The following is an interview with Dr. David Mulligan, Chief of Transplantation & Immunology at Yale-New Haven Hospital. Eyewitness News spoke with him regarding the Becky Houle's case of acute liver failure.
Sometimes there can be a high concentration of something that could've been toxic. When that happens, then you're exposed to this and then suddenly you turn yellow, the whites of your eyes could turn yellow, you get a little confused/fatigued, you may notice that you have either liver pain (pain in abdomen) or you get sick to your stomach and you start to vomit; Blood tests will show that your liver enzymes are through the roof and you have a problem. But, there aren't a lot of signs or symptoms that are there before it's already a serious issue.
Something happened to Becky that she was exposed to, that we don't really know; and many times we don't know the cause. But it caused her liver to develop a disease that continues to fail. Despite the fact that Becky still looks good, she's awake, she can talk to us, and that she has certain liver functions that are in-tact--the liver biopsies, her lab tests show us that there are many functions of her liver that are continuing to go down hill. and it's very *unlikely* that her liver is just going to get better on its own. This course has been going on now for almost 7 weeks; and it's very scary because she still has hope, she still looks for this opportunity to get better; yet the liver function is continuing to deteriorate--and we hope that sometime between now and when the liver would give up that we can get a life-saving liver transplant for her and get her on the path of recovery.
Most of the time when people develop "fulminant" liver failure: they get really sick, really fast, they end up getting sicker over just a matter of days, and they're on a ventilator in ICU, they're in a coma, many times they're on dialysis. We can see very easily that it's life or death in the next few days, the next few hours and we have to get a life-saving liver for them right away. When these more drawn-out liver failures occur, we call it a "sub-fulminant" liver failure. We see the liver get hit by something and we don't know what causes it, it takes a longer period of time for that to draw out and many times, patients have some liver function that seems to be preserved, and it gives us hope--'well, maybe the liver is going to get better and we're going to see her recover'; But there's a lot of the liver function that doesn't--and that's when it gets the most dangerous because we never know when the patient could 'fall off the cliff' so-to-speak, and go into rapid liver failure and if we don't replace that liver in the right amount of time, we can be in a lot of trouble.
SO IT'S BETTER TO BE CAUTIOUS AND GET THAT LIVER WHEN IT'S AVAILABLE?
As long as we're seeing evidence that the liver isn't recovering...If there's any chance that this liver CAN get better and CAN recover--we always prefer that to be the best choice for a patient. We'd love everyone to be able to live with their own liver and not require the medications as a transplant recipient; and that's what we've been holding out to hope for. However, we are realizing that there are a lot of factors that just AREN'T recovering; and if this is the course she's going to be going down--we need to make sure we get the transplant before it gets too late.
HAVE THERE EVER SEEN THINGS TAKE A TURN FOR THE BETTER, AFTER A RAPID DECLINE?
Most of the time when we see things turn around, we start to notice that one thing gets better, then another thing gets better, and the patient progressively recovers the loss of liver function that they were experiencing earlier; and we can get a general trend that with good, supportive medical care, the patient's going to get through this course and through this event and they do recover and they do fine. We don't generally get to the point where we've done a transplant, and then look at the liver to say, 'Oh, we think this liver probably could have gotten better; there wasn't a problem'. That is very rare. I haven't seen that actually occur. We have seen unfortunately, times where someone didn't get a transplant in time and they passed away because their liver went into failure and we couldn't support them any longer.
WHAT'S THE TYPICAL AGE/CAUSE OF LIVER FAILURE?
Either they've gotten Hepatitis C--which is the most common cause of liver disease in this country right now--or they've got other causes of liver disease...and most of those manifest themselves over time in patients in their mid-to-late 40s, or early 50s, and you start to see the cirrhosis cause enough damage for those patients to need a liver transplant. When young people get liver failure, it's usually this fulminant type of a picture; and part of what has been keeping her so well, is because she is young, and she's very healthy, and she has really no medical comorbidities (chronic diseases) or other problems that could be adding to her overall health and so that youth, and strong sense of vitality she has is what's helping her get through this whole process.
WHAT IS 'MELD'?
MELD is the point system that we use that's based on the lab values that any patient has that is able to maintain about how sick they are and what their liver function represents...so that we can get those organs to where we need. The Status 1 situation--like we're dealing with fulminant liver failure--is the top of the list and everyone's list; and those often require a national outreach to get these organs; and so that's why she has an even broad outreach. But with a rare blood-type, we have to make sure that she is getting access to these organs and that there's nobody else closer to where that donor is that's also a status and needs that liver as well that's going to deviate that liver to them because of that proximity to where they live.
WHAT IS THE PROCESS LIKE, ONCE YOU MAKE THE DONOR LIST?
She's got a rare blood type--blood type-B (the 2nd most rare blood type--type-A being the most rare)--and because of that, there's not a lot of other donors that are blood type identical; and she can have a blood type O liver as well--which is the most common blood type--that she'll share in that community. But, a lot depends on the timing. It's all about the timing like so many things in life; and for her since she's been on the list, we've only had a few blood type compatibile donors who are either O or B, that she could've received--but those donors unfortunately were either very old, or had a lot of medical or public helath risks, that have made reluctance to use that organ for her. The fact that she has been holding her own and doing so well, and been very stable, we wanted to make sure--if we were going to put her down that transplant--we want to make sure we're giving her the very possible organ to do so. At this juncture, we've been waiting for just that organ. We haven't been pushed to make say, a really elderly 83-year-old liver be her best choice and put her in a transplant. We've certainly have been trying to give her every opportunity to just recover from this liver failure--if she's going to be able do that--but also holding out, if we're not getting there we better be prepared for a fallback option; and so that's why she's on this list.
Her whole liver would need to come out; but because The liver has this amazing capacity to regenerate; it's really our only solid organ other than our skin that can regenerate when it's been injured or part of it's been removed. It actually grows and reproduces cells, and grows to a full-size organ and is fully functional in as little as 6-8 weeks; and this liver, you can take a portion somewhere between 50-60% of a donor liver and safely perform the surgery on a healthy donor and give that 50-60% of the liver to a recipient who needs a liver transplant, removing their whole liver, and that liver regenerates, grows and it gives them full, normal liver function and saves a life. In both the donor and the recipient, the liver regenerates and grows to full-size and fully-functional in 6-8 weeks; It'll keep growing out for as long as about a year--but they organ is fully functional...you can go about taking whatever medications you need to take, you could do anything that would require liver metabolism and it's all normal in as little as 6-8 weeks.
WHY BE A LIVING DONOR?
A living donor is something that adds to the list; and the reason we do living donors is because we don't have enough of these precious resources of organs to save enough lives; and we've got people waiting for so many weeks, months, and years for life-saving transplants. That's where living donors came a part of is to try to save these lives--and it adds to the opportunity of what we can get from the organ bank in the best possible way.
Our preference is that there's some emotional connection, some kind of relationship between a donor and recipient--because that always seems to work the best. But, with the experience that we've had in kidney paired exchange, we've realized that actually so many wonderful, altruistic people out there that--if they knew there was someone in need, and they had the compassion within themselves to step-forward and to be that hero...that they can save-a-life as long as we can be sure it's safe for them to have the surgery--if that can happen, we're happy to make that transformation and to make that message be known and to save-a-life.
MORE ON THE DONOR SELECTION PROCESS
All of us are built with an immune system that has various proteins on our white blood cells that help go around and police our whole system to tell us what's part of us--what's ourselves--and what's not ourselves...so that the immune system can take care of getting rid of things that could potentially be harmful to us. The organ transplantation depends on that immune system having a special relationship, because now we've given a patient an organ that came from someone else with a little bit different of an immune mapping system...So, the medications that we use help to calm down the effect just enough, so that there wont' be a rejection of that organ by the immune system, but there will be a recognition; and going forward the immune system will get along with this new, donated organ and not cause harm to it but live in a nice symbiosis--so the organ can work and replace the need for whatever that organ replaced, as well as the immune system still having enough capabilities to fight off infections and other things we're exposed to in life and not be completely wiped out, but also get along with the organ so we don't have that rejection process. So, it is very complicated and requires adjustments of medicines in the first month or so in a very careful way to achieve a certain blood level, to make sure that patients are managing any side effects that can come from those medications, and also to make sure that those medications are doing JUST the right job so that they aren't too much or too little....After about 3-4 months, the risk of that goes way down and then we get down to what we call the maintenance phase--and that's something the patient will stay on, just a low-dose for the rest of their lives.
HOW DOES THE DONOR/TRANSPLANT LIST WORK?
The way the system works right now (that we're actually trying to improve), is that everything starts out locally within our own New England Organ Bank.
If there's not a local donor, it goes regional, and then it goes out to a national--with the rest of the country. Right now, because of the way that our population density is based, the number of transplant programs, the number of people that are on a waiting list (in Boston, and in Hartford and other transplant centers throughout Region 1) we're sharing organs amongst ourselves in this highly dense population which is a small geographic area of donor organs; and because of that the number of organs we have in our population that are medically eligible and come from our donors, are very small and give us a much lower opportunity to get a live-saving transplant, then say if we were in FL, or LA, or KS where someone--who is as sick as she is--would probably wait maybe a day or 2 (because there are so many more medically eligible donors there).
We're in the process of solving that...the Secretary of the DOH has mandated that we come up with a better solution to distribute this scarce nat'l resource, so that people don't have to worry about where they live is going to hurt them, to be saved with a life-saving liver transplant. And the truth is, that people who live in areas that are well-served and they do have a much better access, they can wait a little longer without that fear of dying.
THE PROBLEM WITH ORGAN DONORS/TRANSPLANTS
The problem is, there are 122,000 people on that waiting list for solid organ transplants in the country right now, and we only have about 7,000 donors in this country. That's a huge disparity. That same ratio exists in CT as well as in the rest of New England....That's WHY we have to think about things like living donation as a potential solution to really save lives. The good news is, with living donation, the outcomes are phenomenal; even long-term patients who get transplants, do extremely well and even better than many of their colleagues who get the deceased donor transplants.
LOOKING FORWARD TO THE FUTURE
It's so scary. But it can happen to anyone, and not from anything that you do to yourself to create this...and we have to be prepared and we have to be able to manage it. The hope is that we can get a patient through without requiring a transplant; but when we do we need to be able to give that gift of life...doesn't even need to be on your driver's license. Have a conversation with your family. Talk to them and say, 'You know this is would be something I'd really like to do if I no longer can use my organs; I don't want my life to be lost and not leave a legacy that I can really save so many others.
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