We couldn’t breathe without our lungs, but how do we bring fresh oxygen into our bodies and remove carbon dioxide? First, the air is warmed and moistened in our mouth and nose. That way it’s less irritating to the airways in our lungs. Tiny nose hairs trap things like dust and pollen, keeping them out of our lungs. Also, slippery mucus lines our airways, catching any bits that still manage to get through. That way the air can travel through smaller and smaller airway branches, called bronchi, until it reaches tiny air sacs called alveoli. They actually look like bunches of balloons deep inside our lungs. When you breathe in by expanding the chest, these balloons expand as air rushes in. When you breathe out, the balloons relax and air moves out of the lungs. Tiny blood vessels surround each of the 300 million alveoli in the lungs.One important switch happens here. Oxygen moves out of the tiny balloons, which carries it into blood and to the rest of the body. Carbon dioxide, or waste gas, goes from the blood into the alveoli and its breathed out. But in CF, the body makes excess mucus in our airways, blocking them and not allowing oxygen to get in and waste gases to get out. Also, the abnormal mucus clings to bacteria that cause infections in our lungs. Complications of Lung Disease
Chronic Lung Disease
CF airways are clogged by abnormally thick mucus, making it difficult to clear foreign particles and bacteria. The result is constant infections that lead to bronchiectasis, an abnormal stretching of the airways. Several tests can determine the extent of infection, inflammation, bronchiectasis and mucus clogging in a patient. A test called spirometry measures the amount of air we breathe in compared to the amount we breathe out. Changes in the rate of airflow help doctors understand whether the medicines and treatments are helping to control the disease. 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. The word means “air in the chest,” but refers to air surrounding the lungs. Normally, this space contains a small amount of fluid. When air comes in from a rip or hole in the smallest airways of the lungs, the surrounding lung may collapse, and you can't breathe.
Pneumothorax occurs in 20 percent of CF patients older than 10-years-old; half of these patients will have more than one occurrence. Patients may feel sudden chest pain and shortness of breath. The good news is a smaller pneumothorax may not cause problems and may simply fix itself. Also, a small pneumothorax may be treated by breathing from an oxygen tank, while a larger pneumothorax requires a chest tube to remove the air. A chest X-ray tells doctors whether there is a pneumothorax and how big it is. Hemoptysis
Sometimes kids with CF can cough up blood, a condition known as hemoptysis. This can happen when an infection or inflammation changes blood vessels in the lungs, causing them to wear away or form abnormal connections with each other. The good news is that there are medicines that can treat the infections. But medications that affect the ability of the blood to clot, like aspirin, should be avoided. Pulmonary Exacerbation
CF patients may also experience shortness of breath, fatigue, fever and, increased coughing. These problems are caused by bacterial or viral infections and inflammation in the airways of the lungs. Some of these problems can be treated at home with antibiotics, chest therapy, and breathing treatments. But if symptoms continue or get worse, patients may have to go to the hospital for treatment.
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