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Biliary Atresia Education Day 2010

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Nutrition in Biliary Atresia Part Two

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: Nutrition in Biliary Atresia Part Two


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Sarah: The way it works is that when you have an absence of — when this is obstructed — where is my little pointer thing? When this is obstructed in terms of everything, what you lose is really the ability to digest fat and to digest fat-soluble vitamins and that is really what causes all of the issues.

Fat is 9 calories per gram. So every little gram of fat you take in you get 9 calories. It's awesome. Carbs and protein are 4. So they're less than half of that, which is fine. We can — you know carbs, protein they're still fine, you still need them, but you get a lot of calories by using just a very little amount of fat. And when you don't absorb it as well, it's really frustrating because you're not going to be able to grow as well. When you then are not going to be able to absorb that as well added onto the fact that you're not — you have increased needs because you're malabsorbing, like, it's just a very frustrating intuitive cycle.

So why do we care about the fat-soluble vitamins? I get this question a lot actually. Surprisingly. So I thought I'd talk about for a little bit. So since your ADEK, you're A-D-E-K. Vitamin A is really what's important for eyes skin, teeth, and actually your skeleton long term. But mostly it's eyes and skin and teeth is what we're looking more for that. You can — vitamin A is actually supplied in plenty in most of the infant formulas and the breast milk has a pretty nice amount. So there's not always the need to additionally supplement with vitamin A. What you'll typically hear more is D and K and E.

For vitamin D we really are looking at that bone health, we're looking at your skeleton. How healthy are your bones? You're in a formative time. Do the kids have until they're 20 to lay bone? Absolutely. But you want to start off on good solid footing and so that's why it's really essential that you get that done.

For vitamin E has many roles as an antioxidant. It also forms red blood cells, which again is essential and really something that we need, and the nerve health piece is huge with it and again you really only have those first three years for the myelination of those nerve fibers.

Vitamin K plays a factor in clotting. When I used to — at a former hospital, one of the surgeons used to joke that it was, you know — the liver transplants were always the hardest for him because he's like, in the other, you know, when you're transplanting kidneys, you're transplanting a heart, he's like, the clotting factor's working. You know, the thing that actually clots your blood, and as a surgeon, when you're in there, you know, you don't want a kid to have bleeding problems. You don't have to worry about that. With the liver you do. That's an issue. And I always thought it was an interesting point to bring up that it was almost like — again it's almost like a double-edged sword. Not only are you malabsorbing, but you have higher needs. Not only are there clotting issues, but you might have to go to surgery. Like, there's these kind of double-edged pieces with it.

So how do we optimize growth and nutrition? Really what we're looking for is to improve your outcome, which means that you need calories, protein, fat, and especially the fat-soluble vitamins. The other ones are important iron, zinc. They're my friends. I love them too. But really for this talk it's like protein, calories, fat-soluble vitamins. It's not that they're not important. They feel left out.

Fortifying — I'm sure a lot of you recognize this beautiful can right here — see yeah. Basically, fortifying is when you're using additional powder to add calories, carbs, protein, fat, and micronutrients. A lot of times we're going to be adding Pregestimil powder or some of its friends, but mostly Pregestimil because of the fact that it's got a better protein broken down, and it's got more MCT oil than other pieces. We're going to be adding it to breast milk or formula or foods.I say that like hopefully like "and foods!" It's not that well accepted, but I can put it in yogurt. I have. I've got it done. So that's always an option if your kid's old enough to take yogurt. I've put it in baby food. I really don't have any kind of barriers when it comes to that kind of stuff.

When we supplement formula, that means that we're adding calories, but we're not adding everything else to it. This is something we're going to do in kids that are really, really tiny, newborns, things like that. Your kidneys can't handle just adding straight more micronutrients and more protein and all that. Our kidneys are real delicate. They can't handle that piece so sometimes we just want to add it with fat, in which case we'd use it with something like MCT oil.

There's a couple commercial MCT oil products that are out there. Some of them are reputable, some of them aren't. So always make sure that you check with your provider if you're not going to be using the standard beautiful brown bottle MCT oil. It's brown bottle for a reason. So, if you are using other products, remember that. It's brown bottle because it needs to be able to protect it from the light. And again you can add this to breast milk and formula and some food. It's not always the easiest thing but, when you realize what you're up against, you can make it work. You can come see one of us. There's three dietitians that work here. We have millions out in the satellites, so we can help you do it.

Nutrition support was something I was asked to talk about today and for a lot of times these kids — their needs extend their ability to meet them by mouth. And I meet with a lot parents with these conditions and with other conditions where sometimes a lot of what gets brought up is they feel like — parents feel like they're not doing enough. They feel like well, if I can just get my kid to eat enough and gain enough, then they're going to be fine and that kind of thing. And the "tube" as they call it, the tube is seen almost as a failure of sorts or it seems like — I can't get my kid to eat. It's not enough dah-dah-dah, and I think what I'm trying to tell you today is that it's absolutely not any of that. From our perspective we know that improving nutritional status improves outcomes, and we're all about here the outcomes for your kid. We want your kid to have the best shot at having that healthy, normal adaptive life. That's what it comes from. It's not supposed to be a punishment. It's not supposed to be something that's negatory. It's supposed to help you provide what we can for a kid who's malabsorbing and not getting enough. It's not a "normal situation." You're being asked to do a ton, and it's in a lot of ways something that could really assist that process and really change the course of events.

So basically in summary, we need adequate nutrition to support our growth, to support outcomes. Weight and height and skin folds should be all used together. It shouldn't just be a weight-based thing. Here I don't have to say that because they don't do that here. Fat-soluble vitamins are absolutely essential. They are important, not just for short-term issues, but absolutely for long term. Again you're growing a child for life. You're setting them up for the rest of it. Supplementing and fortifying are ways that we can optimize calories, but there are times that we might need to go to using a tube overnight so that they can get calories that time and then eat during the day or something like that.

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