Clinical Outcomes after Pediatric Lung Transplantation at Cystic Fibrosis Care Centers

Abstract

Clinical outcomes remain extremely poor for children with cystic fibrosis undergoing lung transplantation. Since 1961, the Cystic Fibrosis Foundation has maintained a network of accredited Cystic Fibrosis Care Centers (CFCC) designated to deliver specialized, multidisciplinary care for people with cystic fibrosis (CF). While this integrated clinical approach is thought to be beneficial in patients undergoing transplantation, it remains unknown whether lung transplantation at a CFCC is associated with improved long-term outcomes among children with CF. We used the Scientific Registry of Transplant Recipients to identify all children (age <18 years) with CF who underwent first-time lung transplantation between 2005-2018. Clinical, demographic, and donor characteristics were compared between groups. We used multivariable Cox proportional hazards modeling to compare graft survival (defined as freedom from death, graft failure, or retransplantation) in children treated at CFCCs and non-CFCC centers. We included 336 children with CF who underwent lung transplantation during the study period. Of these patients, 229 (68%) were transplanted at CFCCs. During a median follow-up time of 2.5 person-years, 35 (10%) children experienced graft failure or underwent retransplantation, and 7 (2%) children were lost to follow-up. In our multivariable Cox proportional hazards model, there was no significant difference in graft survival (HR 1.13, 95% CI 0.82-1.56) or overall survival (HR 1.10, 95% CI 0.77-1.57). In children with CF, lung transplantation at a designated CFCC was not associated with a significant improvement in overall or graft survival. While treatment at a CFCC may not be associated with improved survival after pediatric lung transplantation, specialized care at a CFCC may result in improvements in other aspects of care which could not be assessed in this analysis.