Do No Harm
I had departed for San Francisco for 5 days of vacation. Leaving one little girl behind in the hospital had made my departure much more difficult. That little girl was my patient Angela, a lovely 6-year-old in whom epilepsy had been diagnosed a year and a half ago. Because all the medication options had been exhausted and her seizures were becoming more frequent and severe, Angela had undergone epilepsy surgery at our center. The neurosurgeon had performed a new procedure: transections of areas that could not be removed safely (because it would have caused permanent weakness).
Most of her abnormal brain tissue had been successfully removed-but the surgery had failed. It was like removing one volcano and trying to plug up another one, only to cause new and more powerful volcanoes to erupt. In more than 200 epilepsy surgery cases performed at our center, this one had been followed by the most unexpected and disappointing result that we had experienced. Some failures are cruel. I had seen fear and pain in Angela’s eyes and in her parents’ eyes. They didn’t understand the medical details, but they knew that things were going from bad to worse. As I left for vacation, Angela lay in the intensive care unit, having brief but very distressing seizures every few minutes. She cried and called for her mommy when she wasn’t seizing. When she seized, her eyes darted out of control and her left side jerked. A few minutes’ rest and then the horrible tape replayed over and over.
We had tried massive doses of medication in a dizzying array of combinations, but we barely made a dent against the frequency or severity for more than several hours at a time. There were no more options for Angela. One more try at surgery was all that was left. The problem was that not only had surgery failed the first time, but it appeared to have made things worse.
Five days of vacation passed in a moment. When I returned to my office on Monday morning I faced the usual avalanche of phone calls, messages from colleagues, and issues concerning the dozen or so inpatients under my care, including several new epilepsy surgery cases. Angela was still in the intensive care unit and was never far from my thoughts. Shackled by the flood of patients, I was unable to spend much time with her and her parents, but I did find time to study her seizures carefully. I kept thinking that this was her last chance.
Two days later Angela returned to surgery. A series of metal electrodes embedded in plastic were placed over her brain to record her seizures and to identify, if possible, where the seizures were coming from. The electrodes could also be used to stimulate her brain with weak electrical currents, to locate motor and sensory areas that could not be safely removed surgically.
Because Angela had seizures every few minutes, the answers came quickly. There was one main seizure focus in an area that could be safely removed, but there was another seizure focus that couldn’t be removed without causing leg weakness. To make things worse, several other brain areas showed worrisome activity. My initial fear- that we wouldn’t find a focus- had been allayed, but my greater fear persisted, that if we removed one hot spot another one would emerge, as it had the last time she had surgery. She had been living in the hospital for 6 weeks with almost continuous seizures.
We held a conference to discuss Angela’s case. Because we were so worried about another surgical failure, we changed our approach and converted two-stage surgery into three stages. We removed and transected (the new procedure to limit seizure spread and preserve functional area) what we could. However, the abnormal activity persisted and it looked nasty. The electrodes were repositioned, and we planned to record her seizures over the weekend. If there were no more seizures over the next couple of days, we would remove the electrodes; otherwise, we would try one last surgical procedure. This additional monitoring would give us one more shot if more volcanoes erupted.
By Sunday we were back where we started. High doses of medication were knocking her out, but every few minutes Angela continued to have seizures coming from the area we couldn’t transect. We discussed the possibility of removing the additional area, which would cause lifelong left leg weakness. But the real possibility that inflicting permanent injury would still leave Angela with seizures (from a new hot spot) made this a very difficult choice. By nature I am an optimist, but my optimism was quickly fading.
We decided to recommend additional transactions. Angela returned to surgery, and with repositioning there was better access to the area deep in her brain that controlled her left leg muscles. The transactions were made in the hot area and in some other areas that still showed epileptic activity.
It was 6:15 am. Just as Angela was recovering from her third surgery, my associate called. Steve, a 19-year-old man with refractory seizures, has recently been started on a new drug. Although his seizures were better controlled on this new drug, high blood pressure developed (possibly from the medication, although his would be the first known case) and antihypertensive treatment was begun. My colleague informed me: “Steve woke in the middle of the night with a headache and then became paralyzed on the left side of his body, unable to speak. He was rushed to a community hospital. His father was screaming over and over that he was going to sue.” My heart sank, not because of the threat of a suit (which certainly didn’t lift my spirits) but because of what might be happening to Steve, a young man with his entire life in front of him. I had a gut feeling-call it necrologic common sense-that he had bled into his brain.
I was weary. It just didn’t seem worth it anymore. “Do no harm” kept echoing in my head. First Angela, now Steve.
Steve was transferred to our hospital. A computed tomography scan showed no blood in his brain, which meant that a stroke was the most likely explanation for his acute illness. However, the MRI was perfectly normal making a stroke unlikely. His speech returned and his left arm strength improved. His left leg remained weak for several days and he was unable to walk.
The pieces of Steve’s picture weren’t fitting together. The findings on his examination were inconsistent. Curiously, he didn’t appear worried or depressed about his weakness. Could he be subconsciously producing this weakness? To answer this question, we injected him with sodium amytal (“truth serum”). His strength returned and he walked. While Steve was under the influence we learned that he was very stressed at work. Conversion disorder, the modern term for hysteria was the diagnosis. How did stress bring this vigorously young man to unconsciously fake a stroke? Exactly where is that line between consciousness and unconsciousness? Exactly how much do patients with conversion disorder know? What parts of the brain are involved with this disorder? What is the anatomy of the unconscious? These are questions that I often wonder about.
Two weeks later, Angela became seizure-free and was taking less medication. Steve was walking. Appearances are deceiving, frames of reference are traps, common sense is sometimes nonsense, but sleep is great.
Three years later, Steve is fine, but Angela still has intermittent focal motor seizures as we desperately try to reduce her polytherapy.
The Brainstorms Healer: Epilepsy In Our Experience edited by Steven C. Schachter, M.D. and A. James Rowan, M.D., Raven Press, 1998, Lippincott Williams & Wilkins
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