A disease handed down
NEW TECHNIQUES ALLOW YOU TO HAVE A BETTER QUALITY OF LIFE
Cystic fibrosis is an inherited disorder that affects the cells that produce mucus, sweat and digestive juices leading to severe damage to the lungs and digestive system.
These secreted fluids are normally thin and slippery. But in people with cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas. Due to advances in screening and treatment people with cystic fibrosis are able to have a better quality of life than in previous decades. The condition can be diagnosed within the first month of life, before symptoms develop. The signs and symptoms vary, depending on the severity of the disease. Even in the same person, symptoms may worsen or improve as time passes. Some people may not experience symptoms until adolescence or adulthood.
People with cystic fibrosis have a higher than normal level of salt in their sweat. Most of the other signs and symptoms of cystic fibrosis affect the respiratory system or the digestive system. However, adults with cystic fibrosis are more likely to have atypical symptoms, such as pancreatitis, diabetes and infertility. The mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause: A persistent cough that produces thick (sputum) mucus Wheezing (whistling sound when you breathe) Breathlessness Exercise intolerance Repeated lung infections Inflamed nasal passages or a stuffy nose
The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these, your intestines can’t fully absorb the nutrients in the food you eat. The result is often: Foul-smelling, greasy stools Poor weight gain and growth Intestinal blockage, particularly in newborns Severe constipation which can lead to rectal prolapse
In cystic fibrosis, an abnormality in a gene changes a protein that regulates the movement of salt in and out of cells. The result is thick, sticky mucus in the respiratory, digestive and reproductive systems, as well as increased salt in sweat. Children need to inherit one copy of the gene from each parent in order to have the disease. If children inherit only one copy, they won’t develop cystic fibrosis, but will be carriers and pass the gene to their children.
Damaged airways (bronchiectasis): a condition that damages the airways, making it harder to move air in and out of the lungs, and clearing mucus from their airways.
Chronic or recurrent infections: Thick mucus in the lungs and sinuses provides an ideal breeding ground for bacteria and fungi.
Growths in the nose (nasal polyps): Because the lining inside the