Publication Date




Embargo Period


Degree Type


Degree Name

Master of Science (MS)


Psychology (Arts and Sciences)

Date of Defense


First Committee Member

Amanda Jensen-Doss

Second Committee Member

Maria Llabre

Third Committee Member

Matthias Salathe


Introduction: Cystic fibrosis (CF) is the most common indication for pediatric lung transplantation and ranks number three for adults. However, using the current criteria for selection and timing of a transplant, the survival benefit of this procedure in pediatric patients is controversial (Liou & Cahill, 2008). Modification of the current selection criteria has the potential to reduce mortality rates both for those on the waitlist and those who receive a transplant. Use of the Cystic Fibrosis Questionnaire-Revised (CFQ-R; Quittner et al., 2012), a well-validated, disease-specific quality of life measure may improve pre-transplant decision-making and thus, reduce mortality. Methods: This multi-center study evaluated whether specific domains on the CFQ-R (i.e., Physical Functioning, Respiratory Symptoms) and parent proxy reports on the CFQ-R (i.e., Health Perceptions, Vitality, Role/School Functioning) predicted mortality 4 years post-transplant for the Transplant group (n=28) and 2 years post-evaluation for the Waitlist group (n=35). These analyses controlled for physical predictors (i.e., age, CF-related Diabetes (CFRD), FEV1 % predicted), to assess whether the CFQ-R added additional and unique variance. Cox Regressions were used to measure the overall impact of the CFQ-R scales and Receiver Operating Characteristic (ROC) curves were used to assess the sensitivity and specificity of optimal cut-points of significant CFQ-R domains. Results: The CFQ-R scales explained additional and unique variance in mortality for both the Transplant and Waitlist groups. The Respiratory Symptoms scale was a significant predictor of mortality for the Transplant group. The Respiratory Symptoms and Physical Functioning scales were both significant predictors of mortality for the Waitlist group. ROC curves indicated that the Respiratory Symptoms scale was a strong measure of survival for the Transplant group (AUC = 0.86) and both scales were good measures of survival for the Waitlist group (both with an AUC = 0.71). Optimal cut-points were: 1) 47.20 for the Respiratory Symptoms scale for the Transplant group, 2) 37.50 for the Respiratory Symptoms scale for the Waitlist group, and 3) 28.48 for the Physical Functioning scale for the Waitlist group. Conclusions: This study suggested that including patient-reported outcomes could aid pre-transplant decision making. More accurate pre-transplant decisions could reduce post-transplant mortality in adolescents with CF. Including the patient’s perceptions of functioning in the transplant decision-making process was shown to be effective and importantly, utilized a patient-centered approach. Respiratory Symptoms scores can indicate when to prioritize and move adolescents forward to transplant when they are approaching the identified cut-point. In addition, Respiratory Symptoms and Physical Functioning scores can be used to identify those adolescents who should be put on the waitlist. Future directions include development of a weighting algorithm to improve accuracy and the possible addition of a lie scale to ensure the validity of the scores. Although this study laid the foundation for use of the CFQ-R in transplant decision-making, to adequately test these relationships, a multi-center study using the LAS in conjunction with the CFQ-R is needed.


Cystic fibrosis; quality of life; transplant; adolescent