Lung and Airway Disorders Chapter 43 Cystic Fibrosis Cystic fibrosis is a hereditary disease that causes certain glands to produce abnormal secretions, resulting in several symptoms, the most important of which affect the digestive tract and the lungs. Cystic fibrosis is the most common inherited disease leading to death among white people in the United States. It occurs in 1 of every 2,500 white babies and in 1 of every 17,000 black babies. It's rare in Asians.
Cystic fibrosis is equally common in boys and girls.
Many people with cystic fibrosis die young, but 35 percent of Americans with cystic fibrosis reach adulthood. About 5 percent of white people carry one defective gene responsible for cystic fibrosis, but the trait is recessive, and the disease develops only if a person has two defective genes. When both genes are abnormal, chloride and sodium transfer is disrupted, leading to dehydration and increased stickiness of secretions. The secretions are abnormal in different ways, and they affect gland function. In some glands, such as the pancreas and those in the intestines, the secretions are thick or solid and may block the gland completely.