Causes Of Cystic Fibrosis

746 Words3 Pages
Cystic Fibrosis is a very serious inherited genetic disease which affects one child in every 2,500 Caucasian births. This disease usually affects the lungs and causes the build up of a thick mucus which causes chest infections. These infections unfortunately usually lead to death during childhood or early adulthood. On occasion, besides affecting the lungs, it affects the bowels and the pancreas, causing blockages in the intestines and poor absorption of food. Cystic Fibrosis may first appear in a newly born baby, or in children or young adults. However, 7-10% of cases are apparent at birth. These newborns are born with “meconium ileus.” Normally, meconium is dissolved in a few days after enzymes from the pancreas act upon it. However, Cystic…show more content…
We inherit a complete set of genes from each parent, which results in pairs of genes. Every parent either contributes a normal gene or a mutated gene. Gene pairs, then, can consist of two mutated genes, two normal genes, or one mutated gene and one normal gene. Because the affected gene (found on the number 7 chromosome) is a recessive gene, one must inherit two copies of the affected gene in order to have the disease. If you inherit only one affected gene than you will not have Cystic Fibrosis but you will be a ‘carrier’. One in twenty Caucasians are carriers of Cystic Fibrosis. Almost 90% of Cystic Fibrosis cases are caused by a mutation called DeltaF508. The mutated genes cannot manufacture a protein called CFTR (cystic fibrosis transmembrane conductance regulator) correctly. The CFTR protein allows chloride ions to exit mucus producing cells and water molecules to follow the chloride ions out. When the process is normal, mucus is of normal consistency. However, when chloride ions are trapped and water cannot escape, mucus becomes thick and sticky. The result is the disease we call cystic…show more content…
Like the lungs, CF also affects the narrow ducts of the pancreas, which secretes enzymes that digest food into the bowel, which become blocked by mucus. The mucus prevents important digestive enzymes from reaching the small intestine. This causes malabsorption syndrome. Malabsorption syndrome affects eighty-five percent of CF patients. Therefore, they require an energy-dense diet high in protein, fats, and vitamins. Patients also receive additional A, D, E and K vitamins. Poor absorption also hinders patients’ ability to gain weight and to grow normally. However, the most serious impact of Cystic Fibrosis is still breathing. Doctors can recognize Cystic Fibrosis by testing the salt content of the sweat; it is much higher in people with the disease. A chest X-ray and tests of the lung function will usually be undertaken. If chest infections occur frequently, a sample of sputum (phlegm) will be analyzed for the presence of bacteria. When Cystic Fibrosis is detected the patient has many treatment options. The overall aims of treatments are to treat infections, keep the lungs clean and free of sputum, maintain adequate nutrition, and to improve

More about Causes Of Cystic Fibrosis

Open Document