Cystic Fibrosis is a life threatening, genetic disease that causes persistent lung infections, and progressively limits the ability to breathe (Cystic Fibrosis, n.d.).Pathophysiology and History of the Disease

Cystic Fibrosis is a life threatening, genetic disease that causes persistent lung infections, and progressively limits the ability to breathe (Cystic Fibrosis, n.d.).Pathophysiology andHistory ofthe DiseaseCystic Fibrosis is caused by defects inthe cystic fibrosis gene.Cystic Fibrosis was recognized over 400years ago in Germany.There is no cure for Cystic Fibrosis.Impact on Adults to the HealthCare System and Nursing PracticeIncreased heath care costsOver crowed clinicsIndividuals received less personalattentionLimited number of hospital bedsNursing educationImpact of the Disease on Childhood Altered body imageImpaired relationship with peersImpact of living with a chronic diseaseImpact of the Disease on AdultsBurden of care increasesTaking more medicines and receivingmore treatments.Lung damage is greater in adulthoodthan earlier in life.Fertility issues (Heintz, 2010, para. 6)Reasons People with CF areliving into AdulthoodGENE THERAPYANTIMICROBIAL THERAPYTHE MODERN CF CARECENTERMUCOLYTICSMUCOCILIARY CLEARANCEImpacts this Disease will haveon the Adult Client.Mentally and emotionallyPhysicallySexuallyEconomically, specifically as it relates to the ability to access health insurance and health careSusceptibility to engage in substance abusePrenatal care and childbearing (see additional slide)Occupational considerations and hazards (see additional slide) Ability to cope with stress (see additional slide)PregnancyPregnancyis nowpossiblewith cysticfibrosisPregnancyImportance of Prenatal CarePregnancy StatisticsFocus on Family PlanningInfertility causesThe majority of menwith cystic fibrosishave associatedcongenital bilateralabsence of the vasdeferens ( mutations,1995). Female infertility isless clear. There isa theory that thevaginal mucosa is tothick for spermpassage. Otherreasons are thoughtto be low BMI, poornutrition, andchronic infection(Hodges, 2008).Choosing a careerCystic Fibrosis should notprevent one from pursuing thecareer they seekSome careers pose greaterhealth risks for Cystic Fibrosispatients(Careers, 2013)Learning to copeThe key is to limit stress by:Knowledge: ask, watch, study and readSupport: support groups, get in touch with other families that know the ropes.Planning: getting through daily life will be overwhelming, write a daily plan.Strength: do not listen to well meaning people withnegative advise listen to your healthcare providers(About, 2014).Outcomes for the Adult PatientAdequate airway clearanceReduction of risk factors associated withrespiratory infectionsOrder Now

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