Emily Grodinsky, MD (left), Edith Graham, MD Neurology Residents (center), David Walker, MD (center), Lauren Koffman, DO, MS Assistant Professor of Neurological Sciences (right)
Aaron Smith is the kind of guy you want to be around. He is 20 years old, with maturity and wisdom beyond his years — forged from having lost his mother at a young age. As a result of his loss, he has grown closer to his father and other relatives, and has developed into someone who is “gentle, reserved, hardworking, loving, and hilarious” according to his niece, Tyler.
After graduating from high school, Aaron went straight into the workforce, accepting a position at Amazon Corporation. Despite the job demanding he be on his feet 40 hours or more each workweek, he hasn’t taken a single day off in two years. He has been devoted to his team and has developed an extended workplace family. Outside of work, he spends much of his time enjoying quiet reflection, often catching up on current events, news and sports. But despite his mature persona, Aaron has a fun and lovable side. He loves texting family and friends funny pictures of animals, especially dogs, and making people laugh.
A Routine Broken
Aaron also thrives on following a routine, and he and his dad John made a habit of going to Dunkin' Donuts together every morning at 9. One day in mid-March, he said he was tired and did not want to go, which John thought was unusual considering Aaron rarely strayed from his habits. When John returned from Dunkin' Donuts around 10 a.m., Aaron was still asleep, and John didn't try to wake him up. When Aaron finally did get up to go to the bathroom around 2 p.m., Aaron said he was "just sleepy." But when John went to check on him again around 4 p.m., he found Aaron passed out and covered in vomit. John immediately called 911, later telling the medical team that he “was so scared because [his] wife [Aaron's mom] passed away like that in her 40s." It didn’t make sense to him, though. Aaron appeared fine the night before. Aaron hadn’t been sick recently and appeared completely healthy when he went to bed the night before. He wasn’t the kind of kid to mess around with alcohol or drugs—he knew better, and because he and his dad spent so much time together, his dad says he’d "spot that in a second."
An ambulance rushed Aaron to the nearest hospital, and he underwent a CT scan of his brain that was initially concerning for a possible stroke in his brainstem—the part of the brain responsible for essential involuntary functions like breathing, coughing, eye movements and being wakeful. As a result, a call was made to the team at Rush University Medical Center to transfer him to Rush’s neuroscience intensive care unit for further treatment. Aaron arrived at Rush in the middle of the night, intubated and completely unresponsive. Little was known about him at the time except that he was young and had previously been completely healthy, having rapidly and mysteriously progressed to a comatose state within a single day. The only backstory that had been passed around was his intended trip to get coffee and the words ”Dunkin’ Donuts,“ which were echoed by the night team while the 20 year old remained otherwise unknown.
Mounting Pressure, Surmounting Odds
There was an initial momentum to Aaron’s case. An MRI was quickly obtained that night and showed seemingly devastating findings—diffuse vasogenic edema, or swelling, throughout his brain, plus hemorrhages in deep areas called the thalami, which are part of the brain’s system that maintains consciousness. It was clear that something was terribly wrong with Aaron’s brain, although it was unclear as to what exactly was going on. Another MRI was done the next day, this time with the administration of a contrast medium to look for signs of breakdown of the blood-brain barrier, and it showed that even more areas of edema had developed in the span of 10 hours.
Because of the high risk that the pressure in Aaron’s brain would rise to dangerously high levels, an external ventricular drain was placed, both to measure his brain’s pressure directly and to reduce it by draining cerebrospinal fluid. A biopsy of Aaron’s brain tissue was also done early on given his rapid deterioration and the mystery surrounding his case. But that’s when all momentum seemed to halt—the following weeks were spent in stagnation, with the fear that the ongoing swelling in Aaron’s brain and the resultant increase in intracranial pressure could kill him at any moment.
To keep his intracranial pressures down, Aaron was put in a medically-induced coma for two weeks with the aid of powerful barbiturates, and for much of this time a special device was also used to aggressively lower his body temperature as a way of slowing down his metabolism. Most importantly, he was given very high doses of steroids and other medications that would suppress his immune system—and hopefully, reduce the swelling—in case the underlying problem was an autoimmune one. This was done after consulting the hospital’s neuro-immunology experts, who had seen cases like Aaron’s before. His brain and body were essentially shut down while his family and the healthcare team waited and hoped for him to get better, with no way of knowing if he actually would.
During this time, the results of Aaron’s brain biopsy came back and revealed his diagnosis. He had an immune-mediated disease called necrotizing vasculitis, in which the blood vessels in his brain had become so inflamed that they were causing progressive destruction of brain tissue. This came as grave news, as most reported cases of rapidly progressive necrotizing vasculitis of the brain ended in death, or at best, severe disability.
Meanwhile, Aaron continued to have persistently elevated intracranial pressures despite the treatment he was already receiving, and the neuro ICU team became accustomed to hearing their beepers going off several times per shift—each time a new alert about a dangerously high value, and each alert leading to another dose of mannitol or hypertonic saline to try to wring out excess fluid from Aaron’s brain. There was even talk of having a laparotomy performed in the direst moments, but the thought of cutting open Aaron’s abdomen to help indirectly relieve some of the pressure in his head was disheartening, and the image of him with a gauze-packed open abdomen was one that felt completely deprived of hope. In those moments it seemed as if the team’s fears had been realized, and that Aaron wasn’t going to be getting better.
During regular meetings with Aaron’s family, members of the team spoke measuredly, trying as best as they could to balance facts and updates with everything that was unknown and unknowable. But despite these often dismal updates, Aaron’s family never gave up hope, even when they kept hearing all the uncertainty about whether he would even regain consciousness ever again.
After more than three weeks, the tides shifted and Aaron’s intracranial pressures began to stabilize. All of a sudden, an entire day would go by without any beepers going off about Aaron’s intracranial pressure being high. The team felt reassured, and slowly started to rewarm his body back to a normal temperature and taper off the barbiturate infusion, watching for any signs of the intracranial pressure going up again. But as Aaron’s body was coaxed back into “on” mode from a prolonged comatose state, the pressure stayed within normal ranges. The team became hopeful once more, expecting him to eventually start to wake up. For several days afterward, Aaron was asked the same basic commands—to wiggle his toes, stick his tongue out or squeeze the fingers placed in his hands—but as the team looked on expectantly, each request was left unfulfilled.
Then, on the morning of April 8, those same routine tasks were asked of him—“wiggle your toes, Aaron” and “wiggle your fingers”—but this time, 10 toes wiggled, and 10 fingers wiggled all on cue. There was a stunned pause from the examiner. “Goodness, Aaron, you can hear me!” Then came the next command: “Show me your tongue!” Aaron’s tongue slid forward alongside the endotracheal tube, and just like that, everyone knew he was on his way back.
His mental status only continued to improve from that point forward. But his body had become severely weakened, and he wasn’t yet strong enough to come off the breathing machine, so he underwent a tracheostomy—a safer and more durable breathing tube that would allow him time to recover without having to feel limited by his breathing. His external ventricular drain was able to be removed, and a repeat MRI showed a stunning improvement in all the areas in his brain that had looked so damaged and swollen before. The steroids and immune-suppressing medications seemed to have worked.
Getting Back to Routine
Like most people in their 20s these days, Aaron quickly reached for his phone when he did become strong enough, and started texting his family and exchanging funny pictures like he had done before. He seemed to be back to the same person again, and his sister Tawanna agreed: “He has the same personality and everything.” His whole family considered it miraculous as they watched him recover. Finally, after a total of 31 days at Rush, he was deemed medically stable for discharge to a long-term acute care hospital (LTACH), where he would continue to be weaned from the breathing machine.
Aaron spent one month at the LTACH, during which time he had successfully come off the breathing machine and was able to have his tracheostomy removed. By early May, he passed a swallow evaluation and was able to eat on his own, requiring only minimal supplementation through his feeding tube. He kept his spirits high and his motivation strong, and with physical therapy by his side, he also walked for the first time since his illness hit him two months earlier.
After leaving the LTACH, he had his first follow up in the neuro-immunology clinic. The neuro-ICU team that had taken care of him in the hospital went to see him firsthand when they heard he was coming back, and they could not believe their eyes when they saw how well he was doing. He was that mythical patient—the one who miraculously pulled through despite long odds—that ICU providers read about but don’t often get to see. Hearing his voice was the reward for all the long days and nights working in the hospital taking care of patients like him.
Since the team could hear Aaron speak, they finally had the chance to ask him what his favorite drink at Dunkin' Donuts was after wondering all this time.
“Coffee,” he replied.
“Of course, but what kind?”
“Just black. No sugar.”
They couldn’t help but smile at the anticlimactic response. After weeks of seeing him unresponsive, in a coma and on the verge of dying, members of the team that had taken care of him could now see the real Aaron being Aaron. It was a simple but immensely gratifying answer.
Because he was doing so well by the time of his clinic visit, Aaron was transitioned to Shirley Ryan Ability Lab for inpatient acute rehabilitation. But the journey to full recovery was far from over—he needed to regain aspects of both his physical and mental strength, and maintain the stamina to constantly strive for improvement. While there, he spent multiple hours each day in sessions with physical and occupational therapists, speech therapists and the rehabilitation medicine doctors to reclaim his independence with daily tasks—even simple ones like walking down a hallway by himself that he could no longer take for granted. As a big sports fan, he kept himself motivated watching basketball games and keeping up with the NBA playoffs all spring.
His strength continued to improve each week, and by the end of spring he had graduated—having been discharged home from acute rehabilitation after spending more than two months in various healthcare facilities. When last seen in the neuro-immunology clinic at Rush, he mentioned he could walk up to half a mile at a time on his own. And by late summer, the long, arduous journey had come to an end of sorts, when Aaron was discharged from outpatient therapy due to his continued improvement. According to his sister, he's been “killing it” with outpatient cognitive testing, and Aaron and his family continue to feel his memory and personality have gotten back to what they were before despite everything his brain went through. He’s been approved to return to work, and plans to do so at the end of this summer.
A big thank you to others involved in Aaron’s care who also included (in alphabetical order): Dr. Fawaz Ahmad, Dr. Hunan Chaudhry, Dr. Christine Chuck, Dr. Rajeev Garg, Dr. Christopher Green, Dr. Sayona John, Dr. Wajahat Lodhi, Dr. Pranusha Naidu, Dr. Allison Osen, Nicholas Panos PharmD, Dr. Alok Patel, Dr. Sebastian Pollandt, Georgeanna Rechner PharmD, Dr. Rodd Rezvani, Dr. David Smyth, Dr. Thomas Shoemaker of neuro-immunology; the majority of the neurosurgical residents/faculty; and the caring and diligent nursing staff, respiratory therapists, and physical/occipital therapists of 11 west neurosciences ICU.