food and lung infections. The body makes mucus as a way to keep itself lubricated and clean (rather like motor oil in a car engine). In healthy people, the lungs and the digestive tract both make mucus. In people with CF, the body makes mucus differently; it is much thicker and stickier. Abnormal mucus causes several problems. The thick and sticky mucus in the lungs of people with CF is hard to cough up. When mucus stays in the lungs, it makes an ideal place for bacteria to grow. This is why most people with CF eventually have lung problems. Children with CF take antibiotics to fight lung infections. Also, airway clearance and breathing treatments (inhaling medicine into the lungs) are used to help clear mucus from the lungs.

In time, almost everyone with Cystic Fibrosis develops lung problems. But the age at which lung problems appear, how often they occur, and how quickly they progress is different for each person. Some children who have CF have few or no symptoms for years. Others have many symptoms from the time they are babies. Symptoms may be mild or severe, and they may vary from child to child, even in the same family.

Thirty years ago, most children with Cystic Fibrosis died from the disease in childhood. That is not true any more. No one can say exactly how long any particular person with CF will live: however, more and more children with CF are living to be adults with happy, productive lives. Although CF is a life-shortening illness, more than half of the people with CF in the United States have lived past their late thirties in recent years. The average life expectancy with CF will improve as research helps find better ways of treating the disease. There is no cure for CF at this time, but medical research continues to make steady progress.

Article sourced from the Cystic Fibrosis Foundation & Baylor College of Medicine: Living with Cystic Fibrosis

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