All About Pediatric Liver Disease and Transplants

Podcast Transcript

Scott Steele: Butts and Guts, a Cleveland Clinic podcast, exploring your digestive and surgical health from end to end.

Hi, everybody, and welcome back to another episode of Butts and Guts. I'm your host, Scott Steele, the Chair of Colorectal Surgery here at the Cleveland Clinic in beautiful Cleveland, Ohio. And I'm very pleased to have Dr. Vera Hupertz here, who is the Executive Vice Chair of Pediatrics. She's also the Pediatric Institute's Vice Chair of Quality and Safety and the Medical Director of Pediatric Hepatology and Transplantation here at the Cleveland Clinic. Finally, she's a Clinical Assistant Professor of Pediatrics at Case Western Reserve University. Vera, thanks so much for joining us on Butts and Guts.

Dr. Vera Hupertz: Thanks, Scott. I'm happy to be here.

Scott Steele: So we always like to start off a bit with our guests to tell us a little bit about yourself. So since this is your first time here on Butts and Guts, where are you from? Where did you train and how did it get to the point that you're here at the Cleveland Clinic?

Dr. Vera Hupertz: Oh, that's great. I think it's a good story, actually. I grew up in upstate New York, Albany, New York to be exact, went to school there at Rensselaer Polytechnic Institute, and then went to medical school at the University of Rochester in Rochester, New York. Headed West to Cleveland, did my residency and fellowship at Rainbow Babies and Children's Hospital. And while there I met my husband on a blind date, no less, fell in love with him and we got married, but he's one of those born in Cleveland and we'll die in Cleveland. And so here we've been, not that I have to say I regret, that I love Cleveland, loved being here. And when I started working at the Cleveland Clinic 20 years ago, it has been a great experience for me.

Scott Steele: And we're so glad that you went out on that blind date.

Dr. Vera Hupertz: I know.

Scott Steele: So for our listeners out there, give us a little bit of an overview of Cleveland Clinic's, Pediatric Liver Transplant Program.

Dr. Vera Hupertz: Yeah. So Cleveland Clinic has been really on the cutting edge of liver transplantation, since the mid to late eighties, 1980s, doing many adult transplants, as well as starting to do pediatric transplants at that point in time as well. Currently, we have a really strong program, we have four board certified transplant hepatologists who are basically, myself included, gastroenterologists who have demonstrated expertise and additional training in liver transplant and therefore liver [inaudible 00:02:25], specifically liver disease in children. And we'll be joined actually by our fifth transplant hepatologist in August. And that time our program we'll just be building and building.

We have three primary transplant surgeons who do the pediatric cases, Dr. Hashimoto, Quintini and Eghtesad who are amazing. They have amazing skill and expertise. Not only do they do the pediatric transplants, but they participate regrowing dozens of adult liver transplants on a yearly basis. So they bring all of that experience as well to our pediatric liver cases.

And in addition, they are supported by incredible surgeons, such as yourself and the rest of the surgical services at the Cleveland Clinic. So there's no end to the expertise that they have. Our transplant center cannot be complete without mentioning our coordinators who help our patients navigate through their process of listing transplant and in the longterm followup. In addition, we're supported by dedicated social workers, dietitians, pharmacists, infectious disease experts, and psychologists who are all trained in pediatrics, as well as the needs of the transplant patients in a hospital. We're covered by every subspecialty service in pediatrics who can assist us if the need arises in addition to our incredible intensivists in the critical care unit there.

Scott Steele: Well, what incredible team you have. And so let's delve right in. And so for our listeners out there who may not be as aware, what are some of the most common liver diseases that your program treats?

Dr. Vera Hupertz: Yes. So we take care of a lot of patients with biliary atresia, which is the most common cause for liver transplants in children. This is a disease that presents within the first few weeks of life, generally with jaundice, which is yellowing of the skin and of the whites of the eyes. There is surgery that is indicated for this disease, but it often doesn't work. It generally you will slow down the process, but eventually transplant is needed in probably about 80% of them by the time they're 20 years of age. So the majority will need a transplant eventually anyways, many of them in the first few years of life.

We also transplant a lot of kids with metabolic diseases. This is where the body is not making the right proteins or chemicals to help the body function appropriately and or the liver to function appropriately. Some of those diseases are things like Alpha-1 antitrypsin deficiency or Wilson's disease.

And then probably the third most common cause we see for liver transplant in kids is permanent liver failure. And very often we don't understand or don't have a good reason why the liver suddenly fails, but because of that rapid failure, it can lead to coma and death in a child if it's not recognized quickly and treated with liver transplant quickly.

Then in our teenage patients, we probably see a lot of the same diseases that our adult colleagues do, are such as autoimmune diseases, autoimmune hepatitis and sclerosing cholangitis, and also drug reactions that can lead to liver failure either due to sort of a bizarre reaction to the drug or due to overdoses.

Scott Steele: So you mentioned jaundice, what are some of the other symptoms that a child may have that could mean that they have a liver disease?

Dr. Vera Hupertz: Yes. Symptoms, and I don't want every parent to go rushing out there thinking that their child has liver disease, but they can be pretty vague. Sometimes it can be due to tiredness or excess sleeping. Kids, when their liver is not working properly can have a loss of appetite or it can result in poor waking or slowing down in the growth of the child or even a change in the school performance.

So symptoms can be vague, but you can imagine that these symptoms can be due to lots of other diseases and disorders as well, or even just to rapid growth. So it really takes a lot of expertise to try to figure this all out and suspicion.

Then there are the more obvious symptoms like jaundice, the yellowing of their eyes, but sometimes the kids can present with excessive itching and that's due to too much bile acids in their blood. One time I had a child sent to me from a dermatologist because the child was just itching so much and it turned out they had a disorder called Alagille's that resulted in that itching. Sometimes they'll have pain in the right upper side of their abdomen. And then in later stages of liver disease, sometimes they'll present with confusion and even sometimes coma, if it's a severe, really an acute process, which is happening very quickly.

Scott Steele: So truth or myth, there are different treatment options for a child with a liver disease than there are for adults?

Dr. Vera Hupertz: Myth, of the diseases that are common in both children and adults like the autoimmune diseases or viral hepatitis, the treatments are generally the same. Sometimes in pediatrics, we're limited if the medications aren't necessarily approved yet by the FDA, but usually if there's a strong need for the medication, we can get it approved and treat the child if necessary. The differences though are where our diseases aren't the same as what the adults see, like some of the metabolic diseases or the biliary atresia, which adults don't really see.

Scott Steele: So in just overall kind of 50,000 foot, when it's a liver transplant required, it can't be that all of these diseases, you have to jump right to liver transplant. There could be some medical treatment options for them, when do you say this is a liver transplant candidate?

Dr. Vera Hupertz: One of the major reasons we see for transplant in children is because the liver is not supporting their growth and natural development of the child. If it's really pushing them back and they're not developing in those critical years of their lives. And it's because the liver is not doing what it's supposed to be doing. Then we would recommend a liver transplant. If the liver is slowly deteriorating, it can sometimes result in increased pressures in the belly that can lead to poor blood clotting and can lead to bleeding. And that could be a cause as well for a liver transplant. And some of those children are at risk for increased infections, which can result in repeated hospitalizations. And then obviously a transplant would be the way to go for them.

Sometimes the symptoms themselves are so horrible that the child's quality of life dictates the need for a transplant. Itching is one of those reasons. Sometimes we see these children with scratches all over their bodies, they're bleeding from the scratches. They develop infections in the skin. Sometimes the children are depressed and not even eating and drinking the way they're supposed to be. So we would actually transplant those children, even if their liver is not absolutely failing, but because their quality of life is so horrible. And then in the permanent cases where the liver is basically dying right before our eyes, we need to transplant those patients very quickly at that point in time.

Scott Steele: Vera, I'm going to walk through some terminology that may be very confusing to our listener out there. And if you could just kind of tell us, as a broad overview, what these are, that'd be great. What's the difference between a living donor transplant, a split liver, a reduced size liver or a whole liver transplantation.

Dr. Vera Hupertz: That's a great question. So let's start with a living donor liver transplant. That's where we take part of the liver from another human being. Often, this is a living related donor. So a parent or a sibling, they have to be over 18 to consent for it, but we can take part of their liver. Usually it's a smaller part of their liver because it's going into a child and we can place that into a child. The wonderful thing about the liver, which is different from many of the other organs in our body, is that it can regenerate. So if we took part of my liver, I won't go into yours, but we'll take part of mine and give it to somebody. My liver will regenerate to what I need it to do for my body to support it. And that can happen pretty quickly within literally weeks to a couple of months.

And then that piece of liver that we transplanted into the child will grow to fit their needs as well. Historically, we've done that with living related donors, like I said, a family member. But a lot of times, if the family member is not able to do it, sometimes we've had neighbors volunteer or other church members volunteer, and we've had anonymous donors who have volunteered to be on a list to donate part of their liver or a child or for another adult either.

Then the second type of transplant you mentioned is to split liver transplant. And that's where we take what's called a cadaveric donor. So somebody who has, and they can split that liver into two pieces, whereas one piece will go into usually a child and the other larger piece will go into an adult recipient. And that way two people benefit from one liver. Our surgeons are very good with this approach and really try to recommend that because of the potential shortage of liver donors out there, this way, two people will benefit from one liver.

And then in what's called the reduced size liver transplant. So if we have an organ being donated by somebody who has died and we're not able to split that for one reason or another, we could take that whole liver, cut it down along the appropriate line to a smaller size where it'll be more compatible with the abdominal cavity in a child. We can't take a huge adult liver and put it into a child because that would be too much pressure on that liver when we try to close up the child's belly. So we have to cut down the liver into a more appropriate size for the child and along with the blood vessels and so forth. And we can actually get really good results with this as well.

And then lastly, you could take a whole liver from another donor and put it into a child or another adult. Obviously you need to have appropriate size matching. Again, you cannot put a huge liver from an adult into a child. So unfortunately you'll have to take either a very small adult, put it into a child or even another child that may have died and put it into another child.

Scott Steele: Only imagine that facing the possibility of a liver transplant is scary for adults. But when you add into that, the pediatric aspect of what you do, that can be quite overwhelming. So for our listeners out there, can you walk us through a family's journey with your pediatric liver transplant program? How did they get in touch with you? What's the first appointment like? And what can that family expect moving forward with future visits and follow ups as their child grows?

Dr. Vera Hupertz: So a lot of times the family will get in touch with us through their own gastroenterologist or through their pediatrician who may refer them to us. If they're still looking for other options, they can find us through the website and find our telephone number and or contact us literally through the website itself. Then we will usually set up a first appointment. And at that first appointment they'll always meet with the transplant hepatologist. The reason for that is we are well versed in all the different diseases that children can experience affecting their liver. And there have been occasions where people may have come to us for a liver transplant. Whereas we find that, well, maybe there are some other treatments that are available or some other options for them. And there have been times where even though they thought they needed a liver transplant, we've been able to turn around their disease process where they didn't need a liver transplant. So I said anything it's always important to meet with the transplant hepatologist.
At that visit, they will meet with our coordinator as well. And if we all are in agreement after reviewing the prior records, the biopsies, the treatments, et cetera, that we should proceed with a transplant, we'll set up a meeting with the transplant surgeon as well. That could actually happen at that first meeting, sort of a meet and greet, if you will, to meet the majority of the team. Once there is agreement that a transplant needs to be done, then numerous appointments are made. And those are for blood tests, for X-rays, for a CT scans for various imaging, as well as meeting the rest of our team, which includes the dietitians, the social workers, the pharmacists, the psychologist, and our coordinators are really good about trying to combine all those visits so that the families don't have to make numerous trips back and forth to the hospital.

Scott Steele: About the time of this taping, we're dealing with coronavirus being as a daily part of our lives. So how has that affected liver transplant and what is the Cleveland Clinic doing in order to continue with this program, but ensure safety?

Dr. Vera Hupertz: Yes, we're trying to do everything we can to protect our patients and protecting our caregivers as well. So we are all strongly encouraging, wearing mask for our patients, being over sensitive, about hand hygiene and cleaning all surfaces between patients. When this all developed, we reached out to all of our transplant patients who are out there and those who are awaiting transplant to give them recommendations of how to protect themselves, how to protect themselves at home, or at school, when school was in session. And also even at work, since many of these young people may even have jobs, looking at their jobs and trying to help them negotiate whether or not they should continue working or whether they should be on a laid off for the present time and working from home as much as possible.

We have had some success with virtual visits, but because, examination of the liver is really an important part of our followup exam, we really do need to get laid hands on those bellies to really feel the liver, to make sure that they're not suffering any consequences after their transplant and make sure everything is great. So in person visits are still the best. And as a result, working with our nurses, our medical assistants, with our schedulers and everybody else, making sure we're all up to date and trying to keep a close eye on each other to make sure we're staying as safe as possible and keeping our patients safe. So I think we have done the best we can to minimize exposure of our patients. They should feel safe coming in. They certainly feel safe calling us and asking us for advice and giving them the best recommendations we possibly can to stay safe during this time.

Scott Steele: So what's on the horizon as far as liver disease and transplant research to help combat or even improve the quality of life for children diagnosed with liver disease?

Dr. Vera Hupertz: Well, certainly, with regards to liver transplant, I think again, Cleveland Clinic is on the cutting edge, we're trying to come up with better ways to keeping those livers that are being donated healthy before they're even transplanted in the patient. Dr. Quintini is working with specific perfusion techniques that are being piloted at the Cleveland Clinic to keep those transplanted liver healthy before they're even placed in the patient.

Dr. Hashimoto and his team are working with living donor protocols where we are actually doing the donor surgery by a laparoscopic approach, basically to a very small incision and taking out the liver to be given into a pediatric patient. As a result, the donors have well, a much shorter recovery time and they're able to get up and moving and back to work are discharged from the hospitals a lot sooner. This is especially important in pediatrics where very often the donor is a parent, a mother or father, historically they would have to stay in the hospital, be on bed rest for a few days. They wouldn't even be able to hold their child after their transplant surgery. But now with this minimally invasive approach, they can literally come over, hold their baby after the transplant. And it really is a great improvement in the care and getting these families back together again, after the transplant, and since it's all done on the Cleveland Clinic campus, it's much easier to do that. Literally walking down the hall practically.
And then in the future in a pipeline, I think for a lot of our metabolic diseases, we're seeing a lot of new [inaudible 00:20:28] coming up to treat some of these genetic diseases and these metabolic diseases so that we may never even need to get to the transplant scenario. Things like Alpha-1 antitrypsin deficiency, these diseases may be treated by gene therapies that will help these kids in the future.

Scott Steele: So we'd like to end up with all of our guests to know you a little bit better with some quick hitters. So first, what's your favorite sport?

Dr. Vera Hupertz: Oh, volleyball, without a doubt.

Scott Steele: And what's your favorite meal?

Dr. Vera Hupertz: Oh, my favorite meal, our favorite meal at home, which is like our just feel good meal is barbecued ribs and macaroni and cheese. Those two things, they go together.

That's wonderful. So what is the last nonmedical book that you've read?

Dr. Vera Hupertz: Well, is it medical or is it not? It's a book called Death and it's a little bit tongue in cheek, but a lot of interesting points about what's happened to body after we die, specifically bodies that are given up for research or donated for further study. And it's a really good book and it's fascinating.

Scott Steele: And finally, what is something that you like about living here in Cleveland?

Dr. Vera Hupertz: I just love the, my family does this a lot, hiking in the Metro Park and there's the Emerald chain around all of Cleveland with Metro Parks in the various counties, as well as in Cleveland itself where you can go hiking and you feel like you're out in the wilderness, most beautiful wilderness in the world. It's wonderful.

Scott Steele: And so what's a final take home message for our listeners regarding pediatric liver disease and transplants?

Dr. Vera Hupertz: Yeah. I think families need to know liver disease is often treatable and it takes really an expert in dealing with this who has been around for a while, who have the expertise to treat these illnesses as well as, needing a team approach to taking care of the patient both before transplant. And if they need a transplant, and after a transplant as well, that's the most important thing.

Scott Steele: Well, that's fantastic. And so for more information about Cleveland Clinic's Pediatric Liver Transplant Program, please visit clevelandclinic.org/pediatricliver. That's clevelandclinic.org/pediatricliver. And to make an appointment with a transplant center specialist, please call (216) 444-1976. That's (216) 444-1976. And in times like these, please remember it's important for you and your family to continue to receive your medical care and be rest assured that here at the Cleveland Clinic, we're taking all necessary precautions to sterilize our facilities and protect our patients.

Hey, I really appreciate thanks so much for doing this and taking the time out.

Dr. Vera Hupertz: Thanks Scott, anytime.

Scott Steele: That wraps things up here at Cleveland Clinic, until next time, thanks for listening to Butts and Guts.