CF patients are more often requiring lung transplants later in life while there is a growing trend in children with refractory ARDS needing transplants.
The most common indications for lung transplantation in older children are cystic fibrosis (CF) and pulmonary vascular diseases (PVDs). In addition to PVDs, surfactant protein deficiencies, interstitial lung diseases, and congenital heart disease are important indications for very young children including infants. Two noticeable trends seem to be occurring in pediatric lung transplantation including CF and severe acute respiratory distress syndrome (ARDS).
Due to advancements in the medical care of patients with CF, there are trends suggesting that children with CF are requiring lung transplantation less often during childhood but later in life during their adult years. In contrast, there is a growing trend in children with refractory ARDS needing to be considered for lung transplantation.
At Nationwide Children’s Hospital, we have had a significant increase in calls by pediatric intensivists to transfer and consider children with refractory ARDS on ECMO (n = 5, mean age 6.4 years, range 4-10) for lung transplantation. Unfortunately, many calls are occurring later during the ECMO course and patients have experienced complications that lower their chance for being considered and/or evaluated for lung transplantation. Looking at this important issue in the United States, our team analyzed the United Network for Organ Sharing Registry including data up to March 2016 to determine outcomes of children with ARDS on ECMO who were listed for lung transplantation. A total of 11 children ages 10-17 (mean 14.4) years were identified requiring ECMO due to ARDS and were listed for lung transplantation between 2010-2015. Of this small group, four children underwent lung transplant, one child died on the waitlist, two were de-listed due to clinical improvement, and two were de-listed for unspecified reasons, and two were still on the wait list at last follow-up. Although the timing of referral for these children for lung transplant was not available, we anticipate that this occurred early based on our experiences at Nationwide Children’s.
Published findings and our expertise at Nationwide Children’s indicate that clinicians caring for critically ill children with refractory ARDS who are not improving or may be deteriorating on ECMO should be considered for lung transplantation. Although no study has identified when to consider lung transplant for children in this setting, we would recommend a referral to a pediatric lung transplant program if the child is either not improving or deteriorating on ECMO support within the first two weeks of the ECMO. Based on our published work, some children in this setting may improve on the waitlist leading to de-listing, but the benefit of considering and referring for lung transplant earlier gives those children who will not improve a chance to be considered for lung transplantation.
Kirkby S, Hayes D Jr. Pediatric lung transplantation: indications and outcomes. Journal of Thoracic Disease. 2014;6(8):1024-1031.
Hayes D Jr, Tumin D, and Frazier WJ. Children with refractory ARDS. Pediatric Pulmonology. 2017; 52: 1249-1249.