The lungs allow you to breathe in oxygen and release carbon dioxide. The lung structure allows you to breathe this oxygen in quickly and, just as quickly, release it into your bloodstream. Air is warmed and moistened in your mouth and nose, so that it is less irritating to the airways, while nose hairs trap particles such as dust, pollen, and bacteria. The filtered air moves through your airways, which are lined with mucus to collect any particles that evade the previous barriers. The air travels through smaller and smaller airway branches until reaching tiny air sacs called alveoli. This is where oxygen is exchanged for carbon dioxide. In CF, problems with electrolyte transport across airway branches lead to abnormal mucus production along the respiratory tract. This mucus is easily dehydrated and difficult to clear from the airways. Eventually, the mucus obstructs the airways, preventing the oxygen and carbon dioxide exchange. The abnormal mucus also clings to foreign particles that ultimately cause chronic lung infections. Complications of Lung Disease
Chronic Lung Disease
CF airways are clogged by thick, abnormal mucus, which make it difficult to clear foreign particles and infection-causing bacteria like Pseudomonas aeruginosa, Staphylococcus aureus, Hemophilus influenza and Burkholderia cepacia. These constant infections cause inflammation and lead to bronchiectasis, an abnormal stretching of the respiratory passages. Several tests determine the extent of infection, inflammation, bronchiectasis, and mucus clogging in a patient. Spirometry measures the amount of air patients breathe in compared to the amount they exhale. Changes in the rate of airflow will detect development, progression, and response to therapy. More extensive airway function tests such as chest X-rays and chest CT scans are also used. Pneumothorax
Pneumothorax is a potential life-threatening complication of CF. Literally, this word means “air in the chest,” but it actually refers to air surrounding the lungs. Normally, this space contains a small amount of fluid. When air enters because of a rupture of the lungs' smallest airways, the surrounding lung tissue may collapse. Pneumothorax occurs in 20 percent of CF patients over the age of 10, and 50 percent of these patients will have more than one occurrence. Symptoms include sudden chest pain and shortness of breath. A smaller pneumothorax may not cause symptoms, and tends to resolve itself. Chest X-rays usually confirm this problem. A small pneumothorax may be treated by breathing from an oxygen tank, but a larger pneumothorax is treated with a chest tube to remove the air. Hemoptysis
Hemoptysis, or coughing up blood, is a common CF complication. This condition varies from streaks of blood in mucus to mouthfuls of blood that are coughed up. Infection and inflammation change the lungs’ blood vessels, including wearing away of the airway blood vessel walls and formation of abnormal connections between different arteries that bleed easily. Infections increase the risk of hemoptysis, but can be treated with antibiotics. Minor hemoptysis is common, occurring in up to 75 percent of adults with CF. Major hemoptysis, defined as more than 200 milliliters of blood (about the volume of a can of soda), occurs much less frequently but requires immediate attention. Medications that affect the ability of the blood to clot, such as aspirin, should be avoided. Pulmonary Exacerbation
CF patients have regular pulmonary problems such as shortness of breath, increased cough and sputum production, fever, tiredness, and occasionally hemoptysis. These problems are caused by bacterial or viral infections in the lung airways leading to inflammation. Some pulmonary problems can be treated at home with antibiotics, aggressive chest therapy, and breathing treatments. If symptoms persist or worsen, hospitalization will likely be necessary for intravenous antibiotics and other treatments.
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