Approximately 30,000 children and young adults in the United States have Cystic Fibrosis. It is the most common fatal, inherited disease today. Cystic Fibrosis affects the body's respiratory and digestive systems. This disease occurs in about one of every 3,000 children born every year. One out of every twenty Americans carries the CF gene. These people do not have the disease, but given the right (or wrong) circumstances might pass it on to their children.

Today, thanks to medical interventions the median age for survival for a person born with CF is 31 years. In the 1950's those born with CF died in early childhood and even in the 1970's the median age was only 10-12 years old.

People who have CF produce abnormally thick, sticky mucus, which clogs the lungs and leads to chronic respiratory infections. Those with the disease may develop a barrel shaped chest. Reduced oxygen absorption leads to a rounding and enlargement of the nail bed in the fingers and toes.

The mucus also obstructs the ducts in the pancreas, preventing digestive enzymes from reaching the intestines. Those with CF have trouble absorbing nutrients, eliminating nondigested food through the bowel causing very large, foul smelling stools. Because those with CF absorb so little food, they have difficulty gaining and maintaining weight. Despite a healthy diet and good appetite, those with CF must constantly be monitored.

The symptoms of CF vary. While some children may exhibit symptoms at birth (a salty taste when kissed by their parents), others may not develop symptoms for weeks, months or even years. The severity of symptoms also vary, with some children displaying only mild respiratory and digestive problems, while others display major malabsorption problems and life threatening respiratory complications.

Although the median age for CF sufferers has risen in past years, most do not have children on their own. Another complication of CF is the inability to reproduce. Females born with CF have normal reproductive organs but their thick cervical secretions block sperm entry, preventing them from getting pregnant. Males born with CF are almost always sterile because they produce too little or no sperm. In the unlikelihood of pregnancy those with CF have a 90% failure rate.

The most common symptoms of CF are:

Salty tasting skin.

Bulky, foul smelling stools.

Excessive appetite with poor weight gain.

Persistent respiratory or intestinal infections.

A barrel chest.

Clubbed nail beds.

The standard diagnostic test for CF is the sweat test. To conduct this totally painless test, a doctor or nurse gives the child a medication that stimulates the production of sweat. Then he or she applies a gauze pad to the arm and wraps it in plastic. After 30 minutes the plastic is removed and the sweat is analyzed. Because newborns do not have active sweat glands, an immunoreactive tryosinogen test is performed.

Other diagnostic tests include chest x-rays, lung function tests and analysis of stool samples. The detection of CF in a fetus is now possible through genetic testing. It is recommended that couples that are pregnant or plan on becoming pregnant, who have a family history of CF, be genetically tested. Genetic testing will identify 90% of all CF carriers.

CF sufferers are living longer, largely because of advances in medical knowledge. Today treatment focuses on both the respiratory and digestive components of the disease. Antibiotic therapy and improved nutritional management has greatly advanced the treatment of CF also.

In the management of respiratory problems, the major focus is the loosen of infection. Daily percussion and postural drainage (clapping on the back), helps to loosen lung secretions and stimulate coughing. This is usually performed twice daily and more frequently when the person has an active lung infection. Family members can perform this for young children, while older children and young adults can learn to do it for themselves. Also regular exercise helps to loosen and move secretions. Antibiotic therapy is also crucial in treating CF lung infections.

People with CF must eat a well-balanced high-calorie, high-protein diet to effectively manage digestive problems. Their reduced absorption of nutrients and vitamins often means that children with CF must consume 150% of the recommended daily allowances to even meet their minimum growth needs. Multivitamins and supplements are also very important. To help digestion, pancreatic enzymes have to be taken before every meal and those with severe digestive problems may need supplemental feedings.

Prevention of CF is currently not possible. With medical intervention the disease can be managed effectively. Researchers are developing more effective medications and hope to someday be able to eradicate the disease entirely.