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Diagnosis Deconstructed

Diagnosis Deconstructed: Sanctuary for an Illness

Morchi, Ravi MD

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doi: 10.1097/01.EEM.0000421869.65752.91

    Home. I can say that historically this disease has gotten the better of humankind. It consumed thousands of lives throughout the 19th century and was especially effective at killing during military conflicts. Some believe it to be the source of a plague that decimated Athens and that shifted power to the Spartans in the 5th century BC. The organism has had its way with our immune system for thousands of years.

    Yet we rarely see it today in the emergency department. Once a potent and feared foe, now it functions as trivia for medical students. And it is promptly dispatched with a course of antibiotics if identified in its first few days.

    Far Away. In this place, on these wards, the air is full of purulence and necrosis. Infants are wailing, and two or more adults languish in a cot originally built for one. It is about 100 degrees, and most of us, practitioners and patients, have become accustomed to sitting in our own sweat.

    Three children in one ward are seizing continuously from cerebral malaria. We have only Valium to give them, and they continue to depolarize violently and build their oxygen debt.

    One child lies under a covered hood with full-thickness burns over 50 percent of her body. We try to keep her comfortable with a drug called Pethidine, and she waits to die.

    A handful of youngsters lay comatose or paralyzed in their beds. Negative lumbar punctures and no signs of improvement days out. Medical mysteries without a diagnosis and practitioners like me without any clear clinical direction to take them. We wait until their families are fed up and ready to take them home.

    Another boy sits with an edematous face and periorbital area that at first glance brings to mind hypoproteinemia from either malarial nephropathy or liver failure due to the hepatitis virus or schistosomiasis. I place an ultrasound on his neck and note bilateral, thrombosed, noncompressible internal jugular veins. He must have an SVC thrombus. Why? What is going on in his mediastinum? I have no idea. Chest x-rays are not easy to get here, and even if I make the effort, what will I do about his mediastinal tumor in this place? I do have aspirin. He is discharged with it to his father. Better to be with family and make the most of time now. He was 8.

    We are a long way from home.

    It is in this place that our disease still thrives. Driven from the western world by a revolution in sanitation, its history remains unaltered here. Water is difficult to come by during the dry season, and desperate villagers will drink from dirty streams and old ponds, and our organism gains entry.

    The patient. Lying prostate on his bed in a pool of his own sweat, a young man mutters to himself, picking at the air. Does he think he is speaking to ancestors long gone? That is the usual presentation. This peculiar psychosis I know. Historically people called our disease “nervous fever” as homage to the encephalopathy. It is characteristic of our illness. I will not LP him, and will not waste the Ceftriaxone. We do not have much of it, and we need it for the children ravaged by true bacterial meningoencephalitis, not for a man whose psychosis originated outside the central nervous system.

    HOB 3/52. This was written on the chart by the medical assistant. This means “hotness of body for three weeks.” Our patient is febrile.

    I look him over, and he recognizes my presence. His eyes are glazed, but he can make eye contact. He is not completely gone. His peripheral pulses are bounding, and his nail beds flush red on release.

    His lungs are rhonchorous, and he coughs a few times as the translator asks him to breathe. Like psychosis, another false localizer. Our disease does not live in the thorax.

    His liver is enlarged and tender to palpation. His spleen similarly so. They are working hard. Macrophages as part of the reticuloendothelial system are trying to clear the plasma of the pathogen. The problem? The pathogen has learned to hide. Not in tissue but in a cell. It finds its home inside phagocytes. Salvation within the very cells responsible for ingesting it.

    RLQ. There is tenderness and guarding here, an exam that brings to mind appendicitis. But the most common cause of an acute abdomen in the western world is not the most common cause here. Not now. Not in this season. Our disease trumps the appendix. Tenderness suggests the pathogen is luminal. Living within white cells that collect in Peyer's patches of the terminal ileum.

    But guarding indicates that the inflammatory process is diving deep to the mucosa and submucosa of the bowel wall. It is creeping through the muscular layers, and will eventually erupt out the serosa. It may have already done so. Typically during the third week of illness, it will perforate into the peritoneal cavity. Occasionally loops of bowel huddle around the perforated, weeping terminal ileum, and an abscess builds between these loops. An interloop abscess.

    At other times, the omentum helps patch the defect. But these men and women have very thin omentum. With long days of work and not much to eat, they do not store fat and so have little protection from intestinal perforation.

    At other times, if the patient does not seek medical attention and is able to live with the breach in his bowel wall, the leaking contents forge their own path to the skin surface. And months later we have an enterocutaneous fistula on our hands.

    I feel his PMI. It is bounding but not displaced to indicate ventricular dilation from rheumatic valvular disease. This is his cardiac clearance. He is OK for the operating theater. He should be able to tolerate the vasodilation of spinal anesthesia. Just one more thing to check before we go.

    MPs 1+. The lab tech has written the results of the blood film. One plus malaria parasites. Not enough. That is not the disease we are looking for. That is background noise. He needs treatment, no doubt, or the parasite will seize the moment and begin to build its own colonies. But malaria is not what has brought him here today.

    Operating Theater. Knowing the organisms are in the terminal ileum, I prefer a short, midline infraumbilical incision through which I can run the ileum all the way up to the ileocecal valve. Spinal anesthesia is adequate provided there is no need to extend the incision cephalad.

    Once in the abdominal cavity, I see pea soup. The characteristic green thick syrup oozing from the terminal ileitis secondary to this organism. I can see many areas of nearly perforated ileum, but usually only one actively leaking site. It often looks like an office hole puncher took a bite out of the ileum. Clean and circular. I trim the edges, and the hole can now be closed in two layers, the abdomen irrigated, the fascia approximated, and the skin loosely brought together to allow the inevitable wound infection to drain.

    Abdomens like this have been looking angrier over the past few years. More and more perforations or near perforations littered along the terminal ileum. Ragged serosa all the way to the ileocecal valve. Decimated bowel that does not lend itself to primary repair. The tissue being too soggy and friable. I find myself considering bringing the injured loop of bowel out as a temporary ileostomy. It is safe and experts in the tropical world recommend it.

    But I know what ostomy care entails in this environment. The 100+ temperatures and the rugged lifestyle do not mesh well with a plastic bag. Patients end up losing lots of volume from their ileostomy, becoming dehydrated and deranging electrolytes we cannot even measure. They stool into their clothes as the seal of the ostomy bag goes bad over just a few days. I imagine them cleaning their soiled clothing in the river nearby. Teaming with our culprit bacteria, their ileal output is released into the water. And downstream, children play and drink. Ostomies in these parts? They may be more of a problem than a solution.

    More and more, I find myself extending that incision cephalad to mobilize the right hemicolon. An incision beyond the range of spinal anesthesia. The patient either needs local anesthetic in the midline or he needs Ketamine. There are no inhaled gases, and there is no intubation. Once mobilized off the retroperitoneum, the right hemicolon can be removed along with the terminal ileum. Then fresh proximal ileum and a healthy transverse colon can be anastomosed. I avoid an ostomy in this way. All the Peyer's patches are gone now. Lying on a specimen table in the back of the operating theater. The organism no longer has a place to call home.

    Residual metastasized bacteria stranded in splenic sinusoids, the biliary tree, and between hepatocytes will have to be eradicated with antibiotics. Chloramphenicol has historically been our best bet. But much like the operative approach, I find that antibiotics have had to become more intense. Chloramphenicol abandoned for azithromycin and quinolones. Now those too are proving insufficient at times, meaning we have to rely on a precious resource, third-generation cephalosporins.

    Typhoid is also known as enteric fever. Diarrhea is the exception rather than the rule. Caused by Salmonella typhi or paratyphi. Vaccines are good but not perfect. The 20th century advent of sanitation and antibiotics in our country declared this killer past its prime.

    But be wary that it has found sanctuary. And it is getting stronger.

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