For Acute Exacerbations
CF pulmonary exacerbations caused by bacteria infections require antibiotics. Pseudomonas aeruginosa is the most common organism found in the sputum cultures. P. aeruginosa is resistant to many antibiotics and therefore difficult to treat. For mild to moderate exacerbations, an oral drug may be adequate. If the P. aeruginosa is resistant or the exacerbation more severe, intravenous (IV) antibiotics are necessary. A combination of two antibiotics is often required and longer durations of treatment (three weeks or more) may be necessary. Patients are often able to administer intravenous antibiotics at home via a central venous catheter. Inhaled antibiotics may also be helpful because they deliver the maximum antibiotic concentration to the lungs.
For Chronic Infections
Since antibiotics do not typically eradicate the bacteria from the lungs of CF patients, ongoing antibiotic therapy may be administered. One concern with long-term antibiotic therapy is the development of more resistant bacteria. The decision to use scheduled antibiotics is based on lung function, exacerbation frequency, and the resistance profile of the bacteria.
Inhaled antibiotics such as a tobramycin improve lung function in patients. In addition, this inhaled antibiotic reduces the frequency of exacerbations. To minimize the risk of resistance, inhaled tobramycin is typically given in 28-day on-off cycles. Colistin, another commonly used inhaled antibiotic, may be associated with more bronchospasm than tobramycin, though pre-medicating with inhaled albuterol decreases that risk. Colistin can be used alone or in alternating 28-day cycles with tobramycin. Azithromycin, an oral antibiotic, has both anti-inflammatory and anti-microbial properties. Several studies suggest that ongoing use of azithromycin, typically given three times per week, may improve lung function and decrease the frequency of exacerbations. Bronchodilators
Many CF patients have asthma-like symptoms such as coughing and wheezing. Bronchodilators, administered via inhaler or nebulizer, relax the smooth muscle of the airways, and prevent or reverse bronchospasm. Bronchodilators come in short- and long-acting forms. The short-acting form (albuterol or levalbuerol) is used occasionally and prior to chest physical therapy to open airways and drain mucus. The long-acting form (salmeterol) is used in patients whose lung function has improved with bronchodilators. Anti-inflammatory Agents
Anti-inflammatory agents, such as corticosteroids, are used to combat asthma-like symptoms. Unfortunately, oral steroids have many side effects including bone loss and gastrointestinal irritation. Oral steroids are usually avoided except during acute exacerbations. Occasionally, long courses of oral steroids will be used to treat inflammation related to the fungus Aspergillus fumigatus, termed allergic pulmonary aspergillosis or ABPA. Inhaled steroids, which have fewer side effects, may be used to decrease lung inflammation. High-dose ibuprofen therapy, used for its anti-inflammatory properties in children, has been shown to slow the decline in pulmonary function. But this therapy can have side effects, including kidney dysfunction and GI bleeding, and is not typically used in adults. Lung Transplantation
Lung transplantation is an option for patients with very poor lung function, and who are considered to have limited survival. This surgery should be carefully considered. CF patients are often good candidates because they are relatively young and familiar with complex medical regimens. The transplanted lungs will not develop CF, but the sinuses and upper airways will continue to be affected. CF patients on high-dose steroids or with severe malnutrition, previous thoracic surgery, resistant bacterial or mycobacterial infections, poorly controlled diabetes, osteoporosis, poor compliance with previous therapy, and psychosocial issues, are not good transplant candidates. Lung transplantation is not for everyone. Successful transplantation requires a tremendous amount of work by the CF patient and caregivers. Also, lung transplantation is not a cure for lung disease. To prevent the rejection of the new lungs, transplant recipients must take strong immunosuppressants that make them susceptible to infection. The average five-year survival after lung transplant is about 50 percent, and additional medical problems may lower this life expectancy further. In some cases the risks of transplant outweigh the benefits. Also, a very limited supply of organs is available. |
