I have now been a physician for 16 years. As a whole, the work has been as good as anything I could have chosen. My personality seems well-suited to the job, and I have had very few complaints. I have had a few bad experiences, however. One of the worst happened when I was in my third year of residency.
I was on call that night - covering the pediatric ICU at Riley Children’s Hospital. Since I am Med/Peds, I had a lot of experience caring for the critically ill. Medicine residents were given far more latitude to make independent decisions when on call. Also, we worked at the VA hospital and the county hospital in Indianapolis, where supervision was a lot less. So working in the pediatric ICU was always a step back in independence - having to call the fellow or attending for minor things. We had the reputation for being “loose cannons” in the ICU - although I think the attendings actually appreciated our knowledge.
This was not the issue on this night. Very early in the evening, I was called regarding a child coming in from an outlying hospital. She had taken a large dose of Acetaminophen (Tylenol) and had high liver numbers. Acetaminophen is broken down in the liver and is basically safe at low doses, but at high doses can cause fulminate liver failure. The key to Acetaminophen overdose is to get to it as soon as possible - as the longer the cells are bathed in the drug, the more cells are killed. Even though the person is asymptomatic for quite a while after a massive ingestion, there is a fairly short window in which the person can be saved. There is a fairly standard protocol for dealing with Acetaminophen overdose, depending on how long after the ingestion and how high the blood levels of the drug are. I went to the pediatric texts to make sure I was up-to-date with how to handle this situation.
In 2005, Acetaminophen poisoning was reported to be the leading cause of liver failure in the US, causing approximately 450 deaths each year. While it is debated by some whether a person can unintentionally ingest too much of it and die, most lay people view the drug as being very safe, and are unaware of the significant danger of an overdosage. This girl was a good example of that.
When the transport crew arrived, they told me the story. This 12-year old girl had been upset about being teased by other girls at school. To show how upset she was, she took a handful of Acetaminophen pills and swallowed them. She had taken the dose over 24-hours before, only telling her family when she started to fell sick. This was not good. They told me that she looked pretty good, and that nausea and vomiting were her only significant external symptom.
My first impression when I walked into her room was that she was a normal-appearing junior high girl. She seemed very frightened by all the attention she was getting and how worried everyone who was caring for her seemed to be. I reassured her that we would do our best and that she just needed to get some rest, but in my heart I was very worried.
Her lab results increased my anxiety. The main tests we do to assess the liver are called transaminases, often referred to as “liver function tests.” This is actually a little misleading, because these are not actually tests of liver function, but instead are enzymes used by the liver to clear the blood of foreign substances. The elevation of these numbers generally reveals direct damage to the cells of the liver. Normal transaminase levels are in the 0-40 range, and hers were over 10,000. The real “liver function tests” are ones that test for the presence of cells doing normal liver functions - producing proteins needed for clotting and clearing the blood of toxins, the most significant of which is ammonia. Her clotting time (PT) was significantly elevated and her ammonia level was also elevated. Her liver was failing.
The liver has an amazing resiliency to it, regenerating function after severe insult. Often the proper way of dealing with acute liver failure is to support the functions of the liver by giving blood products and other medications to keep the person alive while their liver regenerates from the damage. If the damage is severe enough, however, no recovery is possible and the only treatment is liver transplant. After consulting with the attending, fellow, and gastroenterologist (liver specialist), we agreed that she would probably need a liver transplant and the proper protocols to secure her a transplant were initiated. I was left to keep her stable overnight.
To lower the Acetaminophen levels in the blood, I gave N-Acetyl-Cysteine, also known as Mucomist. This substance prevents the formation of the toxic byproducts of the molecule as it is metabolized at the liver. This protects the liver from further damage and allows the body to clear the Acetaminophen. Unfortunately, there was probably not much liver to protect.
There are two big problems you deal with when someone is in liver failure. The first problem is the lack of clotting products produced by the liver causing the person to spontaneously bleed. This is handled by giving blood products containing these substances (specifically, you give something called Fresh Frozen Plasma -FFP). The person can bleed in their GI tract, into the lungs, and most seriously, into the brain. The second serious problem with liver failure is the accumulation of waste products in the blood, especially ammonia. The failure of the liver to clear the blood of ammonia causes swelling in the brain, which can cause such pressure around the brain that the brain is pushed down into the spinal canal. This is called a herniation of the foramen magnum and is fatal.
By early mid-evening, she was becoming sleepy. She had been getting multiple infusions of FFP to keep her from developing a bleed. Her transaminases were dropping - which was a bad thing, indicating the number of functional liver cells was going down. Her bilirubin was going up and worst of all, her ammonia level was going up. As her mental status worsened and the ammonia level went up, we needed to know her intracranial pressure (the pressure of the fluid around her brain). To do so, I called the neurosurgeon to put in an intracranial bolt.
An intracranial bolt is exactly what it sounds like. It is a pressure-monitoring device that is inserted through the skull to give an ongoing log of the intracranial pressure. To have one in, the person has to be put on the ventilator so she can be fully sedated. As we gave her medicine so she could be intubated, I wondered if she would ever awaken again.
For the rest of the night, I was fighting with the intracranial pressure. To bring it down, I gave the medication Mannitol (a diuretic used to lower blood pressure and the pressure around the brain) and used the ventilator to hyperventilate her, lowering the Carbon Dioxide in the blood. As the hours passed, I couldn’t stray far from her bedside. I watched helplessly as her ammonia level and her pressure steadily rose, despite my best efforts. At dawn her pupils became fixed and dilated - a sign of herniation. I called the neurologist and he confirmed the diagnosis of herniation. She was declared dead soon after, as we stopped her life support and her monitoring.
It took me a long time to recover from that night - even writing about it this far out brings back emotion. Here was a girl who was being teased by her classmates. She does a silly and stupid thing, taking a handful of something she thought would not harm her, and suddenly she was dead. I knew I had done my best to save her - her lot was cast before she got to me - but it just seemed like a huge waste.
While I know that “these things happen,” as we doctors have to repeatedly say to ourselves, it is very hard to swallow when you see a life so young needlessly ended prematurely. I wonder why it is not better known that there is this toxicity of Acetaminophen. Why is there not a warning on the label of Tylenol like there is on aspirin for the risk of Reye’s Syndrome? The petition has gone before the FDA, but has never been put into law.
Being a doctor can be incredibly rewarding. There is no other profession I would choose if I had to do it over again. Really. Yet there are moments like these where we are reminded of the tenuous hold we all have on life. So many things happen which seem arbitrary and unfair. While the rest of our country can avoid thinking about these things by turning on Survivor or having a swig of alcohol, I am regularly reminded of this fact. Nobody can explain why all of these things happen - anyone who thinks they have all the answers has not heard most of the questions. Watching this young life flicker and then go out has etched on my mind the truth we all must face at some time. I am not God. I do not hold my patients’ lives in my hands. I can only comfort those who mourn, strengthen the weak, help the hurting, and reach out to the lonely. My help will be temporary, but something tells me that the effects may last far beyond my years.
You have given us a beautiful often unseen side of being a physician. Too often the pain is hidden from the patients and the families. Thank you for sharing.
The labeling of Tylenol seems so easy and responsible, I guess that is why it isn't done. The producers of these drugs could label them with out being forced to by regulation if they chose to be ethically responsible. So much for my soapbox.
thank you.
Naomi Giroux M.Ed., RN
Health Educator, Radio Show Host, Author