Cystic Fibrosis: A Genetic Disorder Affecting the Lungs and Digestive System
Cystic fibrosis is an inherited condition that causes the lungs and digestive system to become clogged with thick, sticky mucus. This mucus blocks airways and can cause infections. It also makes it harder for the body to absorb the nutrients needed from food.
Diverse Symptoms and Severity
Symptoms vary depending on which organs are affected and the severity of the disease. Some people with CF have few or no symptoms, while others experience severe symptoms or life-threatening complications.
Pulmonary Problems: A Common Complication
The most common complication of cystic fibrosis is problems with the lungs (pulmonary or respiratory problems). These problems can lead to pneumonia, lung damage and breathing difficulties. Other problems can include chronic cough, a thick, sticky mucus that forms in the chest and lungs, and inflammation of the lining of the nose.
Weight Loss and Digestive Problems
Other common complications of CF are weight loss and problems with digestion, because the intestines cannot digest foods as well as they should. These symptoms can slow growth, make it hard to gain weight and keep up with daily activities and may require medical treatments.
Early Diagnosis and Treatment
Early diagnosis and treatment of pulmonary and digestive problems can help control the disease and improve your quality of life. Your doctor will recommend treatment that includes medicines to thin the mucus, reduce infection, and prevent lung problems.
Diagnostics and Genetic Testing
Your doctor will also likely do a variety of tests to see how your lungs work. These tests include X-rays and a CT scan, which can show how much air you can inhale and exhale. They can also show the extent of lung and pancreas damage caused by CF. Blood samples can also be tested for the faulty gene that causes CF. Getting this test before pregnancy is recommended because if both parents have the faulty gene, each pregnancy has a 25 percent chance of producing a child with CF.
Revealing Signs through Chest X-rays
Chest X-rays are helpful for revealing swelling in the lungs due to blockages in the respiratory passageways. This can be caused by fluids that have built up in the lungs or scarring of the walls of the lungs.
Pulmonary Function Tests
Pulmonary function tests determine how well the lungs work and are often used to check for a buildup of bacteria or other infections. These tests also can tell whether the lungs have stopped working properly, which is called respiratory failure.
Treatment Approaches
If your lungs are working well, your doctor may recommend treatment that includes medicines to open the airways, antibiotics to prevent lung infections, and breathing devices that help clear the airways. Your doctor can also prescribe a diet that includes more protein and calories to compensate for the nutrients your lungs are missing. Additionally, a special type of antibiotic called a glucocorticoid can be prescribed. It will increase your body’s ability to fight infections and reduce your risk of developing lung infections and pneumonia.
A Brighter Outlook
The outlook for people with CF is improving today, thanks to new treatments. Most people with CF live into their 40s and 50s, and in some cases, longer.
