Cystic fibrosis (CF) is a genetic disease that causes excess mucus production in the lungs and digestive system. This genetic defect makes the mucus thicker than normal, causing it to clog airways and drainage ducts in the body and making it difficult to breathe. It mostly affects the lungs and pancreas, but other areas of the body can be affected. Also, bacteria can be trapped, resulting in frequent infections, which damage the lung tissue and cause respiratory failure. In the pancreas, thick mucus blocks the ability to secrete enzymes that aid digestion and nutrient absorption. This can lead to delayed growth in children due to malnourishment. The known cause of CF is a genetic defect in the CFTR gene. This gene is responsible for the CFTR protein which helps your body move salt and water through the cells. When this gene is defective, the salt is moved to the skin and less is deposited inside the body. Since salt attracts water, not enough gets into the secretions and this causes them to become very thick and sticky. Cystic fibrosis affects over 30,000 people in the U.S and over 70,000 all over the world. It is a progressive lung and digestive disease that can be life-threatening. The majority of cystic fibrosis cases only survive into their teens up to their 20s, but a small number of people with CF may live up to mid-life.
The most obvious indicator of CF is salty skin. This is because the disease causes the body to deposit more salt in the sweat than normal. The other common symptoms include:
Chronic cough and wheezing.CF causes the mucus to become very thick and sticky. It clogs up the airways and is very hard to clear.
Stuffy nose. Both thickened mucus and polyps in the sinuses and nose can cause a chronically stuffy nose and increased cases of sinus infections in CF.
Frequent respiratory infections. Thick mucus can trap bacteria in the respiratory tract leading to more upper and lower respiratory tract infections in CF sufferers.
Shortness of breath. This is due to the airways getting clogged with mucus and reducing the airflow to and from the lungs.
Reduced exercise tolerance. Not only does reduced lung function affect the ability to exercise, but also increased inflammation and muscle weakness. While this is a symptom, exercise is still important to CF patients.
Fatty, foul-smelling stools. This is due to a lack of digestive enzymes normally secreted by the pancreas.
Slow weight gain and slow growth. Not enough digestive enzymes, plus mucus in the intestines reduces nutrient absorption.
Constipation and intestinal blockage. These symptoms have a few different factors. First, the thick intestinal mucus slows down the movement of stool through the intestines. Next, pancreatic supplements are strongly believed to be constipating. Lastly, people with CF have high levels of inflammation in the intestines.