Currently, there is no cure. However, over the past 25 years, treatment for cystic fibrosis has improved dramatically. Improvements in the management of respiratory disease and malnutrition allow cystic fibrosis patients to survive into their early 30s and even 40s. In the United States, about 39 percent of cystic fibrosis patients are over the age of 18 years.
Much of the care has centered on improved breathing through the use of inhaled bronchodilators. New and more effective antibiotics have been better able to manage chronic infections. During severe infections which might require hospitalization, inhaled antibiotics are sometimes used. In a few cases, single- and double-lung or heart-and-lung transplants have been tried successfully.
The treatment of cystic fibrosis depends upon the stage of the disease and which organs are involved. Treatment for the lungs involves breathing exercises or techniques to dislodge and remove the thick mucus.
Chest physical therapy, or vigorous percussion (by using cupped hands) on the back and chest, is a common therapy. The chest vest uses high frequency chest wall oscillations to vibrate and remove the mucus. There are two devices patients can breathe through that also can help. These devices are called OPEP, or oscillatory positive expiratory pressure. As the patient breathes out through the device, airflow vibrations are created that help to break the mucus loose from the airway walls. Pressure is also generated during exhalation, which helps keep the airways open. There are 2 medical devices that provide oscillatory PEP, the Acapella, DHD Healthcare, and the Flutter.
Antibiotics are also used to treat lung infections and are administered intravenously, by mouth and/or inhaled. The antibiotics treat infection of the mucus-clogged airways. Nebulized drugs such as steroids, bronchodilators, antibiotics and agents that break up mucus are also important treatments.
When cystic fibrosis affects the digestive system, the body does not absorb enough nutrients. Therefore, people with cystic fibrosis may need to eat an enriched diet and take both replacement vitamins and enzymes.
To stay healthy, those with cystic fibrosis need regular visits and examinations at a Cystic Fibrosis Foundation-accredited care center. There are more than 110 cystic fibrosis care centers nationwide. These centers provide specialized care for those with cystic fibrosis. Usually patients are seen every six to eight weeks by the specialized cystic fibrosis team. This team consists of a doctor, nurse, respiratory therapist, dietitian, physical therapist, social worker and in some cases a psychologist. Knowing the change or increase in symptoms is helpful. It is important for patients with cystic fibrosis to exercise, eat healthy high calorie diets, and take their medications and treatments as directed even though the treatments take up a great deal of time.
Created: October 2004
Reviewed: May 26, 2005