Children with kidney disease may require transplants

Host Amber Smith: Upstate Medical University in Syracuse, New York, invites you to be "The Informed Patient" with the podcast that features experts from Central New York's only academic medical center. I'm your host, Amber Smith. Today, I am talking about kidney disease in children, including dialysis and transplant options, with Dr. Scott Schurman. He's an associate professor of pediatrics and the director of nephrology at Upstate.

Welcome back to "The Informed Patient," Dr. Schurman.

Scott Schurman, MD: Thanks for having me.

Host Amber Smith: Let's start by going over the most common causes of kidney disease in children. How frequently are babies born with a kidney malformation or related birth defect?

Scott Schurman, MD: Approximately 1% of children have some type of structural abnormality of their kidneys, but almost all the times it is minor, very mild and doesn't affect their long-term kidney function.

More severe structural abnormalities, in which the kidneys do not form properly, so that the number of filters that we generally have at birth are markedly reduced and may lead to future kidney failure, that occurs much less frequently, maybe one or two in a thousand babies.

Host Amber Smith: Now these birth effects, is there a genetic cause for them?

Scott Schurman, MD: Sometimes there is. Sometimes there is an inherited abnormality in which one of the parents will have a problem or a predisposition to a problem, and it'll be inherited by their children. Most of the time, there's not such a direct link. There's a genetic predisposition sometimes, but sometimes there's not even that.

Host Amber Smith: Is it common to see infections that lead to kidney disease in children?

Scott Schurman, MD: Urinary infections in infants and particularly young children are common, much more common in girls than in boys. They sometimes can lead to very high fevers, sometimes even requiring hospitalization.

It's rare, though, that urinary infections lead to severe kidney scarring that could eventually lead to things like kidney failure, reduced kidney function. Most of the time the infections can be treated effectively without that.

Host Amber Smith: Now in children, do you see diseases like diabetes or high blood pressure causing kidney disease like they might in adults?

Scott Schurman, MD: It's definitely different than in adults. Over half of the adults on dialysis have diabetes. Most of them have Type 2 diabetes. Most children, not all, but most children with diabetes have Type 1 diabetes, in which their bodies aren't making insulin properly. It's rare for that to cause severe kidney disease in childhood. When they become adults, it certainly can, but it's rare for them to need something like kidney dialysis or a kidney transplant in childhood.

Hypertension is more and more common in children, as we've seen children become heavier and heavier, like our population in general. Hypertension leading to reduced kidney function, while fairly common in adults, is fortunately uncommon in children. Most of our children with reduced kidney function were either born with abnormal kidneys, kidneys that were born with a reduced number of filters from typical, or they developed some other type of kidney problem before age 10 or when they were teenagers.

Host Amber Smith: Do you ever see kidney damage from injuries in children?

Scott Schurman, MD: That's super rare, fortunately. The the risk of significant traumatic injury is less than one in a quarter million. So in the many years I've been practicing, I've seen renal injuries with various athletic activities, but they are extremely, extremely rare.

Host Amber Smith: Now, what are the signs and symptoms of kidney disease in children? How would you, or how would a parent, know that the child had something wrong with their kidneys?

Scott Schurman, MD: The majority of children born with abnormal kidneys have that discovered on ultrasounds done before birth. So, most mothers have an anatomic screening ultrasound done around 20 weeks gestation. And those ultrasounds can pick up the majority, not all, but the majority of severe structural kidney abnormalities. We then are alerted to that and see the children shortly after birth and then follow them. Some of them ultimately require some type of surgical intervention that may reduce their problems early on, but they might have problems then later in life.

Children, though, that are born with normal kidneys, structurally normal kidneys, and then later develop kidney disease, the signs and symptoms relate largely to some abnormality in how the filters of the kidney function. Each kidney on average has around a million filters, and those filter the fluids and waste products that make up your urine and keep the things you need, like blood cells and proteins. And if those filters are leaking blood cells and proteins, that can lead to symptoms. So in certain cases, they'll be leaking enough blood that you'll see that the urine is red or brown, and that generally leads to some presentation.

Sometimes the kidneys will leak a large amount of protein that ultimately will lead to some fluid accumulation and edema, in a condition called nephrotic syndrome. And that leads to some presentation.

Host Amber Smith: This is Upstate's "The Informed Patient" podcast. I'm your host, Amber Smith. I'm talking with pediatric nephrologist Dr. Scott Schurman about pediatric kidney disease.

So a lot of your patients have the diagnosis before they're even born, so that you know to start caring for them after birth. But some of them develop symptoms later in childhood.

Scott Schurman, MD: That's right. We see many infants. Now, the majority of infants we see with some structural abnormality have some minor abnormality. We follow it for a period of several months to a few years, make sure that it's mild and almost always getting better and there's no long term consequences to those children.

Others have more significant, structural abnormalities. They'll be born with much fewer than that 1 million filters per kidney, and eventually those filters wear down. Everyone, their filters in their kidneys wear down as they get older, and if you're born with fewer, they wear down faster. And so we can monitor those children. We have various interventions to help keep them healthy, to make sure they're growing properly, that their bones are developing normally, that they don't develop anemia and the significant symptoms that in the old days would occur when the problem was silent and the children would present very ill, later in childhood or as early adults.

Host Amber Smith: Now, when we talk about kidney disease, are both kidneys affected?

Scott Schurman, MD: For sure. If you have reduced kidney function, we talk to our patients about their kidney function number. It's called the estimated GFR. That's glomerular, which are the filters. Filtration. And R is rate. And a normal GFR number is greater than 90. Someone needs something like kidney dialysis or a transplant roughly when they're less than 10 to 15.

We, talk to them as they get older, what their kidney function number is and what they can do and what we can do to try to slow the loss of kidney function over time.

Host Amber Smith: Well, let's talk about what's done for treatment of kidney disease. Are there medications that help?

Scott Schurman, MD: Yeah. So, you are born with the number of filters that you will have through the rest of your life, so we don't yet have the ability to make new filters. That may be something that is in the distant future, but it's not something that's anytime near reality. So if you're born with reduced number of filters, we don't have a treatment that can change that, but we can do things to slow the loss of kidney function over time. In particular, we are very aggressive, making sure the blood pressure is normal, because high blood pressure can lead to faster deterioration in kidney function.

We monitor things like urine protein and can intervene as a higher urine protein is a sign that the filters of the kidney are wearing down faster. We're very aggressive with nutrition to make sure the children are growing properly, but not getting significantly overweight, as that can put significant stress on the kidneys.

We have abilities to prevent anemia, which can occur as the kidneys deteriorate significantly. We have treatments to help prevent bone disease, which can occur as the kidneys deteriorate significantly. We have growth hormone therapy to make sure the kids grow properly, so that when I started doing this work 30 years ago the children invariably were very, very short. And now they invariably achieve a typical normal height.

So we do have things that we can do to not cure the condition in that circumstance, but keep the kids healthy, until time that they may need something like dialysis or then kidney transplant.

Someone who it develops some type of kidney disease as they get older, depending on the condition, there are often treatments that can hopefully reverse the condition.

Host Amber Smith: It sounds like there's a lot to monitor in these children.

Scott Schurman, MD: Yes. So the kidneys and what they do touch pretty much every other organ. They're important in child development and proper neurologic functioning. They affect blood pressure, so they're very important in cardiovascular health. They affect various endocrine functions and the proper development of your bones and growth. And so we monitor all those things in the context of their kidney disease.

Host Amber Smith: How do you tell when kidney disease progresses to kidney failure?

Scott Schurman, MD: So we, again, monitor blood work and can then use that to calculate, estimate this GFR number that gives us a good approximation of where they are in the process and what kind of complications we can expect through that time. It also allows us to provide a dialogue with the kids and their families of where they are in the process, how fast or not fast it's progressing, so that they can be prepared for the steps through this process.

Host Amber Smith: Does kidney failure mean dialysis is next?

Scott Schurman, MD: If the kidney failure advances to a point in which it is affecting your ability to process waste products properly, affecting your ability to take proper nutrition, to have the kind of energy you need for daily functions like going to school or later going to work, then the next steps are what's called kidney or renal replacement therapy. And those are dialysis and then ultimately kidney transplantation.

Host Amber Smith: Well, let me ask you to walk us through how dialysis works.

Scott Schurman, MD: Sure. So in particularly in children, we stress to the kids and their families that dialysis is a bridge to a transplant. And sometimes we're able to go directly to transplant without the kids needing dialysis. But sometimes the circumstances are not right for that, and they will need a bridge of dialysis.

There are two modalities, we call them, types of dialysis. Many people in general are familiar with what's called hemodialysis in which blood is taken out of the body and it is pumped through an artificial filter, in essence, an artificial kidney that removes waste products and excess fluids and returns the cleaned blood to the patient. That requires usually a large IV to take the blood out and return it. The children are hooked up to a machine usually three days a week, four or so hours per treatment. It can take a lot of out of them, and it's definitely burdensome for them and their families in terms of normal functioning, normal school and that sort of thing.

We're fortunate here at Upstate to have our own pediatric hemodialysis center, so that we can focus on kids. We have a team of really expert nurses and support staff to be able to do that, and get them ready toward transplantation.

About a quarter to a third of our patients on dialysis get hemodialysis. The remainder get what's called peritoneal dialysis. This is a treatment that we train families to be able to do at home and allows really a more normal life for the kids and their families, including school and other activities. A catheter IV, in essence, is placed in the sac that surrounds the bowels. That sac has thousands of tiny little blood vessels, and there are small machines, about 20 pounds, that we perform the dialysis overnight while the kids sleep. A sterile fluid is pumped into this sack. It draws waste products out of these tiny little blood vessels, and then that is drained out. And that happens continually, overnight again, and then they're unhooked and can go about their day.

And then we see those patients in our office once or twice a month after they've been trained and stable.

Host Amber Smith: How long can dialysis be effective in kids?

Scott Schurman, MD: Well, it can be done and effective for years for sure, but it's rare for us to have a patient that's on dialysis for years and years because, again, it is largely a bridge to a transplant.

Kidneys are much better than dialysis at getting rid of waste products and providing what is necessary for normal function. So kids who get transplants do generally better in school. They are more likely to be employed later in life. They live longer, in general, than persons on longer periods of time on dialysis.

Now there are circumstances in which dialysis is necessary, and sometimes there are circumstances in which transplant is not feasible, at least for a period of time. And so dialysis is definitely a workable bridge. But our goal for all of our patients is to get a good functioning transplant.

Host Amber Smith: So they join the transplant waiting list when they go on dialysis, is that right?

Scott Schurman, MD: That's generally correct. Some of our patients are fortunate enough to have a family member or a close personal friend of the family that is willing, able -- able meaning healthy enough and compatible -- to be a living kidney donor. We work hard to try and identify potential living donors, as living donor kidneys generally work immediately and give a best chance of long kidney function to our kids. Sometimes, though, there is not a family member or close family friend that is able to donate. And so yes, our kids go on what's called the deceased donor transplant list.

You know, this is donors that have died under unfortunate circumstances, such that their kidneys and usually other organs are still functioning well, and their families have agreed to donate their organs.

Children under 18 do have some advantages on the list, in terms of being able to get a good functioning kidney sooner than adults. That was done intentionally just probably three or four years ago, in recognition that younger persons are going to need a good kidney for a longer period of time, and that prolonged periods of time on dialysis for children can have more effects on their development, schooling and future functioning. And so they are provided those advantages on the list.

Host Amber Smith: So are children able to accept a kidney from an adult?

Scott Schurman, MD: The adult donors are actually preferred. So even in children who are say, 18 months to 2 years and need a kidney, children usually have to be around 20 pounds. That's usually the minimum size in which kidney transplantation is technically feasible. Sometimes a little lower than that, but right about that threshold. And even at that age, we prefer kidneys from adults. Now at that size, we'll usually do a smaller adult.

And that is because if you transplant kidneys from children into children, the blood vessels of each are very small, and that can technically be very challenging and lead to certain complications. So generally we prefer adults. Not always. There are certain circumstances in which we'll use younger donors, depending, but generally adults.

Host Amber Smith: You're listening to Upstate's "The Informed Patient" podcast. I'm your host Amber Smith. I'm talking with pediatric nephrologist Dr. Scott Schurman about kidney disease in children.

Now, is the transplant expected to last a lifetime in a child?

Scott Schurman, MD: No, they definitely don't generally last a lifetime. There's been significant improvements in the transplant functioning and the length of time in which they work.

When I started this work, again, over 30 years ago, the one-year survival for a deceased donor kidney transplant in a child under age five was 68%. So 32% did not work even one year after transplant. Now that is 99%, OK?

Host Amber Smith: Wow.

Scott Schurman, MD: So there's been tremendous improvement in surgical techniques, medications to prevent rejection. And so now the average length for a kidney is probably 15 to 17 years in children. So not forever.

We tell our kids that we are going to do everything we can to make this kidney last as long as it can. And then someday they're probably going to need a second one.

There's been some very exciting developments that have been in the news over the last year in terms of what's called xenotransplantation, using kidneys from genetically engineered pigs and using those eventually in humans. And there's some clinical trials now beginning for that, and I'm very optimistic that in the next five, 10, 15 years that this will become fairly routine. And that's very exciting for our patients because our patients are going to need more than one transplant. And it really provides a lot of optimism for our kids and our families.

Host Amber Smith: Well, let me ask you what life is like for a child after a successful transplant. There's no more dialysis, right?

Scott Schurman, MD: That's correct.

Host Amber Smith: Do they still have, quote unquote, kidney disease?

Scott Schurman, MD: About three quarters of kids after transplant have high blood pressure, so we're still treating that.

All of them have to be on medications to prevent rejection.

I tell our kids, "Look, you're going to have to take a handful of pills in the morning, a handful of pills at night. You're going to have to come see me and get blood work periodically. And then otherwise I want the rest of your life to be your life and for you to be able to do what you want to do." And that's pretty much the case.

Now there can be circumstances in which they get certain types of infections, and we monitor for that. And the kidneys generally slowly wear down over time. But for the most part, their lives can be pretty normal.

Host Amber Smith: So they can do activities, there's no restrictions on activity, or diet? Are they able to eat a regular diet?

Scott Schurman, MD: Yeah, pretty much a regular diet. It is important they stay a healthy weight. Being particularly overweight puts a lot of stress on the kidneys. But there's been two men that have played basketball in the NBA after a kidney transplant. So after a successful transplant, I think you can pretty much do almost any activity.

Host Amber Smith: How long do pediatric nephrologists continue caring for a child who received a kidney transplant?

Scott Schurman, MD: Generally we see them until around age 21 or so. I do have some young people I see beyond that, depending on the circumstance. And we've got amazing families, and so it's very bittersweet for them to move on, because they are an amazing part of our lives as well. But that's generally about when we try and transition to the adult teams.

Host Amber Smith: Well, Dr. Schurman, I appreciate you making time for this interview. Thank you.

Scott Schurman, MD: Oh, it's my pleasure. Thank you.

Host Amber Smith: My guest has been Dr. Scott Schurman. He's an associate professor of pediatrics at Upstate and the director of nephrology.

"The Informed Patient" is a podcast covering health, science and medicine, brought to you by Upstate Medical University in Syracuse, New York, and produced by Jim Howe, with sound engineering by Bill Broeckel and graphic design by Dan Cameron.

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