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Biliary Atresia Education Day 2010

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Quality of Life

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: Quality of Life

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Dr. Barbara Haber: He worked alongside the Japanese Dr. Ohi who looked at many of the Japanese patients. And it's a small study where there's 21 patients in the UK and there were 25 in Japan. And in the UK there's actually no impairment in any category. If you look down — body pain, general health, mental status, physical function, role of emotional health, role of physical health, all the way down to the bottom — and you look at the P values, at least in this small group, it looked like quality of life was fine.

In Japan — and it shows you that there's differences from country to country, that their group of survivors — I wasn't very good at drawing this circle — but their group of survivors there should be a negative .01 there — had, in general, felt their health was poor. And then down here there's three more significant figures. They felt emotional, physical, and social functioning were all impaired.

So you walk away from that, and it's a little confusing. Quality of life is good. Maybe it's not. Maybe it's a small study. Maybe it's the wrong form. A number of people in this room possibly have taken part in this. This is part of this national study, and you filled out a form called the PedsQL. It's a short form that only has 20-something questions, and we've looked at 158 patients around the country and so for this group looking at how your health compares in a U.S. population is probably the most important thing to think about.

So the patients fill out a form, if they can write, and the parents fill out a form if they're under 18. So the 2 to 4 year olds only the parents fill out a form, and the over 18 year olds only the person with biliary atresia fills out a form. Along with that we also do physical exams, lab studies, and history of the medical complications at the same time they come in. And so this is a paper that we're currently working on writing and, in this of the 158, there's a fair number of people in each of these categories. Twenty-three in the 2 to 4 year olds. 43, 43, 37, and we nationally don't have that many over 18. There's 12.

About half are female. The group has normal weights. So you can also infer from that that all these long-term survivors are growing pretty well. They have — this is triceps skin fold, which is a measure of energy stores where people do pinches here and see whether or not you put on enough fat. And that too is in the normal range. This has to do with complications. And there is still cholangitis and occasionally a bleed that happens. And this is recording how many children had one episode within the previous 12 months. It's more common to have it when you're young than when you're old and having one episode does not mean that you're going to have multiple episodes. Healthy kids get cholangitis.

GI bleeds are much rarer. So that you can see that the total is 11 out of 158 patients. So about less than 10 percent will have a GI bleed after the age of 2.

We looked at their lab studies and, for the most part, these kids are still clearing their jaundice or are waiting for a liver transplant. And the oldest group has a little bit of jaundice. The rest are normal, not jaundice at those ages and then you can see that you still have a little bit of — ALT is liver inflammation. Albumin is normal in terms of protein stores going all the way down. All of these numbers hemoglobin, PT or INR are normal. White count is, in general, basically normal as well as platelet count. So that's what the group of patients look like. And we gave them the PedsQL. These are just samples of the way in which we assess quality of life.

So for physical questions the questions are: I feel pain. It's hard for me to read. It is hard for me to run. It is hard for me to play sports. I have low energy. And when the child fills that out, they rate it from 0 to 4. They never feel that, or they always feel that. And emotional questions have to do with: I feel sad. I feel scared. I have trouble sleeping. Social questions are: I have trouble getting along with other kids. Kids tease me. And there's a few other questions. And for school: It's hard to pay attention in class. I forget things. It's hard to get all my homework done.

This is the data. And physically, this 158 patients are physically, compared to other groups, are physically normal. So that for that group of kids who don't get transplanted, they move onto this 2- to 25-year-old age group without physical difficulties for the most part. And yet, for reasons we don't understand, and I'll try to tease it out, these scores, emotional, social, school in particular, and psychosocial is really a combination of these three and total is combination of all four of these — these other aspects are the things where kids seem to have some issues, and we don't know why.

The parents had the same perception as the kids. If you matched the parents in each of these different age groups 2 to 4 year olds and going all the way up, the parent and the child perceived their health the same way. So that was sort of interesting so that parents are getting it right for whatever reason. And so they were matching that the school functioning was the area where there were the most difficulties, and physical was the least difficult.

So to try to tease this out, we to do something called "multivariate analysis," and we use all that other data that we had: their height, their weight, their lab values, their cholangitis, their ascites, everything that we could think about, and try to figure out why are there impairments in certain areas? And what we found is for the children scored lower if hemoglobin was low, if the skin fold for the triceps skin fold was low, or if ascites was present. If ascites is present, it's easy for me to understand that that's going to make life difficult. These other two are really a little subtle, and I was surprised. And probably what it shows is that those kids who are most tired, who have a low hemoglobin, who possibly have less energy stores are the ones who are having more school struggles. And we have to look into this more and see whether or not that's really true. And the parents, I thought that this was funny. Even those they got it right, they get it right for the wrong reasons. The parents scored their child lower if they had things that they could see were abnormal. So they scored their children lower if they looked jaundiced. That was by far and away the biggest predictor of whether or not they ranked their child as functioning more poorly, and yet the child didn't see it that way because they don't look at themselves.

There was no significant change in scores across different age groups. It didn't matter whether or not you were in first grade or in your first year of college whether or not you scored differently on that test. We then compared it to different types of chronic illness to see whether or not you could get a better understanding of what this is. And it's a lot data here, but the bars I'd like to you focus on is the first green one is biliary atresia and the red and white dotted one are normal, healthy controls. So that physically — is this not lined up exactly? Physically children were fine compared to the healthy controls and really significantly better than somebody who has cancer. Their psychosocial, functioning which is that composite group, is for some reason lower, and it may be energy level, as I hinted at. Same thing. So where there's a star up here, those are where there's significant difference. So in the psychosocial, emotional, social, there were differences. And school was the biggest area of concern.

So that paper is in progress, and data is being analyzed. And the reason why we do this is to try to give ourselves new ideas of things that we should focus on. Rather than focusing exclusively on the younger kids and preventing cholangitis and doing these medical things is to try to look into, "What are some of these softer findings that might help us focus our medical attention long term?"

So, in summary, I want to say that growing up with biliary atresia can be okay. It's a pretty corny picture, especially for the older kids. Overall, children and young adults without liver transplantation can do extremely well. They're employed, but we'd like them to live life to their fullest. We expect marriages for them. We expect children and jobs and somehow using this quality of life tool we may be able to tease out, "What are the medical things that might hold them back?" And that's it for the beginning.

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