Friday, January 8, 2010
What Is Cystic Fibrosis???
As May is CF awareness month, this is my attempt to educate, anyone who is interested, on the details of this part of our life. Stay tuned for more posts. Please ask questions if you have them. - CF is an inherited chronic condition that affects about 30,000 people in the U.S and 70,000 worldwide - Average lifespan is 37.4 years, death is usually caused by respiratory infections or distress. In 1959 the median lifespan was 6 months. In the 1980's, those with CF were not expected to live past their teens. - A person with Cystic Fibrosis must inherit one copy of the CF gene from EACH parent. - Cystic fibrosis is caused by the defect in the gene CFTR. This gene controls the movement of salt and water in and out of the body's cells. The defect causes the body to produce very salty sweat and abnormally thick and sticky fluid, which builds up primarily in the lungs and pancreas. - The result of this defective gene is an increased production of sweat and mucus to overcompensate for the problem. This causes blockage in the pancreas and lungs, which prevents proper function. - The blockage in the lungs makes it easy for bacterias (that poses no real threat to "normal" people) to collect and permanent damage to develop. - The blockage in the pancreas prevents much needed digestive enzymes from getting to the intestines where they aid in breaking down food. This hinders the digestion of nutrients necessary for the body to function properly. - There is a danger of having an electrolyte imbalance during physical exertion or in hot weather, due to salt being excreted in high amounts through the sweat glands. Last but not least... 90% of all males with CF are infertile - they have normal sperm production but are missing the vas deferens.