Treating Cystic Fibrosis

CF causes the body to produce an abnormally thick, sticky mucus, due to the faulty transport of sodium and chloride (salt) within cells lining organs such as the lungs and pancreas, to their outer surfaces. The thick CF mucus also obstructs the pancreas, preventing enzymes from reaching the intestines to help break down and digest food. Specialized treatments are available to address these problems.

People with CF have a variety of symptoms including: very salty-tasting skin; persistent coughing, at times with phlegm; wheezing or shortness of breath; an excessive appetite but poor weight gain; and greasy, bulky stools. Symptoms vary from person to person due, in part, to the more than 1,000 mutations of the CF gene.

Treating the Lungs
The treatment of CF depends upon the stage of the disease and the organs involved. Clearing mucus from the lungs is an important part of the daily CF treatment regimen. Chest physical therapy is a form of airway clearance done by vigorous percussion (by using cupped hands) clapping on the back and chest to dislodge the thick mucus from the lungs. After lying down in a position that helps drain mucus from the lungs, the person has a helper, such as a parent, gently bang on his or her chest or back to loosen the mucus. There is also a newer device called a therapy vest that shakes the chest, which allows teens to be more independent by doing their therapy on their own.

Other types of treatments include TOBI ® (tobramycin solution for inhalation), an aerosolized antibiotic used to treat lung infections; Pulmozyme ® , a mucus-thinning drug shown to reduce the number of lung infections and improve lung function; and azithromycin, an antibiotic recently proven to be effective in people with CF whose lungs are chronically infected with the common Pseudomonas aeruginosa bacteria. These antibiotics are administered intravenously, via pills, and/or medicated vapors which are inhaled to open up clogged airways.

Treating the Digestive System
When CF affects the digestive system, the body does not absorb enough nutrients. Therefore, people with CF may need to eat an enriched diet and take both replacement vitamins and enzymes.

In the pancreas the small channels (through which the enzyme produced in the pancreas flow to reach the intestines) become blocked with mucus. This results in cysts being formed and these lead to fibrosis in the pancreas. The enzymes produced by the pancreas are vital for normal digestion. Digestive enzyme preparations can replace most of the digestive enzymes produced by the pancreas

Insulin is also produced by the pancreas. However, in most cases of cystic fibrosis, the pancreas usually manages to produce enough insulin in childhood but diabetes is increasingly common in adults.

If you have CF, focus on staying as healthy as possible by following your doctor's treatment instructions, taking your medications, eating right, and exercising. Meeting other teens who have CF via chat rooms is fine, although personal contact between two CF patients can present problems since it carries the risk of passing dangerous