Cystic fibrosis is a relatively rare inherited disease that affects the lungs and digestive system. The disease is chronic and progressive. In the past, it was believed that all cystic fibrosis patients succumbed to their disease by the age of 20. However, we are now finding that many of these people are living into their 50s and 60s. This may be because we now recognize more strains of the disease than we once did.
Because of the unique nature of this disease, it is critical that a team approach be used for long-term care and management. The team should include a pediatrician/internist, pulmonologist/cystic fibrosis
specialist, anesthesiologist, and otolaryngologist. Cystic fibrosis causes the exocrine glands, which produce sweat and mucus, to produce abnormal secretions. This can include unusually thick, sticky mucus that clogs the sinuses and/or the lungs, leading to chronic respiratory problems. The disease can also affect the mucus that is secreted in various parts of the digestive tract, obstructing the ducts in the pancreas and preventing digestive enzymes from reaching the intestines to properly digest food. As a result, people with cystic fibrosis have trouble breathing and absorbing nutrients. They also have trouble eliminating waste. Nasal polyps are another common result of this disease. Some scientists believe that cystic fibrosis polyps are different from the nasal polyps formed in the general population. However, many of the same symptoms occur, including nasal airway obstruction, absent or decreased sense of smell, runny nose, and exacerbation of CAID. Medical therapy emphasizes relieving nasal obstruction with decongestants and topical nasal steroids, and using antibiotics when necessary to fight infection. Mucolytics, such as guaifenesin, and nasal saline sprays are also recom - : mended. By loosening and eliminating nasal secretions, overall sinus drainage improves, and the probability of polyp buildup diminishes. The use of topical steroids has been shown to be beneficial for cystic fibrosis polyps as well. Nasal irrigation can be helpful in controlling infection and for clearing out the thick nasal secretions.
Surgical treatment is needed if there is a poor response to medical therapy. Between 10 and 20 percent of all cystic fibrosis patients will require surgical intervention for their sinus symptoms. Endoscopic surgical techniques allow for safe, minimally invasive surgery. Unfortunately, those with cystic fibrosis frequently suffer a recurrence of both nasal polyps and sinus disease. Interval antibiotic medical therapy slows the progression of disease, but frequent debridement and reoperation are usually necessary.