Alphabetic Guide to Child Care - Cystic fibrosis

Cystic Fibrosis

Cystic fibrosis is an inherited disease in which the child cannot handle the normal secretions of the respiratory tract. There is a lack of ciliary action—the beating movement by the hairs of the cells lining the bronchial tubes of the lungs. Thick mucus collects at the base of the lungs, obstructing the smaller air passages and causing labored breathing and chronic cough.

Respiratory Complications

Bacteria multiply in the accumulated lung secretions, predisposing the patient to chronic bronchitis and other respiratory infections, such as pneumonia. Lung tissue changes can result eventually in severe, permanent damage to the lungs.

Digestive Complications

The abnormally thick, viscous mucus produced by the cystic fibrosis patient tends to obstruct the ducts or openings of the mucus-secreting glands. When such mucus obstructs the pancreas, it interferes with its ability to supply important digestive enzymes to the intestinal tract, thus leading to poor digestion and malabsorption of a number of important nutrients. A child with cystic fibrosis may therefore be poorly nourished in spite of an adequate diet.

Other Complications

Tissue changes in the lungs can restrict blood flow to the heart, leading to increased blood pressure and chronic heart strain. Loss of large amounts of salt through malfunctioning sweat glands can become a very serious problem for youngsters in hot weather, causing dehydration and heat exhaustion. Laboratory tests usually find an abnormally high concentration of salt in the sweat of cystic fibrosis patients. (Indeed, the skin of a cystic fibrosis patient is apt to taste salty.) Some tissues may show three times the normal concentration of sodium and twice the normal levels of potassium.

Treatment

There is no known cure for cystic fibrosis. Treatment includes the use of humidifiers and inhalation medication in the form of aerosols to loosen secretions. Another method used is postural drainage— lying facedown with the head lower than the feet to let gravity help loosen secretions. Antibiotics are used to control infections; special diets with reduced fat intake and added nutrients are prescribed to compensate for abnormal digestive function. Other medical techniques are utilized in individual cases to maintain normal heart and lung function.

Scientists have discovered a molecular defect in the gene that regulates the expulsion and absorption of chloride and sodium ions by lung cells. Using inactivated cold viruses, doctors have been able to transmit a normal gene to the cells of the trachea and bronchi. Because the new normal gene does not become a part of the cells the treatment is not permanent and must be repeated.

Once considered strictly a disease of early childhood, an increasing number of cases of cystic fibrosis have been detected in recent years among adolescents and adults. Symptoms of the disease, including respiratory difficulty with chronic coughing, may resemble those of an allergic reaction.

How the Disease Is Inherited

Cystic fibrosis is transmitted to a child only when both parents are carriers of the trait. When only one parent carries the trait, however, some of the children can become carriers. Through marriage with other carriers, they may then become the parents of a child afflicted with the disorder.

Information on new treatment techniques, the location of treatment centers, and genetic counseling may be obtained from the national headquarters of the Cystic Fibrosis Foundation, 6931 Arlington Rd., Ste. 200, Bethesda, MD 20814.