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Biliary Atresia Education Day 2010

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Clinical Course

Experts including Dr. Barbara Haber and Dr. Elizabeth Rand provided detailed updates on Biliary Atresia at the annual BA Education Day. CHOP's Biliary Atresia Clinical Care Program has been caring for children with the liver disorder since the 1970s.

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Transcript: Clinical Course


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Dr. Barbara Haber: The clinical course in BA — I thought this baby looked cute. My daughters did not, but — the goal is to show you like some people can look and everything is coming up roses. It looks pretty good. These are some of the statistics I think everybody should know. This is a famous paper that describes the outcomes from 1968 to 1983, and it hasn't changed that much.

If you look here, this is close to birth and about half — this is living with your native liver. Half the children have moved onto transplantation somewhere between 2 and 5 years of age. And if somebody says that 20 percent overall will be transplant free, that's where that number comes from. It's very old data though. We don't know if that really applies to today's situation. But many people will tell you 80 percent overall will be transplanted, and it comes from that picture.

If you want to know where you stand compared to the rest of the world, this is the data from around the world. And I would say that being in the United States and being at our center is competitive, meaning we hope that we've delivered as good care as you could have gotten anywhere else and that in the United States the chance of being transplanted by 2 years of age is about 50 percent. This has to do with your post-transplant survival and overall — and I think that our statistic is actually better than this — overall between your biliary atresia, Kasai, and possible transplantation — the overall survival is greater than 90 percent. And it's probably higher than that. So when people are starting out, the thing that I want you to know is that you can either do okay with your own liver or someone else's liver, but overall you should be able to live. That sounds a little morbid, but it's better than what some people think in the first weeks.

In Japan, which is where we looked to some of our data, their native liver survival is a little bit higher perhaps, hard to say. Transplantation is a different situation for them. UK is perhaps a little bit worse in terms of their native liver survival, but these aren't comparing exactly the same situation. But, overall, I would say that being in the United States is a favorable place in terms of medical care.

One of the other facts that I think everybody should know is — What is the hallmark of doing well? When can you predict that somebody's probably not going to need a liver transplantation early on? So this is the need for transplantation by the age of 2 years. So this top curve is if your bilirubin — your total bilirubin — is under 2 three months after the operation, your chance of needing a liver transplantation is less than 20 percent.

If you never have a total bilirubin that goes under 6 at three months, your chance of needing a liver transplantation is quite high. So, within the first three months after surgery, we're able to tell people — Does it look like you're going to need a transplant because that Kasai didn't work.

If it does work — we're going to skip that one — never mind. This is what happens when you put slides together the night before. That's in the next part of the talk. So that was one of the things I thought everybody should know about. The next is how important is the surgical center expertise? And here we do about ten cases a year, and at many of the larger centers it is greater than five cases per year. This data is — comes from England and it became the basis for them deciding to have centralized care and stopping other hospitals from doing surgery.

They found that if you did less than five cases a year, this is the chance of not having a transplant. Almost everybody needed a transplant if you were at one of these smaller centers. And it was even more drastic if you look at the overall outcomes in terms of survival. So it's always better to go to a center and the surgeon that has experience.

The standard medications for the first year — I feel a little bit like I'm preaching to the choir since most of the people have moved beyond this step in this room — but these days we use Urso to help bile flow. We use antibiotics to help with cholangitis, and we use vitamin supplementation to help with some of the fat soluble vitamin malabsorption. And so that was my overview about biliary atresia. Now I have to give the first talk.

So the goal for today is to do a variety of different things. We're going to cover growing up with and without need for liver transplantation. We will talk about nutrition. We will talk about transition, which is something new that we're thinking about at our center. Now that we have more and more people growing up is what do you do when they actually age out of our care? I will try to cover some of the science and then let you meet some of the families that have gone through a variety of different experiences. And before you hear their stories, I want to caution you that sometimes we pick people who have gone through a lot. So we want you to know that you can go through a lot and come out the other end doing well. Not everybody has always as dramatic courses as some of the people that we've chosen.

So I want to do a talk called "Biliary Atresia Long-term Outcomes — the Other 50 percent," the 50 percent who don't get transplanted. It's a topic that's often ignored. And I threw out at about 4:00 o'clock this morning my original talk and anything that you see there because I just thought it focused too much on complications and that's just not what I wanted to talk to you about. I want to show you that people grow up, and they do fairly well. The general management after a Kasai is to optimize nutrition, promote bile flow, and prevent inflammation.

I told you and showed you this curve a second ago which shows that, nevertheless, even with us doing all that, there's a good chance that you may need a liver transplant. But about half won't after the age of 2. And those are the ones who didn't have — who had good bile flow and who had good growth. The two most common reasons to end up with a liver transplant — the first one is that the Kasai didn't work. And the second one is that your nutrition just couldn't support you well enough, that you need to move on and do something that — you need a better liver and a better setup in order to grow better.

So if you didn't end up in that category — and I really think that this picture is a little backwards I feel like the first two years is a tornado, like you've been hit by lightening and then after that things really calm down. So it's, sort of, you move into a phase where things just don't happen nearly as often, and you have to think about things in a slightly different perspective. There's very few papers addressing this older group of patients. Biliary atresia patients, we now know, live into adulthood. They're able to attend mainstream schools. They pursue higher education. And they're, in general, fully employed. They have marriage in their future. They have fathered children, and they've delivered children, you know, so there's light at the end of the tunnel with or without a liver transplant.

So one way of approaching how to assess this older group of patients is to focus on something called "Quality of Life." And quality of life means that we'd like to think about things in a more global way and not just focus on medical complications and how to prevent them.

So there's something called "health-related quality of life" and it's a measure of outcome. So rather than saying — Outcome is whether or not you need a transplant or if you're dead, which it seems like a little bit focused on the negative — to look at quality of life, we now view success as a combination of how you're doing on multiple dimensions. And we managed to take this from the cancer world who realizes that sometimes giving chemotherapy just isn't worth it, if it's going to impair your quality of life to a great extent.

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