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UBC Theses and Dissertations

The burden of cystic fibrosis in British Columbia Desai, Sameer


The landscape of Cystic fibrosis (CF) is changing. Historically, it was a disease mainly affecting children, but now there are more adults living with CF with many additional health problems and evolving needs. The thesis aims to assess the impact and burden of CF in British Columbia (BC). The Canadian CF Registry (CCFR) and multiple administrative data sources were utilized to evaluate an annual, open provincial CF cohort from 2000 to 2017. Epidemiological and clinical trends were summarized. The Global Burden of Disease methodology was used to quantify the overall burden of CF attributed to lung impairment, complications/comorbidities, and mortality. Health care resource use and costs were summarized. A fixed CF cohort was followed from 2009 to 2017 to evaluate characteristics associated with frequent high-cost CF users. The incidence rate of CF has slightly increased (average annual percentage change [AAPC]: +2.9%, 95% CI: -0.1 to +5.9) while the birth incidence rate declined (AAPC: -2.4%, 95% CI: -4.2% to -0.5%) since 2000. The prevalence rate has grown over time (AAPC: +2.1%, 95% CI: +1.7% to +2.5%). Survival outcomes have improved and more growth in the prevalence of CF (up to 50%), specifically in the adult CF population is expected. The clinical status has also improved with less occurrence of severe or advanced lung disease and an overall improvement in lung function over time. The total burden of CF has declined over time (Disability Adjusted Life Year [DALY] rate: -12.3%, 95% CI: -2.7% to -20.9%) primarily due to a reduction in premature mortality. Despite this progress, morbidity due to other CF-related complications and comorbidities has been slowly rising (AAPC: +1.0%, 95% CI: -1.4% to +3.5%) and disproportionately affects adults. Health care resource use and inflation-adjusted costs have also increased during this time, driven by the rising costs of adult care and medications. Frequent high-cost users represent 17% of the cohort but accounted for 32% to 45% of the overall total health care costs. Moderate-to-severe lung impairment, transplantation, liver cirrhosis with portal hypertension, and female sex were the main factors associated with frequent high-costing CF individuals.

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