Cystic Fibrosis

Cystic Fibrosis is an inherited chronic disease that affects the lungs and digestive system of many people in the United States. A defective gene and its protein product cause your body to produce thick, sticky like mucus that clogs the lungs and leads to lung infections, it obstructs the pancreas and stops enzymes from helping your body absorb and break down food. The symptoms and signs of Cystic Fibrosis are: salty tasting skin, persistent coughing with phlegm, frequent lung infections, wheezing or shortness of breath, poor growth, weight gain in spite of a good appetite, and greasy, bulky stools or difficulty in your bowel movements. Today, medical treatments have further enhanced and extended life for children and adults with CF (Cystic Fibrosis). People with the disease can now usually live into their 30s, 40s, and even longer. This disease has about 1,000 new cases of CF diagnosed each year. More than 70% of patients are diagnosed with disease by the young age of 2. A little less than half of the CF patient population have an age of 18 or older. The age predicted to live by CF patients is 30 years old.  Not everyone who has the mutant gene can get CF. You can have 1 perfect copy of that gene, but it takes 2 perfect copies to get it.
      The function of the protein that is defective in CF is usually in the epithelial cells lining the airways of the lung. There are channels in the lining cells where ions can pass. Usually the movement of ions brings water to the surface of the airway and keeps the mucus from getting dry, and staying moist. The gene that is defective blocks the channels, and causes the mucus to dry out. Then it makes it difficult  for somebody to sift the mucus, which becomes prone to infection by bacteria. In the lining of a person with CF, the vital chloride channel is blocked. so there is no movement of chloride ions in the mucus. Having no ionic gradient, theres is no water that moves towards the surface, and the mucus ends up drying out. Sometimes the protein may be present in the cell membrane, but may not function the way it is supposed to.
    There are a few ways that you can treat having the disease of CF and 3 of those ways are; Antibiotics, mucus-thinning drugs, and bronchodilators. The drugs with antibiotics are used to treat and prevent lung infections. They may come in pill form, inhaled in a mist, or delivered intravenously. The mucus-thining drugs are drugs that reduce the sticky mucus that you get, and makes it easier to cough it up, which improves the function of your lungs. Bronchodilators are medications such as albuterol that can be used as inhalers, or nebulizers. They help you keep your airways open by relaxing the muscles around your bronchial tubes. People who have CF have to go through a lot, and you may experience many emotions, but especially anger and fear. They are most common in teens, but openly talking about it can help a lot. IIt would also help to talk to other people with the same problem as you. If you have a child with CF, you can join support groups, or meet with other parents on your own time that also have children with CF. Psychologists are also another a good thing to consider. They can help the children as the grow into their teen years. CF can be a very serious thing that you have to live with throughout your life time.