Individuals with cystic fibrosis (CF) and alpha 1-antitrypsin (AAT) deficiency pulmonary disease face many difficult decisions throughout the course of their lives and illnesses. The events culminating in these events may be called Milestone Life Events (MLEs). Decisions regarding goals of care in patients with advanced disease are particularly onerous. Two such MLEs may include the decision whether or not to pursue lung transplantation, and decisions about advance care planning for the end of life. Patients may not be prepared adequately to face these MLEs and, therefore, may struggle with these decisions. This study seeks to investigate these MLEs and the decision-making issues related to them for patients with advanced CF and AAT pulmonary disease.
The specific aims of this study are to: 1) identify ways to increase patient comfort with making treatment-related decisions, 2) identify the beliefs and understanding of patients about decisions regarding lung transplantation and advance care planning, 3) identify key issues for patients' informational needs around treatment decisions, 4) determine if patient-defined informational needs are expected by clinicians caring for those patients, and 5) plan for development of a survey instrument for a larger population of patients with CF and AAT in order to develop educational material and specific advance directive forms. This exploratory study will utilize the qualitative research tool of focus group interviewing to generate rich descriptive data about these issues. Adult patients with CF and AAT who are seen at sub-specialty CF and AAT clinics will be invited to participate in a focus group. Three focus groups of each disease-specific population will convene. The data will be analyzed by an iterative process to ensure reliability, then further investigations will be planned to develop a survey tool, and eventually educational materials and resources for patients facing these MLEs.
