Physicians and 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, erpetuating 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
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.
Department of Pediatrics, Division of Neonatal-Perinatal
Medicine, University of North Carolina, UNC Hospital,
Chapel Hill, NC, USA
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15 Koogler TK, Wilfond BS, Ross LF. Lethal language, lethal decisions. Hastings Cent Rep
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