Differing perceptions

Freeman JM. On learning humility: a thirty-year journey. Hastings Cent Rep. 2004 May-Jun;34(3):13-6. PMID: 15281722.

When I returned to Johns Hopkins as an associate professor in 1969, the physician's role was quite paternalistic. The concept of informed consent was just beginning to enter the patient-physician relationship, and the idea of shared decisionmaking had not begun. Parents had responsibility for the surrogate decisionmaking for their child, and the idea that disabled persons have the same rights as everyone else was still new. I had been hired to direct the pediatric neurology division at Johns Hopkins. I was also to run the March of Dimes Birth Defects Clinic which primarily cared for children with spina bifida, a congenital malformation of the spine associated with varying degrees of paralysis of the legs, bladder, and bowels, and often associated with hydrocephalus (water on the brain).

During the mid-1960s, newborns affected with spina bifida were sent home or to an institution, typically to die. Children who did not die had treatment begun when they were a year old. As surgical and anesthetic techniques improved, however, surgery for the newborn with spina bifida became increasingly feasible, and John Lorber, of Sheffield, England, showed that early closure of the back to avoid meningitis increased the child's chances of survival. 

When I began to work in the Birth Defects Clinic, early surgery was the norm but was not mandatory. As director of the clinic, it became my responsibility to elicit the parent's informed consent for the operation to repair the back. I needed to discuss the likelihood of the child's future ability to walk, with or without braces and crutches, and to explain the consequences and management of the probable incontinence of bowel and bladder and the likelihood of hydrocephalus and of mental retardation. I had been trained in the paternalistic era when the physician directed the patient's care and convinced the parents to make the correct decision, and this was typically the approach I put into practice myself.

I have recently had occasion to look back on my work and to see what happened to some of the patients I have worked with over the years. What I find leads me to think differently about much of what I have done. I had been a biology major in college and had little patience for philosophy and ethics. I found them lacking the precision that had led me to science and eventually into medicine. Through my medical school and residency training there were no courses in medical ethics, caring and compassion, or communication. We were taught "medicine" and to make good decisions for the patients. But my journey with spina bifida has led me toward humility and, I hope, greater wisdom.

About one year after I began directing the clinic, I saw a newborn with spina bifida whose story has become emblematic for me of my ever-growing uncertainty. The infant had a high spinal lesion, and I explained to her parents that she would spend most of her life in a wheelchair and never have normal control of her bowel or bladder. She was likely to develop hydrocephalus, and if we did not operate to close the back, she was likely to develop meningitis. They were a churchgoing family and asked to consult with their minister. The following day they returned and told me that, with their minister's guidance, they had decided to let God make the decision. They did not want to have a surgeon interfere with God's will. They wanted to take the baby, now named Faith, home and provide her with love and care.

I knew that if she were taken home, she would probably die of meningitis. Was death with love preferable to life with love and suffering? At that time, society's attitudes toward the child with Down Syndrome were changing; the prevailing sentiment now was that if the child had an intestinal blockage she should be operated on, with or without parental permission, and then either taken home by the family, put up for adoption, or placed in foster care. I wondered whether Faiths case was similar, and I worried about my obligations both to her and to her family. My paternalistic bent was to do everything possible to give her the best life available, to close the back and embark on the multiple medical procedures, recognizing that she faced a lifetime of medical complications. She would likely be of normal intelligence and thus aware of her condition. Yet the family was apparently making its decision out of love and compassion, not because they wanted to avoid caring for a disabled child. They wanted to give Faith their best care, and then leave the outcome to higher powers. I decided that it was not my role to interfere, even if it meant a higher probability of Faith's dying. I let them take her home and asked to see her in the clinic in a month.

When Faith returned for the four-week visit, she had been well cared for. Her back had largely healed, but she also had all of the clinical signs of meningitis. I explained to the parents that we had two choices: to hospitalize her and treat her with antibiotics, or to pretend not to recognize the infection and let them take her home to die. Again, after a long discussion, they convinced me of the validity of their choice to leave the outcome in God's hands, and Faith went home.

Three weeks later, the family returned to the clinic because Faith was vomiting and not feeding. Her meningitis seemed to have resolved on its own, but she had now developed hydrocephalus, which if left untreated would certainly kill her, although possibly not for months, even years. A minor operation to place a shunt and siphon off the fluid could control the hydrocephalus. I explained to the parents that if Faith were to survive, she should survive in the best condition possible. She should have her back repaired so it would be less disfiguring; she should begin to have orthopedic procedures on her legs so that they would be as straight as possible. She should have her bladder and kidneys evaluated and treated, if necessary, to avoid permanent damage. At this point, Faiths family agreed to treatment. From their perspective, God had shown His wisdom and had not allowed Faith to die. He would want her to be the best she could be. We performed the shunt.

As a teen she was beautiful, walking with crutches, seemingly happy, but somewhat slow mentally. I lost track of her when she outgrew pediatrics, but I have recently reviewed her medical history. Over the ensuing twenty years, she has had many hospitalizations, many shunt revisions, and many bed sores requiring plastic surgery. She has had repeated renal infections, and one kidney has been removed. In short, she has endured a grueling medical history.

In retrospect, Faiths case has been a lesson in humility several times over. It was, to some degree, a lesson in medical humility: she did well even when the family rejected some of my recommendations for treatment. Thinking about these decisions was also an early lesson in moral humility, as I found it necessary to accede to the family's views about how she ought to be treated. Thinking about the outcomes of these decisions has provided yet another lesson in moral humility. When Faith was a teen, she appeared to be doing well, even without receiving as much care as I had recommended. But at this point in her life, perhaps she feels differently, and I wonder what she and her family would say now about the quality of her life. On the basis of her medical history alone, anyway, I am not sure that the decisions we made back then were right.

Even at the time, these matters were obscure. In 1971, shortly after Faith's early surgeries, Lorber re-evaluated at the results of his recommendations for early surgery for children with spina bifida and concluded that the early treatment of all children with spina bifida was a mistake. 

Half of the children he had treated early and vigorously had died anyway, and a substantial number of those who survived would be in wheelchairs or require bracing and crutches. He believed that they would be better off dead. He published criteria that could predict a child's future impairment and recommended "selection" and nontreatment of those who would be in wheelchairs or walk only with braces and crutches. He stated that such selection, at birth, would reduce the suffering of the children and their families, and reduce the resources needed for their care.

Lorber claimed that infants "selected" would die quickly, but having recently been through the events described above with Faith, I knew that was not necessarily true and wrote a commentary for a pediatric journal titled, "Is there a Right to Die Quickly?" The commentary raised questions about the meaning of "quickly" and contended that the definition was in the eye of the beholder. Having seen children like Faith lie around the wards for weeks or months--waiting to die--I treated most children with spina bifida, but I also wrote, in another article, that "If euthanasia were legal, I might feel differently." 

Later in my career, I debated John Lorber in front of the American Academy of Pediatrics. He articulated the results of his study, stating that those not selected for treatment died quickly. What was not apparent to the audience, and initially not to me, was that in England, infants were fed on demand. If they were sedated with morphine and phenobarbital and fed on demand, they demanded less, and died more quickly. Infants in this country were fed every four hours and were patiently waiting to die without their physicians knowing the "trick."

Recently I was asked to write a review of the ethical issues involved in treating spina bifida. It had been years since I had worked with children who had that condition, and both the field and society had changed. Now, prenatal folic acid may prevent spina bifida, routine amniocentesis and ultra sound examination often detect a lesion early in pregnancy, and the abortion of an affected fetus is common. I wondered if these changes had affected perinatal decisionmaking.

I went to the spina bifida groups' annual Christmas Party and spoke with two young women in their 20s who had been poster children for what we could do. I had always believed that if the child was given the proper medical care, if complications were prevented, if adequate education and psychological support were provided, these affected children could be given a good life. If they were dissatisfied with their lot, it was our fault for not having adequately met their needs. During their childhood and early adolescence, I had considered these two to he among my treatment successes.

Although both of these young women have high paraplegia and are in wheelchairs, they are of good intelligence, have graduated from high school, and hold government jobs. One has had twenty-seven operations for shunt blockage and for hip, back, and kidney problems. The other had only one shunt but many other surgical procedures. Both live at home. When I asked about their lives they both complained about having few friends, no social life, little prospect for marriage, and little independence. I mentioned that I was writing an article about decisionmaking for infants with spina bifida. One said that although she was Catholic and did not believe in abortion, nevertheless, she wished she had not been born and would advise parents to terminate an affected fetus. The other agreed.

I was shocked. I was reminded of Lorber's statement, which I had disputed in 1971, that "most [of his survivors] had a quality of life inconsistent with self-respect, earning capacity, happiness, and even marriage."

I consider myself to be a moral, caring, dedicated, compassionate, paternalistic physician whose life's work has been with children and parents of children with disabilities. Over the course of my career I had became increasingly confident in the correctness of my decisions, and therefore grew even more paternalistic. My confidence may even have turned into arrogance as I found and articulated the flaws in Lorber's position. Now, with time and experience, and partly as a result of speaking to these young women, my arrogance has become doubt and ignorance, once again. I now do not know what to recommend to the family of a newborn with a severe birth defect, or how to counsel the family whose fetus has been diagnosed in utero. I no longer know what is "the right decision," or even the ethics underpinning a proper decision.

My patients and parents have taught me that families have amazing abilities to cope. I am repeatedly struck by the families who rise to meet what I would have considered terrible, ongoing adversities. I am awed by those who continue to care lovingly for the profoundly disabled child who does not interact with them or with the environment, and who has constant seizures. I remain overwhelmed by the parent who is constantly demonstrating love, care, and compassion without the immediate positive feedbacks and rewards that I as a parent have come to expect.

Predicting the future for children with major abnormalities may be fraught with hazzard; so also may be predicting the parental response to a handicapped child. I was taught this by three mothers, each referred to me for counseling late in their pregnancies. Each was carrying a fetus who, by ultrasound, had hydrocephalus. One child, when born, had hydranencephalaphy (no cortical mantle) and made almost no development. Stasia had seizures and died at three years of age. Her parents considered her "a gift" and wrote of their experience. The second at birth was found to have massive hydrocephalus with only a very thin cortical mantle. She was shunted and has become an "A" student in high school and a competitive swimmer. The third, similarly treated, made little developmental progress, had major complications, and a devastated, ultimately broken, family who came to curse me for advising them to carry the child to term. When seen in utero, I could not predict the three fetuses' medical outcomes, let alone their parents' future reactions.

Since these problems were detected only late in pregnancy, the only alternative was the currently banned "partial birth abortion." Would that have been better? For which of the children? How do you work to achieve the greatest good for the greatest number when "the good" is so unpredictable both for the children and for their families?

I realized the depths of the biases and prejudices that continue to underlie my personal ethics and my decision making. As I tried to factor my perception of quality of life into my advice, I also realized how limited is my own view of other people's quality of life--how tolerant we must become of those with a different quality of life.

In reflecting on my career, I find that I have progressed from ignorance to paternalism through arrogance to a new humility. I have become more tolerant, perhaps more compassionate, less judgmental, somewhat wiser, more confused. I am still learning how deep my own biases run, and how little I really know about the lives of those with disabilities. I no longer know the ethically correct answer to the specific questions posed by the patients' problems. I have also learned, much to my continued disappointment, that ethical theories and ethicists are rarely helpful in decisionmaking at the bedside or for the individual.

But one lesson I believe I have learned is that decisionmaking should be a process, and the process for arriving at a decision is far more important than the decision arrived at. ( This process cannot be left solely to the family, or to relatives and friends, with their biases and prejudices, any more than it can be left solely to the physician. Physicians and families together must learn to work out a plan for the handicapped or potentially handicapped newborn--and likewise for the premature infant, for the person with late-stage Alzheimer's disease, for the critically ill, for individuals at both ends of life.