Case narrative

A 7-year-old boy with recurrent medulloblastoma presents to the emergency department with seizures. He receives multiple antiepileptics and subsequently loses the ability to protect his airway. As a result, he is intubated and supported with mechanical ventilation. Over the next several days he requires high doses of benzodiazepines and barbiturates to control his seizures. Eventually, his seizures stop and physicians modify the antiepileptic agents to a maintenance regimen. He is evaluated for extubation. Because of his waxing and waning mental status and frequent apneic episodes (not related to medications) he does not meet extubation criteria. Imaging of his brain demonstrates spread of his brain tumor despite previous aggressive chemotherapy. The neuro-oncologist acknowledges that no therapeutic alternatives remain to lessen tumor burden or halt progression of the disease. The clinicians hold a meeting with the family to discuss care options and goals and create a clinical plan.

Ethical principles and discussion

Medical technologies provide the opportunity to maintain life artificially and, at times, nearly indefinitely. Most of the time life support appropriately allows time for patients to recover from complex surgery or devastating illness. However, at other times technology sustains bodily functions with no realistic possibility of cure or return to what involved parties consider a reasonable quality of life. In these situations determining when to forgo life-sustaining therapies provides clinical and emotional challenges for families and the clinicians caring for the critically ill children. While some argue that the “sanctity of life” is absolute and the full complement of available technologies should always be used to maintain life, others believe in the moral permissibility of forgoing life-sustaining therapies (FLT) in certain situations. In this chapter, we address the ethical issues posed by FLT through a discussion of the steps involved in reasoned decision making: first, recognizing criteria for considering FLT; second, deliberating about the FLT decision; and third, determining whether or not to go ahead with FLT. We also discuss the specific issues pertinent to decisions about discontinuing medically provided fluids and nutrition (MPFN). Finally, we briefly consider issues related to care of patients for whom a decision to forgo life-sustaining therapies has been made.

Case narrative 1: a minor as solid organ donor

A 15-year-old girl with end-stage kidney disease due to Wegener’s granulomatosis has been on dialysis since her diagnosis 18 months before. Her lung disease is quiescent. The transplant team has determined that she is ready for transplant and has identified no psychological or medical issues to delay the procedure. Each parent volunteered to be a living kidney donor, but both were blood type incompatible (they were both type A and she was a type O). The patient is highly sensitized due to transfusions received early in her illness, suggesting a long wait on the deceased-donor kidney list, even with some priority accorded children awaiting a kidney. Her fraternal twin accompanied her to a clinic visit and stated, “I want to give my sister my kidney. I’m blood type O, I know what I’m getting myself into, and I understand the risks. How do we make this happen?”

Should the transplant team accept the twin’s offer and proceed with a donor evaluation? Would the situation be different if the girls were identical twins? What if the twins were 8 years old?

Case narrative 1

Julie G, a 26-year-old banker, has a normal first pregnancy and goes into labor at 41 weeks. All proceeds normally. Her husband stays with her in the delivery room and she has the baby at approximately 4 a.m. When the obstetrician delivers the baby, she indicates that the infant is a boy. Later that morning, a pediatrician from the practice Julie and her husband have chosen arrives to speak with them and examine the newborn. She notices the urethral opening is at the base of the phallic shaft and the phallus seems somewhat short. She wonders if the child might have a disorder of sex development (DSD). Specifically, she wonders if the child might have normal female chromosomes and some form of virilizing congenital adrenal hyperplasia (CAH) – a relatively common form of DSD.

Dr. P sits down with the parents. She explains what she has observed and what it might mean. She emphasizes that the baby appears otherwise entirely normal and should be healthy, unless the child has a form of CAH with disturbed regulation of the body’s important minerals. Both parents want to know whether their baby is “really” a boy or a girl. They have questions about picking a name for the child, what to tell those close to them, and what treatment the child will need.

Introduction

In the case, Ms. L was found to be carrying a fetus with hypoplastic left heart syndrome (HLHS) at 34 weeks of gestation. She and her family, having been informed and having considered the options, elected to pursue neonatal heart transplantation, rather than “palliative” surgery (the Norwood sequence of operations) or a non-surgical approach. (We can only assume Ms. L and her husband had the latter alternative made available to them.)

Unfortunately, the baby did not do as well as do some newborns with HLHS treated with prostaglandin. At three weeks of age, because of a failing cardiovascular system and no available organ for transplantation, Baby L had a first-stage Norwood operation. Seizures some time in the postoperative period led to the diagnosis of cerebral infarction. At six months of age, he had a second-stage procedure and awaited the third stage at the time of the case report. He is said to be “doing very well.”

Dr. Chin argues that Baby L should have had palliative surgery in the days immediately following birth, rather than await transplantation. He believes this to be so because (1) survival following the Norwood sequence approximates that following neonatal transplantation; (2) the Norwood approach involves lower (financial) costs; and (3) the inadequate supply of hearts for infant transplantation should be reserved for patients who have no alternative treatments.

Many think that medical treatment for children is altogether different from that for adults. On the one hand, pediatrics seems to be about maintaining inherent good health through disease prevention, such as immunizations. On the other hand, we think of the obvious, even miraculous, advances in therapies for some childhood cancers (especially the most common, acute lymphocytic leukemia) and for disorders of the prematurely born. Unfortunately, pediatrics also has a less optimistic side. Some congenital anomalies, genetic disorders, and malignancies have not yet yielded to the press of modern medical science. Current technology and medical skill are unable to provide a reliably good outcome for very small preterm infants, certainly those born at 22 weeks of gestation or earlier, and a varying proportion of those born up to 26 weeks of gestation. The scourges of accidental injury, severe physical abuse, and AIDS continue to provide both enormous challenges and a depressing reminder of the limits of treatment.

Two features distinguish the futility confrontation in pediatrics faced by health care providers and the public from the confrontation in the rest of medicine. These are (1) our deeply held belief and hope that childhood should be different and that children should not die before some imaginary natural life span and (2) medical uncertainty in pediatrics, that is, the difficulty in predicting eventual outcomes, especially those pertaining to neurodevelopment. With these thoughts in mind, some issues in the debate over futile treatment in pediatrics are discussed.

In 1979 when undergraduates applied in record numbers for admission to medical school, AIDS was not a clinical and diagnostic category. In 1990 when the applications to medical schools are plummeting, AIDS is unarguably with us, and not just as a clinical entity. AIDS has become what the French anthropologist Marcel Mauss called a “total social phenomenon — one whose transactions are at once economic, juridical, moral, aesthetic, religious and mythological, and whose meaning cannot, therefore, be adequately described from the point of view of any single discipline” (Hyde 1979). For cultural analysts, present and future, the 1980s and beyond are the AIDS years.

This chapter is about the impact of AIDS on the shop floor of the academic urban hospital, an attempt to understand the impact of AIDS on everyday practices of doctors providing inpatient care. Following Mauss, we wish to view AIDS as a total social phenomenon rather than as a mere disease. Procedurally, we shall concentrate on the house officer (someone who, after graduation from medical school, participates in medical specialty training) and the medical student to see how this new infectious disease changes the content of everyday work and the education of apprentice physicians learning how to doctor and to assume the social responsibilities of the role of the physician. We are going to look at professional and occupational culture as a set of shop-floor practices and beliefs about work.