Cystic Fibrosis
Friday, 28 January 2005 01:43
Cystic Fibrosis—or CF—is an incurable, genetically inherited and ultimately fatal disease that is typically diagnosed during early childhood, usually by age three.
The Mayo Clinic estimates that about 30,000 young children and young adults in the U.S. alone have CF. About 1,000 new cases are diagnosed each year. The average life expectancy of those with Cystic Fibrosis is 32 years, but advances in medical treatment have seen an increase in prolonged life with a higher quality of life.
Though anyone can get the disease, the likelihood of contracting it is greater among whites, particularly those who are of Northern European ancestry. Those people from that region have a one in 29 chance of carrying the CF gene. Blacks in the United States have a one in 62 chance of carrying the CF gene, whereas Asian-Americans have a one in 90 chance of carrying the gene, according to the Mayo Clinic.
People with Cystic Fibrosis have difficulty with the way salt, which is made up of sodium and chloride, move in and out of the mucus-producing cells in the body. As a result, mucus in the body becomes dry, thick and sticky. This mucus can prevent organs from secreting normally resulting in abnormal attraction of bacteria. Most of the serious problems that arise as a result of CF develop in either the lungs or pancreas.
Those who develop CF in the lungs, produce thick mucus that block airways which in turn lead to problems breathing and other respiratory complications. In the pancreas, CF causes the mucus to become extra thick leading to digestive problems and an increased likelihood of infections. The consequence of these poor digestive processes is impaired growth.
Those with CF of all ages have a hard time gaining and maintaining weight.
There are many warning signs of Cystic Fibrosis.
They include:
Blockage in the small intestine at birth—about 17 percent of newborn babies with CF have this symptom.
Salty sweat or salty skin.
Poor appetite; lack of energy and weight loss during infancy, usually this is a by-product of the thickening of mucus which prevents digestive juices from flowing in the pancreas.
Pancreatitis, an inflammation of the pancreas, which comes from what experts commonly call a “failure to thrive,” may be the first sign of CF in children.
Irregular bowel movements. Stool may also become greasy; have an especially bad odor as a result of blockages in the intestine; or those with CF may have uncontrollable diarrhea.
Respiratory problems. Those with CF typically have infections particularly of the lung, nose or sinuses.
Coughing and wheezing. Both are especially bad at night or in the early morning hours.







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