This morning I started with a new team, Child Health II. In addition to general pediatrics, they cover child nephrology (kidneys) and rheumatology (autoimmune) services. Otherwise, it is not so different from Child Health I, with whom I spent last week. Each has a working crew of 3-5 very articulate, intelligent and eager residents.
One of them, Dr. Sunil, was checking up on a young boy of about 10 years of age who had come in to the hospital with worsening jaundice and altered sensorium, meaning, respectively, that he was, increasingly yellow in color (particularly the whites of his eyes) and wasn't acting himself. A small boy with stick thin, bony arms lay on his side, resting with his eyes slightly open. His short dark hair was oil-slicked and matted to his head. He appeared tired, sluggish even, as if he couldn't be bothered to care that a couple of strange faces peered over him. Over his legs, hips and buttocks were patches of dark coloured skin, like a rash. He appeared to be breathing quite rapidly, though not uncomfortably, and probably as a result of his remarkable abdomen, which occupied as much space as the rest of him put together. He appeared malnourished, like the children of Ethiopia that we used to see on TV. It would be difficult to imagine that this child could carry the weight of his pregnant belly. His father stood anxiously by his bedside, wondering, i imagine, what we could be saying about his child in a language he didn't understand. The boy stared into space.
Here is some basic biochemistry*: jaundice is caused by an increased level of bilirubin in the bloodstream. Bilirubin can be described as a breakdown product of the hemoglobin that carries oxygen in our red blood cells. When they die, hemoglobin is released and the "heme" is broken down in the blood stream to bilirubin through a series of biochemical reactions. Bilirubin is metabolized (conjugated) in the liver, by which it is made ready for excretion in the urine and the feces. If too many red blood cells break up at one time, if the liver is unable to process the bilirubin, or if it gets backed up in the bile duct (from where it is excreted into the GI tract) then it is possible for one to get jaundiced.
This child was diagnosed a few years ago with a disease called Budd Chiari Syndrome. It is the name given to the clotting of blood, thrombosis, in the major vein that leaves the liver, called the hepatic vein. Now for some physiology*: normally, freshly oxygenated blood from the lungs travels to the left side of the heart and out into the rest of the body, where it delivers the oxygen to eagerly awaiting cells. Some of that blood travels to the intestines, where it delivers its oxygen but also picks up digested nutrients for delivery into the body. The nutrients don't go immediately back to the heart, however. They make a stop at the liver. The blood vessels that carry the blood from the intestine to the liver are together called the portal system. The liver is like a processing center for all those nutrients that get sent in from the gut. Some things they pack up and store for later (like excess sugar), some things get processed to be sent out into the body (like cholesterol), and some, like things that are not good for you, get broken down and excreted into the biliary tract. The liver also makes the protein (albumin) that is in your blood and the clotting factors that allow it to clot when you bleed. So, blood travels into the liver by way of the portal system, it makes it's way through the liver in small passages called sinusoids, alongside of which the liver cells (or hepatocytes) can be found. The blood eventually travels out of the liver through various and so-called central veins until they join together to form the hepatic vein, which we mentioned above.
When a blood clot forms in the hepatic vein, the blood backs up in the liver, causing direct damage to the hepatocytes. Over time, and if the damage is extensive, one loses the ability to process nutrients, remove toxins, and make essential blood components. Without clotting factors, which exist in dynamic equilibrium with factors that prevent blood from clotting, bruise-like lesions appear on the skin. These were the "rashes" we saw on the child. Without protein in the blood, pressure gradients in the blood change and fluid travels by an osmosis-like process into the abdominal cavity, causing an accumulation of fluid (ascites) and the increased size of the belly. Furthermore, pressure increases in the portal system, resulting in what we call portal hypertension, a condition which has some potentialy serious complications. Among them are ascites, as the pressure in the portal system leads to extravasation of fluids into the abdomen, and portosystemic (or hepatic) encephalopathy. The build of of ammonia in the blood stream and its significant affect on one's neuropsychological functioning happens more frequently in those who have had an operation to shunt blood from the portal system to the blood vessels (vena cavae) that carry blood back to the heart. This boy had had such an operation a few years back. Furthermore, bilirubin itself can be toxic to the brain in high concentrations. Distended and unaware, this child was suffering through the extremes of both complications.
While we are treating these complications the definitive treatment for a person in this condition is liver transplant. Asked about the chances of such a transplant, Sunil replied: "unthinkable." "Even if he could get a transplant", he explained, "affording the lifelong immunosupressive therapy that accompanies such an operation would be impossible." Based on several blood markers and symptoms, we are able to classify the severity of his liver disease and suggest a prognosis. There is only a 35% chance that this boy will live beyond 2 years (Child-Pugh Class C). Such odds are slim in a country where resources are limited.
We visited another young child on the ward, a very sick girl of 1 year and 6 months with HIV/AIDS. Underweight, with pneumonia, diarrhea, and other complications of a disease that she'd acquired through no actions of her own, she needs antiviral medications desperately. While there are mechanisms through which adults with HIV can get free treatment in India, there is no such thing for children with severe illness. This child will likely die soon.
I don't mention these two children to depress anyone, although it might. It is good for us to understand that death happens in children, and why. It is only through that understanding that we can find both the motivation and the means to correct the problem. Though Indians have access to basic healthcare through government centers, it is a system that appears to be greatly underfunded. More sophisticated care must be purchased in private hospitals, such as CMC. In that sense, there system of care greatly resembles our own. And where our government leaves off in paying for care of sick children, charity is often standing by to take over. Beacuse of this a child with liver failure is more likely (though not guaranteed) to get the liver transplant and the necessary medications than one in India. A child with HIV/AIDS is more likely to get anti-retrovirals. As for the latter, it's a shame that the cost of antiretrovirals, so much less here than in America, are still out of the range of affordability for most.
Later that morning, on our formal rounds, I stood again by the bedside of the boy with Budd Chiari syndrome, that wasted child with a belly full of fluid. I pulled my camera from my bag, curious if the father might let me have a photo so that i could show others back home how such an illness can progress. I have taken several photos of patients, all with their or their parent's permission. I lifted it up and gestured to the father. "Photo?" i asked. He shook is head in a way that i think meant "no". I put my camera away. He turned his head and started to cry.
In medicine, we have the precarious task of balancing our education and the dignity of our patients. We must be careful that in our ceaseless struggle to learn we do not take from patients the sense of control, the sense of privacy, or the sense of hope that allow us to help them overcome illness. It occurred to me a few minutes later that of the children i'd previously photographed, most had significant, even grotesque complications of various illnesses. But each was likely to survive. I wondered if the child's father felt like i wanted to take a photo to record how a child looks before he dies. If he felt i was capturing not a miracle, but a tragedy. Perhaps my camera made the disease seem somehow more serious. I think this is why he started to cry. As far as that delicate balance, i'm quite sure that today i tipped things in the wrong direction.
justin
*i hope i get this right - i.e. i'd be happy to be corrected where i go wrong.
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