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The Investigation Of Quality Of Life, Perceived Disease Severity, And Adherence In Patients With The G551d Mutation Receiving Ivacaftor Therapy, Heather Young Hathorne

All ETDs from UAB

Cystic fibrosis (CF), a genetic disorder, is a chronic life threatening illness that affects 30,000 adults and children in the United States and 70,000 worldwide. Treatment for CF is complex and consuming, consisting of multiple time intensive regimens. The therapy regimen has been descried as extraordinary when compared even to therapy regimens for other chronic illnesses. Oral and nebulized medication therapy, airway clearance therapy, pancreatic enzyme replacement therapy and vitamin /nutritional supplements are all required for the multiorgan treatment regimen for CF. Daily prescribed therapy can include as many as 40-50 pills, breathing treatments lasting up to tow hours a …