About Cystic Fibrosis

Cystic Fibrosis is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract. It is one of the most common chronic lung diseases in children and young adults. Being diagnosed with the disease may result in early death.

Causes, Incidence, and Risk Factors

Cystic Fibrosis (CF) is caused by a defective gene which causes the body to produce abnormally thick and sticky fluid called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food.

This collection of sticky mucus results in life-threatening lung infections and serious digestion problems. The disease may also affect the sweat glands and the male reproductive system.

Millions of Americans carry the defective CF gene but do not show any symptoms. That's because a person with CF must inherit two defective CF genes - one from each parent. An estimated 1 in 29 Caucasian Americans have the CF gene. The disease is the most common deadly, inherited disorder affecting Caucasians in the United States. It's most common among those of Northern or Central European descent.

Most children with CF are diagnosed by age 2. A small number, however, are not diagnosed until age 18 or older. These patients usually have a milder form of the disease.

Expectations (Prognosis)

Most children with Cystic Fibrosis are fairly healthy until they reach adulthood. They are able to participate in most activities and attend school. Many young adults with Cystic Fibrosis finish college or find employment.

Lung disease eventually worsens to the point where the person is disabled. Today, the average life span for people with CF who live to adulthood is 35 years. Thanks to advances in treatment, life expectancy for those afflicted with CF has seen a dramatic increase over the last three decades.

Death is usually caused by lung complications.