Cystic Fibrosis

Currently, no cure for CF exists. Treatment of the disease mainly involves alleviating symptoms caused by the build-up of mucus. To combat the lung infections, many patients are given large doses of antibiotics to prevent a severe, life-threatening infection. Some people undergo a course of antibiotics four times a year, on a predetermined schedule. Mucus in the lungs also can be broken down by drugs called mucolytic agents. These agents can be orally. Other drugs are inhaled as aerosols.

A drug called Pulmozyme is an enzyme which breaks down the excess DNA present in the mucus of CF patients that accumulates as a result of the inflammatory process. Pulmozyme helps to thin the mucus, allowing it to be more easily expelled. Clearing the thick mucus from the lungs can also be accomplished by physiotherapy. Physiotherapy includes breathing exercises and percussion, the administration of blows to the back and chest to loosen the mucus.

To control the malabsorption of nutrients, many people with CF take pancreatic enzymes in pill form with every meal. A diet high in fat, protein, and carbohydrates is also recommended to increase the nutrient intake. Multi-vitamins can also help prevent deficiencies of certain vitamins. When these methods do not result in adequate weight gain some people supplement their diets with feeding tubes, or a nutrient-rich solution infused through a tube placed in the stomach. Newer advances in the types of pancreatic enzymes and nutritional supplements offered to CF patients are helping such patients avoid malnutrition.

A number of other recent therapies are available for CF patients. These include an inhaled form of the antibiotic called tobramycin. Previously given intravenously to treat infections, inhaled tobramycin appears to improve lung function, while avoiding some of the detrimental side effects associated with IV tobraymycin. There are also other exciting development drugs that are aimed at improving the function of the protein defective in CF.