Reefes’ gruff voice barely wafted to the residents, though he was probably shouting to be heard through the door. “Who is it?”
Susan tripped the latch and toed the door open a crack, enough for sound to carry easily without violating any privacy. “It’s Susan Calvin and Kendall Stevens, sir. We’d like to discuss a patient with you.”
“Come in. Come in.” Reefes sounded more harried than upset. “What can I do for you?”
Kendall pushed the door open, and the residents stepped inside. While he closed the door behind them, Reefes gestured toward a single chair in front of his desk. He sat behind the desk, a massive computer screen in front of him. Framed pictures hung from the walls, mostly hand-painted still lifes and photographs of a woman and four children Susan assumed were his wife and offspring. The desk held a snow globe with zoo animals inside it, a box of tissues decorated with crayon scribbles, a large old-fashioned laser printer, and a sleek multilined telephone.
Susan took the proffered chair, leaving Kendall to glance around, shrug, and slouch beside her. “I’d like to talk to you about Chuck Tripler.”
“Chuck Tripler.” Reefes tapped his fingers on the full-sized keyboard, then sat back, reading. “Charles C. Tripler. Sixty-six-year-old man with a ten-year history of Parkinson’s disease. Currently on Unit 2.” He looked up. “What about him?”
“Well, sir, I’ve been watching him.” Susan rose and stepped around the desk, tapping her Vox. “May I?”
The gesture had become universal. Reefes tapped out his sequence code to allow her to connect the information on her Vox to his larger system. A grainy video of Chuck shuffling around the unit appeared on the screen.
Kendall came around to join them.
Susan got right to the point, “I think he’s been misdiagnosed.”
Reefes sat back with a sigh. Like most attendings and nurses, he had probably grown accustomed to hotshot residents arriving each month with personal theories, trying to demonstrate their genius by unearthing a brilliant diagnosis overlooked by their superiors and predecessors.
Susan tapped the proper buttons on her Vox and brought up a MedVid of a patient with Parkinson’s disease, walking. “This is a typical chronic Parkinson’s walk before L-dopa treatment.” She tapped a series of buttons to highlight her points as she made them. “It’s slow and shuffling, but the cadence is actually increased so that the overall velocity remains essentially normal. The steps are short, the posture flexed and rigid. He tends to freeze in place, with hesitation at the start.” She backed up to the start of the MedVid to emphasize the point. “The step width and height, however, are nearly normal, and you don’t see any rotation angle to the feet.”
Reefes sat back. He wore his dark hair short, with bangs dangling over a broad forehead. His narrow green eyes followed the picture on the screen. Full lips currently held a smirk, though he said nothing, silently waiting for Susan to make a valid point.
Susan switched to another MedVid. “This is a patient with normal pressure hydrocephalus.”
Reefes groaned loudly, gripping the edge of his desk. “Not again.”
Susan froze the video. “Excuse me, sir?”
Reefes shook his head with stiff efficiency. “Sorry, I know you mean well. But this happens every month. Some resident zeroes in on a prospect and becomes convinced he has one of the rare forms of treatable dementia, almost always NPH, and tries to make a case for it.”
As Susan had intended to find a treatable patient and did intend to make a case for normal pressure hydrocephalus, she could hardly claim Reefes was wrong. However, in this situation, she believed she had a strong argument. “Please let me finish.”
Reefes rolled his eyes and started to rise.
Kendall stepped in closer. As he clearly intended to speak, Susan braced herself. Seriousness was not in his nature, and his humor often held an edge. “Sir, I’m sure you see this all the time, but Susan is a special case. Families grovel at her feet. Nurses festoon her with balloons. Researchers throw flowers in her path, and the hospital attorneys seek out her legal advice.”
Susan glared at her companion. She had made some good catches in the past year and she had a decent eye for diagnoses, but exaggeration to the point of absurdity was not going to help her cause. Reefes turned a withering look on him.
Kendall reined in his hyperbole. “Honestly, sir. In our first week of residency, she sent home three PIPU lifers, the staff threw her a party, and Doctors Goldman and Peters asked for her by name to assist in their research.” The latter were well-known as the premier psychiatry researchers in the state; their names topped many prestigious articles in the journals all psychiatrists read. Susan tried not to cringe at the high praise. Though true, Cody Peters and Ari Goldman had requested her because of the nature of the research, not her brilliance. They had a robot-related study and knew her father from previous projects. “On the Adult Inpatient Unit, she cured a patient catatonic for fourteen years, rescued a subdural hematoma from lifelong bipolar therapy, and found a pheochromocytoma masquerading as panic attacks. If Susan sees something out of the ordinary, it’s worth examining.”
Reefes sat back, lips pressed firmly together. “Fine.” He made a grand gesture toward the screen. “Enlighten me.”
Susan restarted the MedVid. “Watching the man with normal pressure hydrocephalus walk, we see he has the same slow, short steps, flexed posture, and freezing on the first step. Except, on closer inspection, it’s clear the freezing is more incessant and profound in the NPH patient. It’s not just the first step, but he wrests every step from the ground, as if his iron shoes have to overcome an enormous underground magnet.”
“Magnetic gait,” Reefes supplied. “That’s the technical term, in case they hadn’t taught you.”
“Yes,” Susan said, ignoring the taunt. Many university specialists saw themselves as superior to private practitioners, disregarding so-called outside docs as too far removed from cutting-edge research and set in old-fashioned ways. They sometimes referred to private practice as cookie-cutter medicine. The private practitioners often considered the university doctors arrogant, dependent on technology, and incapable of truly understanding human emotion or observational diagnoses. Susan did not wish to get dragged into that long-standing conflict.
She continued, “Unlike the Parkinson’s patient, the one with NPH doesn’t increase his cadence to make up for the shorter steps. He simply moves much more slowly. His step width is more open, and his feet are externally rotated, suggesting more balance issues than you see with the Parkinson’s gait. The arm swing is essentially normal.”
Reefes stared at Susan. “All right. That sounds like it would make an interesting study, had it not, apparently, already been done. But we all know people’s illnesses progress at different rates, their reactions span the gamut, and there is no absolute uniformity between patients with the same problem. The days of relying solely on our eyeballs for diagnoses is long gone. According to his chart”—Reefes tapped the right upper square of his computer monitor—“Chuck had a positive alpha-synuclein test when first diagnosed. That means he has Lewy bodies in his brain.” He spread his arms. “Ergo, Parkinson’s, not NPH.”
Susan nodded her agreement. She had read over Chuck’s chart thoroughly before bringing her ideas to Dr. Reefes. She was always most careful with the first patient, the one who formed the basis for the attending’s opinion of her and her competence for the rest of the rotation. A decade earlier, the alpha-synuclein assay was a relatively new and highly heralded laboratory blood test that obviated the need for invasive brain biopsy. Prior to that time, Lewy bodies generally were not seen except on postmortem examinations. “Lewy bodies aren’t pathognomonic for parkinsonism. And I’m not suggesting Chuck has a pure form of NPH, either.”
Reefes’ eyes narrowed. Clearly, he was on shaky ground now, the technicalities Susan had chosen to highlight beyond his zone of comfort. Kendall put his foot on top of Susan’s and pushed down lightly in warn
ing.
Susan ignored her fellow resident to put the grainy images of a walking Chuck back onto the screen. “Now, if we watch Mr. Tripler, we get an unusual picture.” She touched buttons on her Vox to highlight different areas. “If I had to diagnose him solely on the basis of gait, I couldn’t do it.”
“Nor do you have to,” Reefes pointed out.
Susan nodded assent. “Nor do I have to. But if you read the old texts, a competent neurologist could distinguish Parkinson’s disease from normal pressure hydrocephalus more than ninety-five percent of the time, without a single lab test.” She continued before anyone could interrupt. “Chuck’s gait is almost, but not quite, a hybrid of the two entities. He demonstrates the magnetic gait, though inconsistently. He has the bradykinesia of both, the flexed posture of both, the rigidity. His feet practically skim the floor, but he can step. He has an inordinately wide-based gait with significant rotation, which favors an equilibrium problem, like in NPH. Additionally, he has some features that don’t fit either syndrome.” Susan highlighted the proper areas. “His gait is stilted, with a pronounced foot drop, more prominent on the right.”
Now Kendall added his two cents to the diagnosis. “You can see foot drop with parkinsonism, and it’s not necessarily symmetrical. But the stilted…” He moved in closer. “That’s pain, isn’t it?”
Susan bobbed her head vigorously. “I think the man is experiencing nerve-root pain. And there’s one other issue.” She left the repetitive image of Chuck walking on the screen. “There are four parts to the idiopathic Parkinson’s disease diagnosis. Number one, there are the symptoms, which he clearly demonstrates. Number two is the presence of Lewy bodies, which has been satisfied. Number three is the exclusion of other disease processes, which is exactly what I’m debating. Number four is a documented response to dopamine therapy.”
“Which we have,” Reefes pointed out, leisurely leafing through the chart by moving his index finger across the screen.
“Anemic, at best. If we go back to the first MedVid, you’ll see the same patient after L-dopa. His gait becomes almost normal. Chuck never had anywhere near that impressive a response.”
Reefes waved a nonchalant hand. “Which can be easily explained by the fact that different patients respond differently to disease entities and to therapy. How else can you explain how my mother died of ALS ten months after her diagnosis while Stephen Hawking lived with the same disease for longer than five decades?”
Eighty percent of patients with amyotrophic lateral sclerosis died within five years of receiving their diagnosis, and ninety percent survived fewer than ten years. The oddity of the theoretical physicist’s prolonged survival assured he would remain one of science’s most examined phenomena for decades to come, and comparing a rarity to the norm did not prove a point. Susan suspected they could have had a monthlong discussion on that particular matter, but she did not want to get into it now, so she conceded the point. “Agreed. But when you’re using response to a specific medicine as a criterion for diagnosis, that response in any given individual becomes much more germane.”
Reefes shook his head and rolled his eyes simultaneously. Susan had a feeling she was rapidly reaching the end of his patience. “Fine, I’ll give you that one. But in this case, we have a positive alpha-synuclein. Lewy bodies are seen in exactly three diseases: Parkinson’s, dementia with Lewy bodies, and Alzheimer’s. Now, of the three, Parkinson’s is the most treatable, and that is the diagnosis Mr. Tripler carries. The dementia seen in those three diseases is not distinguishable, at least therapeutically. In other words, they are treated exactly the same way: supportive therapy until death, which is forthcoming and inevitable.” Apparently thinking they had finished, Reefes gestured toward the door and tapped the code into his desktop, unlinking it from Susan’s Vox.
Kendall started for the door, his movements a bit hesitant. Surely he had expected something better from Susan Calvin.
Susan had to make her case now. “Unless…”
Reefes glanced back up, looking surprised to find her still in the room. “Unless?”
“Unless Mr. Tripler has secondary parkinsonism. Perhaps related to NPH, which might explain the oddity of his gait.”
Reefes laughed, not cruelly but clearly involuntarily. “NPH causing Parkinson’s. That’s a new one.” He gestured more vigorously to the door. “Please get back to work.”
Susan started to obey, then stopped. She turned back to face Dr. Reefes and found him looking at her as well. “I’d like to get an MRI.”
Reefes’ brows rose in increments, and crimson tinged his face. “Susan, I applaud your effort to try to help an otherwise hopeless patient, and I appreciate your desire to discover something that wiser, more experienced heads might have missed. But you surely know an MRI is an expensive and difficult procedure. It would be uncomfortable for Mr. Tripler, who would have to be sedated. It would waste the time of our staff, who would have to transport him there; the MRI staff, who would have to deal with a confused patient; and the nurses, who would need to prepare and assist him. And it would squander our scarce resources. The taxpayers do not need to be burdened with an expensive study that will not change our therapeutic approach one whit.”
Susan knew arguing would prove fruitless. “Can I at least perform a lumbar puncture on Mr. Tripler? That’s simple, inexpensive, and wastes only my time and that of a single assistant, who could be the least-paid person on staff, if you wish. I could do it with a janitor steadying him for me.”
Reefes stared daggers at her. Susan could no longer hope he harbored one shred of appreciation for her. “Denied.” He thrust his fingers toward the door. “Now get out!”
Susan and Kendall scrambled through the door, pulling it carefully closed behind them.
The instant the door clicked closed, Kendall huffed out, “Well. You seem to have made another friend, Calvin. But this time, I’m not sure your ability, or your tongue, can save you.” He lowered his voice to a stage whisper. “You may have to castrate this one with a scalpel.”
“Funny,” Susan growled. Ignoring the patients and milling nurses, she wound her way into the charting room and dropped into a chair, fuming. It was not the first time an attending had chosen not to listen to, or even to belittle, her. Accustomed to gung-ho young doctors, they had heard it all and developed a jaundiced eye, a practical bent that did not allow for wishful guessing. The logical principle of Occam’s razor existed in medicine specifically for the fresh faces; patients did not come with labels. Symptoms could suggest many competing diagnoses, and the simplest and most common nearly always proved the correct one.
There was a reason why one of the most enduring aphorisms in medicine was “When you hear hoofbeats, think horses not zebras,” and the obvious tautology “Common illnesses occur commonly” was bandied about by attendings, probably through millennia. Medical novices were predisposed to make rare diagnoses for many reasons: the desire to contribute in a substantial and memorable way, because striking diagnoses remain lodged in memory longer and more thoroughly, the excitement of plucking something special from the mundane. Most people chose the medical profession for altruistic reasons. It also attracted people who took joy in learning and lived for the epiphany that came so rarely in science and in life.
Susan rose, opened the door, and surveyed Unit 2 of the Winter Wine Dementia Facility. Without much effort, she picked Chuck out from the group. He sat in one of a line of padded chairs, lips moving silently, staring uncomprehendingly around the unit. Every part of him trembled violently, and she wondered if his sensory system had fully adjusted to it or if the world seemed to jump like an early twentieth-century movie. She tried to put herself in his place, a victim of a degenerating neurological system, adrift in a world that gradually lost all sense or feeling.
Kendall stepped up beside her and spoke softly. “What’s your plan, Dr. Calvin?”
Susan bit her lip, grimaced, steeled herself. The answer slipped from her mouth before she had a ch
ance to consider it. “I’m going to perform an LP.” She headed toward the procedure room to set up her supplies, calling over her shoulder, “Would you like to assist me?”
Kendall trotted after her, smiling. “Calvin, I thought you’d never ask.”
With Kendall’s help, the lumbar puncture went off without a hitch. Apparently accustomed to working with and around Dr. Reefes, the ancillary staff did not bother him, or either of the residents, in the ten minutes it took to complete the procedure. Not wishing to involve innocents in their deception, Susan personally delivered the tubes of fluid to the lab, noting only that the cerebrospinal fluid color seemed yellowish instead of crystal clear. Called xanthochromia, it usually alerted a physician to the possibility of a subarachnoid hemorrhage, which was not a logical concern in this case.
Susan knew other causes of xanthochromia existed, including bilirubin abnormalities, such as liver disease or generalized red-blood-cell breakdown, elevated protein, increased numbers of red blood cells in the cerebrospinal fluid from clots or subdural bleeding, or even an unrealized traumatic tap. She also knew eyeballing the cerebrospinal fluid for coloring, the gold standard prior to the last decade, was unreliable, and the lab would use a spectrophotometer to determine whether the xanthochromia was real or a trick of light and background.
As soon as Susan returned, Kendall gripped her arm and guided her into one of the small staffing rooms. For an instant, dread flashed through her, and she wondered if Dr. Reefes had caught wind of her betrayal. He could not get her expelled, but he could contact the director of the residency program and put a blotch on her record. It would not negate all the good she had done, but it automatically put her beneath those with spotless records and could cause a problem when put together with the time she missed while recuperating from her injuries.
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