Another of the few individual case reports of pigbel in the medical literature describes a young diabetic nurse in the Netherlands who died 24 hours after being admitted with characteristic symptoms, in 1984. He had eaten an unspecified quantity of pork at a party the day before becoming sick, but no other guests were affected. As the paper’s authors write, “It is well known that diabetic patients have a lowered resistance to infections. It is therefore tempting to speculate that this may have been a contributing factor.” Neither the Dutch doctors nor those of us who treated Grace can go any further than that.
Race improved only transiently in the hours following her operation. At first her acidosis responded to treatment, and her blood pressure stabilized. She developed a sepsis-related condition of inadequate blood clotting, called disseminated intravascular coagulation, but it wasn’t severe enough to cause serious trouble. Her subnormal temperature rose to 103, indicating a more appropriate response to infection. On the morning after surgery, we were cautiously hopeful, even though the mottling had decreased only slightly, and blood tests continued to show evidence of rhabdomyolysis. But it became increasingly difficult to maintain the balance of minerals and fluids in her body, and the generalized swelling of her tissues progressed as her kidneys began to fail. Dialysis was begun late that day, shortly after Brian West called me with the diagnosis.
The number of consultants was multiplying. By evening Grace had been seen by specialists in infectious disease, dermatology, neurology, kidney disease, gastroenterology, surgery, and anesthesia, and every one of us continued to monitor her condition closely. Besides the minerals added to her intravenous solutions, she was receiving five medications, three of which were antibiotics. The intern’s summary note takes up seven pages of closely written script in which 14 distinct problem areas are identified: sepsis, recent necrotic bowel, blood pressure, kidney failure, ventilation of lungs, rhabdomyolysis, low calcium, low magnesium, the effect of shock on the liver, disseminated intravascular coagulation, diabetes, pain control, skin mottling, and nutrition. The white blood count, which had dropped to 16,000 in the immediate postoperative period, was beginning to rise again and had reached 21,000. By the next morning, the evidence of worsening sepsis was mounting. Almost certainly, the process in Grace’s bowel was extending to the area that had appeared uninvolved two days earlier. When I made the decision to reexplore her, there was universal agreement. Her belly had begun to distend again.
By then the kidney failure was rapidly worsening. Grace’s tissues had retained so much fluid that her presickness weight of 125 had risen to 185—her entire body was bloated and swollen. It was decided to give her another dialysis treatment and then go directly to the operating room.
Again I went out to speak to my patient’s mother, and again I described the situation to her exactly as I saw it. Anne had not left the hospital since Grace’s admission, sleeping in the MICU waiting room and eating in the cafeteria. When it was permitted, she would stand at her daughter’s bedside, holding her hand and stroking her face—speaking quiet words of encouragement, even though Grace didn’t know she was there. In her thoughtful, analytic way, she listened to every consultant and always came to the right conclusion. Anne had added everything up, and before I said a word she knew what I had come to tell her. Our conversation was a reprise of the one we had had two days earlier, but the outlook was even worse. I had thought it impossible for Grace to have been any sicker than she was before the first operation, and yet the impossible had happened. Anne signed the consent form and took my hands in hers, just for a moment. This time nothing needed to be said.
A radioisotope scan was done. I looked forward to having my fears laid to rest by the absence of any troubling finding, but when I reviewd the study, I felt my knees weaken.
When the abdomen had been sterilized and draped, we reassembled on each side of Grace exactly as we had 48 hours earlier, but now there was a larger group around the head of the table. When a patient is very sick, anesthesiologists cluster about, trying to help each other as much as possible. During 30 years of a surgical career, it has been my not quite tongue-in-cheek observation that a patient’s chance of survival is inversely proportional to the number of anesthesiologists required to get the operation under way; a figure of six or higher is a virtual guarantee of death. As I looked up at the assembled group, I counted six. I made a wry comment that they seemed not to appreciate, and then went right to work.
Grace’s abdomen was bulging so tightly that it strained against the stitches holding it together. As soon as they were removed, the contained fluid and gut exploded out onto the drapes. Quickly, the surgical resident and I put everything in some approximation of order and assessed the findings. Starting just at the point where we had placed the staples to restore continuity, a bit beyond an anatomic point called the duodenal-jejunal junction, the next 18 inches of intestine looked exactly like the segment removed two days before. The preoperative impression was correct—the process of necrosis and clostridial overgrowth had extended and would require further excision. This time Brian West had come to the OR himself. When I completed the removal of the specimen, I handed it directly to him. He scrutinized it silently for a few minutes, and then we spoke briefly about its appearance before he took it off to his lab for further testing.
I carried out the operation much as I had done before, except that this time I closed the wound with a series of individual large stitches of heavy nylon, placed in such a way that they exerted a pulley effect—abdominal distension would bring the wound edges closer together. It’s a time-consuming and not very pretty closure, but the strongest I know of, and I wasn’t taking any chances with the possibility of a burst incision.
Afterward, Grace’s improvement was more sustained. Within 24 hours the rhabdomyolysis had decreased, and her kidneys began to function better—she went from nearly zero urinary production to the beginnings of what would soon be a reasonable amount of output. Moreover, her clotting mechanism was satisfactory, the white count had dropped to 15,000, and the pH of her blood was normal. The evidence of sepsis was much less. The livedo reticularis had begun to recede, and within another day it would be gone. Twenty-four hours after the surgery, Mike Bennick wrote in his note, “Improvement on all fronts.” For the first time, the campaign was beginning to look winnable.
There was to be one more scare, a few days later. Grace’s fever began to rise in a sequence of ascending spikes, and her white blood count went up to 33,000 by the fourth postoperative day. I thought the problem was an infection in one of her many intravenous lines, but I couldn’t find any proof of it. I then began to worry that leaking intestinal contents might be contaminating my surgical wound, but I couldn’t find any evidence of that either. The most frightening concern was the possibility of yet another extension of the clostridial infestation, into the remaining length of bowel. To evaluate this, a radioisotope scan was done, of a type designed to light up areas of infection or necrosis. I looked forward to having my fears laid to rest by the absence of any troubling findings, but when I reviewed the study with the superspecialist who had done it, I felt my knees weaken. The entire length of remaining small bowel showed an irregular pattern of involvement with the process I had seen in two successive specimens of excised bowel.
My mind’s eye could visualize the now-familiar carpet of clostridia lining Grace’s gut. Yet the study, scientifically precise as it was, seemed strangely inconsistent with what I kept finding each time I returned to examine Grace’s abdomen, something I did over and over again. Despite the radiographic appearance of necrosis, her belly was flat and she didn’t grimace or in any other way display evidence of pain when I pressed deeply inward. Through my stethoscope, I could hear the unmistakable and very comforting sounds of peristalsis. Most important, although she was still quite sick, Grace’s general appearance was improving each day. My patient looked hardly at all like the deathly ill girl I had twice rushed to an operating room.
Here was a great deal of pressure on me to open Grace’s abdomen again. High-tech gadgetry is very impressive to young doctors, and has long since, in the hearts of many, usurped the revered place once reserved for the clinical skills of history taking and physical examination. Except for the senior infectious-disease consultant, I was by some two decades older than any of Grace’s panoply of caregivers, and I decided it was time to pull rank. I went upstairs to the MICU and wrote a long note in Grace’s chart, the gist of which was expressed in two sentences: “Her abdomen is simply not the abdomen of a person with necrotic bowel. I do not think she should be operated on.” Then I got up to tell Anne. She was standing at the entrance to Grace’s cubicle, deep in conversation with Mike Bennick.
Anne recalls that morning’s events very well. She had followed every step of the previous days’ evaluations and knew that all the doctors were talking about another operation. She also knew that the operator (she now tells me this is what she and her family called me during the first hectic day in the hospital) seemed reluctant. The operator was now leading her and Bennick into the only empty cubicle in the MICU.
I have no recollection of what I said, but Anne remembers the exact words. Thinking back on them now, they sound unnecessarily magisterial, but perhaps that was what was needed at the time. Anne tells me I looked directly at her and said, “I’m going to make a command decision,” and then to Bennick, “Mike, come with me.” What I do remember is that Bennick and I went to Grace’s bedside and carefully reviewed the physical exam of her abdomen. Anne tells me we took a good long time to do it, but when we emerged from the cubicle, we were of one mind. Bennick had agreed that we should sit tight.
During my training years, I worked with a surgical resident who had been a star athlete at a large southern university, and he seemed to have a down-home Carolina bon mot for every clinical occasion. He would have said that this kind of decision making was “playing guts football”—we absolutely had to be right. Actually, Bennick and I were taking less of a chance with Grace’s life than some might have thought. She almost certainly would not have survived a third operation, in which I might have been forced to remove all of her remaining small bowel. I was betting that only the mucosa of her intestine was involved in the process of necrosis, and her present benign physical exam meant that she had already marshaled the forces necessary to overcome the infection and heal that layer.
Fortunately for all of us, that thesis proved to be right. By the next day Grace had improved sufficiently so that her breathing tube could be disconnected from the respirator. Twenty-four hours later, in her first fully alert moment since admission, she opened her eyes. Within minutes she saw her mother looking down at her, holding a large card printed with the alphabet, which Anne had made ready for just such use. Grace gestured for the card, and pointing very slowly to each letter, she spelled out, “I have a history exam on Friday.” Eleven days had passed since her first operation, and she had lost every moment of them.
“My mother said, ‘No, honey, that was two weeks ago.’ I felt like ‘Oh, my gosh’—you know, total amazement. Then she asked, ‘How did you make it? You weren’t supposed to.’ And I spelled out, ‘I got my strength from my daddy.’ ”
The improvement continued, although very slowly. It took almost three more weeks in the MICU before Grace was ready to be transferred to an acute-care floor. She stayed there an additional two months and then moved to the hospital’s rehabilitation unit. She had lost a great deal of weight and considerable muscle mass in her legs, but she knew that everything was recoverable with hard work. She was finally ready for discharge from the hospital 18 weeks after she had entered it.
It would be another four months before Grace regained enough strength to return to college. Her mother considers her graduation two years later to have been the final step in a triumph not only of perseverance and luck but of Bill’s protecting spirit, too. A few hours after Grace’s return to wakefulness on that joyful morning four years ago, a rainbow appeared in the sky, even though there had been no rain. Anne remembers looking at it and being sure it was a good omen.