Exploring the Developmental Impact of Cystic Fibrosis on Lung Transplant Candidacy: Considerations for Adulthood.

Abstract

The average life expectancy for cystic fibrosis (CF) has increased over the past four decades resulting in a higher rate of adult CF patients. Adults seeking lung transplant to address CF-related advanced lung disease (ALD) represent a small, yet growing, subset of lung transplant recipients. Psychosocial factors such as adherence to medical recommendations, self-management of medical care, and caregiver support have been identified as positive prognostic factors in lung transplant outcomes. These factors are also implicated in the pediatric chronic illness literature and are crucial as patients begin to transition to a more autonomous and independent role in their own health management. Adults with CF facing ALD must navigate through another transitional phase as lung transplant requires additional supports and new expectations. A case series is used to highlight specific psychosocial considerations in this population and to explore the seemingly dichotomous relationship between independent self-management and caregiver support.