Documente Academic
Documente Profesional
Documente Cultură
Cystic fibrosis
Cysticfibrosis(CF)isthemostcommonlifethreateninggeneticdisorderamongstCaucasians.It primarilyaffectstherespiratorysystem(lungs),thedigestivesystem(pancreasandsometimes liver)andthereproductivesystem.WhenapersonhasCFtheirmucusglandssecreteverythick stickymucus.Inthelungs,themucusclogsthetinyairpassagesandtrapsbacteria.Repeated infectionsandblockagescancauseirreversiblelungdamageandashortenedlife.Thepancreasis alsoaffected,preventingthereleaseofenzymesneededtodigestfood.Thismeansthatpeople withCFcanhaveproblemswithnutritionandmustconsumeadiethighinkilojoules,fats,sugar andsalts. People with CF produce abnormal mucus CFaffectstheexocrineglands,whichsecretebodyfluidssuchassweat,mucusandenzymes. PeoplewithCFproduceabnormallythick,stickymucuswhichblockssmallairpassagesinthe lungs.Thiscausesdifficultyclearinginfectionsandcanresultinlungdamageoveraperiodoftime. A range of symptoms PeoplewithCFmayhavethefollowingsymptoms: Persistentcough,withgreatphysicaleffort Somedifficultybreathing Tiredness,lethargyoranimpairedexerciseability Frequentvisitstothetoilet Saltlossinhotweatherwhichmayproducemusclecrampsorweakness Poorappetite.
Oneinevery2,500birthsproducesachildwithCF.Approximately3,000peopleinAustraliahave CF. CF is usually diagnosed at birth InmostAustralianStates,allbabiesarescreenedatbirthforCFusingaheelpricktest(Guthrie Test)whichinvolvescollectionofabloodsample.Iftheresultsofthebloodspottestrevealvery highlevelsofasubstancecalledImmunoreactiveTrypsin(IRT),CFissuspectedandtheDNAinthe bloodisthenanalysedforthemostcommonmutationcausingCF.Asweattestmaybedoneto measuretheamountofsalt(sodiumchloride)inthesweatandconfirmthediagnosis. Somebabiesmaybediagnosedshortlyafterbirthasaresultofanintestinalblockagecalled meconiumileus. MostbabieswhohaveCFarenowdiagnosedwithinthefirsttwomonthsoflife. Treatment aims to slow progress TreatmentforCFcanbeintensiveandtimeconsuming.AtpresentthereisnocureforCF. Treatmentaimstoslowprogressionoftheconditionandincludes: Chestphysiotherapy
Cysticfibrosis
Page1of2
Things to remember
This page has been produced in consultation with, and approved by:
CysticFibrosisVictoria
Cysticfibrosis
Page2of2