Moribund
The most frightening thing about covering the ER is the kids, especially the little kids. And what I knew about pediatrics as a senior medical student covering the ER as an unlicensed, pretend ER physician at Aiken Memorial Hospital in Aiken, South Carolina wouldn’t have filled a thimble. Pediatrics was not a required third year rotation and I had not elected to take it in the fourth year.
I had learned what little I knew from reading and study. And from almost a year of experience in the ER. I knew that a small child with a deadly serious illness might not look very sick. A child with meningitis might only seem listless with a little fever, in contrast to an adult who would have a high fever and a stiff neck. I lived in fear of missing a case of childhood meningitis.
One relatively quiet evening, the two ER nurses, Christie and Brenda, and I were sitting in the main treatment room chatting when the door burst open and a young Black woman rushed in carrying a child of about three who hung limply across her arms. The woman’s face wore an expression of panic. Her voice was urgent as she pleaded, “Please help her. She’s real sick.”
Christie leapt from her seat pointing at the gurney, “Lay her there.”
I said to Brenda, “Call the pediatrician,” then began to examine the child.
The first thing that burst into my consciousness was how cool and clammy she felt, almost like touching a corpse. She was completely flaccid, like a rag doll. Had her skin been less dark I would probably have seen the cutaneous lesions immediately, but I didn’t. Her pulse was thready and barely detectable.
Christie tried to obtain a blood pressure, shook her head and said, “Nothing.” She was in circulatory collapse. In shock. Her respirations were shallow and sporadic. She was moribund, at death’s door.
As I tried to sort out what to do first, intubation, start an IV, the pediatrician appeared as if by magic. He quickly assessed the situation, then said, “Peds ward, quick.”
He wanted to go to the treatment room on the pediatric ward where he would have all the equipment needed: pediatric size laryngoscope and endotracheal tubes for intubation, pediatric IV sets and, most important, pediatric nurses. I scooped the child up and began to run as she flopped in my arms, head lolling. The pediatric ward was on the second floor. I took the steps two at a time.
She stopped breathing as I ran through the door to the pediatric ward, the pediatrician holding the door open.
She was in full cardiopulmonary arrest as I laid her on the table in the treatment room. I started mouth to mouth respirations while one of the nurses began chest compressions, one hand on the tiny sternum. The other nurses and the pediatrician began preparations for inserting an endotracheal tube.
This was my first and only personal experience with real live mouth to mouth resuscitation. It’s a good thing because I’m not sure I would have been able to do it again, since after the first few breaths she vomited copious, acrid, sour fluid into my mouth. What do you do in that situation? You spit it out, clear the patient’s airway and resume rescue breathing.
The pediatrician managed to insert the endotracheal tube and we ran the code for about an hour but it was useless. She was gone. Close inspection after stopping the code showed what looked like scattered hemorrhagic skin lesions.
Afterwards, I felt drained and finished my ER shift like a zombie. I felt morose over losing the little girl and exasperated with her mother for waiting so long to bring her in.
A couple days later, my beeper lit up with an Aiken number. It was the pediatrician.
“Bill, that kid we ran the code on. All of the blood cultures are growing meningococcus. She had overwhelming meningococcemia. You and all of your family members need to take chloramphenicol for ten days.”
This diagnosis at least explained how the child had gotten into such an extreme state before her mother brought her in to the hospital. She was infected with the bacterium Neisseria meningitidis, commonly referred to as meningococcus.
Neisseria meningitidis is the feared cause of deadly meningitis that often occurs in outbreaks in crowded settings such as day care centers, college dormitories and among military recruits, usually involving older children and adolescents. Many organisms may cause meningitis, but when the average person hears the term meningitis this is the disease they are thinking of. Although meningococcal disease often occurs in outbreaks, most cases are sporadic, especially in young children. Children younger than 5 have poor immunity against meningococcus.
The cardinal signs of meningitis include fever, severe headache and neck stiffness. The diagnosis is straightforward when all these elements are present. But disease, any disease, often presents atypically in the very young and the very old. Even children with meningitis severe enough to cause status epilepticus, repetitive seizures, may not have the classic signs and symptoms of acute bacterial meningitis.1
In meningococcemia, the organisms grow, proliferate and circulate in the bloodstream, spreading the infection widely throughout the body so that it involves multiple organs. Most meningococcal infections cause meningitis but some produce meningococcemia as a primary infection, with or without coexistent meningitis.
In meningococcemia, the infection can rapidly reach a fulminant, overwhelming state. The fatality rate for meningitis alone is 4-6%, but for meningococcemia it may rise as high as 40%, often leaving survivors with devastating complications. Neisseria meningitidis is a uniquely virulent organism.
Meningococcemia may evolve into a fulminant form and produce circulatory collapse, septic shock, within hours of onset.2 Herrick, in 1919, said, “No other infection so quickly slays.” 3 Not even Ebola kills as rapidly as fulminant meningococcemia.
Some cases of fulminant meningococcemia with rapid demise are due to a condition called Waterhouse-Friderichsen syndrome, in which low platelets and impaired blood clotting lead to large and small hemorrhages into the skin. The small hemorrhages are called petechiae and the large hemorrhages purpura. Recognizing the hemorrhagic rash is often difficult in dark-skinned patients.
In Waterhouse-Friderichsen syndrome, a major contributor to the morbidity and mortality is hemorrhagic necrosis of the adrenal glands leading to severe, acute adrenal failure. Waterhouse-Friderichsen syndrome occurs in 10-20% of children with meningococcemia.
In our case, the child may very well have appeared well or only slightly sick that morning, perhaps listless with a runny nose and low-grade fever, nothing that would have prompted a visit to the doctor. In the first 4-6 hours, meningococcemia tends to mimic a viral upper respiratory infection, before entering a rapidly progressive phase. She may have grown more listless through the day, perhaps vomiting, before abruptly becoming unresponsive and going into septic shock.
It’s not that this mother neglected her child. No, meningococcus can kill quickly. It could have happened to anyone.
At that time, my family was my wife, Robbie, and two sons, Wes, age 4, and Matt, age 14 months. The Aiken pediatrician thought all four of us should take chloramphenicol for ten days. There are many other choices for post-meningococcal exposure antibiotic coverage now, but in the spring of 1970 the choices were penicillin and chloramphenicol, and chloramphenicol had the edge in potency against a very virulent organism.
The problem was chloramphenicol’s reputation. The drug was widely feared and seen as the antibiotic of last resort in most clinical situations because of its tendency to cause bone marrow suppression and aplastic anemia. Chloramphenicol is toxic to the bone marrow and predictably causes a drop in hemoglobin above a certain cumulative dose. The most serious, fortunately rare but sometimes fatal, side effect is aplastic anemia.
In aplastic anemia, the bone marrow stops producing blood cells of all types: red blood cells, white blood cells and platelets. Examination of the bone marrow shows pancytopenia—all the cells are gone. Lack of red blood cells causes anemia; lack of white blood cells predisposes to infection; lack of platelets causes abnormal bleeding. Untreated, the majority of affected patients die from one of these complications or from transformation into a lymphoproliferative disorder, such as leukemia or lymphoma.
All this was bad enough. I had also heard of something called gray baby syndrome due to chloramphenicol. I did not know much about gray baby syndrome but it just sounded bad. I did not want Matt turning gray.
Robbie and I discussed our situation. I did not tell her every gory detail, kept gray baby syndrome to myself. No need to scare her to death. The word meningitis conjures up fright enough. I was scared aplenty for the two of us, afraid of the disease, afraid of the treatment. I decided to get another opinion, maybe even two.
Only a couple of months before, I had done a one-month rotation on the Infectious Disease (ID) service. ID is a subspecialty of Internal Medicine and we spent most of our time roaming around the hospital doing consults and recommending which antibiotics to use in various situations. The Chief of the Division of Infectious Disease was Dr. William Chew, an experienced and stellar clinician and an inspiring teacher. I had gotten to know him well enough to drop by his office for a chat. He listened to the story attentively, then said, “I think it would be OK if you took penicillin yourself for ten days. Don’t give your family anything but minimize your contact.”
That meant not holding my baby or hugging my son for ten days. Normally, one of the first things I did when I got home in the evening was sit down with the baby in my lap. That would have to stop temporarily. Small price to pay.
I talked to another faculty member, a junior faculty member, not as experienced as Dr. Chew. She seemed uncertain, then recommended we all take penicillin for ten days.
Decision time.
The Aiken pediatrician thought we should all take chloramphenicol. But two medical school faculty members were telling me penicillin was sufficient, so I was not going to expose us to the risks of chloramphenicol. In the end, I took the penicillin and stayed away from Robbie and the boys for ten days, holding my breath the whole time.
Nothing happened. We were all fine. The little girl was an isolated case, not part of any outbreak, and I did not catch it. But what a tense and stressful ten days. Every ache, every pain, every kiddie cough and sniffle set us on edge. The last dose of penicillin felt like emancipation.
And the story proves the lesson, once again: it never hurts to get another opinion.
