The liver is an amazing organ when it is working properly. It is the largest and heaviest internal organ. According to wikipedia--a fantastic source of medical information, I know, especially to all of the medical and lab professionals out there--it is thought to be responsible for ~500 separate functions in the body. It is important in many types of protein synthesis, all sorts of metabolic cycles (e.g. carbohydrates and lipids), produces blood coagulation factors, stores such substances like vitamin D and iron, and breaks down toxic substances (many of you know that about drinking alcohol, for example).
Bottom line: when you have a failing liver, you will have a lot of different problems to look forward to.
It was clear a few years ago that Dad started to have trouble breaking down toxic substances. Again, Dad never really drank, so this is not an issue about alcohol. Or drugs--he doesn't do that either. The liver plays a key role in converting ammonia to urea. Ammonia (NH3) is the product of oxidative deamidation reactions. The reaction is part of the breakdown process of amino acids (they are the building blocks of proteins, for the non-scientists). To humans, ammonia is toxic even in small amounts. In a healthy person, ammonia is converted to urea via the urea cycle and then excreted from the kidneys. When someone has end-stage liver disease, they could potentially have a build-up of ammonia levels. This is not good.
When someone has a build-up of ammonia (and other toxins that the liver isn't working properly to convert and/or help excrete out of the body), they can develop hepatic encephalopathy--we will call this HE from now on. This has been a horrible aspect about Dad's liver disease. Honestly, this has been the most terrifying (may be tied with variceal bleeding--this is a close call) symptom for me to handle as a daughter/caregiver. I learned all about HE in the summer of 2010 when Dad first showed signs of acute HE while in the hospital after a variceal bleed.
What exactly is HE and what does it mean?
From a drug company marketing materials:
Here are some of the criteria of HE grades:
Where does this leave us today?
First, as described above, the fact that he had the odds stacked against him in May and was lucky to survive the complications from his liver disease, it never changed his status on the waiting list--at UCLA or Indiana. If I were to ever change the system, boy do I have some recommendations for UNOS.
Second, I am absolutely terrified of another episode like May where he has an acute HE 'attack'--whether from a bleed or another cause. Not only was he facing ridiculously high odds of fatality while in a coma last year, but seeing a loved one in a coma is.... indescribable.
Third, I think that he suffers from a milder version of HE on a daily basis. I can talk about this topic all day [to my father's dismay] since it's so incredibly frustrating to describe and experience from my perspective. My perspective being that I know my father very well yet am not experiencing the actual symptoms personally. Some days are worse than others for him in relation to his HE. What's so frustrating for me is that Dad doesn't notice or acknowledge the symptoms even when I point them out--my guess is as it's impacting his brain it is hard for him to be objective. He has many other symptoms of HE frequently including hand tremors, frequent loss of balance, and trouble concentrating.
I REALLY started to get scared when he started forgetting things. That was the red flag right there that something was not right. My father can recall any date or fact from his life or any point in US or World History from memory in a flash (or facts needed as a working physician--that memory he thankfully still has, though). What was his address when he was 2 years old? What year did a particular Civil War Battle take place? What countries border Ukraine? What is my friend's name that lives in Florida? All of these types of questions are usually something that almost supernaturally comes second nature to him. Yet, the past several months (or years ?) he has slowly gone from struggling to remember to simply not remembering details at all. Short-term and long-term memory are gone. Like that. Thanks, HE!
For him, the most noticeable symptom is inverted sleep pattern. Dad cannot sleep at night. He usually attributes this to other issues, but I am convinced more than ever that this is due to his HE. It has gotten worse ever since the first acute HE episode of Summer of 2010. Anyhow, he cannot sleep at all at night and is sleepy during the day so he never feels quite right from all of this lack of sleep. I certainly wouldn't either.
And those are just some aspects of HE that I have noticed over the past year and a half or so. It certainly has been worse since he was in a coma in May 2011. I'll save some of the detailed stories of what he was like with acute HE symptoms (but not in a coma) for a little later, but it was traumatic for me. It was traumatic for Dad at those times, too, however he doesn't even remember now because of his memory loss that I attribute to his HE--is that a good side to this? He can't remember exactly how crappy he felt at various low points throughout the last few years?
When the world seems to conspire against Dad, it seems to hit me just as hard. At least the emotional aspect. Obviously, I cannot begin to understand what he is going through physically. However, it is incredibly difficult to see such extreme changes in someone that you have known all your life to be a strong, incredibly intelligent, and healthy role model. Not to mention the memories imprinted in my mind of sitting for hours in his ICU room talking to every physician in Simi Valley and UCLA that knew his case trying to figure out how to make sure he was the 20% that survives. And these days, even though I talk to him multiple times everyday and am very thankful he indeed was the 20%, I just hope that his HE isn't too bad that day and he remembers when I last called.
My mazel.
Bottom line: when you have a failing liver, you will have a lot of different problems to look forward to.
It was clear a few years ago that Dad started to have trouble breaking down toxic substances. Again, Dad never really drank, so this is not an issue about alcohol. Or drugs--he doesn't do that either. The liver plays a key role in converting ammonia to urea. Ammonia (NH3) is the product of oxidative deamidation reactions. The reaction is part of the breakdown process of amino acids (they are the building blocks of proteins, for the non-scientists). To humans, ammonia is toxic even in small amounts. In a healthy person, ammonia is converted to urea via the urea cycle and then excreted from the kidneys. When someone has end-stage liver disease, they could potentially have a build-up of ammonia levels. This is not good.
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| http://smmhc.adam.com/content.aspx?productId=39&pid=1&gid=000372 |
When someone has a build-up of ammonia (and other toxins that the liver isn't working properly to convert and/or help excrete out of the body), they can develop hepatic encephalopathy--we will call this HE from now on. This has been a horrible aspect about Dad's liver disease. Honestly, this has been the most terrifying (may be tied with variceal bleeding--this is a close call) symptom for me to handle as a daughter/caregiver. I learned all about HE in the summer of 2010 when Dad first showed signs of acute HE while in the hospital after a variceal bleed.
What exactly is HE and what does it mean?
From a drug company marketing materials:
HE is a condition that occurs when a damaged liver cannot remove the toxins that a healthy liver normally would. These toxins then pass into the blood and travel through the system until they reach the brain. They can then damage the brain and cause HE. People with liver damage, which is also known as cirrhosis, are most likely to get HEOne can notice the signs of HE when someone starts having certain brain symptoms. There is a grading system based on the symptoms called the West Haven Criteria. The grades range from sleep disruption and anxiety to coma and an awful lot in between. Dad has had every symptom of HE including one of the worst weeks of my life when Dad was in a coma from HE in May 2011.
Here are some of the criteria of HE grades:
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| http://www.medscape.com/viewarticle/578716 |
For the record, if you are in a coma from HE, you actually have a pretty low chance of recovery. Last May when Dad went into a coma after a variceal bleed, I learned that coma associated with hepatitis has a fatality rate of 80%. As I boarded the flight to California from New Jersey in May I thought my Dad only had a 20% chance of even surviving. Once I was out there, he was unresponsive for several days at that point.To add to the fun of that week, his physician and transplant coordinator at UCLA told me this condition (80% change of death, no eyes open, no verbal response, and no reaction to simple commands) doesn't change his status on the transplant list. The liver transplant criteria always comes down to MELD score as I explained about in a previous post and his MELD score still did not reflect his actual risk of dying as it was originally intended to do. Meanwhile, my father had about 6 doctors on his case while he was in a coma: his local GI, a pulmonologist, a cardiologist, a infectious disease specialist, and a few more for good measure. While he was in ICU in a coma his entire body seemed to be failing. He had pneumonia at one point, he had a major cardiac event in the middle of his coma, his liver was worse (but he only got up to a MELD score of 21 or 22 from the teens--not even close to the 30+ needed at UCLA for a transplant), and he had multiple infections that had to be managed. Yet, he's not even close to needing a liver transplant according to UNOS/UCLA. RIIIIIGGGGHHHHTTT. This national transplant system totally makes sense!
By the way--the last 2 acute HE 'episodes' that Dad experienced were related to variceal bleeding. These are connected. When he starts bleeding internally from his stomach or esophagus, he is releasing blood into his system--more specifically into his bowels where the liver is supposed to be working to convert the blood into substances that the body can remove. Blood contains proteins and amino acids that cannot be broken-down in his cirrhotic liver, however, and therefore Dad will develop HE from the toxic build-up of ammonia. To get an idea of how abnormal his system was after the last bleed: normal ammonia levels are less than ~40 mcg/dL and Dad was over 250 mcg/dL when he was in a coma.
First, as described above, the fact that he had the odds stacked against him in May and was lucky to survive the complications from his liver disease, it never changed his status on the waiting list--at UCLA or Indiana. If I were to ever change the system, boy do I have some recommendations for UNOS.
Second, I am absolutely terrified of another episode like May where he has an acute HE 'attack'--whether from a bleed or another cause. Not only was he facing ridiculously high odds of fatality while in a coma last year, but seeing a loved one in a coma is.... indescribable.
Third, I think that he suffers from a milder version of HE on a daily basis. I can talk about this topic all day [to my father's dismay] since it's so incredibly frustrating to describe and experience from my perspective. My perspective being that I know my father very well yet am not experiencing the actual symptoms personally. Some days are worse than others for him in relation to his HE. What's so frustrating for me is that Dad doesn't notice or acknowledge the symptoms even when I point them out--my guess is as it's impacting his brain it is hard for him to be objective. He has many other symptoms of HE frequently including hand tremors, frequent loss of balance, and trouble concentrating.
I REALLY started to get scared when he started forgetting things. That was the red flag right there that something was not right. My father can recall any date or fact from his life or any point in US or World History from memory in a flash (or facts needed as a working physician--that memory he thankfully still has, though). What was his address when he was 2 years old? What year did a particular Civil War Battle take place? What countries border Ukraine? What is my friend's name that lives in Florida? All of these types of questions are usually something that almost supernaturally comes second nature to him. Yet, the past several months (or years ?) he has slowly gone from struggling to remember to simply not remembering details at all. Short-term and long-term memory are gone. Like that. Thanks, HE!
For him, the most noticeable symptom is inverted sleep pattern. Dad cannot sleep at night. He usually attributes this to other issues, but I am convinced more than ever that this is due to his HE. It has gotten worse ever since the first acute HE episode of Summer of 2010. Anyhow, he cannot sleep at all at night and is sleepy during the day so he never feels quite right from all of this lack of sleep. I certainly wouldn't either.
And those are just some aspects of HE that I have noticed over the past year and a half or so. It certainly has been worse since he was in a coma in May 2011. I'll save some of the detailed stories of what he was like with acute HE symptoms (but not in a coma) for a little later, but it was traumatic for me. It was traumatic for Dad at those times, too, however he doesn't even remember now because of his memory loss that I attribute to his HE--is that a good side to this? He can't remember exactly how crappy he felt at various low points throughout the last few years?
When the world seems to conspire against Dad, it seems to hit me just as hard. At least the emotional aspect. Obviously, I cannot begin to understand what he is going through physically. However, it is incredibly difficult to see such extreme changes in someone that you have known all your life to be a strong, incredibly intelligent, and healthy role model. Not to mention the memories imprinted in my mind of sitting for hours in his ICU room talking to every physician in Simi Valley and UCLA that knew his case trying to figure out how to make sure he was the 20% that survives. And these days, even though I talk to him multiple times everyday and am very thankful he indeed was the 20%, I just hope that his HE isn't too bad that day and he remembers when I last called.
My mazel.
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