Pushing the ECMO Envelope for Children With Genetic Conditions*

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Attitudes regarding medical procedures in children with genetic conditions have changed over time. Decades ago, children with trisomy 21 (T21) were not routinely offered surgery for congenital heart defects (1), and it was not until 1996 that Sandra Jensen became the first person with T21 to undergo a heart transplant (2). Similarly, children with other genetic conditions such as trisomy 13 (T13) and trisomy 18 (T18) were previously presumed to universally die as infants and many interventions were therefore avoided. Such predictions could, however, become a self-fulfilling prophecy (3). In the current era, many children with these diagnoses undergo a variety of surgical procedures, and those children who survive infancy may live for many years (4). It is also true that the concept of a “genetic condition” has changed over time, as our ability to diagnose such conditions grows exponentially.
Some changes in attitude likely arose because of shifts in perspectives regarding disability, with greater recognition of the rights of people with physical or developmental disabilities to have equal access to education, work, healthcare, and other benefits of life. Perspectives also shift as medical care changes—as procedures become safer, more widespread, and more “routine,” they become the sort of thing that people would demand access to. Extracorporeal life support or extracorporeal “membrane oxygenation” (ECMO) is now a common therapy in pediatric cardiac and respiratory failure refractory to conventional therapies. Additionally, it is being increasingly used for rescue therapy, and many institutions use ECMO as standard of care in the setting of cardiopulmonary resuscitation (5). Routine use leads to increasing expansion of indications such that ECMO has been used for toxic ingestions (6), chemotherapy-induced ventricular dysfunction (7), bridge to new devices or long-term lung recovery, and to preserve organ function for purposes of donation (8). This leaves the practitioner faced with decisions regarding ECMO usage in patients not previously eligible. Additionally, parents face decisions about whether ECMO is right for their child (9). Finally, as ECMO use is expanding exponentially for adult indications, media and popular culture are now dramatizing use of this therapy for standard and unique indications.
The retrospective analysis in this issue of Pediatric Critical Care Medicine by Furlong-Dillard et al (10) hopes to provide outcome data for clinicians and parents facing these decisions. Such data are important, as many children with genetic conditions also have heart disease and many centers now consider surgical interventions to extend life, or prolong quality of life, for these children. The authors used a large, national dataset to examine records of children undergoing cardiac surgery over a 10-year period (2004–2014), of whom, 4,300 children were placed on ECMO. For their analysis, they grouped patients into three categories: 1) T21; 2) T13/18; and 3) “other” (a wide range of genetic conditions, reflecting the increasing diagnostic categories that exist). Compared with all children undergoing cardiac surgery, patients with T21 were less likely to receive ECMO, and those with “other” conditions were more likely to. Reassuringly, it seemed that children with genetic conditions were not excluded from consideration for ECMO, and mortality rates suggest that the lower usage in those with T21 may have more to do with their associated cardiac lesions and surgical procedures being in a slightly lower risk category than with decisions regarding escalation. Although some subgroups had higher mortality than the general cardiac surgery ECMO population, this was not universal.
The group of patients with T13/18 are worth specific mention because all five patients in this category treated with ECMO died.

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