Anencephaly - organ transplantation?

• Author: Byrne, Paul A.

A Fort Lauderdale infant with anencephaly, Theresa Ann Campo Pearson, was recently the subject of many newspaper articles, television shows, and medical and legal discussions. Her parents had filed a petition in a Florida circuit court for the "right" to authorize the excision of Theresa Ann's unpaired vital organs.(1) The petition sought a judicial determination that anencephalic infants be considered legally dead for the purposes of organ transplantation.(2) The trial court's order denying the petition was summarily upheld on appeal.(3) The Florida Supreme Court affirmed, thus rejecting an expansion of Florida's common law to include anencephaly within the legal definition of death.(4)

In this article, we will consider the case of Theresa Ann in the context of the brain death controversy. We will show how the attempt to declare death in a living baby with anencephaly is but another step in the growing acceptance of something less than actual death as legal death for the purpose of acquiring transplantable organs.

Theresa Ann was diagnosed antenatally as having anencephaly. Soon thereafter, plans were made to remove her organs for transplantation following her birth. After she was born, Theresa Ann was intubated and received ventilatory support. The ventilator was removed after about one week. Theresa Ann then breathed on her own for a period of time, clearly indicating that the brain stem function governing spontaneous breathing was intact. Other brain stem functions that control heart rate, blood pressure, salt and water balance, pituitary-endocrine organ functions, as well as many other organs and systems, were presumably also intact and functioning in Theresa Ann's body.

At least some of the physicians treating Theresa Ann knew, and made others aware, that her brain was functioning. Based on this, the Florida Supreme Court ruled that Theresa Ann could not be "brain dead" under Florida law.(5)

Anencephaly

Anencephaly results from a failure of the neural tissue to completely close at the cephalic end. Even though anencephaly literally means the absence of the brain, functioning neural tissue is always present. The telencephalon is usually absent, but the brain stem is present. Absence of the cranium (acrania) is a constant finding. Closure of the cephalic end of the neural tube normally occurs between the second and third week of development. Thus, anencephaly is a manifestation of an abnormality of development that occurs sometime between conception and two to three weeks of gestation.(6)

Prenatal Diagnosis

Alpha-fetoprotein (AFP) is a glycoprotein normally synthesized by the fetal liver but not by the adult liver. Some AFP is normally present in the amniotic fluid. When the neural tube remains open (in anencephaly or spina bifida with myelomeningocele), there can be abnormally large quantities of AFP in the amniotic fluid, and as a result there is elevation of AFP in maternal serum. Screening of maternal serum for elevated amounts of AFP, analysis of AFP in amniotic fluid, and ultrasound examination of the fetus can lead to a diagnosis of anencephaly. (One must be aware of the potential for error in all of these tests.)

Postnatal Diagnosis

The appearance of an infant with anencephaly is characterized by the absence of bone and scalp over the part of the head posterior to the forehead. The exposed brain is covered by a thickened angiofibrous stroma. Recognizable cerebral hemispheres are absent.

Brain Death Law

The Uniform Determination of Death Act (UDDA) was recommended for adoption in all jurisdictions of the United States by the President's Commission for the Study of Ethical Problems in Medicine and Biomedical and Behavioral Research.(7) The UDDA reads as follows:

An individual who has sustained either (1) irreversible cessation of

circulatory and respiratory functions, or (2) irreversible cessation of all

functions of the entire brain, including the brain stem, is dead. A

determination of death must be made in accordance with accepted

medical standards.(8)

The Florida Supreme Court affirmed the lower court's ruling that Florida state law "[does] not permit a determination of legal death so long as the ... brain stem continue[s] to function."(9) Since Theresa Ann had obvious functioning (or functions) of her brain, the statutory criteria of irreversible cessation of all functioning (or functions) of the entire brain had not been fulfilled.

When so many patients today are being legally declared brain dead in the presence of some brain stem activity, why was the same not permitted for Theresa Ann? The answer lies in the second sentence of the UDDA, which states that "[a] determination of death must be made in accordance with accepted medical standards."(10) Whereas Theresa Ann's case presented the question of whether a certain class of infants should be considered legally dead by definition, these other patients are declared brain dead on a case-bycase basis under more ambiguous and flexible criteria, i.e., accepted medical standards.

Like the Florida court justices, many people are not aware that accepted medical standards may include the presence of some functions of the brain when a determination of brain death is made.(11) For example, it was reported that ten out of ten organ donors at a leading university hospital showed a dramatic rise in blood pressure and heart rate as soon as the scalpel was applied during organ removal and that such an occurrence "appears to be common during organ harvesting."(12) Is there any way that this could have happened in ten out of ten patients without a functioning brain stem? The reflex centers for control of cardiac and hemodynamic functions are in the brain stem. How could Judge Moriarity be expected to know that, under current medical practice, it is "acceptable" to declare brain death in the presence of some brain functions, when the law so clearly requires the cessation of all functions of the entire brain?

Thirty Ways to Be Declared Dead

What has society accepted in brain death? Death is a fact, but is it fulfilled when brain death is declared? Before the acceptance of brain death as legal death, the criteria for the determination of death were based on the definition of death in Black's Law Dictionary: "The cessation of life; the ceasing to exist; defined by...