Prompt, aggressive and comprehensive treatment can help extend the lives of people with cystic fibrosis (CF) and improve their quality of life. Treatment of CF depends on the stage of the disease and the organs involved.
Treatment options include:
Airway Clearance Techniques (ACTs)
ACTs help people with CF breathe easier and stay healthy by loosening the thick, sticky mucus in the lungs, which can then be more easily removed by coughing or huffing. Clearing the airways helps to reduce the occurrence of lung infections and may improve lung function.
There are several different types of inhaled medications, including:
- Bronchodilators, such as albuterol, open the airways to help clear mucus and make breathing easier. They are given before performing ACTs.
- Mucolytics, such as Pulmozyme® (DNase), are medications that make mucus thinner, so it is easier to cough up.
- Mucoactives, such as Hypertonic Saline, are medications that help to stimulate cough and help to moisten thick mucous in the airways.
- Anti-inflammatories, such as inhaled steroids, reduce inflammation of the airways.
- Antibiotics treat infections and are taken after performing ACTs. With CF, inhaled TOBI® (tobramycin) is commonly used.
Lung infections caused by bacteria are common in people with CF. Medicines known as antibiotics stop bacteria from colonizing in the airways, making them an important part of CF therapy. Some people with CF need daily antibiotics; others need this type of medication only when they experience an exacerbation.
CF affects the pancreas in a majority of patients. As a result, the body may not be able to absorb enough nutrients to stay healthy. Weight has been significantly correlated to pulmonary function making every patients weight important. People with CF are encouraged to eat a high-calorie, high fat, and high protein diet regardless of a diagnosis of CF related diabetes. Taking enzymes before or during each meal and snack is necessary for those who have pancreatic insufficiency. In addition, supplemental vitamins are also important to take daily and should be taken with meals as these vitamins are fat-soluble and require enzymes for absorption. People with CF who cannot maintain a healthy weight through daily meals may be asked to take oral supplements daily or be advised to have a G-tube placement for assistance.
Lung transplants are serious and challenging procedures — and as such, they are not for everyone with CF. But their growing success rate makes them an option for some people with severe lung disease. Lung transplants are not a cure but they give those severely compromised with lung disease a better quality of life. Most lung transplants utilize lungs donated by a person who has died. Today, however, “living donor” lung transplants (also called lobar lung transplantations) are possible as well. The lungs have five lobes, and a healthy person may donate one healthy lobe and still live normally. In lobar transplantation, there are two donors; each gives one lobe to the recipient.
The Center for Cystic Fibrosis at Keck Hospital of USC is one of only a handful of adult CF programs in the U.S. to offer lobar lung transplantation.
Exercise is vitally important for people with CF. It helps to loosen mucus and stimulate coughing to help clear the mucus from the airways. It also strengthens breathing muscles, and improves the fitness of your heart and lungs. Daily exercise reduces your risk for respiratory infections and hospitalizations.
People with CF must take extra precautions to avoid infections. Frequent hand washing and use of antibacterial hand gels helps prevent the spread of germs, and annual immunizations help to protect against flu and pneumonia. CF patients should also refrain from smoking and avoid second-hand smoke and other environments and/or substances that can irritate the airways.
For a physician referral, call 1-800-USC-CARE.