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The Lost Patient

Doron Weber
Damon Weber, the subject of Immortal Bird, by his father, Doron Weber

As the second year of medical school drew to a close, our education moved from lectures in the classroom to rounds at the bedside. We assumed the role of apprentices, expected to model ourselves not only on the clinical acumen of the senior physicians, but also on how they communicated with patients.

Each student followed a sequence of assignments to the different wards, and my first months were spent with the surgeons. Despite the grueling hours and heavy workload, the experience was exhilarating. I watched with awe as a woman’s chest was opened, exposing a gritty stenotic aortic valve, which was replaced with a functioning prosthesis. The procedure took many hours, and demanded a precise choreography of care among the heart surgeon, anesthesiologist, and cardiologist. As the last sutures were placed, the resident remarked how it was “a great case.”

While surgery offered the drama of dexterous skills, my next assignment, to internal medicine, prized broad thinking. The aim was to generate comprehensive lists of possible causes for the patient’s symptoms and physical findings, so-called “differential diagnoses.” It was like assembling a large jigsaw puzzle, but you did not receive the pieces all at once: rather, you had to guess which next piece to seek by ordering one diagnostic test or another—should it be a certain X-ray, or a particular serology, or a tissue biopsy? Then you evaluated whether the new piece of data fit and solved the puzzle, or whether more was needed to form a coherent picture. “Great cases” on the internal medicine ward were ample. I recall how our team of residents and students identified a rare form of malaria in a botanist with raging fevers who recently had returned from Africa, and discovered an overlooked lymphoma that was the cause of a patient’s kidney failure. After surgery and internal medicine, I was assigned to pediatrics, and there became frozen in my tracks.

Some of my day was spent in the outpatient clinic, where mostly healthy children arrived with sore throats or ear infections, and our prescriptions for acetaminophen or antibiotics were rewarded by a parent’s relieved smile. But the other part of the day was on the wards, among scores of children with incurable illnesses. There were listless babies with a dusky hue whose malformed hearts defied surgical repair, toddlers with cystic fibrosis struggling to breathe through airways encased in cement-like mucus, middle-schoolers with brain tumors that blunted their vision and unleashed violent seizures.

In the evenings, the images of these children could not be moved from my mind. I was unable to concentrate on the medical articles assigned for reading, or prepare presentations for the next day’s rounds. I could not summon the cognitive dissonance that allowed labeling a patient “a great case,” and had no capacity to marvel at the complex biology of a child’s disease. As I looked at these suffering youngsters and their anguished parents, all I felt was pain and loss. I realized that I had reached my limits, that I would not be able to function effectively as a pediatrician, to care competently for such desperately ill children, to stand in charge at their bedside without being overwhelmed by emotion.

Doron Weber’s Immortal Bird is the story of his son, Damon, born with a malformed heart incompatible with life, and cared for on the same pediatric ward where I was a medical student. The narrative is wrenching, and there were several times I had to put the book down. Not long after I finished reading it, I met a young pediatrician who directed the intensive care unit at her medical center. I asked how she balanced her mind and her heart, the need to think coolly while caring deeply. “You learn how to manage your emotions,” she replied. “And for the children who do not survive, I feel it’s an honor to have been with them at this point in their lives.”

Damon Weber was born with one rather than two ventricles in his heart. A complex surgical repair, called a Fontan procedure, was performed to allow adequate flow of blood to his organs, and for many years the repair succeeded. Under the watchful eyes of Dr. Constance Hayes, a pediatric cardiologist at Columbia Presbyterian Medical Center, Damon flourished. But when he entered eighth grade, his parents noted that he wasn’t growing well; despite a healthy and nutritious diet, his body had begun to lose vital proteins. Some 10 percent of children like Damon who undergo the Fontan repair later develop a syndrome termed protein-losing enteropathy, or PLE. The gastrointestinal tract leaks albumin and other vital proteins. This disturbs the nutritional and fluid balance in the body, so that growth is stunted and organs swell with retained water.

Doron Weber was a Rhodes Scholar at Oxford, worked in communications at the Rockefeller University, and now is at the Alfred P. Sloan Foundation overseeing programs in the public understanding of science and technology. Shealagh Weber, Damon’s mother, is a psychotherapist. Both parents are conversant with medical terminology and Doron Weber in particular is able to tackle clinical research studies, identifying their methodological limitations and critically weighing their results.

“They think it’s something called PLE,” Shealagh says in a low, fraught voice. “He’s not keeping protein in his body.”
“Never heard of it…What’s the treatment?” I ask, assuming a new medication.
“Dr. Hayes says they’ll see if they can fix the problem in the cath lab or redo his original operation, but otherwise he will need a heart transplant.”
“What?” I shake my head in disbelief. “Hold on! How do we go from ‘He’s doing so well’ in one checkup to ‘It’s all falling apart’ in the next? Where did this come from?”
…I have one basic rule: if we can’t understand an issue or a medical approach makes no sense to us, it’s the doctor’s problem, not ours. No mumbo-jumbo. We must always know exactly what’s going on so we can help ensure the best decisions are made. The most capable physicians supply the clearest explanations—equally clear about what they don’t know as what they do—and only the mediocre take refuge in obfuscation or omniscience.

Weber is certainly right that a doctor’s skills in communicating clearly with her patients reflect her clinical acumen. But there is another dimension to the importance of providing comprehensive information. Much is made in the popular imagination about maintaining an optimistic attitude in the face of severe illness. Yet there is a key difference between optimism and true hope. An optimist believes that things will generally work out for the best. Life teaches that this often is not the case, and Doron Weber does not depend on an optimistic outcome in his son’s case. Rather, he seeks true hope by being clear-eyed and complete in his understanding of every potential clinical problem and pitfall. Only that way can he see a possible path through the looming risks and dangers. To be unaware or deny a possible complication or reversal leads to the complacency of false hope.1

Weber reports that Dr. Hayes did not inform the family that this serious loss of protein could one day occur in Damon’s case:

I cannot grasp how the same voice that has seen us through two open-heart surgeries and countless medical difficulties to the verge of young adulthood, the same voice that has bantered with us about skiing and schoolwork and Damon’s plays,…not once…breathed a word about the possibility of a new disease—which, if he has it, has been developing for some time—how this same voice could so suddenly change its tune….
If the leakage cannot be stopped, the patient will eventually “starve” to death. No one really knows what causes PLE—though many suspect a link to higher pressures in the heart—nor has anyone found a cure.

We learn that Dr. Hayes’s omission was not consciously intended, not meant to spare Damon’s family worry. She explains that she was “so encouraged by his tremendous success that I didn’t want to believe it myself.

Here is the risk of emotion, when a caring doctor cares so much that affection can cloud thinking. I have been in the same place as Dr. Hayes. My empathy for an exhausted, feverish cancer patient caused me to omit an intrusive physical examination. Taking a shortcut to spare him discomfort, I missed an abscess deep between his buttocks. The patient soon went into shock from sepsis. Fortunately, he survived my error.

Medical mistakes have been studied over the past two decades. Newspaper headlines feature cases of surgeons operating on the wrong limb, of incompatible blood products given to recipients, causing a life-threatening reaction to the transfusion. Initially, the focus was on such technical and logistical errors. Much progress has been made in implementing systems based on so-called “cockpit rules,” with multiple checks on each step of administering anesthesia or accounting for surgical instruments, so none is left in the body after an operation. But more recent research indicates that the majority of medical mistakes are not caused by technical errors but are due to thinking traps.

Cognitive psychologists have identified an array of biases that we employ in making judgments under conditions of time pressure and uncertainty. For example, “anchoring” occurs when we latch onto the first bit of clinical data, skewing our thinking. Anchored on a premature conclusion, we fail to consider the full range of possible causes for the patient’s condition. These pitfalls account for most cases of delayed or incorrect diagnosis.2

As Weber recounts the unfolding struggle to combat PLE, he reveals Damon not only as a patient but as a person. The teenager is a student with a keen mind who gained acceptance into one of the most competitive public high schools in New York, a talented actor who readily masters his roles, and the leader of a collection of fun and quirky kids. David Milch, the screenwriter, is a friend of the family and creates a part for Damon in the HBO series Deadwood. Despite blistering California heat during the shoot, the young actor displays the determination and stoicism that characterize so many children who are afflicted with maladies from an early age.

In this book Doron Weber does a great deal of kvelling, the Yiddish term for effusive parental praise for a child’s accomplishments. Weber also portrays Damon’s remarkably mature grasp of his condition. While watching a TV program about John Kennedy, Weber tells Damon how, from a very young age, the future president suffered from a series of “grave ailments” including scarlet fever, colitis, back injury, Addison’s disease, weight loss, and sleep disorders. JFK took ten to twelve medications a day and was getting six daily injections for his back pain. Whenever Doron speaks to his son, he uses the nickname “D-man.” “Sound familiar?” he asks Damon.

“It’s inspiring to see what people can accomplish despite early setbacks. How overcoming illness and adversity builds character…Hey, D-man, maybe one day, you’ll be president.”
  1. 1

    See Jerome Groopman, The Anatomy of Hope: How People Prevail in the Face of Illness (Random House, 2004). 

  2. 2

    See Jerome Groopman, How Doctors Think (Houghton Mifflin, 2007). See also Daniel Kahneman, Thinking, Fast and Slow (Farrar, Straus and Giroux, 2011) as well as Freeman Dyson’s review in these pages, December 22, 2011, and Daniel Kahneman’s reply in the January 12, 2012 issue. 

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