Lethal language, lethal decisions.

Neonatal and pediatric critical care have dramatically improved survival for children over the past twenty years. Neonatologists continue to reduce the gestational age and birth weight that a newborn must have achieved to survive, and advances in pediatric surgery and pediatric organ transplantation mean that complex congenital heart disease and biliary atresia no longer always result in death. They are no longer "lethal," because with treatment, an infant can survive these conditions and even enjoy a good quality of life. (1) Yet obstetricians, pediatricians, geneticists, and neonatologists continue to regard a category of congenital syndromes as "lethal anomalies. " (2) Examples are trisomy 13, trisomy 18, and anencephaly. Although most professional discussions do not provide a specific definition of "lethal anomaly," the term is generally used to refer to a child with (1) severe neurological compromise and (2) structural anomalies and/or functional disabilities that, if untreated, would cause death within a few months. The structural anomalies in these children include airway malformations, congenital heart disease, and gastrointestinal defects; the functional disabilities include swallowing dysfunction, aspiration, and apnea. These anomalies and disabilities are usually treatable, and invariably are treated in an otherwise healthy infant. What makes them sometimes "lethal," then, is the decision not to repair the anomalies or treat the disabilities in light of the child's poor neurological prognosis. In effect, as we will argue, "lethal anomaly" is not an accurate clinical description; instead, it serves to convey an implicit normative view about quality of life. The hidden significance of the phrase is problematic because many parents may not rate the child's quality of life as dismally as their child's health care providers do. Data show that health care providers rank severely disabled health states lower than parents do, (3) which can partly explain why parents typically prefer more aggressive treatment than health care providers. (4) Data also show that what health care providers tell parents about a child's prognosis is influenced by the providers' attitude toward neurological impairment, with the effect that what is told to the parents depends on the provider. (5) Since this information influences family decisionmaking, (6) the phrase "lethal anomaly" obscures the normative nature of the decision and interferes with authentic parental autonomy. An Historical Overview The practice of recommending that invasive treatments be withheld or withdrawn from infants with "lethal anomalies" first received critical attention in 1973, when Raymond Duff and AGM Campbell challenged the prevailing "social taboo" by making public and explicit their reasons for not providing invasive treatment to children with neurological disabilities. (7) They reviewed 299 consecutive deaths in the Yale Special Care Nursery and found that 43 (14 percent) were related to withholding treatment. They explained that the decisions were made by the physicians and parents because the child's prognosis "for meaningful life was extremely poor or hopeless"--a quality-of-life judgment that rarely had been publicly stated previously. (8) Once the Pandora's box was opened, medical ethicists, theologians, and clinicians came out in support of and against this practice. (9) The dispute came to a head in the 1980s, when disability advocates and right-to-life groups publicized the issues surrounding the withholding of treatment, including nutrition, in handicapped newborns. They focused public attention on the fact that hospitals and physicians were allowing children with congenital causes of developmental delay, such as Trisomy 21, to die from correctable birth defects. One such case to reach national attention was Baby Doe, who was born in 1982 with Trisomy 21 and a correctable gastrointestinal defect. …