One morning in 2009, young New York Post reporter Susannah Cahalan woke up certain that she had bedbugs. Something just felt wrong. She was overcome with a desire to scour everything in her tiny apartment, and to have an exterminator in to spray—despite his assurance that he could find no evidence of bedbug infestation anywhere in her apartment.
Because of the nature of my illness, and its effect on my brain, I remember only flashes of actual events, and brief but vivid hallucinations, from the months in which this story takes place.
Cahalan suffered from anti-NMDA-receptor autoimmune encephalitis. Essentially, this means that her brain was inflamed, so her capacity to reason, remember, form new memories, or have a coherent sense of self, was obliterated. During her months-long ordeal, she essentially "wasn't there" in her own body. Afterward, she applied her tenacious reporter skills to hunt down medical reports, videotapes, and interviews with the people who helped her through this ordeal in order to piece together an understanding of what happened to her, and (essentially) who she was while her brain was so badly malfunctioning. She was blessed in two ways: She was surrounded by benevolent "guardian angels"—her parents, boyfriend, friends, and coworkers—who stood by her (literally) throughout her ordeal. And she had the best medical care possible—including the brilliant medical researchers who identified what was happening to her and successfully treated her.
Most people believe that the mind and brain are separate entities that somehow communicate with each other. This belief system is called dualism. A contrary position is physicalism—that the mind is nothing more and nothing less than what the brain does. Thinking is neurons firing. Learning is modifying neural connections. Knowledge, skills, and memories are neural circuits. Remembering is activating those circuits. Damage the brain, and you damage the mind. Damage the mind, and you damage the person's identity—who he or she is.
One of the perplexing questions that interviewers ask Cahalan is what it felt like to be mad, because mad she certainly seemed to be during that period. She became unpredictable, violent, childishly demanding, and paranoid—certain that everyone from her nurses to her father was out to get her. But as she points out, she can't really answer that question because "she" wasn't there. What was there inside her skull was a malfunctioning brain, a brain that could not effectively distinguish between true events and hallucinations, fitfully forming "memories" of both in ways that made them seem equally true. When she viewed videotapes of herself, she saw someone she knew was her very sick self, yet she frequently had no memory of the experience captured by the camera's unwavering eye. The "true" Cahalan remained only in the circuits that the disease had not yet damaged, and those circuits were fighting a losing battle to survive.
Getting a diagnosis proved as challenging as seeking the Holy Grail, a maze of false steps and genuine progress that began with her trusted (but stumped) gynecologist and ended with a truly gifted neurosurgeon. The first neurologist she saw dismissed her rapidly disintegrating self as simply "too much drinking, too much partying, and working too hard", carefully recording her admission of drinking two glasses of wine each evening with dinner as drinking two BOTTLES of wine each evening.
Was this a case of medical incompetence? No, simply medical ignorance. As Cahalan puts it:
In the spring of 2009, I was the 217th person ever to be diagnosed with anti-NMDA-receptor autoimmune encephalitis. Just a year later, that figure had doubled. Now the number is in the thousands. Yet Dr. <X>, considered one of the best neurologists in the country, had never heard of it…He is a by-product of a defective system that forces neurologists to spend five minutes with X number of patients a day to maintain their bottom line.
To Cahalan, the problem is that doctors spend too little time with patients. But one could argue that her story carries a different moral: That too many physicians spend too little time reading the medical literature. Cahalan's story had a happy ending, because her case was handed off to a Syrian-born, American educated neurologist who coupled a dogged determination to get to the root of a problem with a driving compassion for his patients. Think Gregory House with a heart. But unlike House, he did not arrive at the right diagnosis by ordering countless invasive tests using expensive technology. He arrived at it by asking the right questions and employing simple pencil and paper tests that led him to suspect that one of her cerebral hemispheres was inflamed. After confirming that suspicion with a lumbar puncture and white blood cell count, what happened next saved Cahalan's life:
Dr. Najjar then recalled a series of cases in the recent medical literature about a rare autoimmune disease that affects mostly young women who had come out of the University of Pennsylvania. Could that be it?
I cannot emphasize enough the importance of that statement. Had Dr. Najjar not been reading the medical literature or attending medical conferences where such new findings are presented and discussed, he (like Dr. X) would have missed the diagnosis. It would not have mattered whether he had spent 10 minutes examining his patient or 10 hours. And Cahalan would now probably be either dead or institutionalized.
Most patients are not aware that having an M.D. medical degree is not sufficient to practice medicine in the U.S. Instead, physicians must renew their licenses periodically, and to do so, they must complete a number of hours of Continuing Medical Education (CME). The number of hours and the content of the education varies from state to state. Here is an example from the state of Pennsylvania.
CME programs are typically developed, reviewed, and delivered by university faculty at teaching hospitals who are experts in their individual clinical areas. While commendable in its intent, CME is often criticized as pandering to drug and device manufacturers who often use their financial sponsorship to bias the programs toward their own products.
In my own experience, a litmus test of medical competency is simply this: Ask your doctor for information regarding a drug or procedure that he or she has decided to prescribe for you. If your doctor refers to published medical studies, you are probably in good hands. It means his or her judgment is based on the best evidence currently available about the safety and efficacy of the treatment plan. If instead, your doctor refers you to paraphernalia given to him or her by a drug/device representative, consider it a warning flag. It means his or her judgment is based on information given to him or her by people who stand to financially benefit from the treatment plan. Oftentimes, the prescribing physicians themselves receive financial incentives for every patient treated using the drug or device.
What most people also do not fully appreciate is this: Medical research takes a good deal of money, and the question is: who is putting up that money? If the research takes place in a teaching or research institution (such as a teaching hospital or university), it is usually funded with public money—grants from the federal government. The results of such research must be made available for public viewing through summaries (abstracts), usually through websites such as PubMed or Medline. If instead it takes place within a drug or device manufacturer, then the funding comes from the company's profits. On balance, there is less risk of bias when there is no financial incentive for positive outcomes of the studies undertaken. For this reason, information that comes from studies undertaken in research facilities and published in medical journals is more trustworthy than information that comes from commercially-funded research.
The tubes of cerebrospinal fluid taken from Cahalan during her lumbar puncture were shipped to a medical researcher at the University of Pennsylvania for analysis. The researcher, Dr. Dalmau, was the senior author of a medical research paper published in 2005 in the medical journal Annals of Neurology that described four cases of young women who suffered from the same symptoms as Cahalan. The paper went on to describe careful studies that isolated the source of the symptoms—an autoimmune reaction to a specific type of neuroreceptor, the NMDA receptor. These receptors are like wall sockets in which only plugs of a certain shape will fit. In these patients, antibodies attached themselves to the receptors in ways that made it difficult (or impossible) for these receptors to do their jobs. He and his colleagues had also developed a protocol for successfully treating the disease. Cahalan was diagnosed as having the disease, and the protocol was begun. Improvement was seen almost immediately, although it would take months before Cahalan was back to her old self.
But even though her former memories, skills, intelligence, and personality have returned, her memory of her ideal is sketchy at best. Some true memories come rushing back at the smell of hospital disinfectant. Others, she knows, must be memories of hallucinations because, no matter how real they seem, they simply are too bizarre to be true. Large segments of that time are simply irretrievable, undoubtedly because the hardware necessary to form memories was severely compromised.
Cahalan ends her book with this illuminating insight:
Someone once asked, "If you could take it all back, would you?"
At the time I didn't know. Now I do. I wouldn't take that terrible experience back for anything in the world. Too much light has come out of my darkness.
Copyright Dr. Denise Cummins February 18, 2014
Dr. Cummins is a research psychologist, a Fellow of the Association for Psychological Science, and the author of Good Thinking: Seven Powerful Ideas That Influence the Way We Think.
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