Intellectual disability in pediatric transplantation: Pitfalls and opportunities

Whether or not to transplant a child with intellectual disability remains a topic of much debate. On the one hand, denying patients a transplant solely because of intellectual disability would represent a gruesome deed of discrimination. On the other hand, transplanting every child with intellectual disability presenting with end-stage organ disease would also not be appropriate. Indeed, pretransplant screening to select those patients who will benefit most from transplantation seems reasonable in times of organ scarcity, with the following questions emerging: (i) Will a patient with intellectual disability equally benefit from receiving a transplant, both in terms of survival and quality of life? (ii) Will he/she be capable of understanding the implications of the transplant procedure and the subsequent need to follow a complex therapeutic regimen? , and (iii) Is there a sufficiently strong and sustainable social network available to support these vulnerable patients in achieving good post-transplant adherence? In fact, these questions do not differ much from those to be answered for every transplant candidate, irrespective of their intellectual capacities. Previous reports already indicated that posttransplant patient and graft survival of both children and adults with intellectual disability are excellent and comparable to those without disability (1–5). Yet, up till now, only case reports or small cohort studies were available. Consequently, one might question whether publication bias might exist, as centers might be more enthusiastic to publish about their success stories and reluctant to report on failures. Wightman and colleagues for the first time performed a largescale retrospective cohort study, using a UNOS dataset consisting of almost all children (n = 2076) having received a first kidney transplant between 2008 and 2011, thereby largely ruling out the risk of publication bias (6). No significant difference in graft or patient survival between patients with definite or probable intellectual disability and those without disability were observed up to three yr post-transplant. The excellent survival rates confirm previously documented positive results and might ultimately take away clinicians’ doubt whether transplantation for children with intellectual disability in general represents a lifesaving option. Yet, these positive results certainly do not mean that intellectual disability offers patients an unrestricted permit to transplantation. Indeed, estimates on projected survival benefit should always be made on a case by case basis, as intellectual disability is often caused by genetic syndromes or chromosome aberrations, and may go hand in hand with high comorbidity, which might seriously hamper the success of transplantation. In other words, transplant centers might have very good evidence-based reasons why transplantation is not a realistic option for an individual patient. Unfortunately, the lay public does not always understand the complexity of these decisions, and, often inflated by sensation seeking media, pinpoint on discrimination rather than on fair, well-grounded medical decisions. We currently do not know how many patients with intellectual disability presenting for pretransplant screening are deemed ineligible for transplantation. In addition, it remains obscure how many patients with intellectual disability are never referred for pretransplant screening, representing a hidden form of discrimination, also conflicting with the United Nations Organization’s Convention, stating that “persons with disabilities have the right to the enjoyment of the highest attainable standard of health without discrimination on the basis of disability” (7). However, although the results of Wightman and colleagues show that excellent survival in patients with intellectual disability is indeed possible, the authors limited their follow-up to three yr post-transplant. If children with intellectual disability would have high degrees of nonadherence, a concern that is, raised by many