Cystic Fibrosis

Cystic fibrosis is an inherited disease that affects sodium channels in the body and causes respiratory and digestive problems.

Causes, Incidence, And Risk Factors of Cystic Fibrosis:

Cystic fibrosis affects the mucus and sweat glands of the body and is caused by a defective gene. Thick mucus is formed in the breathing passages of the lungs and this predisposes the person to chronic lung infections.

When someone has cystic fibrosis many pancreatic enzymes involved in the breakdown and absorption of fats in the intestine are absent, causing malabsorption (inadequate absorption of nutrients from the intestinal tract) and malnutrition.

Most males with cystic fibrosis are infertile. About one in 2500 Caucasians is affected, and 2-5% of Caucasians carry the cystic fibrosis gene.

Cystic fibrosis is the most common fatal hereditary disorder affecting Caucasians in the United States. It is most common among Caucasians of Northern or Central European descent and is much less common in other ethnic groups. Risk factors include a family history of cystic fibrosis or unexplained infant death

Treatment of Cystic Fibrosis

Early recognition of cystic fibrosis and a comprehensive, multidisciplinary treatment program can lengthen survival time and improve quality of life. Specialty clinics for cystic fibrosis can be found in many communities and may be helpful.

Medications for cystic fibrosis include antibiotics for respiratory infections and pancreatic enzymes to replace those which are missing. Vitamin supplements are sometimes prescribed. Inhaled bronchodilators are used to relieve chronic obstruction of the airways.

A recent study showed that the pain reliever ibuprofen may slow lung deterioration in some children with cystic fibrosis. The results were most dramatic in children ages 5 to 13. More research is being done.

Learn more about cystic fibrosis at the Brenner Children’s Hospital web site.

Cystic Fibrosis