It was 1988, eleven at night, the fifteenth hour of a twenty four-hour shift, and the emergency department was busy. Our ER has five beds, and they were all occupied. Our two beds reserved for major cases were occupied with patients being evaluated for heart failure and respiratory distress. They were not critically unstable and so I went on to see the little girl in bed three with complaint of headache.
Her parents brought in this three and a half-year-old blonde girl with a sibling in tow. They stated that she had a headache and fever, sick for just the past day. They weren’t sure but maybe it was an earache, as she had these before. She hadn’t vomited and denied sore throat, cough, or abdominal pain. Sounded typical....
On physical exam, she was a cooperative child, appeared ill or at least uncomfortable, but not severely so. Her temperature was 100 degrees F.; her right ear was a little bit red, but not enough to account for her total picture of illness. The rest of the exam did not reveal anything specific so I got a blood count to see if this internal index of physiology might point to a more specific diagnosis.
I noticed that when her blood was drawn, she lay on the gurney without moving, unusual for a child this age. The typical sick kid will resist with crying and attempts to withdraw the arm away from the lab tech. Some kids scream and wrestle in a more angry resistance to being restrained. I worry when the child is too cooperative. Another clinical finding was skin pallor and I remarked to the mother about this.
"Oh, this is just how she looks", she said. "She always looks pale and white."
I took this as some degree of reassurance that there was nothing too out of the ordinary going on here. Still, there was a small voice inside me whispering that this kid was sicker than with the typical viral flu. I had to find evidence to either support this sense or support the conclusion that this was a benign viral illness. Of a hundred kids who appear to have a simple virus, only one or two percent will turn out to have something more threatening. The routine is that they come back for a recheck when the illness has evolved to be more obviously serious, and further therapy is instituted. Often, they are better and nothing more is needed.
While waiting for the blood count to come back, I thought about talking to the family practitioner who was on-call for patients without established doctors. If I were unsure about the seriousness of this illness, a second opinion would be valuable. The majority of patients we see in our emergency department do not have their own doctors. I looked to see who was on-call for backup. I saw that it was a doctor who tends to be resistant to helping, generally looking for a way out of work ("sounds surgical, give it to the surgeon", and other lines of unwillingness to pitch in). I figured that if called him, he would say something like, "I trust your judgment, Jon, do what ever you think is right", and I would be back to square one. Bear in mind that these considerations were fleeting thoughts. Most of me thought this little girl had a benign illness. Her fever was not especially high, she did not have a stiff neck (as is seen with meningitis), and she could walk to the bathroom and didn’t throw up. Her mom said she always looked like this.
The blood count came back normal. The white blood cell count, an index of infection or stress response in the body, can be diagnostically useful if it is abnormal. Sometimes it reliably points to a viral or bacterial infection. Sometimes it doesn’t and sometimes it is completely normal when collapse of health is imminent. It all depends on the context in which it is found. A happy, active kid with a high fever and abnormal blood count is probably all right. A morbidly ill child with a normal blood count is probably as sick as they appear and the blood count is useless. The rest of the kids fall in between. A mildly or moderately ill child with a normal blood count may or may not have major illness. Probably not, by odds. Statistics also tell us that sometimes, the opposite will be true. The one thing we can count on in emergency medicine is that sooner or later, an exception to anything will be seen. There is a certain amount of luck, dumb luck it seems, to finding and treating disasters before they occur. If there is anything to the ‘art’ of medicine, it is sensing the unseen, the impending storm, when only a light mist is evident.
And so, I had to add up the pieces of the puzzle. Odds were on my side, seemingly on her side. Normal blood count, normal skin color ‘for her’, low-grade fever, supple neck, not vomiting, slight ear redness, all pointed to a benign illness. A degree of lethargy pointed in the opposite direction. Could I have known more at that moment?
I told the parents that I could not be certain about her diagnosis, but most signs pointed to a virus as the cause and there wasn't anything specific to do about this now. As it was nearing midnight, I instructed them to check her periodically, offer acetaminophen (Tylenol) for pain and fever, and come back or call for any problems. I would be there all night and available for any concerns they might have. They signed out and although this little girl stood next to them at the desk while I gave them instructions, the dad carried her to the car. Nothing too unusual for a late night, sleepy, slightly ill child her age.
It took another ninety minutes to finish up with the rest of the patients still in the ER. By one something in the morning, I dragged my weary butt up to the on-call room and collapsed for the night. Well, almost for the night. There were two interruptions, strategically placed by the gods of sleepless torment, to break up any semblance of deep sleep or dream continuity. They weren’t major cases, just an ear infection in a toddler with a cold, and a scalp laceration on a young man who attempted to leave a drunken bar fight, met head on with a flying ashtray. Nothing unusual.
The next day was Friday, the mornings on which we have our medical staff meetings or a scientific session for continuing medical education. As I often work Thursday nights, I am usually sleep deprived for the meetings. Our medical staff of about a dozen doctors is mostly a congenial bunch, so we have a pretty good time together. Senses of humor and candid comparisons of notes and patients makes the meetings roll along enjoyably for my work battered brain. My replacement for the next ER shift came in promptly at eight and I was happy to be off call. As miserable as sleep deprivation is, there is a small joy at the end, the release from the pain of being hammered on the head, in the cerebral cortex, by the endless flow of problems to be solved, the endless profound responsibility for so much. It seems so unnatural at these moments. It probably is.
Something was going on. There was a call to the conference room for the radiologist to check a chest x-ray on a child. There were calls over the paging system for respiratory therapy to go to the ER ‘stat’ (right away). Whatever had come in after my shift was over was now in the able hands of Dr. Ben Peters. At the end of the meeting, I decided to swing by the ER before going home. I was curious and, as the department chief, it was the right thing to do.
I was blown away by the scene before me. My little blonde girl from just eight hours ago was in critical condition. She was in shock (not enough blood pressure for survival of vital organs), had ecchymoses (pronounced-ecky mo sees, bleeding into the skin) on her legs and arms, and was semiconscious and limp. Oh my God, she had full blown Waterhouse-Friderichsen syndrome, the life threatening horrible effect of toxins made by the bacteria Meningococcus. This is the same germ that can cause a very severe form of meningitis, a brain infection. The form of the disease she had was a bloodstream infection. Her spinal tap showed no brain infection so it was only in the blood. It is one of the worst diseases anyone can ever have. The toxin produced by the bacteria causes a disruption in the normal blood clotting mechanism so that patients form clots in their blood vessels and they also leak blood out of the vessels. It causes shock, depriving the brain, kidney and heart of enough blood pressure for survival. Even when antibiotics are used to kill the germ, once the toxin is there, the body is ravaged.
Ben called the pediatric intensive care unit at Oakland Children’s Hospital. The staff there gave him directions for treating the shock and other stabilizing maneuvers to prepare for transport to the medical center. She had several intravenous lines placed and was given two potent drugs to flog as much pumping power out of the heart as possible. She was given extra fluids to increase her blood volume. All of these moves worked, at the moment. The blood pressure increased to a more life sustaining level. Her blood oxygen was improved by giving oxygen through a facemask. She had not stopped breathing yet so an airway tube was not placed into her throat.
The parents were frantic. The ER staff was stressed to their limits. We deal with a lot of critical adult illness but very little severe pediatric illness. Meningococcemia (infection with this germ in the blood stream) is uncommon and most of us haven’t seen more than one or two cases, ever. Treatment is often futile and we are shaken to our roots by such an assault on an innocent child. We are soaked by the tidal wave of cosmic injustice.
I left the department and went outside to one of the picnic benches on the hospital grounds and wept. Sleep loss and empathic connection shred my emotional defense mechanisms. My own blonde daughter was not too many years older than this child was and, but for the grace of God, could have been her. My daughter had been a preemie and the images of her tiny body in the isolette, wired and tubed to the big outside world, bounced around my inner eyeballs as I grieved for this child and wondered what I could have done differently. That two or three percent of me that had felt something more was wrong, the instinct that I pushed down and out with hard data - should I have acted more on this sense? What would I have done? If I had demanded that the on-call physician come in to see the patient with me, could I have gotten him to do this? And, would he do the same thing I did? At least if that would have been the case, two of us could share the outcome. Or maybe, if I acted on suspicion, I might have given her a shot of an antibiotic (one of the modern ‘gorillacillins’-antibiotics that will usually kill the major bad germs) and the course might have been benign. Or, I might have given the antibiotic but the toxin was already being made and the antibiotic doesn’t work immediately and the toxin would still have ravaged her little body. I thought and I wept at the likely outcome of this girl’s life and felt responsible. I felt horrible.
I went home and told my wife about this tragedy. After a shower and running several miles in the hills, I tried to play my guitar, but there was no spark. There was nothing to do except allow myself to go through it. When the kids got home from school, I held my daughter and told her how special she was to me.
My daughter was nine years old at the time, still very innocent, sweet, and dependent on me. She is a teenager now, driving around with her friends, doing what she pleases. How things change….
I called the pediatric intensivist at Children’s Hospital to see how she was doing and to ask what I could have done differently. Although not of tremendous relief, it was somewhat reassuring to hear that basically nobody can diagnose this illness before it’s too late. Their uniform experience (and I'm sure there are exceptions somewhere, sometime) is that once there are diagnosable signs of this illness, the physiologic damage is rapid and rampant, and nothing can be done. Most people die, but some survive, with variable consequences. At the first conversation, I was again told that her brain was not involved, unusual for this illness, but the circulation to her arms and legs was a problem with the bleeding abnormalities. They were considering amputations. She was still quite critical.
I felt a little gun shy about repeatedly calling the pediatric hospital, not wanting to know the grim news, but the information came anyway, as our staff called regularly to get updates on progress. I called irregularly. She was in the intensive care unit for about three months, during which time she had amputations of both legs, one arm at the forearm, and some of the fingers of the other hand. My heart still sinks with the image of this child, her future wrenched away from her by the infection from hell. Her brain, however, was remarkably and incredibly unscathed. Even when infections do not directly injure the brain, many illnesses of this severity rob the brain for some of its potential. Sometimes a lot. In her case, she not only woke up and spoke and thought, she was actually already adapting to her new different body. She even had a sense of humor about what she had left. Her bravery made it in to the newspapers. She had her moment of fame, morbid and touching as it was.
The family never came back to our town. The little girl would need any number of support services, not available in our rural community. She would need medical care and special physical therapy. I imagine she would be fitted with modern prostheses to partially overcome the loss of her limbs. She must be about 16 now and I wonder how she has made out. I pray that she has adapted to her disabilities and got to experience some of the simple joys of childhood, despite our grownup sense of reality. I will never know if I could have made a difference, if I had acted on the little voice inside and my self-doubt, only partially tempered by the reassurance of the experts. I am left wondering, connected, forever.
are supposed to practice medicine based on a foundation of accepted facts
and statistically significant new data. A single case does not make
for statistical significance. A single case doesn’t mean anything
in the big picture. One event might be a fluke or a mistake or a genetic
mutant. But, all physicians modify their medical behavior, at least some
of the time, when rattled by a dramatic or otherwise gut wrenching case.
It may make terrible economic sense, but the psychological need to avoid
personal and professional pain determines some of what we do. Though I
will likely never again see a case of ‘flu’ that is really
smoldering meningococcemia, if I do, I will act on the long odds and draw
blood cultures and start some big gun antibiotics. Not that this is what
I do for any viral illness, just the ones that make that little voice inside
whisper, ‘something’s wrong,’ no matter how quietly or
subtly. I must try to protect the innocent victim, even if it costs more
money and risks allergic reaction and the other downsides of antibiotic
use. I have to live with myself, and I may have to live with my patient.
All Rights Reserved © 2004 Jon Sterngold