S2E2: "You're Not Alone", CAKUT with Vincent Ko

The Kidney Collective™ co-hosts Tamara Ruggiero and Mike Spigler are joined by Vincent Ko, founder of KidneyFuture (formerly the CAKUT Foundation), to discuss congenital abnormalities of the kidneys and urinary tract (CAKUT). As a parent of a child living with CAKUT, Vincent shares how he felt learning about his daughter's diagnosis before she was even born and how he has used his personal experiences to create KidneyFuture. 

The three discuss how CAKUT differs from other types of kidney diseases and the unique challenges people living with CAKUT face, including navigating the transition from being a pediatric kidney patient to an adult patient. Vincent also shares how he discovered the two greatest desires of pediatric nephrologists and of CAKUT patients/parents are aligned: (1) a better understanding of whose CAKUT progresses and whose doesn't and (2) more therapies that are safe and effective for children living with CAKUT. He also explains to Tamara and Mike how the kidney community may not be too far off from those goals.

More resources: 

• Congenital Abnormalities of the Kidneys and Urinary Tract (CAKUT) 
• Caring for someone with kidney disease 
• Kidney disease in children 
• Camp Connections
• Kidney Kitchen® 
• Genetic testing and counseling for kidney disease 

Transcript

Vincent Ko 0:00

When I talk to parents, they're like, oh, you're you're like the first parent that I've talked to that has had the condition. I thought I was all alone. And I think the one thing is that I just want to say that like you're not alone. Actually, collectively, congenital kidney diseases like CAKUT actually make up close to the majority of what they see in a pediatric clinic. There's great research out there, and there's ultimately community out there as well.

LaVarne Burton 0:28

Hello, I'm LaVarne Burton.

Tamara Ruggiero 0:31

I'm Tamara Ruggiero.

Mike Spigler 0:32

And I'm Mike Spigler.

LaVarne Burton 0:34

Welcome to season two of the American Kidney Fund's Podcast, The Kidney Collective, which gives an intimate voice to the many facets of living with kidney disease.

Tamara Ruggiero 0:44

Our theme for this season is Rare Voices Relatable Stories.

Mike Spigler 0:48

Over the next few months, we'll be talking about rare kidney diseases and explore the shared experiences that connect the entire kidney community.

LaVarne Burton 0:55

Join us for these candid conversations that offer both information and inspiration.

Mike Spigler 1:05

Welcome everyone. On this episode of the Kidney Collective, we are joined by Vincent Ko. Vincent is the executive director and founder of Kidney Future, formerly known as the CAKUT Foundation, an organization dedicated to bringing together patients and families living with CAKUT with clinicians, researchers, and advocates to improve outcomes for people living with CAKUT.

Tamara Ruggiero 1:25

Just to start, what is CAKUT? What does that acronym stand for?

Vincent Ko 1:28

CAKUT stands for congenital abnormalities of the kidney and urinary tract, but it's really just a fancy way that doctors referred to pretty much anyone or a kid that's born with a kidney difference. So that could be a kidney or urinary tract difference that ultimately affects their kidneys as they grow up.

Tamara Ruggiero 1:45

And you're the founder of Kidney Future.

Vincent Ko 1:48

Yeah, that's right.

Tamara Ruggiero 1:50

And so what tell us what inspired you to found Kidney Future?

Vincent Ko 1:54

It all stems from my experience as a father. So my daughter was born with a kidney difference or CAKUT. And for me, just like a lot of other parents, it was about finding answers. So finding out what's going to happen to my daughter, what's going to happen in their future. And it ultimately came down to asking uh kidney doctors what they wanted. And we found out that there was a very big void in the space, uh, despite it making up the majority of pediatric morphology. Apparently, no one's working on it. So decided to take the reins and started the foundation.

Mike Spigler 2:24

How did you find out about her diagnosis? I mean, was this something that you find out kind of in utero or after she was born? And anytime you find out anything is happening with your child, obviously it's a it's a very emotional experience. I'm curious kind of how you dealt with that, what your reaction was.

Vincent Ko 2:39

Yeah, so the majority of parents in modern day times, they all usually find out through a prenatal ultrasound. And unfortunately, it happens uh as the mom is pregnant. You know, the doctor comes in and says, you know, we see something uh of concern. In some cases, it resolves itself, but in other cases, uh, like the those in our community, unfortunately it leads to something that uh is confirmed once a child's born.

Mike Spigler 3:02

And had you ever heard of what she was dealing with before? Had you ever heard of CAKUT?

Vincent Ko 3:07

Actually, I didn't hear about CAKUT, the term CAKUT, until maybe a couple years into the journey. I think as parents, we hear about your kid was born with one kidney, abnormal kidneys, they have a blockage, they have uh a UTI, uh, these kind of general terms. Um, it was only until a doctor about five years later actually explained to me that CAKUT is this lump bag term that the doctors use. So I had never heard about it. Um and I never heard or met anyone that was born actually with a kidney difference.

Mike Spigler 3:37

You know, it's fun, it's funny because I will go out in the community sometimes or just even at like uh, you know, a dinner or something and meeting people socially, and they'll ask where I work, and I'll say that. And more people than I would expect will say, you know, I was only born with one kidney, or I have three kidneys. It, you know, it's it's something that people don't seem to really talk about that much, but is I think more prevalent, at least, you know, in the circles that I run in, I guess.

Vincent Ko 4:00

Yeah, absolutely. And I've I've had that same experience too, where you're at a dinner and you're saying, Oh yeah, I have a sister that had this, or I myself have was born with a kidney difference. It's more common than we think.

Tamara Ruggiero 4:10

What was it? If you could just talk through a little bit about what your experience was like when your daughter was born, um, being, you know, was she your was this was your first child?

Vincent Ko 4:20

Yeah, so it was my first child. Yeah.

Tamara Ruggiero 4:22

That must have been a very emotional. You probably went through all kinds of emotions when she was born, learning that she had a kidney difference. Talk through what that experience was like.

Vincent Ko 4:32

Yeah, so it was a double whammy. It was right in the middle of COVID. So not only did it feel like the world was going kind of crazy, at the same time, um, we had a daughter that was on the way, and we knew that we would have a series of hospital visits. And I think, yeah, the one thing that unfortunately unifies the community is that it's this fear, uh fear of the future, fear of the unknown. Um, because it's your child. You want them to have a good life when they grow up. But when you hear this, your mind only wanders of what's going to happen or even the negative scenarios. So, yeah, the first reaction truly was fear, unfortunately.

Mike Spigler 5:03

And you know, you mentioned kind of the the acronym meaning and the congenital, you know, obviously for those listening, means that this was something that she was born with and may run in the family. Does anyone else in your family have kidney or urinary tract issues that you know about?

Vincent Ko 5:17

Yeah, great question. So no one in our family has it. And through our foundation, we have found out that actually for CAKUT, this lump bag term, about 10 to 14% can be tracked back to genetics that we understand today. Uh the remaining 80 or so percent, uh, we either don't know enough information or it is just random. So in our particular family, we did genetic testing. Um, that genetic testing enabled us to have our second child because it wants to genetic. Um, but in our case, yeah, parts of the community are genetic based, and then the remaining are not.

Mike Spigler 5:50

Did you have to push hard or convince the doctors to do the genetic testing? Was it something they offered, or did were you the one that had to kind of push for it?

Vincent Ko 5:58

Funny story. So actually, uh through our foundation, we were looking at actually a genetics testing company who was looking to partner with us. And part of that experience, I actually just asked my doctor, I said, Have you heard of this genetic testing company? Uh, we're about to possibly work with them. Do you know about it? And the funny thing is, she said, actually, we have boxes right here. Let me go get one. So they're like in the back, they they existed. Uh, it's just like not top of mind when it comes to congenital kidney disease. And so it's not the top thing that doctors think about right away.

Mike Spigler 6:28

You know, one of the things that come up very often in in genetic testing is something where there's a there's a gene variant that's um uh you know, maybe not one-on-one associated with the disease, but there's there's something that they're tracking, and they usually call that a variant of unknown significance or VUS. Did you encounter any of those?

Vincent Ko 6:45

Oh yeah, uh no. So I think right now with CAKUT, if you take, for example, like a Natera test or I believe an Invitae TAP test, uh, so there are actual genes that are correlated with uh kidney differences or CAKUT. And so from my knowledge, I'm not familiar with um variants of unknown significance at the moment.

Tamara Ruggiero 7:04

Can you talk to us a little bit about how your daughter is doing now and how this how this has affected her life and how is she progressing? How is she managing her kidney difference?

Vincent Ko 7:14

I think that this may be also for other parents with kids that are born with kidney differences. I think in her mind, there's nothing different about her. Um I think the one thing that differentiates our community versus maybe other communities or school married or adults is the fact that as parents, you want your kids to be normal, so you try as much for them to be normal. So there's a chance, you know, she may never even know that she's different. Um, maybe when she's 18, she realizes I went for in for a lot of blood draws. I saw the doctor a lot more than my friends. Um, but our goal as parents from day one has actually to make her as normal as possible. Interestingly enough, before starting this foundation, I would say four people knew about my daughter's condition, my parents and my parents-in-law. That was it. Um, and I think that is probably also the case with a lot of families. We kind of um keep it to ourselves. And so, yeah, my daughter, we don't do anything different. The main thing we do differently is obviously go to the doctors a lot as well as maintain diet um and things like that.

Tamara Ruggiero 8:14

That resonates with me. My nephew was born um missing part of his right arm. And his his, you know, at first it was such a shock, and his parents went through all of the emotions, and he's now 16. And in the family, we don't even really think about his arm. We don't even notice it. And he does everything perfectly normally, you know, he has no issues. They have to restrain him from taking chances on things. He's he's kind of a daredevil. He told my brother he'd like to go into extreme sports when he grows up, which I don't know. We'll see about that. But it's interesting how kids adapt and it's what they know and it's what they live with, right?

Vincent Ko 8:57

One of the challenges, though, I just came back from a pediatric nephrology uh conference. And one of the challenges that was described is that for other diseases where it may happen later in life, maybe when they're an adult or team, they can kind of understand what's going on. The issue with CAKUT and congenital kidney disease is the kid doesn't know. And so that transition time is actually really difficult. So when they transition from adolescence and they go out of the nest, uh adherent and understanding, so it's a double-edged sword.

Tamara Ruggiero 9:22

That's a big issue going out of the nest and being adherent with their medications. How are you thinking about preparing your daughter for that?

Vincent Ko 9:31

Great question. My daughter's only five. Um, so I haven't thought about that. Others in the community have thought about it. But yeah, I know it's a challenge for sure from others that I've heard.

Mike Spigler 9:39

If there's anyone listening, another parent that's just finding this out, you know, what advice do you have from them kind of on the early stages of this and and kind of adapting to this new reality?

Vincent Ko 9:49

I hear this over and over again, but like when I talk to parents, they're like, oh, you're you're like the first parent uh that I've talked to that has had the condition. I thought I was all alone. And I think the one thing is that I just want to say that like you're not alone. Actually, collectively, congenital kidney diseases like CAKUT could actually make up close to the majority of what they see in a pediatric clinic. And so, one, you're not alone. And two, um, find resources, find resources like our foundation, find resources like uh an American Kidney Fund. Um, there's great resources out there, and there's ultimately community out there as well.

Mike Spigler 10:21

Well, that that's a great transition. And, you know, uh a nonprofit like yours that is really aiming at trying to bring, I mean, lots of things, but one thing that I think is so valuable is bringing people together that really have not met someone else that that is dealing with this. Um, I'm sure that's probably one of many reasons, but could you talk a little bit more about Kidney Future and why you founded it?

Vincent Ko 10:41

It was actually founded by my old nephrologist. So my daughter was born abroad, and in a recent trip back, um, actually have like this model pen. I literally have this pen. And I literally asked her in the meeting, I was like, I was just curious. I was like, imagine this is a magic wand. What does it do for pediatric nephology? And like she didn't hesitate. She's like, You really want to know? I was like, Yeah. She's like, your daughter falls into this bucket we call CAKUT. The first time I ever heard the term, by the way. And she says, This makes up the majority of what we see in clinic. And I have two magic wands, like without hesitation. She said, one, I want to know within the CAKUT bucket who progresses and who doesn't, because by the time they start losing kidney function, it's too late. Uh and number two, I wish for more targeted therapies. She said that all the medications we have right now in pediatrics are blunt. And she was referring to uh like high blood pressure medications. She says, I wish for targeted therapies. And I was like, Okay. Uh and I went back to the United States after that trip to Asia, uh, and I asked my American nephrologists, and they said the exact same thing. And then I would just go around the country and I would hear the same thing over and over again. And then I talk to parents, and you ask parents, what are your two magic wands? And they'll say the exact same thing. I want to know, is my kid gonna progress or are they not gonna progress? Are they in the highest bucket, lowest bucket? What's gonna happen to my kid? And number two, one day if something goes south, kidney preservation is my number one priority, and I want therapies there. And so when we realized that the doctors want the same thing, the patients want the same thing, then I spoke with some doctors and asked if anyone anyone is working on it, and they said no. Uh, but other patient foundations had really paved the way for future therapies and understanding their disease. So uh that's ultimately what led to us creating Kidney Future along with some other parents.

Mike Spigler 12:26

Well, a crowdsourced and consensus mission sounds like a very good one. So, congratulations on doing that.

Tamara Ruggiero 12:32

So, what are some of the projects Kidney Future is working on now?

Vincent Ko 12:35

The very first thing is obviously uh the patient voice. And so we're not trying to overlap on resources. Um, so for example, there's like a great diet advice at American Kidney Fund with uh the Kidney Kitchen uh and other resources. We're trying to build resources that we don't see, uh that parents want. So the first thing would be simple medical explainers using fifth grade language on various terms like uh protein in the urine or living with one kidney. And it's for the kids, but we found out it's actually as much for the parents because when you bring it down at a fifth grade level, uh they get it. Uh, number two, we have children's books and stuff like that. Once again, it's for the kids, but also it says as much for the parent to understand what's going on. So that's on the patient advocacy side, bringing the kidney together. Uh on the other front is that those two magic wands that I mentioned, uh, who progresses and doesn't and why. And number two, uh therapies. And so what we have been doing is bringing together the community. So whether that's uh parents, patients, researchers, academics, but also people like industry, pharma companies, regulators, because they really make up this ecosystem that allows us to have hopefully kidney protective therapies one day. So we spend a lot of time as our foundation really making sure that that community comes together to try and solve those problems together. We're making progress, which is pretty exciting.

Mike Spigler 13:55

Fantastic. Can you talk a little bit about that, especially on the research side? Because, you know, especially when there really has not been a lot of innovation and progress in an area, sometimes it's hard to even know where to start. I've definitely talked to a lot of organizations where kidney disease organizations starting, and I'm always amazed about you know the power that that they have to bring these researchers together. Tell me kind of like your plan of attack and your strategy for how you're doing that.

Vincent Ko 14:19

Yeah, absolutely. So we'll go back to one of the very first magic wands, which is like in CAKUT and congenital kidney diseases. Is there a way for you to, doctors, to realize which kid is going to go downhill and not earlier before they've already lost kidney function? And actually, just came back from a conference uh for academics uh in Miami last week. And I heard a doctor from Yale speak on this, and you know, they've been researching this for their career. This is what they do. And it's very interesting to see that actually there may be someone in at Yale, there might be someone in Boston, and there might be someone in Heidelberg, Germany, all working on the same thing. And our goal is to bring those people together so collectively they can all answer the question. And so if Europeans see it and in Asia they see it, in America, they see it, ultimately that's how science moves forward. And so our goal is really just to unify the science and then hopefully answer that first magic wand who's gonna progress, who doesn't, how can we intervene earlier. On the second one, it's therapies, and therapies is really bringing together uh industry, which we know as pharmaceutical companies, they make therapies. Unfortunately, the way it is currently, a lot of therapies are only for adults. Um, and we are trying to figure out whether those therapies make sense for our community and whether they are safe and effective because children are precious, children are the world to the parents, so we need to make sure that is the case.

Tamara Ruggiero 15:36

Yeah, I was gonna ask about that. I know that uh cancer therapies, for example, are are very harsh on children oftentimes. And is the same true for kidney therapies?

Vincent Ko 15:46

So, in short, for CAKUT, a lot of the therapies that we are looking at are therapies that are have been proven to be safe and effective in adults. And so CAKUT is not like an autoimmune disease, it's not like IgA Nephropathy. It's actually believed to just be people are that are born with less nephrons or filtering units, and it's just hemodynamic, meaning they just need to make sure their kidneys last a long time. So a lot of therapies that are standard of care in adults, like SGLT2 inhibitors, MRAs, these are classes of existing therapies, are very likely the first therapies that we are looking for for our community. So it's really about getting adult therapies and making sure that the ones that are safe and effective are safe and effective in children. It's not novel drugs, it's not new. It's really just making sure where you are either the second, third, or fourth indication for a therapy.

Mike Spigler 16:37

Vincent, we talked about the importance of and and the the beauty of really connecting parents to understand this, but you know, there's so few kids that are dealing with kidney problems overall, whether it's it's a rare kidney disease or a congenital issue. Is that something that you're looking at with Kidney Future as well as kind of bringing kids together? I mean, we've found we have uh a virtual camp that we run uh for kids that are on dialysis or have gotten a transplant. And it is just amazing to see them light up and the bonds that they form with each other, despite being potentially even on different coasts, right? To do that. I mean, um, have you seen that? And do you have plans with kidney future to try to get those kids together as well?

Vincent Ko 17:17

Yeah, great question. So, this is an example of uh do not replicate, do not duplicate. So, I mean, in an ideal world, I think we at that center place, but I would love to actually refer kids to your camp. I've seen the camp. It looks super cool. Kids come together, and whether that kid has born with kidney disease or whether they acquired it from something like a glomerular disease later, I think ultimately they'll be able to relate with one another. So we do not have any plans to run summer camps. Um, you shouldn't you shouldn't try and beat something that's already been doing going really, really well. So if it's anything, we would actually probably be one of your top prefers in the future.

Tamara Ruggiero 17:53

Yeah, we have some great stories from that camp. We have uh two little girls that we call them the two Emmas. They live far, far away from each other. They both have uh cystinosis, and we brought them to our gala, The Hope Affair this year, to meet together on stage. They've become best friends as a result of being in the camp, which is just such a great story.

Vincent Ko 18:11

Absolutely, yeah. Shared experience at the end of the day is what connects people, right?

Tamara Ruggiero 18:15

Yeah. What about um bringing the researchers? You mentioned bringing together researchers from all over the world who are working on researching um in this area. What has that meant for for researchers? What do you hear from them about the ability to collaborate with each other?

Vincent Ko 18:30

For a lot of these researchers, for a lot of those in pediatrician nephrology, they understand that this has been a huge missing piece and makes up oftentimes the majority or at least the number one reason what they see in clinic. And so, as far as gathering the community, it actually has been pediatric nephrologists are like the nicest people. And so when they hear about this movement, um, they are all willing to help. Every doctor has been willing to introduce me to someone, introduce our foundation to someone, uh, join, support our medical advisory board. And so we found that actually uh it's a mission that a lot of doctors can get behind, especially pediatric nephrologists. So I don't want to say it's been easy, but I've we just had a lot of support from the community.

Tamara Ruggiero 19:10

It's probably been very much welcomed, it sounds like.

Vincent Ko 19:13

Exactly.

unknown 19:13

Yeah.

Mike Spigler 19:14

Now that you've talked to lots of parents, you've you've talked to the researchers, it seems like you're you're really doing a great job of bringing the right people together. Let me ask you a moonshot question. You know, we talked about cancer. There's there's a cancer moonshot program that's been started. Looking ahead over the next decade or two, what do you think is one breakthrough that would be a meaningful inflection point for your community?

Vincent Ko 19:35

If you asked me a year ago, it would be that magic wand question that I asked that doctor in Asia uh a long time ago. It's can we predict who is going to progress earlier before it's too late? And are there therapies that can really protect the kidneys or at least uh help with the decline? That would have been like the 10-year moonshot, 15-year moonshot. A year later, I would say, with the community coming together, I I would say it's not even a moonshot at this point. I say that I can see the future. I would say, what's the term for moonshot, but a five, 10-year moonshot? I don't know what that is. Like uh stratosphere shot. I'm not really sure. Stratosphere shot, yeah, yeah.

Tamara Ruggiero 20:13

Asteroid shot.

Vincent Ko 20:15

It seems it seems doable. Um, and I will admit that a lot of this is not because we are special, uh, we follow the tracks that have been laid by other patient foundations, whether it's a NephCure or whether it's an Alport Foundation or the IgA Nephropathy Foundation, they've done it. They've cleared the path. And so I think if we had done this 10 years ago, yeah, it would be a moonshot. But because other patient foundations have accelerated and cleared out that forest, the path is quite clear. So that moonshot, I would say five to ten years, not a 20-year moonshot.

Tamara Ruggiero 20:48

Well, Vincent, thank you for being here with us today.

Vincent Ko 20:50

Yeah, no, thank you for having me.

Mike Spigler 20:52

And Vincent, if anyone is is uh not looking at the show notes and they would just want to listen to this and type it in, what is the link to your to your website?

Vincent Ko 20:59

KidneyFuture.org.

Mike Spigler 21:02

Well, Vincent, thank you for being here. We really appreciate it.

Tamara Ruggiero 21:06

To find out more about the topics we discussed in this episode, or to access resources to learn more about kidney disease, go to the episode show notes on your podcast listening platform, or go to kidneyfun.org slash podcast. And if you haven't already, be sure to subscribe or follow the kidney collective wherever you listen to podcasts, and leave us a like or review to help us reach more members of the kidney community.

Mike Spigler 21:28

You can also follow AKF on all major social media platforms, including Facebook, Instagram, TikTok, Blue Sky, LinkedIn, YouTube, Twitter X, and whatever comes next. Join us next time. Remember that even in the toughest moments, there's hope ahead, and the kidney collective and AKF are here for you now.