All ETDs from UAB

Advisory Committee Chair

Connie Kohler

Advisory Committee Members

Jeewoon Cheong

Kathy Harrington

Steven Rowe

Eric Sorscher

Document Type

Dissertation

Date of Award

2015

Degree Name by School

Doctor of Philosophy (PhD) School of Public Health

Abstract

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 day, and chest physiotherapy sessions tow to three times a day for up to 20 minutes each. Ivacaftor is a new oral cystic fibrosis therapy for the treatment of CF approved specifically for patients with the G551D mutation in January of 2012, and is only available to fewer than 5% of the total CF population. Although this type of therapy is not yet perfected for any other mutation and therefor is not available for CF patients with any other mutation, ivacaftor is the first treatment to alter CF with great positive impacts on patients. The purpose of this study is to evaluate disease perception. The primary purpose of this research is to explore the effects of ivacaftor on 1) on patient reported health related quality of life (HRQOL), 2) patient reported perceived disease severity, and 3) medication adherence and adherence to scheduled clinic appointments.

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