Get the Facts about Cys­tic Fi­bro­sis

Wellness Update - - What Doctors Know And Your Should, Too! -

Cys­tic fi­bro­sis (CF) is an in­her­ited dis­ease char­ac­ter­ized by an ab­nor­mal­ity in the glands that pro­duce sweat and mu­cus. It is chronic, pro­gres­sive, and is usu­ally fa­tal. Due to im­proved treat­ments, peo­ple with CF, on aver­age, live into their mid to late 30s. Cys­tic fi­bro­sis af­fects var­i­ous sys­tems in chil­dren and young adults, in­clud­ing the fol­low­ing: • Res­pi­ra­tory sys­tem • Di­ges­tive sys­tem • Re­pro­duc­tive sys­tem There are about 30,000 peo­ple in the US who are af­fected with the dis­ease, and about 1,000 ba­bies are di­ag­nosed with it each year. It oc­curs mainly in Cau­casians, who have a north­ern Euro­pean hered­ity, al­though it also oc­curs in African-Amer­i­cans, Asian Amer­i­cans, and Na­tive Amer­i­cans. Ap­prox­i­mately one in 31 peo­ple in the US are car­ri­ers of the cys­tic fi­bro­sis gene. Th­ese peo­ple are not af­fected by the dis­ease and usu­ally do not know that they are car­ri­ers.

How does CF af­fect the res­pi­ra­tory sys­tem?

The ba­sis for the prob­lem with CF lies in an ab­nor­mal gene. The re­sult of this gene de­fect is an atyp­i­cal elec­trolyte trans­port sys­tem within the cells of the body. The ab­nor­mal trans­port sys­tem causes the cells in the res­pi­ra­tory sys­tem, es­pe­cially the lungs, to ab­sorb too much sodium and wa­ter. This causes the nor­mal thin se­cre­tions in our lungs to be­come very thick and hard to re­move. Th­ese thick se­cre­tions put the child with CF at risk for con­stant in­fec­tion. The high risk of in­fec­tion in the res­pi­ra­tory sys­tem leads to dam­age in the lungs, lungs that do not work prop­erly, and even­tu­ally death of the cells in the lungs. The most com­mon causes for in­fec­tion in the lungs of the CF pa­tient are the fol­low­ing bac­te­ria: • Staphy­lo­coc­cus aureus • Haemophilus in­fluen­zae • Pseu­domonas aerug­i­nosa (PA) Over a pe­riod of time, PA be­comes the most com­mon bac­te­ria that causes in­fec­tion and be­comes dif­fi­cult to fight. A large per­cent­age of res­pi­ra­tory in­fec­tions in the CF pa­tient are due to PA.

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