Speaker A
Mhm. Welcome to those who have joined us were a few minutes before our starting time and we'll wait for everyone to join.
ESPGHAN webinar on biliary atresia for families: liver assessment, complications, transition to adult care, nutrition, physical activity, and research, with patient Q&A.
Key Takeaways
- Biliary atresia requires lifelong, multidisciplinary follow-up including hepatology, nutrition, and transition planning.
- Early detection of cholangitis, portal hypertension, and vitamin deficiencies is essential.
- Nutritional and physical activity support improve outcomes and quality of life.
- Families' perspectives and psychosocial needs should be central to care decisions.
- Research into causes and treatments such as steroids remains active, with evidence still limited.
What the video covers
- Opening remarks: first ESPGHAN patient-and-family education webinar on biliary atresia, aiming to share reliable knowledge and connect families internationally.
- Presenter Zohar Eitwise Rotman, pediatric hepatologist in Tel Aviv, chairs the ESPGHAN Biliary Atresia Special Interest Group.
- Dr. Emer Fitzpatrick (Dublin) explains liver anatomy, dual blood supply, bile duct function, and how liver health is monitored at clinic visits, including lab markers.
- Discussion of portal hypertension, its complications, and the importance of monitoring over childhood.
- Care after Kasai procedure: focus on cholangitis (bile duct infection), vitamin deficiencies that can precede symptoms, and general liver care.
- Transition from pediatric to adult medicine is highlighted as a difficult process for both physicians and families.
- Nutrition in chronic liver disease, including specialist-guided supplementation and MCT oil use, and the role of physical activity, where children after transplant showed lower exercise capacity than controls.
- Psychosocial aspects: children may feel different due to scars, jaundice, or medical devices; online information can conflict with medical advice.
- Research updates cover possible causes of biliary atresia, including a hypothesized viral trigger, and variability in steroid use across Europe with limited trial evidence.
- The session closes with a live Q&A and input from patient representatives, emphasizing listening to families' priorities.
Chapters
- 00:00Welcome and webinar setup
- 07:00Introduction and goals of the webinar
- 16:00Liver anatomy and monitoring with Dr. Fitzpatrick
- 30:00Complications, cholangitis, and vitamin care
- 45:00Transition from pediatric to adult care
- 60:00Nutrition and physical activity in chronic liver disease
- 75:00Research updates and open Q&A with patient representatives
Full Transcript — Download SRT & Markdown
Speaker A
Hello again and for all who have joined, um, we'll wait another 2 minutes before we start because I see there are still many people joining us. So, a few minutes, um, until we start and then, uh, I'll show you how also to turn on the various
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translations. Okay, we'll start in 1 minute. Okay so we're on and we're going to start our very, very exciting webinar for today.
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And I have to say that while we were waiting for those minutes and I saw in the chat people from all around, not just all around Europe, but also around the world. So, this is really touching.
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So, it's really a great start. Before we start and even before I introduce myself, I just want you to make sure that everyone is comfortable with the language. The webinar will be held in English. But, there are available captions in your language. So,
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all languages are available. And here you can see on the screen that I'm sharing how to um have your own language. So, you click on the more option on the Zoom.
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And then you can have the caption in your the language that you choose. So, hopefully everyone can be comfortable.
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I'll wait another minute for everyone to follow those steps. And then we'll start. Okay, so my name is Zohar Eitwise Rotman and I'm a pediatric hepatologist um practicing in Tel Aviv at Schneider Children's Medical Center. Um I'm we're
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all part of the ESPGHAN, the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and I'm also chairing the Biliary Atresia Special Interest Group uh and my colleagues and friends of the faculty are part of them are here uh with me today.
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So um we really thought among us that we need to do a webinar to include the patient and actually this is the first time the first event of its kind in ESPGHAN and I hope the first of many others uh to
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follow. And why do we want to do it? Why do we think it's very important? So we think it's our responsibility to share reliable knowledge with our patients. We see you often in clinic but we don't have the enough time to sit and share
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the knowledge that should be uh transitioned to you. So this is uh really uh important focus of today's uh webinar. Of course we will not cover everything but I'm hoping it's going to be a tradition and perhaps after getting
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your feedback from today we'll have it will be the first of others to follow.
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I also think we also think it is very important to establish relationship within families across different countries. This is a rare disease and as such perhaps we should be united and families and patients should be united from across the world to get better
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information and perhaps better resources for uh patients and families with BA biliary atresia. And really one of the more important goals of today webinar is to hear your voice. And this is why we're going to have a session at the end
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answering your questions and also including patient representatives who agreed to join us for the discussion.
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And our ultimate goal, and I think it's a mutual goal, to advance both clinical and also clinical practice and research in biliary atresia to ensure even better quality of life to all patients with biliary atresia around the globe.
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So the agenda for today and um uh which will be held uh from and we'll hear talks from my colleagues and friends from across Europe. So we'll start with understanding liver assessment in biliary atresia from Dr. Emer Fitzpatrick from Dublin
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and then we'll continue hearing about complications of biliary atresia from Dr. Eckart Sturm from Tubingen. Uh we'll hear about transition from pediatrics to adult medicine which is hard for us as physicians and also for families to do. We'll hear it from Dr. Muriel
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Girard from Paris. Um hear about nutrition in chronic liver disease from uh really an expert in the field Dr. Sara Mansell from London. We'll hear about the importance of physical activity in chronic liver disease from Dr. Tudor Pop from Cluj.
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Um and we're going to also try to have you hear about some recent uh science and research in the biliary atresia uh field from Dr. Van Jane from London. And then we'll have our last part um of answering your questions. So, during
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everyone's talk, you can put your question on the Q&A on the chat. And we will answer the questions at the end.
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And we'll also hear from patients representatives. So, I want to start with thank you because again, I'm I'm really touched from everyone's work and involvement and even before it started, I know it's going to be a success.
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So, I really want to thank first the ESPGHAN leadership who agreed to have [snorts] this you know different or a new concept for our society to have this patient's involvement. So, thank you for allowing me allowing us to do it. I want
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to thank the pathology committee which is a part of ESPGHAN who again is part of this project. All faculty who did a really strong work and hard work putting into this webinar. The patients representatives who agreed to join and
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to thank all of you for your really warming responses and being involved in the field.
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So, with that we'll start with Dr. Emer Fitzpatrick. So, Dr. Fitzpatrick is pediatric hepatologist from Ireland from Dublin.
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Dr. Fitzpatrick chaired the biliary atresia for the hepatology committee up until recently and is involved in lots of biliary atresia and other chronic liver diseases in pediatrics research and clinical care. So, thank you so much for joining us.
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Thank you so much, Orith. Hopefully, you can see my slides. Yes, we can. We just need you to go to to the presentation mode, Grace.
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So, it it is a tremendous privilege to be here. Thank you so much, Orith, for organizing this. I agree, this is a really exciting opportunity for us all.
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Um and and you know, today, I'm not going to talk to you about anything you don't know already. Um but we just wanted to start somewhere. And I think most of what I'm talking about today will be very familiar to everyone on
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this call. Um I'm going to start off with what the liver does and what liver disease means. And I guess that mean what it means to me. Um and every everyone has their own concept of what liver disease means. But, you know, I
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think it's it's important we kind of kind of shared language in this. Um I guess the majority of the presentation is how we check liver health and what we monitor at clinic visits. And and I guess arm us all in in terms of thinking
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from the other side. What what what should I be asking you guys and and what should you be asking me?
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Um so, I I you know, I love talking to other subspecialists about the liver because it is by far in the way the most sophisticated organ in the body. It does more than 500 things. Um and really, it
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is it is irreplaceable, but we have this wonderful um a concept of liver transplantation that we can replace the function.
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So, I'm just going to talk a little bit about the anatomy first of all. So, you know, like looking at a picture of the liver, we always talk about the right as the left and the left as the right as the
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right. I.E., the right side of the liver is is the right of the child or the patient. And the left side is is the left. And the liver is supplied by a dual blood supply, which is fairly unique. So, it's
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supplied by the portal vein, which takes all the blood from the gut and processes into energy. And it take it's supplied by the hepatic artery. And the importance of that is the hepatic artery takes the oxygenated blood particularly
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to the bile duct cells. And we talk about that in a minute. Um so, you know the gallbladder normally sits under the liver here. And the bile ducts all drain into the gallbladder and then into the gut. Biliary atresia, as you know, this
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is slightly different. Just to mention, I mentioned left and right liver and that's important when you when you get talking about liver transplant, but the left left lateral segment is obvious is something that, you know, we'd often transplant into a little child. And this
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is the right lobe. So, you can split that liver. And this is one of the other most amazing things about the liver.
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The liver at a more microscopic level is organized into little lobules. So, you remember I was talking about these vessels, the portal vein, the hepatic artery, and the bile duct. And they all sit in this portal triad. And blood
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comes in here and across [snorts] the lobule and goes out into the hepatic vein. And the importance of this is you get all of that amazing blood from the gut filtered um through the liver so it can do something with it.
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And in my mind, the liver is really the engine of the body. Um it does, as I said, so many things including removing waste. Um it it it can take uh um waste products, put them into bile, and then excrete them out. Uh it
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makes and transports bile. Um and it it does this through the hepatocyte initially and then through the bile duct cell. It also most importantly makes energy. So, it takes all that good stuff from the gut um and it processes it into
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energy. And it also makes proteins. And this is part of the way you can monitor liver function.
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It processes fat. Um and it removes uh uh other type of waste products. It also makes stores and uses sugar. And this is only a fraction of the things that it actually does.
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So when we check liver health, we're looking at all of that stuff. You know, what is the liver doing? What is it not doing? How do we need to approach that?
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And the first thing we always do is we take a history and examine the child.
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And then we would often do blood tests. So I think there's very few children unfortunately that escape blood tests in one of our clinic visits. But I'm going to try and explain why they're so important to us. And I'm going to talk a
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little bit about scans too and the various type of scans that we employ. So symptom checker, so you'll be really familiar with all of these questions.
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You know, we really focus on growth, growth, growth. That's why, you know, Sarah will talk to us about how nutrition is kind of the be all, end all in terms of really what we can do to change the trajectory of disease.
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Tiredness or or or just not having that good quality of life, particularly in older children affecting school or daily life, being more tired than other children for example. And bleeding is an important one that we'd always ask about, bleeding
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or bruising and that's for a number of different reasons that we'll we'll talk through during this webinar. Most importantly, bleeding from the gut, okay? So blood in vomit or black poos.
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Fluid in the tummy is you know, it is something that's generally fairly obvious, but we always ask about a bit of a swollen tummy. And and children with liver disease often will have more frequent infections because one of those important functions
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of the liver is in the immune system. And then you all know yellow skin or eyes or jaundice or itching, really important symptoms to for us to look for.
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And when we're examining children, again it's growth, growth, growth. We use these centile charts so we can plot where a child should be for their age and developmental stage versus where they are.
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And we examine the baby or the child for a big liver or a big spleen. And and then there are more subtle signs like clubbing of the of the finger fingertips or of the little spider nevi. Now, up to
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five spider nevi are completely normal in children, but you know, you you get a sense when you when you look at a child's skin whether or not there's manifestations of liver disease.
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This um just demonstrates itch. So, you know, itch can be a very significant symptom and we always need to look for it. And the ascites here um not always obvious, but but sometimes children can can present with swollen tummies.
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So, moving on to blood tests. So, bilirubin, I don't have to talk to this audience about. You're all hyper aware of bilirubin. And I'm not I'm talking more in the post Kasai setting um where you've got an eye on the bilirubin, both
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the total bilirubin. So, as you know, bile or bilirubin is a breakdown product of red cells. It goes into the liver. It gets stuck onto a sugar, which allows the liver get rid of it through the biliary tract and through the gut.
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Um if there's hold up in that, uh there is a build-up of indirect or conjugated bilirubin. Um or if the liver is quite irritated and inflamed and and scarred, there can also be a build-up in that.
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I'm going to talk next about gamma GT. So, you know, in the liver there's you you've got your liver cells and you've got your bile duct cells. So, they can be assessed using different uh blood tests. So, gamma GT and alkaline
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phosphatase are blood tests or enzymes that come from the bile duct cells. So, if your bile ducts are irritated, if your bile duct cells are releasing gamma GT, that tells us, you know, something about the bile ducts rather than the
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liver cells. Um I don't look particularly at alkaline phosphatase or alk phos because that is also a reflection of growth. So, alkaline phosphatase not only comes from the liver cells or from the bile duct cells, but also from the bone cells. So,
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alkaline phosphatase in a child could just be my um uh measuring growth. AST and ALT are enzymes that come from the liver cells themselves, but honestly, uh particularly um uh in children with biliary atresia, they're not hugely relevant once you
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know, you know, what's going on in terms of a diagnosis. AST and ALT can be very helpful uh to to monitor liver health after a transplant, for example.
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The real liver function tests, however, uh are more in those proteins that the liver is making. So, albumin and uh and and clotting or INR international uh normalized ratio are how we measure actually what the liver is doing at that
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point in time. Ammonia and lactate are also blood tests that you'll have heard about, uh and again, they monitor how effective the liver is at detoxifying stuff.
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Bile acids are a relatively new kid on the block over the last few years, um and we measure them to try and and and and predict how children will do long-term after Kasai procedure.
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And then finally, I just want to mention white cells and platelets, um and you know that in portal hypertension, which I heart's going to talk about later, um you can get a big spleen. The spleen is a recycling bin for cells. Uh white
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blood cells and platelets in your full blood count or, you know, those those bloods uh blood cells in your blood are recycled quicker if your spleen is bigger, and that's called hypersplenism.
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So, we look very carefully at uh white cells and platelets in the context of chronic liver disease to see what, you know, how are you in terms of portal hypertension.
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And that brings me to ultrasound. So, again, this is is such an important part of liver assessment. Um and you know, this very grainy picture, like, what can that tell? Well, it tells us is huge amount. The first thing is, if we look
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at the liver surface, you know, what does that look like? And and you you do have to be someone who's doing an ultrasound and pretty much every day of your life to to get the nuance here.
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But, you know, our ultrasonographers and our radiologists are really experienced in telling us, well, this parenchyma, this liver tissue looks brighter than it should. Maybe there's some inflammation going on. They look for evenness and particularly any specific lumps and
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in the the parenchyma, the tissue. They also look at the liver surface, okay? So, is it smooth or is it kind of nodular? Is it lumpy? And and the size of the liver.
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In in the context of biliary atresia and indeed post-transplant, they look at the bile ducts inside the liver. So, often in biliary atresia you will get lakes or irregularities of the bile ducts and we like to know that because,
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you know, that that can sometimes go with a cholangitis. And and then finally, as I said, we look at the spleen and the blood vessel flow into the liver. The spleen size, too, of us is really important. The spleen is a
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gland, so with colds and coughs it can get bigger. But, if you've got a a progressively enlarging spleen, we need to think about potential portal hypertension.
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The other thing to mention here is and and many of you will be aware of this, the FibroScan is something that's kind of common to practice over the last 15 years and it's an ultrasound-based technique to measure how stiff the liver
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is. And so, it helps us detect scarring of the liver alongside other tests, of course. It takes about 5 minutes in the clinic.
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For toddlers who are quite wiggly, it's not the easiest test, but it's harmless and certainly in the older children and who are suspicious of of of portal hypertension, it can be useful.
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I'm going to mention everybody actually. After diagnosis, it is pretty rare for any of us to ever ask for a liver biopsy in a child with biliary atresia before the point of transplant. But just I just wanted to explain the concept and
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and what we see. What we're really looking for and in terms of liver disease is the onset of scarring, okay? So and you know, the liver biopsy tells us loads of things and you can have a very what we call
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biliary picture. You can have a lot of inflammation so loads of of blue dots.
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And but in any liver disease really what we care about most and this is in the follow up of children with any liver disease is scarring. Now nowadays we can talk about scarring or fibrosis and using more non-invasive measures and but
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just to get the concept, you know, there's a load of different ways that you can develop scarring. But scarring for you know, for pretty much 90% of liver disease is kind of the final common pathway and that can lead to cirrhosis or advanced
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scarring. But you know, you can be absolutely symptom and sign free with quite advanced disease and it only really matters then if you decompensate.
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So I'm going to talk a little bit about that because that's an important concept when we're talking about the clinic visit.
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And during these few talks you're going to learn an awful lot more about what we're focusing on in the clinic.
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Again, the one thing that we really can have a good chance of changing is nutritional status. So Sarah's going to talk all about that and how growth and development are really key to everything we do in pediatrics.
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And then Eckert's going to talk about portal hypertension. But just to point out, you know, I'm talking about a stiff liver here and then the back pressure leading to this recycling bin or this spleen getting bigger and then the
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consequences of that are which are recycling your cells too fast and then these little varicose veins that can develop mainly because of back pressure.
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So I mentioned the word compensation and decompensation. So, this is important because no matter how scarred your liver is, is the liver still working? It's still working, okay? Um and it's carrying out its main jobs and you would
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be no the none the wiser. It's when things become decompensated and decompensated is defined by a few different things like the gut bleeding that you you will have heard of like new onset jaundice. I'm not talking about jaundice because I am talking about new
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onset onset jaundice. Fluid in the tummy or ascites um if it's from uh liver problems uh or confusion on or unusual sleepiness or encephalopathy. So, these complications, if they develop, you know, they kind of take things to a
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different level from a clinic perspective. So, portal hypertension and its complications can be a big concern and are a big concern for us over um the span of of time that we uh look after children. And when complications and in
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particular when decompensation occurs, then we start to look at liver replacement. And this isn't a talk that I'm going to talk about transplant and and the indications for because there's a long list of them, but essentially, that's what we're looking for in clinic.
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We're looking for well, how well are you Are you managing to get to school every day? Are you having fun? Are you growing well? Are you having a normal life and have you no problems that would indicate decompensations because that's really
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what we care about most. I'm going to mention liver nodules very quickly if I have time. So, we look at these long-term post um in in children with any chronic liver disease, not just biliary atresia. A growing nodule is always a bit of
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concern for us. You'll get a lumpy bumpy liver in most cases with biliary atresia, but as long as it's compensated, it's not a big deal in general. But if there's one growing lump, we want to take a closer look,
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okay? And we monitor a blood test called alpha-fetoprotein. And regular checks certainly in in my practice and and in Kings, I mean we I think this is kind of universal. It's not an exact thing, but at every visit
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we'd always measure a child. We'd usually do liver blood tests and an examination and we'd listen to your concerns.
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In terms of ultrasound, it's not an absolute, but we tend to do one reasonably soon after diagnosis in Kasai and then every one to two years perhaps and a fiber scan maybe every one to two years if it's available. When you
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develop advanced liver disease and we tell that usually from non-invasive measures or portal hypertension, we tend to do ultrasound and examination every 6 to 12 months.
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So that's in summary, just you know, a look at what we review at clinic. So growth and development are always number one in pediatrics. Sarah's going to talk about this further, but nutrition, measuring fat-soluble vitamins and you know, providing support with itching and
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this is all to do with cholestasis or not just jaundice, but those bile acids being a bit too high.
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We look for cholangitis, which you will all be familiar with or signs of bile duct infection.
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And we always check for portal hypertension, signs and symptoms and blood tests like low platelets that can give us a clue. And we also also look for any liver lumps.
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And then of course, we need to cover things like vaccinations, healthy habits and support for your family being fundamental to everything we do.
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Thank you. Thank you Eimear for this fantastic talk and we'll get back to you at the end some questions from the audience. That was great.
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So now it is my pleasure to introduce my friend and colleague Dr. Eckart Sturmbe head of pediatric gastroenterology at Tubingen.
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Uh Dr. Storm is uh real expert uh in uh pediatric liver diseases and biliary atresia. And uh Eckard will be discussing uh complications of uh the disease. So, please, Eckard.
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Thank you, um Hold on. I just have to reorganize my screens. There you go. Thank you, Reet and uh the ESPEN team for organizing uh this event. I think it's really a fantastic opportunity to exchange ideas and thoughts
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uh with um an important group patient and families representatives. I think uh we should really build on this and continue uh this format. And I'm very excited to contribute.
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Um my focus today will be to talk about complications of biliary atresia. Uh complications in children that live with their own liver after Kasai surgery.
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And I'm glad because uh I have too many slides and I can cut them down because we already heard a great talk from Emer on liver function. We will focus on a little bit uh on some details of what
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the liver does, but Emer summarized it in a very elegant way. And you will hear more on other issues that I will touch upon like nutrition, physical activity. So, it's great to hear others also on the topics that are
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part of my talk. Um let's see. Um we have heard from Emer the important functions of the liver. Uh just very briefly, we have heard that the liver, of course, makes bile. This has an important uh function in the gut because
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bile helps us to absorb fat and vitamins that are very important. The liver is a great manager of energy in our body.
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The liver makes blood proteins, which are so important for blood clotting and fluid balance. And of course, it handles medicine and waste. And as Emo pointed out, it's very important also for the defense for infections and is a is a very immune
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active organ. The functional issue that really I want to dive a little bit deeper into is the recycling loop of bile acids in the body. We call it enterohepatic circulation. So, it's the gut liver recycling loop.
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And this picture, just keep that in mind, shows normal anatomy. It's not the anatomy after Kasai surgery.
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The bile acids that I'll focus on are one ingredient of bile. And if you follow the moving dots, you can see that bile acids travel through the liver, through the bile ducts into the small intestine. And there, they have their
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actions. For example, important actions in absorbing fat from the gut. Most bile acids are importantly are taken up in the terminal ileum, as you can see here.
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And blood in the portal vein carries them back to the liver for reuse. So, only a very small amount of these bile acids is really lost in the stool.
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And this is the enterohepatic circulation. You have to keep in mind though in biliary atresia, poor bile drainage means that bile acids may not reach the gut.
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That bile acids are not active in fat in fat absorption and vitamins. And you have to keep in mind if bile acids cannot be transported into the gut, they are retained in the liver.
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And this may contribute to significant inflammation and scarring. So, what kind of problems can happen after Kasai? Uh, Kasai the Kasai procedure helps the bile uh to drain.
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Uh, uh but uh the liver still needs care after this procedure. Uh, and uh we'll focus on uh three main topics. We'll focus on the issue of infection of the bile ducts uh that we call cholangitis.
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Uh, we will focus on uh the problem that might occur that too little bile is reaching the gut. Uh, having an effect on growth and vitamins as I've pointed out and we will hear later more on that. Uh, we will um
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we have to keep in mind that and I pointed that out already, if bile acids are retained in the liver, this can lead to significant inflammation, scarring to um a state that we call fibrosis and in the end stage we call it cirrhosis.
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And this these effects on liver scarring may affect blood flow and um talked about this already leading to the state of portal hypertension. So, it's clear even when after Kasai jaundice clears, we have to follow up uh the
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patients and we have to check for problems that may occur in the course after Kasai.
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If we talk about cholangitis um um cholangitis means infection and inflammation uh in the gut. Bacteria may move up uh I'm sorry.
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Cholangitis means infection and inflammation in the bile ducts and bacteria may move up from the bowel through the drainage uh route and reach the liver and they have an effect on uh the bile ducts and lead to inflammation
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and infection. And one of the hallmark signs is uh if the patients develop fever of 38° or more, I think this is a point where you should really uh contact the liver team promptly and, uh, discuss whether further, uh, procedures are necessary or
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whether you should present, uh, to the liver center. Uh, so, 38° is a is a practical, uh, threshold. Uh, it may be different from center to center, but most centers will agree that 38° C is, uh, the level of temperature where
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you should, uh, check, uh, whether the child needs to be presented, uh, to uh, to a clinic.
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Uh, you should have a written plan, uh, available what to do in that case.
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Uh, you might also notice other changes like, uh, more yellow skin or eyes. You might see changes in urine or the stool color.
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Uh, the child may be may have difficulties feeding or may may be unusually tired or irritable. Vomiting may occur and also pain in the abdomen in the tummy may be a symptom that you, um, may observe.
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So, please do not If you observe the fever, please do not wait for every one of these signs. Uh, you might the child might need IV antibiotics.
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Uh, so, it's important to contact, uh, the liver team and, um, arrange um um, a checkup of your child.
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And just be aware if you see that the child is hard, uh, to wake or has severe breathing difficulties, you should really, uh, consider this is this an emergency and you should use the emergency services that are available
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for you. So, how can you reduce the risk of cholangitis and what's what's a reasonable way to act early? You should keep a written plan. You should know who to call. You should know where to go and what to bring in that case.
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Um, you should follow the prescribed care. Uh you should be aware that of course in some centers we use preventive antibiotics.
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You have to keep in mind that the liver team will tailor this plan individually.
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Uh so if you have a plan available, you should follow this plan for taking medicines and you should not change the antibiotics without um rediscussing with the team of experts in your center.
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If you see and observe on new symptoms, new fever, get advice uh even while taking preventive antibiotics and not change uh antibiotics by yourself.
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Let's uh focus on growth now. You heard uh from Emer that this is a very um it's it's in the focus of basically every pediatrician. And as a pediatric hepatologist, we all like to really take a close look on uh on growth and try to
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observe whether uh slow growth is a medical problem. Uh for sure it is not uh in any cases a parenting failure.
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So, what should we do to find out whether this is really a medical issue?
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We should watch the trend. We should watch uh weight weight and height change over time.
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And we should be aware if uh feeding difficulties occur. And we should be aware of that early in the in the time course.
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We will hear more from Sarah on what a dietitian can do to tailor food and feeds. So, I'm not going to dive into that deeply.
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Uh as a liver team, we should be um available early to add support. And in some patients actually, if feeding is insufficient, um if uh the intake of feeding is a problem, and even fluids are not be taken uh cannot be taken sufficiently.
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Tube feeding is in is a is a way of feeding that can really help.
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So, importantly, I think we have to say that changing the feeding is a very individual is a very individual approach. So, not every child will need extra calories.
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The majority probably will need extra calories, but it is important to individualize. And it is important that you always stay in contact with your dietitian and discuss the right composition of the feeding and whether you should change things or optimize things in order to
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achieve the goal of sufficient growth, which is so important for the outcome of the patients that we care for after the Kasai procedure.
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In the context of feeding, we have to focus on couple of vitamins that really need special attention.
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It is important to note that you can change you should be aware of low vitamin levels. They can be present before symptoms appear.
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And we talking about the vitamins A, D, E, and K. They have different functions.
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They are important for vision, for bone structure, for nerves and muscles, and for blood clotting.
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It is important that many of the patients after the Kasai procedure will have a prescriptions for supplements, but equally important is that we check regularly whether these supplements are sufficient and that we adjust the dose if needed.
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There many multivitamin preparations around, but the ordinary everyday multivitamin preparation may not be enough. So, sometimes it really needs specialized preparations prepared for the needs of a child with liver disease.
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You should be aware of that and you should be aware that you should not add extra doses or change the dose yourself.
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I might just introduce you to the issue of portal hypertension. This is a very important This is a very important pathophysiological problem in children after the Kasai procedure.
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It describes the problem that pressure builds up in the vessels coming from the gut to the liver.
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The so-called portal vein. And the problem is that this that the volume of blood coming from the gut will not easily go through a liver that is severely scarred or as we say fibrotic.
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And as a consequence in patients with this problem, we see specific changes. We We see that the spleen may grow.
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And in consequence that the platelets may fall. I might just pointed out that importantly the vessel structure in in the food pipe or the stomach may change. The veins may enlarge in these organs and we may see a problem
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the varices development. And we'll talk about that in the next slide. And another problem may be that we observe fluid collection in the tummy. Just be aware when we talk about blood pressure in the context of portal hypertension, it's not the blood
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pressure we normally take on the arm. It's a specific problem caused by the scarred liver leading to a pressure build-up in the vessels leading to the liver.
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What kind of clues do we have to study this? I think as we said already spleen size is an important symptom and the platelets the platelet count may drop and this help us helps us to assess the risk.
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We have specific scans available. Ultrasound of course is helpful and we have different liver stiffness scans that might help us to to to quantify the degree of liver scarring actually and E were already pointed you to the FibroScan and there are other
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techniques around that may deliver this information. Importantly, the only way to directly study varices and the change of vessels in the food pipe and stomach the only way we have available to directly study those structures is endoscopy.
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And this is a camera test under anesthesia to really look at the varices and you see a bleeding varix on the right.
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You see it's bleeding here from a from a lesion in this vessel and this is a risky situation.
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And what can you do to to address and to treat these varices, high risk varices that may be may be seen with with red whales, red spots.
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And the size of course is an indicator of the risk of bleeding. They may be treated with bands, with rubber bands as you can see on the right.
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And as a newer approach, we are also using medication in order to treat and to decrease pressure in these vulnerable structures in the food pipe and the stomach.
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So there's no fixed time point when you should use endoscopy and the liver team many times will have will choose an individual approach in each patient to recommend endoscopy and if needed endoscopic therapy in order to address the problem of varices.
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Now if a bleeding occurs from these varices, you have to be aware that this is an emergency situation. You might see blood in the vomit, you might see red or or like coffee grounds in in vomit or you might observe black
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and sticky tar poo. It is important that you do not wait for assistance. You should call your local emergency number directly. You should explain briefly what the background of the disease of your child is and that the child may be
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bleeding from enlarged veins. So, please do not wait for an appointment or routine callback. Treat this as an emergency and get in touch quickly with the next emergency pediatric emergency department.
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Looking at a longer perspective as as a lifelong follow-up for the for the child as a whole, even if the child does clear jaundice after the Kasai operation, I try to focus for you on a couple of issues, the issue of growth
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and nutrition, and you will hear more about this the changes of the liver morphology, the structure of the liver, scarring of the liver, and blood flow through the liver.
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So, we have to be aware when tests like endoscopy need to be performed, but most importantly, we should also really focus on everyday well-being. Issues that might influence that include itch, sleep energy school physical activity, and we will hear more on that,
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and of course, emotional health. We are focusing so much on the best way to help the child grow up with biliary atresia, and this includes plan on medicine on vaccines that we recommend, and and we will hear more on that,
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transition to adult care. How often should you be seen in an in an outpatient clinic? I think this is also a individualized approach, and your team will help you to find the right right schedule for your child.
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I think my time is up, so briefly to summarize, Uh, should be aware when extra support is needed. So, um, when should you ask yourself, "Do we need to review the plan that we have?" And one indicator might be that your
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child has repeated infections, if growth failure occurs, if fluid collects in the tummy, or if worsening liver function is observed and measured in blood tests.
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And this is the time to adjust treatment, to adjust maybe nutrition, to adjust the medication, but also to in some cases discuss whether transplant assessment, uh, should be performed at that time point.
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You should be aware that transplant assessment means planning. It does not mean that there's an immediate transplant to be, uh, performed, but it is very important, I think, to discuss the perspective of transplantation in a situation where there's no urgency,
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because you need to adapt yourself to the perspective, to the problems, but also, um, to the vision of living with, uh, with a transplantation, and to the benefits that you can, uh, receive from that.
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So, this is the last slide. Be aware, uh, when to call emergency services. Be aware that bleeding can be a problem. Be aware that if you see, if you observe, uh, that your child is hard to awake or
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has severe breathing difficulty, this really is an emergency. And you you should contact, um, medical help um um, very early.
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You should call the liver team if you observe fever of 38° or above, if you observe changes in school in stool pigment, uh, after the Kasai operation, more jaundice, or swelling of the tummy, which might indicate fluid collections.
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And then, of course, for the everyday, uh, observations, follow the, um, recommendations for feeding, the vitamin and the medicine plan, and keep the appointments even when your child seems well.
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Just be aware that you have a list of 24-hour contacts available and a care summary if you are seen by somebody not familiar with your child.
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You should share that with with air with every caregiver that that is there to help you.
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I think that's it and I'm looking forward to the talks of my colleagues and thank you again for uh the opportunity to present. Thank you Orith.
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Thank you, Ekkehard. This was a fantastic, a very comprehensive and interesting talk. Thank you so much.
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So, next I will It's my pleasure to introduce Dr. Muriel Girard, the pediatric hepatology from Paris, France, an expert on biliary atresia.
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Muriel will talk today about transition from pediatric care to adult care. And I'm sorry, I'm I'm supposed to share the slides, so we'll do it in a second.
Speaker A
Thank you. And thanks Orith for the invitation and it's a very nice opportunity to share with patients and family, so uh thanks a lot for this opportunity.
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Thank you and of course the slides, so Okay, I'm not talking. Okay, it's good.
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[laughter] It's so weird. Sorry for the delay and Okay, yes, please. So, hello. Today I will ask if you perhaps at the end of the talk and the talk of everyone, we can share our questions but it was important for us to talk about
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transition in liver disease and in particular in BA patients. Next slide, please. So um uh the transition is uh how to pass to adult care.
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And um it's important because growing up with liver disease, uh it's uh something that it's very painful, and it's important to prepare uh the the the passage for adult hepatology uh units.
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So um today we will see what is exactly the transition, why it is important to talk about transition, um is it uh a need of specific preparation for that, uh how the transition need to be organized, and when we need to organize
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transition. Next slide. So, few definitions. The transition is a gradual process uh that leads to the child and adult young adults to go to the uh adult hepatology.
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And the transfer is the move to adult care unit. So, you move to the pediatric hospital that you are uh with follow-up from uh uh very often uh very uh early in your life, and to the another hospital with
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another doctor and another way of doing things. So, it can be uh very uh stressful.
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So, next slide. So what is exactly the transition? Is indeed preparing the child for the adult life, but also to prepare, we need to help the child to prepare him to his adult life because at adulthood he will manage alone his
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condition independently and it's not so easy. So, what does it mean? It means that he the the child need to have knowledge about the disease.
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But not only the disease, also knowledge about history of the disease. This is very important and we can discuss after.
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The child need to know the treatment. What medication is for, when need to be taken.
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The child need to be autonomous to take the medications. The child need to be autonomous to book medical appointments and also for several administrative task that can be a very important and heavy day-to-day.
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Next slide. What is important? Oh, so sorry. Why is it important to talk about transition? It is important because it is difficult difficult to acquire all these skills that we already seen together. And this take time and it is important to
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take this time and it is necessary to anticipate it. So, every medical consultation that we do should be an opportunity to talk about transition and how it will be organized when it will be organized. Next slide.
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And is it a specific preparation we need? Yes, because in the case of biliary atresia, as you know, this is a very early disease in life, and there is a relationship built since infancy.
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And the pediatric team and pediatrician knows very well the child and knows very well the family and the family history.
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Most often, the people knows sister and brother, how is the child into um the family, knows the parents, knows grandparents, and sometime pets, and the school, and so on. So, this is not a child, but it is a life, and a life in a family.
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And so, that's why it is emotionally very difficult to leave the pediatrician to go to adult practitioners.
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Uh, next slide, please. So, uh, the pediatrician hold the complete medical history since birth, and one of the question is will the adult hepatologist be as knowledgeable as a pediatrician for the disease and for the child? The pediatrician also looks after the liver,
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but also everything else, vaccination, but heart problem, brain problem, digestive problem, renal problem, and one of the question is how how will the global care will be managed at adulthood in a different hospital with different team and different
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doctors? Next slide. And how to organize the transition? The goal is really to reduce anxiety because as you've seen it is very there is a big anxiety at the time of the transition. So, for that we need to
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identify adult physician very early. Also, paramedical coordination should be identified early. Uh we need to identify psychological and social needs for the child and the family that will need to be um said or transmitted to the adult uh
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unit. Um also to involve the young patient uh in preparing the documents and the clarity role of the parents uh and the parents with him uh at the adult consultation.
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And um prepare the family uh and both team, pediatric and adult team, to share a care plan.
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And also uh very often question that is asked is is it a need for join or alternating consultation?
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Uh it depends. It depends of the patients. It depends of the family and also the pediatric and adult um unit um because the habits are not always the same uh around the world and uh um depending the town and organization of
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several teams. Uh next slide. And the last uh thing is when to organize the transition.
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The The timing depends of the readiness. Readiness of the patient, of course, the health of the patients and the receiving service, of course. So, to choose to begin the transition, usually it need to be gradual, gradually introduced, and perhaps around the age
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of 12, 14, we can discuss it. And we need each consultation to explain the illness directly to the child and the young person. Perhaps we can propose also to have a part of the consultation alone with the young without the parents.
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Think each consultation to talk about the future, the future for the disease, of course, but the future of the the the young for school, for example, if you want to what you want to do in the future, how
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we see the future, to identify adult team early enough. So, we can talk about all those things together during the transition period. And then, how to choose the timing of the transfer?
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Most of the time it take place around age of 18, but it depends from countries and peoples, probably.
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It's important to choose a period of stability for the disease. The full independence for the child is not it's the best, but it's not a prerequisite for everyone, and we can adapt each situation.
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The transfer should not be presented as a sanction. It's important to to see that it is a positive thing to grow up.
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Um it is also important to maintain a pediatric and adult coordination during this period and for the initial phase.
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And um also uh the support uh continues after the first adult appointments. So uh it means that after the first uh meeting with the adult uh team we need to have um contact with the young to um be sure
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that uh all is okay uh on on his side. And I think it's my last slide. Yes. And of course we can discuss at the end of the the meeting.
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Muriel, thank you for this uh fantastic uh talk and I'm sure we will discuss uh further at the end.
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Um and now it's my pleasure to introduce uh Dr. Sarah uh Mancha. Uh Sarah is a doctor because she completed her PhD in nutrition and she's really the world leader um on nutrition for patients uh for pediatric patients with liver disease in
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general and uh of course biliary atresia. So we're very happy to have you Sarah.
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Thank you so so much for the for the kind words. It's [clears throat] really fantastic. Can you hear me okay? All right.
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Oh. You're breaking down. I don't know if it's just me. No, not is it okay?
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Okay, yeah. Now it's good. Okay. Sorry about that. Um yeah, fantastic. So we're going to talk about the nutritional management of children with biliary atresia. I've completely focused on biliary atresia for this talk. And I think the The most important
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first question is why is nutrition important in biliary atresia? And we talked about it with Eckard before that growth is one of the key indicators for health for children, but why in biliary atresia? And of course we we know that
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we need that better nutrition leads to better overall health, that nutrition is associated with brain development, with quality of life, but we also have some evidence that with better nutrition we get better outcomes. And whether that be time in hospital either before or after
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transplant, we have a small amount of evidence that maybe improved nutrition could reduce or or cause cholangitis to to to be present a little bit later. And we definitely have evidence for our transplanted children that better nutrition leads to better graft health.
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So [clears throat] pardon me, thinking about malnutrition in biliary atresia then, I think it helps to think in these four areas, which I'm sure you're really familiar with.
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So of course our children with biliary atresia may be just eating less. Maybe they have vomiting, reduced appetite, or perhaps because of that extra fluid in the tummy or that large liver, they're pressing on on the gut and and affecting
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appetite. It doesn't feel great. And then the liver as as liver disease advances, we we heard already how important the liver is for metabolizing, so breaking down all our nutrients and producing energy. It's also a major place that we store energy. That's the
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reason that you can usually last overnight with enough energy that you're not breaking down your body's stores.
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So it we have this a reduced ability to make protein to help with growth and repair. And then, children start using their fat stores and their muscle stores for energy because of these changes that are happening in the liver. There's also
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some evidence that maybe children with biliary atresia, on top of all of this, need extra calories, need extra energy.
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This could be because of infections or ascites affecting breathing. And maybe it's just because of this the liver being a little less good at using nutrients. And so, it needs a bit more energy for that.
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And then, we heard already about fat malabsorption, but there can also be some protein malabsorption, especially around portal hypertension.
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So, is there anything we can do to help reduce malnutrition? Unfortunately, the answer is yes.
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And I think this really, as liver disease advances, we know that the liver is not as good as storing the energy. So, I know it's very easy to say, but more difficult to manage, which is to try and avoid
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fasting wherever possible. And with investigations, this is kind of inevitable. But, we the time overnight, that's why it's called breakfast, because we're breaking the fast overnight. And we have some evidence that even just a carbohydrate-rich snack before bedtime with a something
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called a low glycemic index food, so something that releases sugar nice and slowly overnight, so that we have that extra energy available when the liver is a bit less good at at doing that. And I've shown examples here of
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high-carbohydrate foods, which would be a great kind of snack to have afterwards or snacks during the day and meals.
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So, why do do do children with biliary atresia have difficulty absorbing fat? And we know, we talked about bile flow being a problem. But, when that fat reaches the gut, so here we've got a massive globule of fat in usual
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absorption and then the little yellow with the green dots, those are bile salts. And we know that each individual particle of fat needs to be surrounded by these bile salts or bile acids in order for that fat to cross into the
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intestine and be taken to the liver for nutrients. And in biliary atresia, the fat globules reach the intestines but then there are not enough bile salts or no bile salts there and the fat then is excreted into the
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poo. So we get a loss of energy which is really important from fat, we get a loss of vitamins and essential fatty acids.
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So you'll be asking and I'm sure you're you're aware already, is there an alternative fat that we can give?
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Unfortunately, there is. So medium chain triglycerides or known more commonly as MCT. The main sources of these are actually from coconut and palm kernel oil. So if you look at your special liver formulas, you usually you'll find those on the ingredient list.
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There's also a very small amount in breast milk and in in dairy. And the real advantage of MCT is that it can be absorbed without bile being present in the gut. Which has a huge advantage in biliary atresia.
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And this is a busy slide but what I wanted to show you here on on one side we see long chain triglycerides, that's just another name for regular fat and those are the bile acids again. So those are needed so that that fat can enter,
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this is the gut here, the squiggly bit, that can enter the gut and travel through the lymphatic system to the liver. But on the right we have medium chain triglycerides, they don't need bile acids and they can just go straight
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through and be an energy source. And a question I'm really interested in for children with biliary atresia and and this [clears throat] was the subject of my research was how much MCT should we give?
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Um so I looked at all the available evidence um and reviewed the medical notes of 200 of our children with biliary atresia and recently carried out a small trial on 30 infants with biliary atresia and compared them having low or
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higher amounts of uh MCT. And what did I find from all this research? Well, actually there's very little research that's been done on MCT even though it's used around the world.
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Most of the evidence is from over 30 years ago and we still don't know exactly how much MCT we should be giving. What was interesting though is that when we gave low MCT versus high MCT to the infants, all they did, if
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they were having low MCT, they just drank more formula milk. And maybe this is because they were absorbing slightly less energy and they had to compensate um compared to the high MCT. It was a very small study so we need to replicate
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it. So um what are the recommendations then based on what we know so far?
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We would recommend providing uh a formula milk with 30 to 75% of the fat is MCT so you'll find that that's most of the MCT products out there. It's important that we don't give 100% MCT because regular fat or LCT provides
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really important things like essential fatty acids that you can't make in the body and uh they're important for fat-soluble vitamins. Um and for young infants, we would always uh recommend breastfeeding alongside MCT formula milk if possible.
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So are additional vitamins and minerals needed in the diet? And we already heard how important fat-soluble vitamins are so A, D, E, and K. We give high-dose supplements and and supplements in a in a special form so that they can be
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absorbed even when there are no bile acids. Um but water-soluble vitamins and minerals we wouldn't give um, routinely for our children with biliary atresia, only when we see evidence of deficiency.
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And then later on, we do see problems with immunosuppressant medication, for example, um, affecting potassium, magnesium. Those might be instances where we're watching uh, and measuring and making sure we replace any deficiencies, for example, with um, bleeding. Um, they might need iron
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monitoring. So, how should growth be monitored? Um, as the most important indicator of health, I'm sure you're all familiar with the different ways. Weight first, um, on baby scales, and then standing length, and then height. Um, but one of
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the key measures that we use, um, and I'm sure you're familiar with, is mid-upper arm circumference. And that's a really good measure because it's not affected by a large liver or fluid in the tummy, both of which could have have impact on weight, of
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course. In our older children, we would sometimes do triceps skinfold thickness, um, but and that would give us an idea of muscle mass, so that's really handy, but it can be a little bit painful, so we we wouldn't do it as often.
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And then, how how do we increase energy uh, and protein intake in those who need it? And as as Eckard said earlier, it's very important that we're identifying who actually needs that extra energy and protein intake.
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So, in our infants, as dietitians, the first thing we'd be looking at for our babies is, could if we change something about the pattern of their feeding, would it be possible for them to just drink a little bit more? And usually the
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answer is no, they're having exactly what they want to have. Um, and so our next step might be seeing if we could maybe make the formula higher in calories. If it's powder formula, we would add slightly more powder per
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volume. It's very important that this is only be done by a specialist, not just sort of adding in some extra powder, cuz that can have serious consequences. Um and then as a next step, we might be adding MCT oil um or an emulsion to the
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diet. And here's just a little example of how the calories might change as we do each of these steps. A standard MCT formula milk might have 86 calories per 100 mil. And then by going up a step and
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adding a bit more powder with your dietitian, um you might get an extra 10 calories per 100 mil and a little bit of extra protein. Or by adding some extra MCT oil, get almost 20 calories um extra. So, just to give you an idea of
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what we would be what our approach would be in infants. And then in older infants and children, we want to make uh eating and food as delicious as possible and as family-friendly as possible. So, when we're adding extra calories, it would be
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trying to sneak the calories in without affecting um the normal diet. So, it can be just simply adding a teaspoon of oil to the diet. You could add an extra 45 to 50 calories that way. Or a a spoon of
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nut butter so that you get protein as well as calories. Um using an infant high-energy formula milk to make up um cheese sauces and things. And then choosing these higher-energy, higher-protein, really great options.
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And tube feeding, um we need to do this early if we're really seeing that oral feeding's not possible. Um or if children are unable to take nutrients by mouth. Um it's very important that we're staying on top of growth and that we're
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doing everything possible um to make sure um children are growing properly. And we do have some small amount of evidence that just inserting a tube in some cases can lead to better growth compared to children just having oral
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feeding. And so, in conclusion, I think the most heartening thing is that we think that better nutrition can improve outcomes.
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Our dietary management should be trying to avoid fasting wherever possible and making sure we have this good carbohydrate source on board to support the liver.
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MCT supplementation is recommended as well as fat soluble vitamins, regular growth monitoring, and then high energy and protein diet.
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I want to thank you very much for this opportunity and I also just wanted to tell you quickly about we're going to be doing a biliary atresia public engagement nutrition project where we're getting parents of biliary atresia to tell us
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their opinions and things. And this would only be like a 20-minute amount of your time eventually, but if you would like to leave your details by using this QR code, I'll get in touch with you in the future. That's it from me. Thank you.
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Thank you so much, Sarah. This was fantastic and maybe we'll put another second for you to scan the QR code.
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So that would be fantastic to have all of your input for this survey. And I could maybe put it in the chat as well if that's helpful.
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Perfect. Perfect. I'll stop sharing now. So thank you and as expected there are lots of questions and we'll answer everything at the end.
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So stay tuned and stay with us. So um now it is my great pleasure to invite Dr. Tudor Pop. Tudor is an expert pediatrician hepatologist coming from Cluj. He's the chair of the hepatology committee of ASPAGAN and we're very
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happy to have you here Tudor talk to us about physical activity and how or should we even improve our patients' muscle mass. So please Tudor.
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Hello. Hello everyone. Thank you Orri. I think it's okay, the the slides are seen.
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I would like to thank you Orith for this initiative and for this opportunity to be together with the parents, families, doctors, and experts from ESPGHAN. And I think it's great and we should build on this on the benefit of our children.
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So, today I will have to answer to three questions. What is safe for your child?
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How do we protect and build muscle? And how do we talk about the condition with school, sports club, and other children?
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So, my main message is most children with biliary atresia can be active and being active helps.
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So, the default answer is yes. Your child should move. The WHO recommends an average of 60 minutes of day per day of moderate to vigorous activity for ages 5 to 17. That includes play and not only sports.
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The Canadian guidelines add also muscle and bone strengthening at least three days per week. And finally, any amount beat none. But here there is an issue. These guidelines are for all children and there is no separate liver specific
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target. So, our question would be what to adapt and not whether to move. The advice depends on where the the children the child is.
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We know that after Kasai operation about half of the children will still have their own liver at age of two.
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And about a quarter of those will need a transplant by age of 10 and more later in life.
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So, we have three situation. With their own liver but no portal hypertension. There is should be a full participate participation in school physical education and sport.
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If the child has own liver but with portal hypertension that meaning and as we saw before a large spleen or varices, the child should stay active but protect the tummy from falls and hard knocks and also the there should be a
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plan agreed with the medical team. After liver transplantation the child should be active and timing should be agreed with the medical team and also the contact sport the participation in contact sports should be agreed with the medical team. This is could be a
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practical framework not validate classification of this kind situation. Many parents assume that a sick child should rest but the data from the literature show opposite.
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We can see in a Dutch study uh included 46 children with the biliary atresia half of them score low on motor skills.
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The second thing after liver transplant peak oxygen uptake was lower in children after transplant than the controls.
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And finally in a study of 26 transplanted children only one of them reach an hour of moderate to vigorous activity every day.
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But we have to be sure to say that these evidences are mixed and there is a gap real gap but not universal because the activity and the fitness were close to norms but only strength, fatigue and quality of life
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were worse. Now coming to sarcopenia. What is sarcopenia? We are losing the muscle. Sarcopenia means low muscle mass, but together with low muscle strength and function. It's more than being thin.
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Across different studies, the results are different coming from 24 to 70% because the definitions differs.
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If you are looking to the children with liver transplant before liver transplant after liver transplant, we saw numbers around 40% of them 36 after liver transplant with sarcopenia.
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But we if you are looking to the weight, the weight could be normal as we saw already. It was discussed.
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In children with end-stage liver disease, the weight is not equal with the muscle. We can see 23% of them muscle was lower than the others.
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Why this muscle matters for us? And how we check this muscles? It's important because in some study, low muscle was linked with longer intensive care stay during the transplant period period. Also, the complication longer intubation, repeat surgery, and the serious infection and that was
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linked with lower muscle. How we can check and this would be an an issue that should be checked by the doctors and also the parents should ask always how the child muscle strength is and you saw before from Sarah that we should measure
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arm circumference better. So, we can check the strength of the muscle by hand grip strength, by sit-to-stand, push-up, and stair climb.
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6-minute walk test. There are different tests that could show us the strength of our child muscle and not only the uh area by scanning.
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So, we should feed the muscle and then we should train the muscle. We saw before that we should not restrict protein and in some children there is an increased need for proteins.
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We should avoid long gaps between meals. And for clear the needs are higher when the bile flow is reduced. So, when we have a cholestasis, it's clear that we the caloric needs are increased. So, we need a dietitian, we need a nutritional
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expert connected to us, connected to the family to give all the data that they need in order to have a good nutrition to fuel the muscle. But after fueling the muscle, we should train the muscle.
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So, we should play, we should exercise, the child should exercise at least 3 days a week after transplant. There are studies that showed that 2 weeks of home resistant band program leads to increasing gain in the muscle, increasing the number of push-ups, and
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longer work test so that will lead to increase in the strength of the muscle.
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[sighs and gasps] It's clear that in more of the data in the literature are coming from the adults, but we should adapt this and we should start gently with our children and we should progress their exercises with guidance.
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And how we can For example, we can see here a good week of movement. What means that? So, we should aim for an average of 60 minutes a day of exercises in any mix of exercises and play, build up
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gradually. So, we should train the heart and lungs, we should have running games, cycling, swimming, dancing, also more than three days per week, the child should play with climbing or resisting bands at home, push-up, carrying something, and also jumping, sky skipping, and running
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could be good for the bones. But, here are there three tips. So, the child should choose what they enjoy. The children should choose what they enjoy. They should do the exercises together with you or with a friend. And if there is a
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tired day, the exercises should not be skipped, but only shortened. And always with the exercises, we have to be a careful with some restriction.
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There are few restriction that are specific and even individual, but should be careful we should be careful with this.
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We should know that some of them could have an enlarged spleen. The rupture is rare. Mostly after falls and road accidents than sports.
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But, there is a reality. In a study where the doctors were asked how they recommend advice different sports, 60 of the 60% of them advised about against football, hockey, wrestling because of the danger of enlarged spleen ruptures.
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Regarding varices, there are no data and here you the parent should discuss with the medical team.
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And also about exercising after liver transplant, there are some discussion in the literature. There are some indication from 8 weeks to 3 months after liver transplant to try to do some activity and going slowly to increase the activity.
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But, anytime the all the advices should be discussed with the medical team. When to pause and when to call the hepatology team or emergency emergency call. We should know that if there is a belly pain after a fall or
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blown, if there is vomiting, blood in the vomiting or black stools, if the child is fainting, severe dizziness or very pale skin, if there is an unusual breathlessness or bluish lips, fever or chills or new yellow eyes or skin,
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all of these are signs to call the hepatology team and sometimes to go to emergency.
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These are clinical consensus. They are not coming from trial evidence. Your medical team could add some other stop signs or emergency signs, but the aim is not to create fear for you, but is to be clear and to help our children as quick
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as possible if there is some some issue. And we should say out loud what many parents feel. I think you know better than us this this sentiment, these feelings.
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So, I think there is a fear of injury. And this fear of injury shapes what children are allowed to do. There is a tiredness, so the fatigue that was above healthy normal in some children after transplant.
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The idea of feeling different, so using a spring guard or a yellow eyes or a scar can make the child stand out. And also there is a worry about the conflicting advice from the online websites, internet and whatever.
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But the evidence here are weak and each worry should have a four step should have a written can do list, shorter session, discussing discussing uh writing plans from the medical team. So, all of these could be addressed by
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simple things from with the help of the doctors. Why we should talk about it? Why we are choosing this subject? Because in average, children with biliary atresia report more difficulty with everyday life and compared to healthy children.
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So, regarding physical quality of life, social quality of life, school functioning, they are all lower.
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The difference are small to moderate and not all the children are with this kind of problem. Many children do well.
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So, there is a risk, but this is not a rule. There is a risk of having this kind of problem. What would help? Would help to have some school education programs that will improve knowledge and attitude and the
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study shows that after school education programs, the teachers comparing it with the classmate, the effect for increasing knowledge is better in teachers and sometimes that is a good thing for our children because there are some issue in different
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schools. So, coming to this, we should know that how we should train our children, we should prepare how our children will have to discuss with the teachers and coaches in sport.
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There should be one page, one conversation, one page of activity plan. Planned also with the hepatology team that should cover what the children can do, including the team role.
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If sport is restricted, what to avoid with the reason, the stop signs, who to call, practical needs like different water, snacks, when should be rest breaks and need of uh some changing room privacy.
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So, all of these could be prepared from the beginning, prepared in writing and in person. And there are some all over the There are different foundations that could help you with different examples how to do this.
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So, with classmates and teammates, the child should lead. And you and your child should decide what should be shared.
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And all of these should be kept short, true, and positive. Before going out, the child should practice the words at home and to agree from the beginning what to do about teasing.
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For different ages, there are different things that could be shared and should be shared. And keeping the privacy and keeping the child as a leader of these kind of conversations.
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So, finally, five things that we should remember. The first one, the activity by default. So, 60 days of per day is an average in any mixed playing or sports.
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Second, we should build muscle with strengthening by playing three or more days a week with enough protein coming from the nutrition.
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The third one, there are few and specific limits and you should ask the team about spleen varices and activity after transplant.
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The fourth one, know the stop signs and communicate this these with the professor and coaches and communicate who to call when they need.
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And five, prepare the adults with one page, with one plan created with your team.
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Most of the children with biliary atresia can enjoy sports and play and being active should be a part of everyday care. Thank you very much.
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Thank you very much Tudor for this very important topic and a great talk. So, it's my pleasure to introduce the last but not least speaker, my friend and colleague Dr. Vanja from King's College London who will try to
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make us all up to date with a science and research in biliary atresia. So, thank you so much, Van.
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Thank you, Orret. Sorry, I'll just go into slideshow mode. Is that okay, Orret? Can you see that?
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Yeah. Perfect. Um yeah, so thank you, Orret. I have been given the last talk. So, hopefully everyone's feeling awake and I I'm actually going to be summarizing some things that people have already mentioned but just reiterating it as
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well. So, I'm Van. I'm one of the hepatologists who works here in London in King's.
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So, thank you very much. So, really the way I mean it's only 10 minutes and to having to talk about where we are with research in biliary atresia or what are the where's the evidence that we've got is quite tricky. So, I sort of thought
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of doing it in terms of what are the questions that parents and families ask me routinely in my my practice. So, you know, biliary atresia, you know, more like the what, when, and why. Like what is it? When does it happen? Why
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does it happen? And can we do anything to slow down the disease and improve the disease and reduce the need for liver transplantation?
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And what happens when my child gets older? You know, what's going to happen to my child? If they don't need a transplant, how are they going to be anyway generally? And then I mean there's lots of exciting areas that are
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happening in terms of the world of research in biliary atresia but I thought I just sort of highlight one particular area as well that is I think very exciting.
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So, I mean in terms of what is biliary atresia, again, I don't need to explain that to anyone in this audience and I know that all the talks have sort of set that up. The reason I'm sort of saying
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that is because there's very There's lots of unknowns in biliary atresia, but there are some things that we do understand and you know, that is that in biliary atresia, your bile ducts are blocked and you know, there are some
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missing bile ducts and that leads to build up of bile and and then that causes damage to the liver.
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With inflammation and scarring, which actually I put it the wrong way around. And the you know, so that's the one thing we know from research that has been done looking at the at the livers at the time of Kasai and then the
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ones that sort of get to liver transplant, we know that there's inflammation and damage and scarring in these livers. And you know, the the reason we do the Kasai at the beginning is I'm sure again, you all know, is to
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try and unblock these these, you know, tubes and see if we can try and reduce get the bile to flow nicely and try and reduce some of this inflammation and scarring, but we know that actually even if you do unblock all the ducts and you
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do improve the bile drainage, there in you know, in a large proportion of patients, this liver inflammation and scarring still continues.
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So, then the common question again that families ask me is, so when did this happen? You know, how how did this When when along the sort of from when my baby was born to When when did it happen and is it Was it from
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before the baby was born? Was it from birth? Was it after birth, you know? And I think we've often sort of said, look, we're not really sure exactly when this happens and it will be different in different sort of cases of biliary
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atresia. But I think we're a little bit more, you know, sort of of the feeling that this this the damage to these bile ducts happened most likely early on, so antenatally and you know, maybe there was some ongoing issues post
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post when the baby was born as well. And I think evidence pointing towards this I've just put in, you know, three three pieces of evidence here, but a group in Texas have really I think helped to understand this a bit more by what they
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did is they did blood tests at birth, so they did screening blood tests at birth in, you know, huge populations of their patients babies, not not patients, just, you know, babies that were born, and then went ahead and saw which ones developed
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biliary atresia. And then went backwards and looked at their blood test at birth and found that their bilirubin and their conjugated bilirubin was actually higher at birth, which kind of suggests that there was probably something going on from before this baby was born before
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the babies were born, and you know, maybe something else happened as well after birth, but certainly there's that feeling that something was happening with these bile ducts before the babies were born.
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We also know that in a proportion of babies they have a specific type of biliary atresia where you can actually detect this on an antenatal scan you can see the gallbladder looked abnormal. And also in some babies you can see that
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they have other organ abnormalities, so with their heart, with their spleen, or with their gut. And this again sort of points towards something happening before the baby was born in the way that their sort of development of their
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organs happened. So I think we're a little bit more, you know, aware of that now.
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So then families will often say, well, could this have been picked up earlier? Is there anything that that research or evidence is suggesting that could have helped with picking up biliary atresia early, especially if we're sort of saying that, you know, potentially there
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was this damage happening from a very early on. And really where we are with that I've just put two things down here at the moment, and that's one of them is this poo color chart, which every country will have their own form of a
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poo color chart, but certain countries are using this as a kind of national screening tool in a way, especially in countries where it's more prevalent, more at the high incidence is higher like uh Japan and Taiwan, but also some
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countries in Europe. And it's basically where, you know, families are given these stool charts um and you can see here the bottom ones are the more worrying abnormal stool uh poo uh compared to the ones in the top. And
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what they're showing is that and then when families see that their poo, you know, the baby's poo isn't isn't uh a normal color, uh will sort of highlight this to their doctor or their midwife or, you know, early. Um and what they're
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showing the countries that are using this as a kind of national screening tool are are showing that they are picking up biliary atresia as quicker and they are able to do the Kasai operation quicker. But I think it's
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still not clear which countries benefit that not all countries will benefit from this. And certainly in the UK we're not using it, you know, families are certainly given this as part of, you know, um their general sort of health uh
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checkups and things, but it's not rolled out as sort of in a in an official book or in a, you know, in the sort of we call it the red book that they get here, but but it because we I think the
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evidence for us at the moment doesn't suggest that it will necessarily change the outcome or make make the Kasai significantly earlier that will actually affect outcomes in a good way, but but it's certainly something that, you know, countries and I know that, you know,
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people in our in certain countries are also there's digital apps as well and, you know, more advanced technology in that way.
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Then in terms of the blood test, as I mentioned to you in in Texas there was this, you know, they did these blood tests and they found that they could potentially detect uh biliary atresia from that. The problem and and you know,
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certainly some areas um more so in America are trying this, but I think the ideal would be if you could maybe as part of the routine blood test that babies have um and again that may differ in different countries. I'm not sure of
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all the different uh schedules, but whether you could um add that to that blood test and see could you put you know, the direct conjugated bilirubin in there. But at the moment there's lots of like technical errors with that in terms
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of the actual measurement and obviously you don't want to have a predicament where you then have like false positives or negatives. You know, you've got to be a bit more [clears throat] sure that this is actually going to
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help. But these are a couple of a couple of ideas that are out there and research is still very much going on with that.
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So why does it happen? And that's the question that I think I don't know we all probably sort of sigh a little bit at this because unfortunately we don't fully know why and it's probably not as simple as one one thing is causing it.
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It may be a multiple multiple different things and it may be different for different babies which makes it very challenging. Um but here I mean I've just put people say is it genetic? You know, is there a genetic could I did I
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pass it on to my my child? And really the evidence at the moment is there's not one faulty gene. You know, you don't pass on this condition. There are some abnormalities being found in certain groups of patients and you know, that
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may that are in terms of forming the bile ducts but again, it's not you know, this is not sort of in every patient and it's still unclear but maybe some patients do have some slightly altered genetics whereby they're more vulnerable bile ducts are a
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bit more vulnerable. Then there was this theory as to is there a virus that maybe sort of causes this damage either you know, when the baby before the baby's born or at the time of birth.
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But again, you know, the the viruses haven't been seen in all patients and it's it's really not clear whether this maybe is the trigger in some patients and it and not in others.
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There's also this you know, and families will often say is it something I've eaten? Is it something I may have done or is it? And you know, there's certainly nothing that we have found at all in that way but there has been this
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interesting sort of observation in in lambs in in sort of sheep pregnant sheep that have been grazing on this plant in way in in Australia and led to to lambs being born with with what looks like a biliary atresia picture. So
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again, it just makes and and they've started sort of work out what in this plant is it that could be affecting the bile ducts and you know, lots of research has been going is going on in that in the labs to see, but this
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isn't sort of related to to human biliary atresia as far you know, there's no link that we're finding there, but again, it just makes you think is there something and you know, this is why research is really important to see is
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there something or some kind of chemical or toxin or something that could be contributing to it. And obviously, there's lots of other ideas. My personal interest is is looking at the gut bacteria and there was There's always new areas of research that are coming
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out say it's suggesting, you know, potential new ideas. So, like for example, in mice, they've done lots of studies and they found that the bacteria in in mothers of mice, you know, mother mice can affect what their offspring's biliary
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ducts look like. So, you know, is there something like that? And this is really important because if we can find things as early as possible, maybe there's a lot, you know, potential that we can change things. But as I said, big sigh,
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don't really fully understand it and I think it won't be. I think we all feel it won't be something one thing is causing it. It will be probably a multiple group of things that are causing biliary atresia.
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So, then really when people say can we try and prevent biliary atresia? As you can imagine, we're not really there because we don't we need to understand it more to understand what exactly is we need to do to try and prevent biliary
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atresia. Then the next big question that I I'm so I'm sure that a lot of the doctors on this call and it will be asked is, you know, well well, you know, the baby's got biliary atresia, they've had the
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Kasai, will they need a liver transplant? Like doctor, will you tell me? Do Do you think my child is likely to need a liver transplant? And we know from the literature and I know that Tudor mentioned this earlier that, you
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know, these are sort of the the this the numbers that are given, you know, and it will depend to country to country, center to center, but roughly, you're looking at 50 to 55% by year by 5 years of age may need a transplant. A few more
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by 10 years and then by 20 years of age, you know, about 80% need a transplant.
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So, we will often say, and this will vary, but we will often say, you know, majority of patients will need a transplant.
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Oh, and and understandably families want to know what, you know, what how do we how can they know for their particular child whether they're going to going to be in that 80% or not. Um and really the only things we know from the
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evidence at the moment is that ideally the earlier you do the Kasai, the less likely you are to need a transplant, but it's not a given, you know, you can have a very early Kasai and still need a
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transplant, but overall, the earlier you do the Kasai, hence why we try and pick it up as early as possible, you know, the generally the better the outcome. Um and also if you clear your bilirubin, you clear your jaundice by 6 months,
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again, you know, a better outcome, but still, as I said, in those patients, the inflammation and scarring can still continue and you still have a chance of needing a a liver transplant. So, really what what is there that's new that we
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can add to this to help families to decide, you know, or to help them understand that that what's going on in their baby's liver or their child's liver. And one particular protein that's been being measured more in the research field, yeah, it's not
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come out really clinically at the moment, but could be something to watch and wait what how that how that comes out, but is this a protein called MMP7, which is a protein that's released from damaged bile ducts in biliary atresia. So, the
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higher MMP7 in [clears throat] your blood, the more damage in theory for from your bile ducts and this also has been shown to be correlating with scarring as well in the liver. And lots of people have already mentioned the
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FibroScan, but that as well is becoming a very useful tool to measure the scarring within the liver and it may be that we'll, you know, with these sort of new tools that we can put them together and try and sort of understand the liver in
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that in particular child without having to actually put a needle into the liver and look at the scarring, which as Emma said, we know, we don't do after the Kasai.
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So, then So, that's So, that's sort of looking at how can you predict what's going to happen? And then, obviously, can any medicine help that you give after the Kasai? So, you know, the the medicines that we give after Kasai will
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slightly differ in different countries and I think the big thing to sort of understand here is that it's really difficult to understand if a medicine is going to work. It helps in biliary atresia because, as you know, biliary atresia is rare and so to get
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enough patients who have the treatment who and who don't have the treatment to compare them is very difficult. And so, we often have to unite different centers, which is why ESPGAN is a great, you know, tool because we can use we can
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all get together and use our centers and it's difficult still, but to try and get bigger groups of patients to try and do some good research. And really, I've just put some of the more common drugs that are used after Kasai and
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really, I I think the main thing, I don't for for ease of time, I won't go into each and every one of them is that really antibiotics, you know, we we know is good for treating cholangitis, but as
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we mentioned earlier and Echo mentioned, in terms of preventing cholangitis, there's not really clear evidence for that yet. So, different centers will do different things and we recently did a survey and I think about 80% of Europe were using them and 20% not and I can
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you can see why if there's not good evidence one way or the other. And antibiotic resistance is something that we worry about.
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Then you've got ursodeoxycholic acid, which a majority of centers use to try and promote the bile flow in the liver, but again, no really good studies have been done to show Urso versus no Urso and really look at what
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actually is the difference in outcome. Steroids is again 50/50 in terms of Europe whether you use steroids or you don't use steroids. The idea is to calm down the inflammation, but really the clinical trials that have been done show maybe you can reduce the
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amount of jaundice, but in terms of the actually reducing the transplant problem, there's not very good evidence for that. And I just wanted to highlight here, there's been some excitement around these bile acid lowering drugs, especially in other liver diseases in in
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children. And there has been a you know clinical trials in biliary atresia, again using centers from across the world, but unfortunately at the moment they haven't shown any significant improvement in biliary atresia. And I've just selfishly put my probiotics down there because you
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know I think to understand the gut bacteria whether we can try and change modulate the gut bacteria to try and improve that to help with the liver because the gut and the liver are so closely related. At the moment very
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early days. And again I'm not going to go and then so we talked about the older child and and I think you know that's been beautifully described in the transition talk that we had, but it's important you know we're not just looking after the
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liver. We also have to do research to look into the holistic aspects of of child with biliary atresia as they're growing up and I think we've already highlighted that studies out there at the moment are suggesting that quality of life and
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school scores are generally lower in biliary atresia compared to healthy children, but we have to be really aware that these studies are not perfect. You know they're they're they're small and they're kind of based on questionnaires so they're not completely reliable. And
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I think this for me is a real highlight that we need more research in this area.
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As our as our children are growing older with biliary atresia, we need to understand this area more like what is actually their quality of life, how is their education? You know we need to do bigger research studies and we need to
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do more formal ways other than just questionnaires. We need to look maybe do some more formal scans, psychological assessments and that's something that people are doing a lot. I know know certainly at King's we're we're definitely we're this is an area
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that one of my colleagues is very interested in. Then for the final slide and this is the exciting area as of research in biliary atresia. These what we call these mini 3D biliary atresia bile ducts so organoids. It's
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still very you know fresh and new in the area of research in biliary atresia, but I think it's holds a lot of promise and I just wanted to share this as the final slide really. And essentially what what
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people are doing in Oris lab is is very much interested this and we're certainly doing it as well in in London, but you you can basically get some of the biliary atresia liver and actually and the bile ducts and grow the bile ducts
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in the lab. So, this is a very sort of basic picture, but just to to you can grow them in the lab and they call these sort of organoids and you can create these biliary bile ducts with hollow
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interiors. And really this is this has been shown now to to to be quite successful. And really the reason this is important is now we can actually try and understand and look at the bile ducts in more detail and see what we
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think's happening. And certainly there's been some work that's shown, you know, that there's some immaturity in the way the bile ducts have been formed. We can also use this now as a new tool to test things, you know, like do the viruses,
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bacteria and, you know, when I said how hard it is to test new drugs because we don't have enough patients to be able to compare them. We can now maybe use these bile ducts and like, you know, put them
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with the drug drugs and see what happens to the bile ducts, you know? So, it's a very exciting area and we don't have to sort of wholly rely on like mice studies and and things that we used to do. And
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this [clears throat] is probably pie sky thinking, very like, you know, I think skeptics out there will think this is probably too too far out there, but just just to say that actually with tissue engineering and these 3D printing kind
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of becoming very very big now, some in in especially this is actually a particular lab in Cambridge which is doing this where they take healthy they grow healthy bile ducts. So, not from biliary this is not in biliary atresia.
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This is this is, you know, different adult conditions, but they grow the bile ducts in the lab and then they can basically see this red bile duct that they've grown. They can then, you know, reconstruct a damaged bile duct
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and they're trying this in different conditions and to sort of basically try and repair the bile ducts. I think this is going to be very very very difficult for biliary atresia because it's a lot more widespread, but it's just again just to tell you
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that there's a lot more happening in the world of tissue engineering and I think this could be something very interesting for our patients as well.
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So, that's it from me. That is fantastic, Van. Thank you so much. So, with that, we finished the faculty talks, formal presentations, and we're reaching our last session for today. So, I would ask the participants to turn on their camera
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their videos, their camera, so we can all gather together and I would like to introduce a few new faces for you that haven't spoken thus far and these are some of our family representatives that we're very, very thankful and grateful
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for your participation. So, we have here I'm trying to look at it to see if I see all of the faces.
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So, we have Laura Duray, the director of the French Family Association. So, thank you for coming and I'll let you all speak in a few in a second. We have Nina von Baden from Germany who is very much
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involved in family organizations as also the ERN, the European Rare Disease Network specifically focusing also on the biliary atresia working group.
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We have Enav Sarala Vitch from my my from my patients from Israel from Haifa. Enav is a PhD in nursing. So, you may have some other perspective also for our discussion.
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And then we have uh Ekaterina from uh King's College from London um and mother of a patient. And we have I see uh Nick and uh Corinne also uh coming to us from London.
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So, um we will start before we go to our uh questions session, um I'll ask each of you to um share with us um your thoughts on how uh we can promote better clinical care or discuss what you
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think um the patients' needs that are not being addressed uh well enough or in what we should do as a society um to really promote the care for uh biliary atresia patients.
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Okay, so um sorry, I'm having I think I'm not seeing everyone. I'll work it out in a second.
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Okay, so uh Laure, would you like to start? And would you like me to share your presentation?
Speaker A
Yes, it's fine. Thank you, Orit. Thank you very much. Well, um I won't be too long, promise, 5 minutes because I think we'll need to discuss all those topics in the next webinar, Orit, yeah?
Speaker A
Sure. I highlight four important challenges faced by children with BA and their families. Uh the first one is pediatric feeding disorders. The second one is permanent tooth discoloration. We didn't talk about it tonight, but I think it's very
Speaker A
important subject. The third one is education, work, and life course. And the the last one I pick up for tonight is the impact of the whole family, um the impact of the disease on the whole family.
Speaker A
We can't maybe go to the next slide. Thank you very much. Uh I will briefly say a say a few words about each of these this year. The first one so is pediatric feeding disorders. It has been said tonight that the nutrition and
Speaker A
growth in biliary atresia is definitely very important for for children. But what we observe is that parents they feel an enormous pressure when it comes to feeding their children and making sure they eat enough.
Speaker A
Uh what we observe is that some children refuse food. Some others have problem with some different texture with different textures. So to resume is to say that meal times can really become an issue and very stressful for the whole family. And I
Speaker A
think it's very important to discuss about that. I know that the ESPGHAN has a group works on complex feeding problems. My question to you Orit will be is the the ESPGHAN SIG EAT group works together with ESPGHAN biliary atresia and the patients
Speaker A
community. But I will you might not be able to answer me tonight. Maybe the next slide if you if you can.
Speaker A
Um yeah, the permanent tooth discoloration is very important because we know that some children who had cholestasis when they were very young have discolored permanent teeth the tooth teeth, sorry. And of course children with BA are definitely concerned especially when the liver
Speaker A
Kasai surgery didn't work well. So permanent tooth discoloration can definitely affect how young people feel about themselves. It can affect affect also their confidence and their social life.
Speaker A
Maybe especially during teenage years, but we believe also after. So um I think that we think that recognizing this color parent disease as a medical consequence of cholestasis would definitely be a real real step forward.
Speaker A
So, that was my second point. Uh the third one maybe yeah, education work and life school.
Speaker A
Um many children with BA reach adulthood thanks to you all doctors. But uh we still I think we we believe we still more information about their school years.
Speaker A
Uh about further education and working life. In in 2018 the French national liver um rare disease network conveyed uh a survey and we find out that 62% of the parents declare that their child disease had definitely affected the school life.
Speaker A
So, I think it would be very very interesting to collect more information about these discourse.
Speaker A
And the last the last Thank you very much. The last one um is that how the disease impact the whole family because we know that children with BA spend a some time they spend a lot of time in
Speaker A
hospitals. Uh hospital because it's a rare disease hospital can be far from home. Uh those children can also need to go back to hospital very often. So, one parent may stay with the child for weeks or months sometimes while the other
Speaker A
parent may stay at home to keep working and to take care of the siblings. So, we believe that BA, because of all those reasons, because of all that, BA definitely affect the whole family life.
Speaker A
The parents relationships, works, money, but also siblings. And we think it would be interesting to to see to know a bit more about about that. To see how how the family how the parents and the siblings live that together.
Speaker A
And then this last slide, I won't be this longer. Um doctors, you all have uh medical knowledge. Thanks to Well, we're very grateful for that, of course, because you save our children.
Speaker A
But families have definitely everybody expert everyday, sorry, expertise. In France, we work together um with the doctors to try to identify um needs that are sometimes maybe missed.
Speaker A
And ask um we we try to to ask research uh the research question to to that matters to families. So, I think working together is very important. So, thank you very much for tonight. That webinar is great, Glenn. Thank you very much. But we I
Speaker A
think we need another one because there's so many so many topics we need to discuss. Thank you very much.
Speaker A
Thank you for this uh really I think you covered uh many of the questions on the chat. Um so, I'm actually thinking I would ask uh the experts um to um to say their thoughts about your questions because you raised a few
Speaker A
important topics. Uh and then we'll continue with the uh other family representatives. Um so, um Eckerd, I'm sure you have something to say. So, [laughter] I would let you start.
Speaker A
Thank you, Aurit. And thank you, Laura. I think you raised very important points. We have all observed the tooth changes in children, in particular those that were exposed to high bilirubin levels at an early time point.
Speaker A
And I think this is a call to action. And I think it it would be good to partner up with our colleagues from dental health to really work out a plan on how to address that. I think there
Speaker A
are individual experiences on that and some may be more helpful, but others are not. And I know that you have worked on this in France in a scientific approach. So I think it would be would be good to share that and to sort
Speaker A
of come up with recommendations on how to treat especially this point, which is maybe a very challenging and difficult issue, cosmetic issue for our patients.
Speaker A
So I really like your point that you raised there. Again, the other points that you pointed out are equally important. And I really, without getting into much detail, would highlight the collaboration with family with patient and family organizations
Speaker A
and ESPGHAN maybe to work on and collaborate on research questions addressing the issues that so far need more attention in biliary atresia.
Speaker A
The school performance is surely one. So I really like the points you raised and it definitely needs more work to help our patients. Thank you for that, Laura.
Speaker A
I found it very important. I would ask you also one of Laura's points about feeding difficulties and lots of issues with feeding that continues on.
Speaker A
So what do you think we should do better as a society to handle that?
Speaker A
Really really good point maybe from an ESPGHAN perspective for us to to merge with the with the the the the special interest group in that particular area.
Speaker A
And I love that idea. I mean it is a problem that we all face every day.
Speaker A
We're most of us are lucky enough to work with amazing dieticians. Certainly in my country speech and language therapists are more difficult to come by, but this is an obvious area for us all to collaborate. And I know
Speaker A
you know we for our patients we don't have access to a a feeding clinic as such, but we should advocate for that. You know, and I think we should probably do that at an ESPGHAN level too just even by you know promoting
Speaker A
information and knowledge. That's a it's a it's a really important issue. Any of the faculty wants to address other points that are raised or Okay. I would just say that your point about family representatives and the communication with
Speaker A
you know physicians or the medical society is I think is really important. It was one of today's goals, but I think I think we didn't think ahead of time on how we would better communicate afterwards. So while we were
Speaker A
having the great talks, we were thinking about a few options because I think we have we had so many people here that I'm sure would be interested.
Speaker A
So perhaps one of you can also suggest how we can connect better. And also I would like to encourage you to go on to the ESPGHAN website to the same page that we had the biliary atresia family day post
Speaker A
that perhaps will post there what would be a good way to have a distribution list uh and we also have the office the Aspen offices a backup but we'll also we will coordinate what would be the best way to
Speaker A
get in touch with everyone unless you have other suggestions. Okay, I'll ask Sarah to discuss some fitting difficulties later but perhaps we can have Nina from Baden perhaps you can discuss a few points you wanted to raise.
Speaker A
Sure, thank you so much for then thanks really everyone for this very very interesting and exciting session that we just had.
Speaker A
So before I came in we also discussed well with with some of the other parents to to kind of summarize some topics that that might seem very obvious but still are very critical for for the families also for the parents of the well of the
Speaker A
young children with biliary atresia and I think three points so one that Vandana already pointed out in her presentation is really the early detection so how can can it be detected earlier and I think for that there is still a lot of room to improve
Speaker A
education or information for that early detection for the primary care doctors or pediatricians also for the parents and for midwives or for anyone who is in touch or in contact with the newborns in those in those early days because as as you've all
Speaker A
pointed out it's it's very critical and sometimes weeks can you know really make a big change in the in the course of the of the disease later on and the earlier Kasai is possible also you know, you have you have potentially
Speaker A
better outcomes and and and you can postpone transplantation or these things. So, so really I know there are a lot of activities already going on. There are applications and things, but I think there's or we think that there still is
Speaker A
is a lot that could be could be done to to improve that. Um Another one is that usually those the parents that are being faced with it, you know, the new reality maybe or the reality of that new disease or or
Speaker A
the medical condition, this is usually something that's very very um difficult to understand. And especially also if the information and the medical information might also be quite complicated. So, can that information be crafted in an easier manner to you know, to understand
Speaker A
easy wording, also maybe to take away the panic piece, the threat that usually comes with this.
Speaker A
Um this I think in general this is not only early onset, but also throughout um maybe the well, the care of the of the patients.
Speaker A
And and then the third one overall, but I think this is also what why this is so important here and I think everyone who is on this you know, this this group here is already working on that, but how
Speaker A
can the communication between the doctors and the medical staff and the families or the parents and patients even be improved. Um It's We had one example from from from the parents group where um it's really also around maybe a surgery or around another
Speaker A
stay at the hospital, each small comment or each small information that's just been dropped on the family can cause a major impact. So, also, you know, to to have the medical staff be aware of the impact that they
Speaker A
also have on on the families, which I know all of you here for sure have, but this is definitely an issue that also came came up and wanted to raise.
Speaker A
Thank you. Thank you. Tudor, would you like perhaps to discuss what we think what we can do best on what based on what we heard, what we think we can do from ESPGAN side to improve some of those points.
Speaker A
Yes. So, we as European Society, we should in a way try to uniform the situation and all these kind of issues because there are big differences in Europe regarding the access, the possibility of communication between children between parents and doctors.
Speaker A
In some countries, there are organization family or parents organization that easy to access to information in some other countries. And if you are looking also in the chat, there are some ideas from Africa because there are big differences
Speaker A
between countries. So, as ESPGAN probably should organize more and more activities like this. This is an example of how we can improve the the knowledge, the link between parents and doctors.
Speaker A
It's clear that everyone from here have a group of parents they are working well with them.
Speaker A
But um as at the European level, probably we should try to do more of this kind of activities to increase knowledge, to to share the knowledge that the parents need in their language, not in some scientific terms, but in um
Speaker A
words that could be very well understood in in the base of the field, we can say.
Speaker A
And to to try to listen their voices because sometimes what we consider that it's important is not the same important for the parents and for the family.
Speaker A
Also, the problem with the How can I say? The the relationship with the school, with the children, other children, with all the people around this patient with special needs, uh depends on the place. It's different if you are in, for example, coming from my
Speaker A
country. It's different if you are in a city, big city, and the professors are well trained or they know something about that, or you are coming from a village where the child is exposed to a lot of problems and the family
Speaker A
are exposed to a lot of problems in the community. So, we should do more to train the people that are taking care of the children, I think.
Speaker A
Thank you. Um now, Elena, I'll ask you as a mother, as a nurse, as a researcher, uh to perhaps give us your point of view.
Speaker A
Thank you. Thank you for inviting me today. Um as Zoe said, I have a 7-year-old daughter, uh Kasai only. I hope she will start like that. I know that the the odds are not good, but still hoping. And
Speaker A
I think that the main issue as to digest it as a mother is not it's it's also for as a medical professional is to educate the parents to recognize the risks that the red flags. But then as a mother I am
Speaker A
interesting of how can I prevent the the deterioration of the disease. And I think from what I saw in the literature so far, there are not so many studies that found any recommendations evidence-based that we can actually go through and say, "Okay, if I will do
Speaker A
that and that, I know that I can prevent in that odd the the risk for deterioration of the of the liver function. And I think that this question is regarding how can we prevent from Kasai only to get to the to the
Speaker A
transplant eventually. Um for I know that Accord talked about the vitamins. So, great. So, I know that I can provide vitamins to my to my kid. It's it's a good thing, but can I do anything else from the nutritionist perspective? Not
Speaker A
just to prevent the the malnutrition, but even if my child is um, the growth is okay, can I do something to make sure that it will keep keep her okay.
Speaker A
Um, and I think that all of these I'm coming to my point that I think from what I read, most of the studies, if not all, are based on very small sample sizes, very small cohorts of of children
Speaker A
or adults with that are BA. And I think maybe um, you can think about creating a registry of patients across Europe, across the the world of BA patients or their parents if those are children um and and follow them
Speaker A
over the years and do the the questionnaire. Self-report it's still valid. People you talked about using the the Urso and maybe it can prevent, but we don't know. But maybe if we'll have for longer time people that will be in a
Speaker A
registry and will provide this information we might have some data that can be translated eventually to evidence-based to help the parents uh to prevent maybe the next deterioration of the disease. So this is the main issue, but through the uh
Speaker A
presentation of uh I'm I'm hope that I'm pronouncing correctly uh Muriel, am I saying correctly? Um so you talked about the transition to adulthood and I was wondering since that the BA is so rare, why? Why do we need to move
Speaker A
from the pediatrician to the adults? If we're so if there are so so small amount of people that uh suffer from this condition, so why can't we just provide like a small area in the clinic for those people that will
Speaker A
keep their treatment with their um their their professional staff that knows them from the age of almost zero.
Speaker A
So this is what I thought during this those presentations which which were really really uh important and thank you for providing them. So thank you.
Speaker A
Thank you Elena. So Maria, perhaps you can answer. Yes? Yes. For the question that why children need to be followed by adult hepatologist, I think yes, it's I think it is a um very important uh to say that uh
Speaker A
children need to grow up. [laughter] And uh pediatric hepatologists are pediatrician. And uh we are not able to follow um uh people with long um and last um disease, for example, uh what if there is a pregnancy?
Speaker A
Uh we are not able to follow uh a woman with a pregnancy, for example, um but there is some things that uh uh need to be followed by uh adult hepatologist.
Speaker A
And it is important to to to train our colleagues. Of course, it's a rare disease, but biliary atresia it's not so so rare disease. It's one of the first cause of liver transplantation uh in children. So, we need to
Speaker A
to train our colleague uh adult hepatologist to um that they they better know the the disease and first and after, as you said, um children uh are not always transplanted, but most of the time they will require a liver transplantation.
Speaker A
So, we need colleague that uh are knowing the disease and then can follow the people uh after the liver transplantation, also. So, I think yes, it's a rare disease, but we we need to train our adults uh hepatologist colleagues for that.
Speaker A
It's important. Thank you. Heckard, please. Uh enough, I think. You raised very important points and I just wanted to briefly comment uh the issue of uh conducting trials in rare diseases such an important issue, but it raises severe
Speaker A
logistical and financial concern of challenges, which are not easy to address. And maybe this is one reason why uh these trials are um not performed as often as we would like them to be performed.
Speaker A
But you raised the point of registries, and this is something that we picked up.
Speaker A
And since 2 since '23, we now have a registry uh collecting data from many centers throughout Europe and beyond. And we have now 300 patients in this database.
Speaker A
Uh and I think we're collecting a lot of very valuable information to learn more which patient are doing better, which patients are doing less good after Kasai, in order to find out how can we improve the outcome. But most
Speaker A
importantly, we're also trying to install the option to um integrate patient-reported outcomes, uh which is now technically possible. So I think you raised this point that we would like to integrate and use that in the future. Thank you.
Speaker A
Thank you. Um we have a few questions that I tried to summarize by groups from the audience. But before uh uh Nick, if you wanted to uh say something, you can Corinne, um you're welcome to do that.
Speaker A
Um thank you. Yes, I think it would be uh of interest to us to share a few words about our experiences. There's been a lot here that we could relate to as as we've been here. So so thank you
Speaker A
for the invite. That's been fascinating for us. But I think, you know, starting very early on in our days, we we relate to a lot of the early points made there that the um from our experience, there wasn't the
Speaker A
earliest possible diagnosis and intervention um for our son when he was first being diagnosed. So we we feel that um first opportunity to identify actually that he had biliary atresia was was missed.
Speaker A
Um he was jaundiced from an early age and that that was dismissed was our reflection from the health visiting staff at that time. So, the experiences that they fed back to us was a routinely um they they saw actually that the you
Speaker A
know jaundice would self-clear and so they they were unconcerned by that and so that was a that was a a missed opportunity for health care services and ultimately that led to a a later Kasai procedure and then you would then then
Speaker A
we hear from them and understand from our past and be recommended. So, it always leaves you with a question of, you know, what what impact did that have potentially on on the outcome that we had as we we followed on them down the
Speaker A
transplant journey. Do you wish to add? No, I I I I think it's the um the teaching parents um the signs that they need to look for that just wasn't there with us. But also it's it's the communication. That's the
Speaker A
hardest thing to crack, isn't it, between parents and doctors and advisers, but it's um it's it's the not being listened to at the beginning. I think that that changed a lot a lot for us.
Speaker A
Then I I know you did a survey um that you presented among UK pediatricians.
Speaker A
Um perhaps you can um say what your recommendations were based on that. Um I'm not sure but I mean in terms of what I I 100% agree and I think it is it is tricky because it's very it is rare
Speaker A
and so what we found is, you know, when we did yeah, we did like a survey in the UK just to see how um you know, yeah, how many of the the biliary atresias that we had were missed
Speaker A
opportunities and could, you know, how many had presented to the hospital had presented with jaundice or maybe a who would asked them even asked about pale stool? And I think a lot of it is because it is it is a rare condition and
Speaker A
it's it's something that someone may a midwife or a health visitor or a doctor may only ever see once or never in their career and it and it's because that's what makes it really challenging.
Speaker A
But, you know, it means that like you said we need to you know, like obviously I've mentioned a couple of things that are going on in terms of trying, you know, stool charts and whether blood tests, you know, but I
Speaker A
think really it's how we improve the education. And I think although there is that, you know, the the the delay in some cases, I think what is slight what is really encouraging we did a a BA survey across Europe and actually and
Speaker A
even in the UK as well. It's the we are I think there are improvements and I think, you know, it's definitely a long way to go, but there are the age of Kasai is coming down, you know, and it
Speaker A
does seem to be improving and you know, obviously there'll be cases where things could be better and of course that, but I think yeah, education certainly I mean is the big key, but how to do that in the maximal way to to to
Speaker A
really stick. Um yeah, that's that's the thing, but yeah, I I yeah, completely empathize with that what you've said though. It must be really really hard at the beginning, especially if you had gone to with something that wasn't quite right.
Speaker A
So, yeah, think it's our job to educate more and better. Okay, now I'll summarize the questions into groups. Um Sarah, there were questions about NG feeds and feeding oral feeding difficulties that uh infants are not interested in food
Speaker A
because of getting NG feeds. Uh what do you have to say on that? Yes, I was just furiously trying to make sure everybody's questions from nutrition point of view were answered.
Speaker A
Um yeah, feeding difficulties I'm glad that was um raised earlier by I think Laura. Um it's a really important um issue and it can be so hard for us uh for us to manage. Sometimes I try to help parents understand that for a child
Speaker A
who's now afraid of certain foods, try to think of it as if someone asked you to eat a cockroach.
Speaker A
And um you probably wouldn't be very interested. But you might be able to manage like the foot of a cockroach or maybe like a toe of a cockroach if cockroaches have toes. And that's the kind of um start that we should be
Speaker A
trying to make to to gradually get children to eat the foods we want them to eat. Like it's can be so scary for them. Um so I really understand that that's difficult and it would be really brilliant to benefit from our other
Speaker A
dieticians and specialist groups um because they're all over this kind of advice and they have great tips maybe that parents haven't tried yet. Um some other questions people asked were I would just quickly respond to that I think about sometimes babies given um
Speaker A
some of the MCT formula milks unfortunately can get a little bit constipated. Those formula milks are designed to be as close to breast milk as possible but they're never going to be as good as breast milk because it's
Speaker A
so um special. Um they've got less water in them per volume than you will have in in breast milk. So we can sometimes see this and there are different MCT formula milks we can try to see if that if that
Speaker A
helps. Um it's really important to work with your dietician. Must be so stressful for parents to be just trying their best and breastfeeding and then they're they're seeing problems like this. Um there were a couple of other things about whether um there's any
Speaker A
specific foods to have for to get your vitamins A, D, E, and K. It's actually just a balanced diet because those vitamins are spread across fruit, vegetables, whole grains, seeds, etc.
Speaker A
So, there's not not something special, particularly from a food point of view. Um something about weaning, that would just be normal weaning advice for your um your country um and some more people with feeding difficulties. So, I really understand
Speaker A
and I think we need to improve on that. I don't know if there's anything else you wanted me to answer on.
Speaker A
I would like to ask Eimear, there were questions about uh vaccinations, both before transplant and also post-transplant.
Speaker A
Um I think this is a very important topic we missed. But perhaps um you can um uh say what you think and what you So, so you know, in terms of of transplant and live vaccines, um certainly in the early post-transplant
Speaker A
period and and 4 weeks before live vaccines are generally contraindicated because uh there's a lot of immune suppression around that time. This kind of topic is not completely agreed anyway in, you know, in the hepatology community and we
Speaker A
are working we are working on it in terms of live vaccines post-transplant, but at the moment, certainly in my practice and I think in in in in many others, but not all, uh we don't uh vaccinate children post-transplant with
Speaker A
live vaccines currently. But we absolutely get every going vaccine that's killed that we can possibly get to try and prevent every possible disease that you can get both pre- and post-transplant. So, I think it's a watch that space. There are centers
Speaker A
doing it a long time post um liver transplant, but um certainly in terms of of of national guidance, uh there isn't um there there we are not all on the same page as of yet.
Speaker A
Thank you. And Mariela, there were questions about some natural remedies. Um if we recommend them, if we allow them for patients with chronic liver disease.
Speaker A
What do you mean as natural? [laughter] But, you know, some uh supplementations, herbs, um homeopathic medications, uh this group.
Speaker A
Depend what for the nutrition state the best as we said before is to have a very very good nutrition state because it is important for um the best best life before transplantation and for this transplantation it's a it's a critical
Speaker A
point because if there is malnutrition there is a risk as uh Sarah said that infection um bad cicatrization and so there is a lot of um complication after liver transplantation. So, before transplantation it is to have the the best nutritional state but
Speaker A
we said that vitamin soluble within in lipids are very important and this there is no other medication natural or also that can be of help, I think.
Speaker A
Yeah. I would say my the way I look at it in terms of I get patients asking me about natural stuff that we're not really sure what's in it and at least I am really afraid recommending to use those
Speaker A
like supplementations to promote liver health. First, they are not evidence-based. Second, we don't know what's in there. The fact that it's actually written on this unsupervised herbs, herbal, or homeopathic medication that that's what's what's written on the box
Speaker A
does not necessarily mean that that is what is inside. So, personally, I recommend against using, you know, those supplementations. But, if any of my colleagues think otherwise or have any other thought, um you're welcome to say that.
Speaker A
Um I'll try to finish. Um I think we'll have one last question. Vance, for you about probiotics. Do you recommend using probiotics?
Speaker A
I was just typing it into the chat for the person who asked. Um so, at the moment, there's I think I think you know, it's certainly adults, they're they're a bit further ahead in that in the with liver disease, obviously not
Speaker A
biliary atresia, but they're finding that if you do change the gut bacteria and make it more friendly, healthy bacteria, you can potentially improve the liver disease. But, still, you know, very much in the research field. Even they're not using probiotics standardly
Speaker A
in adult liver disease. But, I think so, I think at the moment for the I know somebody asked that question. I think there isn't a specific probiotic. We I think we're still very much in the research field. So, I think if people
Speaker A
did have a question about that, they should ask their their clinician and see what what people what they recommend.
Speaker A
But, yeah, I'm hoping this will be something that we can change in the future. But, at the moment, it's still research-based, I think.
Speaker A
Great. So, unless there is any other comment from the faculty, I would first want to thank the patients. I also want to personally thank patients for agreeing to participate in clinical and and also basic science research.
Speaker A
I know I'm asking personally all of my patients to have a piece from their livers so to grow into organoids, but this is just one example.
Speaker A
But I just want to stress that and say that we need research and your participation is crucial and really helps to actually advance our field. So thank you very much. This is a very very important um help and contribution on your side.
Speaker A
And I will go back to the webinar. This has been I think a fantastic fantastic evening and or afternoon depends where you are around the world.
Speaker A
I was very very excited to see patients from Africa, from the Philippines, and obviously from across all of Europe.
Speaker A
So this is was a success and we'll have a recording available for people to to watch. And definitely all of your input and the discussion will make us think about our next steps and how we can address those important points
Speaker A
to advance the field and take a better care of the patients. So thank you everyone and with that we will end the webinar.
Topics:biliary atresiapediatric hepatologyESPGHANKasai procedureliver transplantationcholangitisportal hypertensionpediatric nutritiontransition to adult carefamily education
