ABSTRACT

I. Introduction Over the last two decades, advances in the diagnosis and treatment of cystic fibrosis (CF) have led to significant improvements in health outcomes and life expectancy. Median life expectancy in the United States is now 36.7 years (1) and the majority of pediatric patients diagnosed today are expected to live into adulthood. These improvements in life expectancy have shifted the focus of CF research and clinical care toward the complex interplay among behavioral, psychosocial, and health outcomes. This change in focus is epitomized by the growing prominence of patient-reported outcomes (PROs), such as health-related quality of life (HRQOL), and new efforts to address behavioral and emotional challenges, such as adherence and depression (2). This chapter introduces a developmental biopsychosocial model that highlights the bidirectional influences of age-related development on disease management and progression, and presents current research on these influences.