Fragile Beginnings

by Adam Wolfberg, MD

  However, even as Kelly and I and the nurses and doctors stared through the Plexiglas at this thriving newborn, we couldn’t avoid worrying about the devastation to the left of the midline within her skull.

  Here was a child who had a dubious prognosis—the risk of devastating disability was still high—but who was completely stable.

  Stephen Wall and John Colin Partridge, pediatricians at Northwestern and the University of California at San Francisco, studied the records of 165 newborns who had died in the San Francisco NICU over a three-year period. Of these babies, 121 had died after doctors withdrew life-sustaining care or decided not to provide the additional care needed to prolong life. The most common reason for limiting or withdrawing life support was the physician’s belief that death was inevitable and that additional treatment was futile. Doctors who stopped care also expressed concern that the baby would suffer as a consequence of futile treatment.

  What is remarkable was that in each of these 121 cases, the parents and the physicians agreed on the decision to stop treatment. In not a single case did the parents and the doctors disagree.1 Parents and doctors make these decisions every day in NICUs all over the United States. And although these decisions are accompanied by great sadness, there is rarely any controversy.

  Usually, these choices are simplified because babies tend to make the big decisions themselves. After some uncertainty, they get progressively better: the ventilator is replaced by simple oxygen supplementation, feeding is begun, and the overall care requirements diminish. Or one complication follows another—intracranial bleeding is diagnosed, increasing doses of medication are needed to maintain blood pressure, and the oxygen requirements go up, not down.

  The fact is that parents and physicians share a common desire to do everything medically reasonable to save the life of a premature baby. These are not tepid resuscitations or halfhearted attempts but all-out efforts with extraordinary resources marshaled to save the baby’s life. Parents typically see committed teams of doctors and nurses working around the clock, and they derive confidence and reassurance from the compassion and commitment that motivate their baby’s caregivers.

  It may not be surprising, then, that when the doctors realize that death is inevitable and approach the parents with this devastating news—and it is almost always the doctor who initiates the discussion about ending care—parents are receptive to the doctors’ suggestions.

  The withdrawal of life-sustaining care has been a part of medicine for centuries. With each advance in the care of newborns, both full-term and preterm, physicians and parents found themselves asking, as child psychiatrist Leon Eisenberg put it, in a 1972 article, “At long last, we are beginning to ask, not can it be done but should it be done.”2 But in an era of relative medical paternalism, the conversation was held in hushed tones.

  The public discussion on this topic began in 1973 with the publication in the New England Journal of Medicine of a lucid and thoughtful article, “Moral and Ethical Dilemmas in the Special-Care Nursery.”3 It was written by two Yale pediatricians who looked at the causes of death of the 299 babies that died in the Yale NICU over a two-and-a-half-year period. In 14 percent of those deaths—most of them children with congenital anomalies—care was withdrawn with the agreement of parents and caregivers.

  Most of the injuries and illnesses described in the article were cases where the majority of reasonable contemporary pediatricians and parents would agree that further care was only delaying death: the child with respiratory distress syndrome on a ventilator requiring a lot of oxygen who developed heart disease and seemed to be suffocating from oxygen deprivation; the child with multiple abnormalities in the structure of the pelvis and pelvic organs, severe spina bifida, and, as a consequence, a buildup of pressure around the brain.

  The 1973 article described how, in making this decision, physicians and parents considered the quality of life for these children, as well as the potential for physical and emotional suffering. But parents and physicians also made the decision to withdraw support for children who would likely survive but would suffer severe disabilities; they considered the toll that would take on their families and on society, as well as the financial burden that their care would cause. Based on these deliberations, care was withheld from babies with conditions that, even in 1973, were survivable, including an infant with Down syndrome and a correctable malformation of the intestines who did not receive surgery and was allowed to die.

  In most of these cases, physicians and parents agreed that death was the preferable outcome. In the few instances in which the physicians thought the prognosis was dismal but the parents wished life-sustaining treatment to continue, it did.

  Although it seems inconceivable, there is no evidence of public disagreement with the doctors, no case in which the parents asked that care be stopped and the physicians insisted that it continue. This may reflect those times—the patient-physician balance of power was even more skewed than it is today—or a prejudiced belief that disability, physical or cognitive, was incompatible with a high quality of life. Either way, in these early years of neonatology, parents and their doctors had relatively few constraints on their freedom to make life-and-death decisions for newborns like Larissa whose prognosis was uncertain.

  “We believe,” wrote Raymond Duff, lead author of the 1973 article, “the burdens of decision making must be borne by families and their professional advisers because they are most familiar with the respective situations.”

  Some years after Larissa was born, I admitted a woman to our Labor and Delivery service whose membranes had ruptured at twenty-three weeks and four days. The patient was highly educated, as was her husband, and they were accompanied by an adorable three-year-old daughter, who charmed our staff. The woman was not in labor, and so we counseled her that there was a reasonable chance that she would remain pregnant for a few days at least, maybe longer.

  “I have to warn you,” I remember telling her, “that most women who rupture their membranes do go into labor within forty-eight hours. But the longer you go without labor, the higher the chance that you’ll remain pregnant for an extended period of time.”

  As a matter of routine, I called the NICU for a consult, and one of the neonatologists came and spoke at length with my patient.

  A couple of hours later, I returned.

  “The neonatologist said that if my baby is born today, survival is about fifty percent,” she said, “but she is almost certain to be injured. And if I stay pregnant for another three days, and I get two full doses of steroids, survival will increase to maybe sixty-five percent.”

  “That’s good,” I ventured.

  “What are my options?” she asked. A severe tone had entered her voice.

  “What do you mean?” I asked her. I had prepared to be hopeful.

  “Can I end the pregnancy?” she asked. “Can I have an abortion? Or is it too late?”

  I was taken aback. I explained that the legal limit for abortion in Massachusetts was twenty-four weeks, and so in theory she had three days. “It may be hard to find a doctor in Massachusetts who will do an abortion for you so close to the legal limit,” I told her. “And besides, your baby could do really well, or you could stay pregnant for weeks . . .”

  “Or my baby could be born tonight and end up with catastrophic brain damage. The other doctor I spoke to mentioned brain damage. Isn’t that true?”

  I had to admit it was.

  Our discussion continued for close to an hour. When I left the room I went to the nurses’ station. “She wants an abortion,” I said.

  The nurses looked at me like I was crazy. “She wants what?” one of them said, incredulous.

  They took it as a personal affront, this highly trained group of professionals who were preparing to do everything necessary to save the life of a severely premature baby, that my patient would decline their care and seek
an abortion.

  Grudgingly, we disconnected the woman’s IV; she changed out of her hospital gown and got back into her street clothes, walked out of the hospital, and took a taxi across town, where one of my colleagues at another hospital performed her abortion.

  Most of my colleagues, as well as the nurses who were personally wounded by this woman’s rejection of their skills and good intentions, would characterize themselves as strong advocates of abortion rights. Yet it felt wrong to these providers of women’s health care that my patient had ended a pregnancy that might have resulted in a lovely sister for her delightful three-year-old because she didn’t want to risk the chance that her child would be disabled. They felt she had made the wrong decision, but no one called the hospital’s lawyer or phoned the attorney general; they questioned her decision, not her right to make it.

  My patient chose abortion at twenty-three weeks and four days, and yet, had she gone into labor and delivered, her baby could have received all the resources our NICU had to offer. Because these decisions hinge on mere days, it is tempting to group together the abortion debate and arguments over who should make decisions about the care of sick or premature newborns, but the legal and medical traditions of the two situations have entirely different origins. Society has debated the morality of abortion for centuries, but at the center of the argument is a disagreement regarding the balance between a woman’s rights and the rights, or lack thereof, of the fetus. Abortion in the United States has always been either outlawed or restricted; it has never been a private medical decision made legally between a woman and her doctor. My patient considered her options within the highly structured system of American abortion laws.

  In contrast, the legal framework for making decisions about which babies get the overwhelming resources of a modern NICU and which babies are allowed to die is less mature and hence more tenuous. The decisions in the nursery involve a baby who has rights and who is entitled to full legal protection. The debate isn’t about whether the child has rights (it does), but rather who is empowered to make decisions for the child.

  Until the 1970s, these decisions were the respected province of parents in consultation with physicians, in part because societal prejudice against the disabled aligned conveniently with the presumption that parents would make appropriate decisions in the best interest of their child.

  Then conservatives, having lost the abortion debate in Roe v. Wade and fearing the breakdown of society, began to look for ways to shore up social structures. They found it in the case of Baby Doe, a child with Down syndrome born in Bloomington, Indiana, in April of 1982. Soon after birth, the child was diagnosed with a tracheoesophageal fistula, a condition in which the esophagus fails to communicate with the stomach and instead links sideways to the trachea. A relatively simple surgery restores the channel so sustenance can reach the stomach and blocks the fistula that would otherwise allow food and liquid to leak into the lungs. Without the surgery, the child would die of malnutrition; with surgery, survival was nearly guaranteed.

  The parents refused to sign a consent form permitting surgery. Outraged, the child’s pediatricians and the administrators at the hospital where the child was born went to court to obtain the needed permission. The Indiana Supreme Court sided with the parents, and while the hospital was in the process of appealing to the U.S. Supreme Court, details were leaked to the media, and the case gained national attention. Six days after he was born, with several families declaring that they would be willing to adopt the child, and with the high court appeal pending, Baby Doe died of malnutrition.

  Outraged by the court’s decision, President Ronald Reagan ordered the Department of Health and Human Services to protect future Baby Does. The political charge was led by the surgeon general at the time, Dr. C. Everett Koop, a pediatric surgeon and founder of one of the nation’s first NICUs. The resulting regulations, known as the Baby Doe rules, undermined the premise that parents, with the guidance of caregivers, were the ones best suited to make decisions for their children.

  The month after Baby Doe died, government health administrators informed hospitals that they could lose federal funding if they withheld treatment or nourishment from handicapped children. Another set of regulations that followed required hospitals to post large signs stating DISCRIMINATORY FAILURE TO FEED OR CARE FOR HANDICAPPED INFANTS IN THIS FACILITY IS PROHIBITED BY FEDERAL LAW. The posters provided the number of a twenty-four-hour, toll-free, anonymous hotline for people to report violations.

  NICU physicians began looking over their shoulders, fearful that anyone who disagreed with one of their decisions might trigger the arrival of groups of federal investigators that came to be known as the God squads. Without warning, a group composed of federal civil rights investigators and consulting doctors would show up at the hospital and demand access to the child, the child’s caregivers, and all the related documentation. Whether or not the investigators found a violation, their visits were chilling.

  Angered by the Baby Doe rules, which not only disempowered parents but also dictated how doctors ought to practice medicine, physicians fought back. They argued that the government had no business usurping the decision-making rights of parents and their children’s doctors, particularly with rules so broad and overarching that they forced doctors to continue life-sustaining care on terminally ill children regardless of the pain and suffering this futile care caused. In a lawsuit decided by the U.S. Supreme Court, the Baby Doe rules were rescinded and the God squad posters came down. But by that point, the era in which parents and caregivers could reach a decision about what was in their child’s best interests without considering the legal implications of their actions was over.

  A second set of Baby Doe rules was enacted in 1985, this time by Congress, imposing similar legal requirements on the care of premature and sick newborns, although without the God squads or combative posters. Language in the new rules included the phrase reasonable medical judgment, which assuaged the ire of the American Academy of Pediatrics, and a détente set in. When Congress passed the Born-Alive Infants Protection Act in 2002, pediatricians were quick to point out that the new law didn’t change the status quo. The God squad period had frightened pediatricians into accepting a set of legal guidelines that governed decisions made about sick or premature newborns, but because federal officials weren’t aggressively imposing a whistle-blower environment inside hospitals, the new rules didn’t spark a full-fledged revolt.

  The environment in the newborn nursery shifted from one that granted caregivers and parents the autonomy to make decisions for their children to one that was open to controversy, and, inevitably, disagreements between parents and doctors began to end up in court.

  The best-known contemporary disagreement involved Sidney Miller, now an adult who cannot walk, speak, sit up, or care for herself. She is blind in one eye and has minimal vision in the other. She has cerebral palsy with spastic quadriplegia, a seizure disorder, and hydrocephalus requiring a shunt to drain fluid from around her brain into her abdomen. She has mental retardation with a cognitive capacity equivalent to a six-year-old’s. Her mother, Karla, provides around-the-clock care for her routine needs—diaper changes, feeding, and dressing—as well as her unique medical ones.4

  Karla arrived at Woman’s Hospital of Texas on August 17, 1990, with symptoms of preterm labor. An ultrasound estimated that Karla’s fetus weighed 629 grams—just over one pound—and was approximately twenty-three weeks gestational age. Soon thereafter, Karla’s obstetrician, Mark Jacobs, diagnosed chorioamnionitis—an infection in the uterus—and recommended delivery. The alternative would put Karla’s life and the life of her fetus at risk.

  Jacobs and the neonatologist on call, Donald Kelley, counseled Karla and her husband that their daughter probably would not be born alive, and if by chance she was, she would likely suffer from the disabilities that plagued severely premature newborns: brain hemorrhage, blindness, and lung disease
.

  The two doctors asked Karla and Mark Miller to decide whether or not they wanted their child aggressively resuscitated at birth, and after weighing the information they had received, the Millers told the physicians they wanted “no heroic measures” taken when their daughter was born. Dr. Kelley documented the Millers’ request in Karla’s chart. Mark Miller left the hospital to make funeral arrangements for the daughter who would soon be born and would then die.

  The decision not to fully resuscitate the baby alarmed hospital staff, who conferred in a series of rushed meetings and then informed Mark Miller on his return that that hospital policy as well as state and federal law required the staff to resuscitate any infant born weighing more than five hundred grams. When Mark requested a copy of the policy, he was told it was an unwritten one, and when he asked how he could prevent a resuscitation from happening, he was told he would have to remove his wife from the hospital. Given Karla’s worsening infection, Dr. Jacobs made it clear that moving Karla was not a safe option.

  Karla’s water broke that evening, labor was induced, and at eleven thirty, she delivered Sidney, at twenty-three weeks and one day of gestation and weighing 615 grams.

  Although Mark Miller had refused to consent to his daughter’s resuscitation, the neonatologist on call at the time of Sidney’s birth, Dr. Eduardo Otero, evaluated Sidney and decided that the baby was alive and had a reasonable chance of living. He resuscitated Sidney, intubated her, and put her on a ventilator.

  Sidney’s first days in the NICU were stable, but she quickly developed a significant intraventricular hemorrhage that resulted in the devastating disabilities that remain with her today.