‘Physicians & Medical Professionals


Lethality Begets Lethality

Journal of Perinatology (2011) 31, 630–631; doi:10.1038/jp.2011.52

Courtwright et al.1 report that the diagnosis of ‘lethal’ conditions prenatally leads to care that is less intense, lower cost and of similar survival benefit when compared with postnatally diagnosed cases. Based on these results, the authors conclude that ‘highly aggressive interventions did not prolong survival in these cases and should not be offered.’ Based on such a definitive assessment one would assume that despite aggressive efforts to support infants after a lethal diagnosis, there was no significant prolongation of life. The authors do not report data that would allow such a determination to be made. Given the lack of such data, the phenomenon of the self-fulfilling prophecy (SFP) is a likely explanation for the authors’ findings.

The SFP describes a situation in which a false description of a circumstance evokes behavior that makes the original false conception come true. ‘The specious validity of the self-fulfilling prophecy perpetuates a reign of error.’2 In intensive care, the SFP is apparent in decisions related to withdrawal of life support on the basis of high predicted mortality. In such cases there may be a possibility of a lethal outcome, but the likelihood for lethality increases as a result of prediction, which is not true. As regards predicting a condition is lethal, potentially lifesaving treatments are withheld. As a result, patients who might have survived will then die, perpetuating the belief that the condition is lethal.2 There are multiple reasons that the SFP should have been considered operational in this study, especially in the largest group of anomalies, the aneuploidies of Trisomy 18 and 13. Courtwright et al., however, make no mention of SFP influence. This lapse occurs despite the fact that aneuploidies were considered lethal in this cohort, reports that families are universally told prenatally that Trisomy 18 is incompatible with life,3 and reference to the lethal views of aneuploidy held by neonatologists.4 This statement by a neonatal ethicist synthesizes the views of many involved in the care of infants with aneuploidy, ‘We have the technological ability to save these infants. This leads us to the ethically hard question: Because we can, does this mean we should?’5

A plausible reason for the findings in this study is that patients were maintained on intensive support until a lethal diagnosis was established. A median time of death of 4 days for postnatally diagnosed infants coincides with the time at which an aneuploidy diagnosis would likely be received. Once a lethal diagnosis was confirmed in critically ill infants, decisions were made, based on lethality, to discontinue support, including nutrition and hydration. Critically ill infants would expire quickly, consuming a greater amount of intensive treatment per day of survival. Without accounting for the overwhelming bias introduced by the SFP, it is unempirical to conclude that ‘highly aggressive interventions did not prolong survival.’ This renders baseless the recommendation of an ‘ethical stance in which treatment options offered to parents in the neonatal period are limited to non-intensive modalities.’

The effect of the SFP would be a non-issue if the designation of lethality for the aneuploidies was incontrovertible. This is not the case. The authors define ‘lethal’ as ‘death before a year of life in nearly all (X85% when statistics are available) cases.’ Of the two studies cited by the authors as supporting lethality for aneuploidies,6,7 only one reports actual survival rates.6 This study cites survival rates of 5–10% but with equipoise adds the following caution, ‘we are unable to address whether people who have Trisomy 13 or 18 and survive longer receive more aggressive care.’ The common deficiency in population studies evaluating survival in aneuploidy patients is the assumption that all Trisomy 13 or 18 patients are created equally and lethally. Courtwright et al. cite a textbook reference supporting lethality. This text describes the clinical course of Trisomy 18 infants with an additional reference, ‘Baty and colleagues documented the natural history of this
disorder.’8 Unstated, however, is that Baty et al., in that publication, report survival rates at 1 year of 42% for Trisomy 18 and 38% for Trisomy 13.9

The authors cite other references reporting on prolonged survival rates for infants with aneuploidies, but dismiss them as ‘small case series’ and not employing ‘contemporary comparison groups receiving lesser amounts of treatment.’10,11 One reports on 24 patients with Trisomy 18 receiving intensive care with a 25%
survival rate at 1 year.10 Another describes survival rates after cardiac surgery, for Trisomy 13 and 18 infants, of 44% at 1 year, with median survival rates of 243 days.11 There are other references not cited that further substantiate that Trisomy 13 and 18 are not lethal. Bruns12 reported on 21 cases of Trisomy 18 surviving to a mean age of 6 years. The 2005 and the 2010 Neonatal Resuscitation: AHA Guidelines identify Trisomy 13, but not Trisomy 18, as ‘a condition associated with high mortality and poor outcomes for which withholding resuscitation may be considered reasonable.’13 Mercurio14 recently reported on a Pediatric Ethics
Committee review of a case of a Trisomy 13 infant who underwent a successful ventricular septal defect repair. The Committee supported the surgery based in part on the view that ‘mortality data were thus understood to be generally grim but uncertain.’ Finally, Courtwright et al. report that two of their four infants with
hydranencephaly lived longer than 2 years. They state that ‘classifying this diagnosis as ‘lethal’ should be reconsidered.’ Based on this standard and the evidence cited, Courtwright et al. also need to reconsider their classification of the

The intent of this commentary has not been to provide unrealistic impressions. Individuals with Trisomy 13 and 18 have shortened lives. They generally have profound neurodevelopmental impairment. There are significant challenges related to long-term care of such children. Despite the possibility of longer survival, some families may very reasonably decide that the burden of therapy for such infants may well outweigh the possible benefits. The persistent characterization of aneuploidies as lethal, however, is disingenuous. The ‘label is not only inaccurate but also dangerous’.15 It uses the language of medical determination to mask what for many infants is ultimately a judgment about the child’s quality of life. It is a characterization that wrests from parents a decision that generally only they can make for their child. As medical providers we have an obligation to assure that non-directive counseling and care for infants affected with Trisomy 13 and 18 are not withheld based on the perpetuation of a false SFP.15 There is an important lesson to be learned from Courtwright et al. They make a convincing case that lethality begets lethality.

MJ McCaffrey
Department of Pediatrics, Division of Neonatal-Perinatal
Medicine, University of North Carolina, UNC Hospital,
Chapel Hill, NC, USA
E-mail: martin_mccaffrey@med.unc.edu

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