Pediatric Blood Disorders

hello everybody. Welcome to Health chatter today's show. We're really digging deep into the medical arena. Today, our show is on pediatric blood disorders, and we have a great guest with us, Dr Kate garland. I'll introduce her in just a second,

but it'll be interesting, because, frankly, I certainly don't know much about this, this arena, but hopefully by the end of the show, everybody will be a little bit more knowledgeable. I want to thank our crew. As always, there's their second to none. Maddie Levine Wolf, Aaron Collins and Deandra Howard do our background research. Sheridan Nygard also does research for us marketing and helps us with recording. Matthew Campbell, our production person. And of course, there's Dr Barry Baines, who's our physician consultant on our show and provides us with good medical insight. Then of course, there's Clarence. Clarence Jones and I have have partnered on this for now close to three years, and it's been a it's been a wonderful endeavor. And we really, we, frankly, even though we've been in the healthcare being a long time, I think I could speak for you too, Clarence, that you know, we've we've learned a lot along the way, and we hope you, you the listening audience does as well.

We also have a great partner sponsor for these shows, the health chatter shows, human partnership, great community health organization. You can check them out at human partnership.org,

and we can check us out at Health chatter podcast.com,

and you could you'll see all the research that we do for the shows there, as well as the transcriptions of the shows. If you'd like to read, as opposed to listen to podcasts, you can read about the information on our website. So thanks to everybody. So today we have Dr Kate garland.

She attended medical school in East Tennessee, State University. Actually, I was in Tennessee over the summer. It was, it's an interesting state to visit. She completed her pediatric residency and a chief residency year at Children's Hospital of the king's daughters in Norfolk, Virginia. She's, she's done a lot of research, and you can, you can tell it's one thing you know, as I've read her bio, it's one thing to to look at a clinician, but it's another thing to also see that they're they're involved in research, and my sense is, is that research is hopefully to help make a difference in these in this arena, she serves as a local principal investigator for multiple large, large national, International clinical trials, and she's being primed for a future co director role of the children's Minnesota hemophilia treatment center here in Minnesota, HTC, right hemophilia treatment center. So welcome to you, and thank you so much for being part of health chatter today. So let me get the ball rolling here, and then, believe me, everybody's going to be chiming in on this one.

You know, as I read through the background research that our crew provided here, it's like, all right, how is it that you identify

somebody who's got any of these pediatric blood disorders, do they present themselves with particular symptoms? Is it? Is it a function of testing, for instance, because there's genetics involved? Help me, help me identify how it is that you, as a clinician, identify somebody who's got a blood disorder. Well, thank you for having me everybody today. It's a true pleasure and honor.

So how do you identify somebody with a bleeding disorder? It can be a real or any sort of blood disorder really. It can be a real challenge.

First, I suppose you have to understand what a blood disorder is.

I think what we're going to be talking about today is things that affect the blood or the blood cells that are considered non malignant, so not cancer, not oncology,

and that doesn't mean that they are benign or doesn't affect somebody's life. But in general, if you have something of the blood, they can really be identified two ways. One, you have a family history of it, so maybe there is a genetic component that can be passed down from generation to generation or two, you are the brand new patient of the family who.

I happened to get this, it was sort of acquired, either genetically or as a result of being exposed to something outside in your life. And really patients come to us with these things, our hope as a clinician or as a doctor is to pick up on those patients who maybe have a family history of it, so we can counsel and talk about what the future risks are. But aside from that, really, people might come to us with, now I'm bleeding and I don't know why, or now I had a clot and I don't know why, or now I have this pain or rash and I don't know why,

and it's our job to figure out why.

So they a level of seriousness. Okay, so it's like, okay,

I'm bleeding all of a sudden. Well, okay, maybe that'll just heal itself. And thank you very much. But at what point does an actual patient present themselves to you? It seems to me that there's got to be this. Wait a minute, something's really not right here. So help me with that.

It can be anything from at birth to late in life. So for instance, if we're talking about bleeding disorders, or things in your blood that make you bleed more heavily, sometimes a patient could come to us because the parents noted, or a pediatrician noted that with the child's circumcision that they bled too much.

Sometimes they come to us because they are now two years old and bruising more easily now that they are running around. So there's they're covered in bruises, which, of course, can be sometimes misdiagnosed as abuse or missed as abuse, or sometimes you have teenage girls who really have had no problems throughout their life, and they start menstruating and now they have very heavy menses. But these things can be missed, because parents and clinicians alike can write it off as I bled a lot. They bleed a lot. I bruised a lot. They bruised a lot, and maybe it's not always picked up until later in life.

All right, so, you know, Clarence chime in on this one, because I, you know, obviously we're dealing with, you know, some diseases that that affect the the African American population.

Let's talk about

there's kind of this cadre of things here, but treatment Alright, so in general, our blood disorders treated pretty much the same way. Or does each specific blood disorder have a particular treatment that's maybe different from the other ones you stand can I let me jump into here real quick? Because I appreciate that, because I was going to, I was going to go, kind of go down this, this lane. I am African American, and all of my life, I have heard about sickle cell,

and I think that part of the the understand, I like to know is number one, I don't think that we know much about it, or it's not talked about much in our community. And so when we look on here about sickle cell, and totally honest, what I heard is that they have it, and they're probably going to die soon.

Okay? And so I would, you, would you kind of explain what it is, and then talk about, go back into the treatment of it, because I think that, you know, sometimes, like I said, it's like the kiss of death, you have sickle cell, you're going to die, you know, I mean, and so I know my, I know my question is not formed very well, but that's because, you know, I don't know. So I hope you understand where I'm trying to go. Absolutely. Let's talk about what sickle cell is, because it certainly is not benign, and it's scary, and it can be life threatening, and

it can be a big change for families when they know about or find out about the diagnosis. So in your blood you make cells. We all make three cells, white blood cells, that fight infections and carry our body's DNA or genetics. We make platelets that help us to clot and stop bleeding. But then we also make red blood cells. And red blood cells carry the oxygen throughout the body. So their job is to be a little round cell and pick up oxygen in the lungs, transport it throughout the body, deliver the oxygen to the tissues or the organs, pick up the carbon dioxide there, transport it back to the lungs so that you can breathe out the carbon dioxide. And then repeat, pick up an oxygen.

Okay, in sickle cell, there is, well, I should say, in general, in red blood cells, we all have something that sits inside the red blood cell and gives its shape. We call it hemoglobin. So this hemoglobin is a protein. So all proteins are made from your DNA. So you have inherited this DNA or genetic structure to build this protein called hemoglobin. Unfortunately, this hemoglobin needs to be a very specific structure in order to be able to do its job or function well, of carrying oxygen. And if you have a very slight spelling mistake in that genetic code which you can inherit, then it makes the hemoglobin less likely to hold on to the oxygen, or less likely to pick it up. And when it does pick it up or delivers the oxygen, it deforms. So when the hemoglobin or the red blood cell is not holding on to oxygen, the round red blood cell instead turns into a more sickle type shape. And if a sickle kind of has two points on the end, it kind of looks like a crescent moon. And those pointy ends can cause a lot of a lot of damage to the body. And obviously, if you did not carry an oxygen very well, that can also lead to a lot of damage and shorten your life.

So go ahead. So

you know, it's like,

you know, I keep having all these Whys in my head. Why? Why, specifically do we, we connect sickle cell

a lot with the African American population. What's going on here?

I don't I don't know if we know exactly why. We know that somewhere along the line, there was a genetic change in a human, and that has gotten passed down. And like anything, you get two copies of every gene, one from your mom, one from your dad, and if each copy of the gene is affected, then you are more severely affected, because you make no normal shape.

There are people that have theorized, and there is some racial bias in this, that maybe this genetic change occurred because a red blood cell that is not normally shaped, does not hold oxygen as well, and maybe is harder for certain diseases to infect it. So one of the more common diseases across the world, that is more prevalent in Africa and other tropical places, or I guess, along the equator, is malaria. So malaria can affect the red blood cells. That's how how it transmits. And if the red blood cell has a more sickle shape or a different shape to it, then maybe the malaria doesn't want to infect it as well. So some scientists theorize that maybe this genetic change occurred as part of evolution and made it harder for malaria to infect people.

It tends to malaria tends to occur in these areas across the world where there is more heavily a black population. And so if this has been going on for generations and generations, 1000s of years, then your black population that lives in these areas have been more severely affected. But that does not mean that anybody cannot have this disease. So if you it's genetic. So if you are a black person or a white person, it doesn't mean that you you can get this disease equally, depending on who your parents are and who are passing it on.

So, alright, so you've been in this, in this world for for a while.

Are you optimistic about

treatment? Are you optimistic about maybe prevention? I'm a doctor. I have to be optimistic. Okay,

alright, I'll give you that.

You know what I one of the things that that's interesting is that I remember, I want to go back to this, my own personal experiences, that I remember I had a person in my life that had sickle cell. They thought they person would, would would pass on when they were 21 but they lived to be 45 so I want

to get back to that thing that that, that statement about Stan and treatment, um, how have treatments changed in the last 20 to 30 years? We're talking about addressing this issue.

So you bring up a valid point that he's now living they're now living to 40 something, but that's still not the normal average. So this is an extremely morbid disease, as are others. The treatments have changed because we've learned more about the disease. We've learned more about.

The genetics. There has been a lot of scientific research in treating with genetics, in treating with making the hemoglobin more stable. So, for instance, 2030, years ago, maybe even a little bit longer, the only treatment we had was to give somebody blood that wasn't sickled. So to transfuse to give their body good hemoglobin.

We know that the life cycle of a red blood cell in a normal body is about three to four months, whereas if it is abnormally shaped, then your body is going to break it down or hemalize it sooner. So in a patient with sickle cell, not only are their red blood cells not lasting as long, but they're then getting transfusions. So when you get a transfusion, it's still not going to last as long, because by the time the blood's prepared, it's transfused, it just doesn't last as long in the body. So now you're getting a lot of transfusions, and now you're getting a lot of extra problems that come with getting transfusions. I'm giving you a lot of less extra iron. There's more iron in the blood. So now you have iron overload. Your organs have iron deposited on it.

That's the major side effect. But you're also not growing as well. You're not your whole your body's not functioning as well. So what we used to do is treat all the complications that went along with sickle cell by transfusing blood. If you happen to get a pneumonia, here's the treatment for pneumonia. If you happened to get a spleen problem, your spleen is the one that's breaking down the red blood cells. It's like a sponge. So the more

full it gets with degraded blood cells, the less it works. So you're more prone to infections. So now you're dying of diseases. So we're treating those infections. We used to take out spleens. Nowadays, now that we know a little bit more about the genetics of it, they're doing things like gene therapy. Now that is not without risk. I mean, I have to edit your own genome and wipe out your immune system so that your own immune system doesn't attack the new genome.

We are giving antibiotics preventatively to prevent infections. We are making sure our patients are vaccinated, we are there are some some medicines that we can use. There's an old chemotherapy medicine. Chemo is the term for

adjusting your immune system to so it doesn't attack itself. But there's one called hydroxyurea that we have been using for the last 20 to 30 years,

that helps to stabilize that hemoglobin shape so it doesn't deform.

We've started using that younger and younger so that patients don't develop the severe consequences.

So we've started adding in medicines, adding preventative medicines. We've talked about gene therapy. I mean, if you ask me, I don't think the field has moved forward fast enough. I do think there's a lot of racial bias that goes into treatment and into designing treatment, interest in treatment, interest in

pharmaceutical companies designing medicines that work, we were fortunate to have a secondary medication that worked similarly to

help the hemoglobin attract and hold on to the oxygen, but that was just pulled from the market about a month or two months ago because it had some severe unintended consequences, including death. So there, I'm hopeful that we're going to get places with treatment, but I am also concerned that there's racial bias in and that we don't have enough quick enough. Thank you for that, Barry. I know that your hand is up. Let me just want to do a real quick follow up question. I know that. I mean, I took a look at the other research. Diony did a phenomenal job, but there were some words in there I could not even pronounce, okay, but I did want to talk about anemia. I mean, that's one that that, you know, we talked a lot about how it affects people. But Barry, I don't know Barry, if you had a question, a different question, or a follow up question, I'm just, but I just want to, I want to make sure that we talk about anemia, because that's something that we are. I'm, I can pronounce that, yeah, okay. I just, I just wanted to

comment on where Kate was, you know, just, just presenting with that and looking at the research. So it seems like, on the one hand, that really, transfusions have remained one of the mainstays of tree of treatments. And so I'm going to do my this is, I'm going off script here, guys and gals, a public service announcement. Because,

you know.

If you need blood transfusions, then that means that someone has to be donating blood. And, you know, and the reality is, only 3% of the population are blood donors, and the need for blood is, you know, is great. So that's my public service announcement. Is that if you're able, you know, to give, to give blood. Think about that, because that's still not only for sickle cell, but also, you know, for some of the other blood disorders that we were talking about that that's in the toolbox, and something that we need to to be, you know, to recognize and to be cognizant of,

and so. And then my other question was that it's sounding like

as the world moves forward, because when I went to medical school, it was always a crisis. You know, these blood disorder was that, you know, that was then and now, you know, we didn't have gene therapy then, and it's sounding like gene therapy. Am I correct that that would be holding the greatest promise for having almost like turning some of these acute diseases into diseases that can be more managed, more like a chronic disease? Because I you know, you can't be you know, you can get blood transfusions every three or four months, but eventually they say iron overload, as you you know, as you pointed out that there it's, it's not a benign treatment. It's great to have that available. But I was, my question was going to be pointing more toward the the horizon for, you know, for some of the gene therapies and and then you pointed out some of the limitations and some of the racial biases that we have that might not make that as high a priority, right? I could talk all hour just on anemia, sickle cell related or not.

I think there's a to back up a bit when you have sickle cell disease or anemia? It's sickle cell disease causes an anemia. So what is anemia? Anemia is when your red blood cell number. Us doctors say hemoglobin because we're lazy, and that's just what we call it. But technically, when the red blood cell quantity or number goes down, it's not normal. So if it's not normal, then you're not getting good oxygen carrying capacity, so you're lacking oxygen and starving your tissues. If the cells are abnormally shaped, you're going to break them down or hemalize them more frequently, so you get a lot of complications related to that. So when you break down a red blood cell. It's your spleen, or the big organ on the left side of your body that's doing that. And like I said, it's a sponge, so it's constantly degrading these abnormally shaped red blood cells, and over time, that sponge will get full and stop working. So your spleen is important for fighting infections. It's also important for clearing these red blood cells,

so you become at risk for certain infections and very sick infections that can cause death.

You also release a lot of bilirubin, which is inside of a red blood cell, when you break it down or hemalize it. So bilirubin is usually pooped out. Am I allowed to say that on this program?

Okay, so it goes to your gallbladder, and then it gets pooped out. And if it sits in your gallbladder too long, then you'll cause gallstones. But I think the most severe complications come from really the abnormal shape and plugging up your blood vessels. So if you these blood cells don't flow right through the tubes in your body or the blood vessels, they're going to irritate the lining of your blood vessels and cause pain and cause a lot of inflammation. Or white blood cells to come in, and they're going to block up your blood vessels. So they're going to cause pain, they're going to cause swelling, and in the case of blocking up your blood vessels in your brain, that's a stroke. So not only can you have silent strokes, so things that occur over time that you might not know about, you can have major strokes.

They can plug up the blood vessels in your lungs, that's pneumonia or acute chest syndrome, they plug up your spleen. So there's a lot of side effects that occur just because of this abnormal shape and decreased oxygen carrying capacity. And what we used to do, to speak to Barry's point is just treat the symptom

rather than prevention. And now, in the last 20 or 30 years, we have a few, not as many as I was hoping to help prevent some of these consequences, infections and strokes and with transfusions, but now we have the ability to really treat or minimize the severity of disease with gene therapy.

So, you know, it's interesting, because I you know,

you know it's like when people.

Have blood disorders.

My question is, what do they die from? Okay, so, like when you put on a, you know, on a death certificate, do you put, you know, sickle cell, or do you put

a stroke?

Okay, it's so, you know, the it seems like this disease leads to

whatever heart attack or or stroke, for instance. So I'm just curious how it is that we we monitor this, and certainly for research purposes, keeping tabs on how many people are have this

absolutely you,

you die from the complications of the disease. I think epidemiologists would argue that you die from the disease and but when a

when somebody writes on a death certificate, they're going to say that you died of a stroke, they might note that you had sickle cell disease, or they might note that you had a bleeding disorder and had a bleeding type stroke, but they might not, and that can hinder our ability to capture these patients and track them, not that we want to track them, but I guess, from a research standpoint, track and see, what are the complications? What really are people having? What are they dying from? And that brings a whole nother can of worms that Clarence might want to talk about as doing research in these populations. You know, these

are people who maybe have been a part of some bad research studies in the past, taken advantage of, maybe not as medically illiterate, because they don't have doctors who look like them treating them,

and have trust in the, I guess, Doctor world, so they're not participating in as many studies, and maybe not getting educated. These are also patients who need to come into the clinic frequently for a lot of treatment, and getting to and from the doctor's office might be a hardship. So there's a lot of compounding social factors. Yeah, I was, I was going to say that you and I had that conversation prior to the starting the program, is that there's so much

value loss and the lack of of transparency, but also in translation. You know, you know doctors are using these big words. You know other and other people are using smaller words. And it's almost like it's a it's taboo to translate, and so therefore people do are suspicious. People don't want to participate. And one of the things that you know that we're trying with you, man, is to is to try to

erase some of those barriers. Because we, we do need researchers. You do need community members, you know. So we need each other, but it's just, it's about, how do we have this conversation where we understand that there's not a loss in us talking to each other. So I appreciate you saying that. Kate, nice.

Sheridan, you you have a comment or two. I have a question, actually, and it's in the same it's in the same vein of,

you know, some medical mistrust, and with you being a specialist in pediatric blood disorders. Kate, I was wondering, do you record recommend every newborn gets the vitamin K shot, and can you address any misinformation that you've heard in your own practice?

It's a great question. We are going to switch gears now from sickle cell to bleeding disorders. So

we talked about so to answer your question outright, yes, we do recommend the vitamin K shot to prevent hemolytic

Hemorrhagic Disease of the newborn. Sorry, hemorrhagic disease of the newborn. I'm going to explain what all those words mean to make Clarence happy and our listeners,

so we talked previously about the three cells that float through our blood, but then there's all this liquid stuff that is just blood, and we call it plasma, as doctors. And inside this plasma, or liquid part, you have super, super tiny, tiny little proteins, again, made from your DNA that you inherit from your parents. So these super tiny, tiny little proteins, there are 14 of them that I care about as a blood doctor that we call factors. Why? Because some doctor who discovered them called them factors. That's really the only reason. And these factors are important for building scabs, so they are important to stop bleeding. So the cell called a platelet, plugs up holes and initially stops bleeding. But these factors come in and form a scab to prevent ongoing bleeding and to build these factors your liver, mostly and some other cells require vitamin K, so.

If you don't have vitamin K, then at least factors 279, and 10, if not others. So at least four of the factors don't have the vitamin that's needed to be built, and therefore you don't make them, and therefore you bleed because they're not there. What happens in a newborn is that when the baby's inside of a mom, the baby require relies a lot on what the mom builds. So, you know, they're connected through that umbilical cord. The mom is building all these factors. The mom is keeping the baby safe. But when the baby's born, you cut that umbilical cord, and now the baby's liver says, Oh my gosh, I have to make these factors. I don't know how I need time. I need I need the vitamins, I need the sustenance to build these factors. And if we don't provide that vitamin K at birth, then the baby's liver is left without the vitamin that it needs to build these factors, and then it's at a huge risk to bleed. So way back when, you know, 50 plus years ago, we saw Hemorrhagic Disease, which is called bleeding disease of a newborn.

Kate Garland: If you don't have vitamin K, at least factors 2, 7, 9, and 10, if not others, can't be built properly. Without these factors, the body can't form clots, which can lead to serious bleeding.

When a baby is in the womb, it relies on the mother's body to produce these clotting factors. The mother provides the necessary nutrients through the umbilical cord. After birth, when the umbilical cord is cut, the baby's liver has to start producing these factors independently. However, the liver needs vitamin K to make them, and newborns are often deficient. Without vitamin K, the baby faces a significant risk of bleeding.

Decades ago, hemorrhagic disease of the newborn was common. It caused severe bleeding, particularly in the brain, leading to strokes and even death. To prevent this, we started giving newborns a vitamin K shot at birth.

Unfortunately, misinformation has spread from those who oppose this practice. While everyone is entitled to their opinions, as doctors, our role is to build trust with patients and promote safe healthcare choices for their babies.

Stanton Shanedling: Wow, yes, trust is so important, especially when dealing with complex blood disorders.

Kate Garland: Absolutely.

Stanton Shanedling: On a related note, how do you handle the mental health impact of these diagnoses? It must be overwhelming for families.

Kate Garland: It can be very difficult. As providers, we have to deliver life-changing news that a child has a chronic condition related to bleeding or clotting. Parents often go through denial before accepting the diagnosis. They need to understand the condition medically and commit to prevention and treatment. As the child grows, they also need to learn how to manage their health.

We're fortunate to work with patient advocates and licensed social workers who help families process these challenges. We also encourage families to seek additional support from others who have had similar experiences.

Stanton Shanedling: It sounds incredibly complex. Clarence, you had something to add?

Clarence: Yes, thank you. I was just thinking about how vast this field is. I heard there are over a thousand blood disorders.

Kate Garland: That's right. There are many.

Clarence: That seems overwhelming. How do you manage dealing with so many conditions and unique patient needs?

Kate Garland: Medicine has evolved a lot over the years. We have general practitioners like family doctors and pediatricians, but we've also developed more specialized fields. I'm a pediatric hematologist-oncologist, though I focus primarily on hematology—specifically bleeding and clotting disorders.

Patients are often referred to me for specialized care, and diagnosing blood disorders can be complex. Sometimes I consult even more specialized colleagues for rare conditions. I’m fortunate to work in a well-resourced center, but many patients in rural areas don't have the same access to care.

Stanton Shanedling: It seems like collaboration is essential, right? Working closely with primary care providers and specialists to ensure proper care?

Kate Garland: Absolutely. It's a team effort. Clarence mentioned earlier that it's a process, and that's true for both providers and families. It takes time for primary care doctors to recognize these issues and for specialists like me to reach a diagnosis. Building trust with families is essential, especially when procedures like blood draws are involved. It all takes patience and teamwork.

Kate Garland: So technically, they sort of prevent or stop clotting. They cause bleeding in different ways. So depending on which drug you're on. For instance, the blood thinners we typically think about are Coumadin or Warfarin. There's some newer ones called Xarelto or Eliquis. There's Lovenox shots. There are a ton of them. They all have different mechanisms of action, so you have to stop them before surgery in different ways. A platelet medicine you might want to stop maybe 5 to 10 days before surgery, whereas a blood thinner, depending on which one you're on, you might want to stop 2 days before surgery.

Stanton Shanedling: Thank you for that clarification. That's an important one, and I hope the listening audience heard that as well. So, I became knowledgeable about this. The other thing I want to talk about is comorbid manifestations here. Let's just say somebody's diabetic and also suddenly anemic. How do we deal with these types of situations?

Kate Garland: It can be a challenge, and going back to Barry and Clarence's point, it takes a village to manage patients. Unfortunately, diabetes is more common and becoming more common in children, so pediatricians are having to become more knowledgeable about it. But previously, it had mostly been followed by endocrinologists, another subspecialist. So now you have 2 or 3 different subspecialists involved in their care. What I do is try to stay educated on things and talk to other providers. "Hey, I'm going to fix your anemia, but how is it going to affect your diabetes management?"

Stanton Shanedling: Right, right. Okay, then I guess I'd be remiss not to bring up, and we've brought this up in other shows too, things that hit us as human beings. Covid, for instance. All of a sudden, everybody's hit with that. So, tell me how you, as a practitioner in this area, had to deal with Covid, vis-a-vis patients dealing with blood disorders, and on the other hand, now they have Covid.

Kate Garland: It was scary. It was scary for providers and scary for patients, because initially, we just didn't know. I think what we understood quickly is that Covid itself, as a virus, caused a lot of inflammation, swelling, and immune system reactions. When that happens, the major side effect from a hematologist standpoint is clotting. So when you have a lot of inflammation, not only is it going to cause clotting, but it’s going to trigger your underlying disease. If you have an underlying, abnormal-shaped red blood cell like sickle cell, it's going to trigger them to sickle. So now you're going to experience worsening complications of sickle cell disease on top of clotting.

Kate Garland: Initially, we were throwing blood thinners at everybody hospitalized to prevent blood clots, and we still do. We were learning more and more about how to treat Covid. We threw steroids at them to decrease inflammation and swelling. We were using antibody therapy because if you get a blood clot, especially in your head, belly, or leg, that can be life-threatening too.

Stanton Shanedling: Yeah, scary.

Kate Garland: Scary.

Stanton Shanedling: Wow. Yeah, it's stuff that hits us, and good or bad, we have to deal with.This has been really enlightening. I learned quite a bit on this show, and I greatly appreciate it. I greatly appreciate you as a clinician in this arena, and we're lucky to have you. It's really... Barry, last thoughts?

Barry:  Yeah, I'm going to keep it brief here. This was really amazing. From the perspective of bleeding disorders, the ground we covered and the perspectives presented were really very educational, very helpful, and were explained extremely clearly. So I thank Dr. Garland for being a guest on our show. Who knew that bleeding disorders could be so fascinating and interesting, covering such a wide base of things? But that's what I have to say. So, thank you.

Kate Garland: Thank you.

Stanton Shanedling: Clarence?

clarence: Well, we often talk about sending shows out to our family and friends, and as we were going through this show, my mind started racing about all the people who should be listening to this show. So, I just want to thank you for your insight and for your information. Thank you for your transparency. It was a good show.

Kate Garland: Thank you.

clarence: Thank you.

Stanton Shanedling: Sheridan, I know that you chimed in. Any thoughts?

hello everybody. Welcome to Health chatter today's show. We're really digging deep into the medical arena. Today, our show is on pediatric blood disorders, and we have a great guest with us, Dr Kate garland. I'll introduce her in just a second,

but it'll be interesting, because, frankly, I certainly don't know much about this, this arena, but hopefully by the end of the show, everybody will be a little bit more knowledgeable. I want to thank our crew. As always, there's their second to none. Maddie Levine Wolf, Aaron Collins and Deandra Howard do our background research. Sheridan Nygard also does research for us marketing and helps us with recording. Matthew Campbell, our production person. And of course, there's Dr Barry Baines, who's our physician consultant on our show and provides us with good medical insight. Then of course, there's Clarence. Clarence Jones and I have have partnered on this for now close to three years, and it's been a it's been a wonderful endeavor. And we really, we, frankly, even though we've been in the healthcare being a long time, I think I could speak for you too, Clarence, that you know, we've we've learned a lot along the way, and we hope you, you the listening audience does as well.

We also have a great partner sponsor for these shows, the health chatter shows, human partnership, great community health organization. You can check them out at human partnership.org,

and we can check us out at Health chatter podcast.com,

and you could you'll see all the research that we do for the shows there, as well as the transcriptions of the shows. If you'd like to read, as opposed to listen to podcasts, you can read about the information on our website. So thanks to everybody. So today we have Dr Kate garland.

She attended medical school in East Tennessee, State University. Actually, I was in Tennessee over the summer. It was, it's an interesting state to visit. She completed her pediatric residency and a chief residency year at Children's Hospital of the king's daughters in Norfolk, Virginia. She's, she's done a lot of research, and you can, you can tell it's one thing you know, as I've read her bio, it's one thing to to look at a clinician, but it's another thing to also see that they're they're involved in research, and my sense is, is that research is hopefully to help make a difference in these in this arena, she serves as a local principal investigator for multiple large, large national, International clinical trials, and she's being primed for a future co director role of the children's Minnesota hemophilia treatment center here in Minnesota, HTC, right hemophilia treatment center. So welcome to you, and thank you so much for being part of health chatter today. So let me get the ball rolling here, and then, believe me, everybody's going to be chiming in on this one.

You know, as I read through the background research that our crew provided here, it's like, all right, how is it that you identify

somebody who's got any of these pediatric blood disorders, do they present themselves with particular symptoms? Is it? Is it a function of testing, for instance, because there's genetics involved? Help me, help me identify how it is that you, as a clinician, identify somebody who's got a blood disorder. Well, thank you for having me everybody today. It's a true pleasure and honor.

So how do you identify somebody with a bleeding disorder? It can be a real or any sort of blood disorder really. It can be a real challenge.

First, I suppose you have to understand what a blood disorder is.

I think what we're going to be talking about today is things that affect the blood or the blood cells that are considered non malignant, so not cancer, not oncology,

and that doesn't mean that they are benign or doesn't affect somebody's life. But in general, if you have something of the blood, they can really be identified two ways. One, you have a family history of it, so maybe there is a genetic component that can be passed down from generation to generation or two, you are the brand new patient of the family who.

I happened to get this, it was sort of acquired, either genetically or as a result of being exposed to something outside in your life. And really patients come to us with these things, our hope as a clinician or as a doctor is to pick up on those patients who maybe have a family history of it, so we can counsel and talk about what the future risks are. But aside from that, really, people might come to us with, now I'm bleeding and I don't know why, or now I had a clot and I don't know why, or now I have this pain or rash and I don't know why,

and it's our job to figure out why.

So they a level of seriousness. Okay, so it's like, okay,

I'm bleeding all of a sudden. Well, okay, maybe that'll just heal itself. And thank you very much. But at what point does an actual patient present themselves to you? It seems to me that there's got to be this. Wait a minute, something's really not right here. So help me with that.

It can be anything from at birth to late in life. So for instance, if we're talking about bleeding disorders, or things in your blood that make you bleed more heavily, sometimes a patient could come to us because the parents noted, or a pediatrician noted that with the child's circumcision that they bled too much.

Sometimes they come to us because they are now two years old and bruising more easily now that they are running around. So there's they're covered in bruises, which, of course, can be sometimes misdiagnosed as abuse or missed as abuse, or sometimes you have teenage girls who really have had no problems throughout their life, and they start menstruating and now they have very heavy menses. But these things can be missed, because parents and clinicians alike can write it off as I bled a lot. They bleed a lot. I bruised a lot. They bruised a lot, and maybe it's not always picked up until later in life.

All right, so, you know, Clarence chime in on this one, because I, you know, obviously we're dealing with, you know, some diseases that that affect the the African American population.

Let's talk about

there's kind of this cadre of things here, but treatment Alright, so in general, our blood disorders treated pretty much the same way. Or does each specific blood disorder have a particular treatment that's maybe different from the other ones you stand can I let me jump into here real quick? Because I appreciate that, because I was going to, I was going to go, kind of go down this, this lane. I am African American, and all of my life, I have heard about sickle cell,

and I think that part of the the understand, I like to know is number one, I don't think that we know much about it, or it's not talked about much in our community. And so when we look on here about sickle cell, and totally honest, what I heard is that they have it, and they're probably going to die soon.

Okay? And so I would, you, would you kind of explain what it is, and then talk about, go back into the treatment of it, because I think that, you know, sometimes, like I said, it's like the kiss of death, you have sickle cell, you're going to die, you know, I mean, and so I know my, I know my question is not formed very well, but that's because, you know, I don't know. So I hope you understand where I'm trying to go. Absolutely. Let's talk about what sickle cell is, because it certainly is not benign, and it's scary, and it can be life threatening, and

it can be a big change for families when they know about or find out about the diagnosis. So in your blood you make cells. We all make three cells, white blood cells, that fight infections and carry our body's DNA or genetics. We make platelets that help us to clot and stop bleeding. But then we also make red blood cells. And red blood cells carry the oxygen throughout the body. So their job is to be a little round cell and pick up oxygen in the lungs, transport it throughout the body, deliver the oxygen to the tissues or the organs, pick up the carbon dioxide there, transport it back to the lungs so that you can breathe out the carbon dioxide. And then repeat, pick up an oxygen.

Okay, in sickle cell, there is, well, I should say, in general, in red blood cells, we all have something that sits inside the red blood cell and gives its shape. We call it hemoglobin. So this hemoglobin is a protein. So all proteins are made from your DNA. So you have inherited this DNA or genetic structure to build this protein called hemoglobin. Unfortunately, this hemoglobin needs to be a very specific structure in order to be able to do its job or function well, of carrying oxygen. And if you have a very slight spelling mistake in that genetic code which you can inherit, then it makes the hemoglobin less likely to hold on to the oxygen, or less likely to pick it up. And when it does pick it up or delivers the oxygen, it deforms. So when the hemoglobin or the red blood cell is not holding on to oxygen, the round red blood cell instead turns into a more sickle type shape. And if a sickle kind of has two points on the end, it kind of looks like a crescent moon. And those pointy ends can cause a lot of a lot of damage to the body. And obviously, if you did not carry an oxygen very well, that can also lead to a lot of damage and shorten your life.

So go ahead. So

you know, it's like,

you know, I keep having all these Whys in my head. Why? Why, specifically do we, we connect sickle cell

a lot with the African American population. What's going on here?

I don't I don't know if we know exactly why. We know that somewhere along the line, there was a genetic change in a human, and that has gotten passed down. And like anything, you get two copies of every gene, one from your mom, one from your dad, and if each copy of the gene is affected, then you are more severely affected, because you make no normal shape.

There are people that have theorized, and there is some racial bias in this, that maybe this genetic change occurred because a red blood cell that is not normally shaped, does not hold oxygen as well, and maybe is harder for certain diseases to infect it. So one of the more common diseases across the world, that is more prevalent in Africa and other tropical places, or I guess, along the equator, is malaria. So malaria can affect the red blood cells. That's how how it transmits. And if the red blood cell has a more sickle shape or a different shape to it, then maybe the malaria doesn't want to infect it as well. So some scientists theorize that maybe this genetic change occurred as part of evolution and made it harder for malaria to infect people.

It tends to malaria tends to occur in these areas across the world where there is more heavily a black population. And so if this has been going on for generations and generations, 1000s of years, then your black population that lives in these areas have been more severely affected. But that does not mean that anybody cannot have this disease. So if you it's genetic. So if you are a black person or a white person, it doesn't mean that you you can get this disease equally, depending on who your parents are and who are passing it on.

So, alright, so you've been in this, in this world for for a while.

Are you optimistic about

treatment? Are you optimistic about maybe prevention? I'm a doctor. I have to be optimistic. Okay,

alright, I'll give you that.

You know what I one of the things that that's interesting is that I remember, I want to go back to this, my own personal experiences, that I remember I had a person in my life that had sickle cell. They thought they person would, would would pass on when they were 21 but they lived to be 45 so I want

to get back to that thing that that, that statement about Stan and treatment, um, how have treatments changed in the last 20 to 30 years? We're talking about addressing this issue.

So you bring up a valid point that he's now living they're now living to 40 something, but that's still not the normal average. So this is an extremely morbid disease, as are others. The treatments have changed because we've learned more about the disease. We've learned more about.

The genetics. There has been a lot of scientific research in treating with genetics, in treating with making the hemoglobin more stable. So, for instance, 2030, years ago, maybe even a little bit longer, the only treatment we had was to give somebody blood that wasn't sickled. So to transfuse to give their body good hemoglobin.

We know that the life cycle of a red blood cell in a normal body is about three to four months, whereas if it is abnormally shaped, then your body is going to break it down or hemalize it sooner. So in a patient with sickle cell, not only are their red blood cells not lasting as long, but they're then getting transfusions. So when you get a transfusion, it's still not going to last as long, because by the time the blood's prepared, it's transfused, it just doesn't last as long in the body. So now you're getting a lot of transfusions, and now you're getting a lot of extra problems that come with getting transfusions. I'm giving you a lot of less extra iron. There's more iron in the blood. So now you have iron overload. Your organs have iron deposited on it.

That's the major side effect. But you're also not growing as well. You're not your whole your body's not functioning as well. So what we used to do is treat all the complications that went along with sickle cell by transfusing blood. If you happen to get a pneumonia, here's the treatment for pneumonia. If you happened to get a spleen problem, your spleen is the one that's breaking down the red blood cells. It's like a sponge. So the more

full it gets with degraded blood cells, the less it works. So you're more prone to infections. So now you're dying of diseases. So we're treating those infections. We used to take out spleens. Nowadays, now that we know a little bit more about the genetics of it, they're doing things like gene therapy. Now that is not without risk. I mean, I have to edit your own genome and wipe out your immune system so that your own immune system doesn't attack the new genome.

We are giving antibiotics preventatively to prevent infections. We are making sure our patients are vaccinated, we are there are some some medicines that we can use. There's an old chemotherapy medicine. Chemo is the term for

adjusting your immune system to so it doesn't attack itself. But there's one called hydroxyurea that we have been using for the last 20 to 30 years,

that helps to stabilize that hemoglobin shape so it doesn't deform.

We've started using that younger and younger so that patients don't develop the severe consequences.

So we've started adding in medicines, adding preventative medicines. We've talked about gene therapy. I mean, if you ask me, I don't think the field has moved forward fast enough. I do think there's a lot of racial bias that goes into treatment and into designing treatment, interest in treatment, interest in

pharmaceutical companies designing medicines that work, we were fortunate to have a secondary medication that worked similarly to

help the hemoglobin attract and hold on to the oxygen, but that was just pulled from the market about a month or two months ago because it had some severe unintended consequences, including death. So there, I'm hopeful that we're going to get places with treatment, but I am also concerned that there's racial bias in and that we don't have enough quick enough. Thank you for that, Barry. I know that your hand is up. Let me just want to do a real quick follow up question. I know that. I mean, I took a look at the other research. Diony did a phenomenal job, but there were some words in there I could not even pronounce, okay, but I did want to talk about anemia. I mean, that's one that that, you know, we talked a lot about how it affects people. But Barry, I don't know Barry, if you had a question, a different question, or a follow up question, I'm just, but I just want to, I want to make sure that we talk about anemia, because that's something that we are. I'm, I can pronounce that, yeah, okay. I just, I just wanted to

comment on where Kate was, you know, just, just presenting with that and looking at the research. So it seems like, on the one hand, that really, transfusions have remained one of the mainstays of tree of treatments. And so I'm going to do my this is, I'm going off script here, guys and gals, a public service announcement. Because,

you know.

If you need blood transfusions, then that means that someone has to be donating blood. And, you know, and the reality is, only 3% of the population are blood donors, and the need for blood is, you know, is great. So that's my public service announcement. Is that if you're able, you know, to give, to give blood. Think about that, because that's still not only for sickle cell, but also, you know, for some of the other blood disorders that we were talking about that that's in the toolbox, and something that we need to to be, you know, to recognize and to be cognizant of,

and so. And then my other question was that it's sounding like

as the world moves forward, because when I went to medical school, it was always a crisis. You know, these blood disorder was that, you know, that was then and now, you know, we didn't have gene therapy then, and it's sounding like gene therapy. Am I correct that that would be holding the greatest promise for having almost like turning some of these acute diseases into diseases that can be more managed, more like a chronic disease? Because I you know, you can't be you know, you can get blood transfusions every three or four months, but eventually they say iron overload, as you you know, as you pointed out that there it's, it's not a benign treatment. It's great to have that available. But I was, my question was going to be pointing more toward the the horizon for, you know, for some of the gene therapies and and then you pointed out some of the limitations and some of the racial biases that we have that might not make that as high a priority, right? I could talk all hour just on anemia, sickle cell related or not.

I think there's a to back up a bit when you have sickle cell disease or anemia? It's sickle cell disease causes an anemia. So what is anemia? Anemia is when your red blood cell number. Us doctors say hemoglobin because we're lazy, and that's just what we call it. But technically, when the red blood cell quantity or number goes down, it's not normal. So if it's not normal, then you're not getting good oxygen carrying capacity, so you're lacking oxygen and starving your tissues. If the cells are abnormally shaped, you're going to break them down or hemalize them more frequently, so you get a lot of complications related to that. So when you break down a red blood cell. It's your spleen, or the big organ on the left side of your body that's doing that. And like I said, it's a sponge, so it's constantly degrading these abnormally shaped red blood cells, and over time, that sponge will get full and stop working. So your spleen is important for fighting infections. It's also important for clearing these red blood cells,

so you become at risk for certain infections and very sick infections that can cause death.

You also release a lot of bilirubin, which is inside of a red blood cell, when you break it down or hemalize it. So bilirubin is usually pooped out. Am I allowed to say that on this program?

Okay, so it goes to your gallbladder, and then it gets pooped out. And if it sits in your gallbladder too long, then you'll cause gallstones. But I think the most severe complications come from really the abnormal shape and plugging up your blood vessels. So if you these blood cells don't flow right through the tubes in your body or the blood vessels, they're going to irritate the lining of your blood vessels and cause pain and cause a lot of inflammation. Or white blood cells to come in, and they're going to block up your blood vessels. So they're going to cause pain, they're going to cause swelling, and in the case of blocking up your blood vessels in your brain, that's a stroke. So not only can you have silent strokes, so things that occur over time that you might not know about, you can have major strokes.

They can plug up the blood vessels in your lungs, that's pneumonia or acute chest syndrome, they plug up your spleen. So there's a lot of side effects that occur just because of this abnormal shape and decreased oxygen carrying capacity. And what we used to do, to speak to Barry's point is just treat the symptom

rather than prevention. And now, in the last 20 or 30 years, we have a few, not as many as I was hoping to help prevent some of these consequences, infections and strokes and with transfusions, but now we have the ability to really treat or minimize the severity of disease with gene therapy.

So, you know, it's interesting, because I you know,

you know it's like when people.

Have blood disorders.

My question is, what do they die from? Okay, so, like when you put on a, you know, on a death certificate, do you put, you know, sickle cell, or do you put

a stroke?

Okay, it's so, you know, the it seems like this disease leads to

whatever heart attack or or stroke, for instance. So I'm just curious how it is that we we monitor this, and certainly for research purposes, keeping tabs on how many people are have this

absolutely you,

you die from the complications of the disease. I think epidemiologists would argue that you die from the disease and but when a

when somebody writes on a death certificate, they're going to say that you died of a stroke, they might note that you had sickle cell disease, or they might note that you had a bleeding disorder and had a bleeding type stroke, but they might not, and that can hinder our ability to capture these patients and track them, not that we want to track them, but I guess, from a research standpoint, track and see, what are the complications? What really are people having? What are they dying from? And that brings a whole nother can of worms that Clarence might want to talk about as doing research in these populations. You know, these

are people who maybe have been a part of some bad research studies in the past, taken advantage of, maybe not as medically illiterate, because they don't have doctors who look like them treating them,

and have trust in the, I guess, Doctor world, so they're not participating in as many studies, and maybe not getting educated. These are also patients who need to come into the clinic frequently for a lot of treatment, and getting to and from the doctor's office might be a hardship. So there's a lot of compounding social factors. Yeah, I was, I was going to say that you and I had that conversation prior to the starting the program, is that there's so much

value loss and the lack of of transparency, but also in translation. You know, you know doctors are using these big words. You know other and other people are using smaller words. And it's almost like it's a it's taboo to translate, and so therefore people do are suspicious. People don't want to participate. And one of the things that you know that we're trying with you, man, is to is to try to

erase some of those barriers. Because we, we do need researchers. You do need community members, you know. So we need each other, but it's just, it's about, how do we have this conversation where we understand that there's not a loss in us talking to each other. So I appreciate you saying that. Kate, nice.

Sheridan, you you have a comment or two. I have a question, actually, and it's in the same it's in the same vein of,

you know, some medical mistrust, and with you being a specialist in pediatric blood disorders. Kate, I was wondering, do you record recommend every newborn gets the vitamin K shot, and can you address any misinformation that you've heard in your own practice?

It's a great question. We are going to switch gears now from sickle cell to bleeding disorders. So

we talked about so to answer your question outright, yes, we do recommend the vitamin K shot to prevent hemolytic

Hemorrhagic Disease of the newborn. Sorry, hemorrhagic disease of the newborn. I'm going to explain what all those words mean to make Clarence happy and our listeners,

so we talked previously about the three cells that float through our blood, but then there's all this liquid stuff that is just blood, and we call it plasma, as doctors. And inside this plasma, or liquid part, you have super, super tiny, tiny little proteins, again, made from your DNA that you inherit from your parents. So these super tiny, tiny little proteins, there are 14 of them that I care about as a blood doctor that we call factors. Why? Because some doctor who discovered them called them factors. That's really the only reason. And these factors are important for building scabs, so they are important to stop bleeding. So the cell called a platelet, plugs up holes and initially stops bleeding. But these factors come in and form a scab to prevent ongoing bleeding and to build these factors your liver, mostly and some other cells require vitamin K, so.

If you don't have vitamin K, then at least factors 279, and 10, if not others. So at least four of the factors don't have the vitamin that's needed to be built, and therefore you don't make them, and therefore you bleed because they're not there. What happens in a newborn is that when the baby's inside of a mom, the baby require relies a lot on what the mom builds. So, you know, they're connected through that umbilical cord. The mom is building all these factors. The mom is keeping the baby safe. But when the baby's born, you cut that umbilical cord, and now the baby's liver says, Oh my gosh, I have to make these factors. I don't know how I need time. I need I need the vitamins, I need the sustenance to build these factors. And if we don't provide that vitamin K at birth, then the baby's liver is left without the vitamin that it needs to build these factors, and then it's at a huge risk to bleed. So way back when, you know, 50 plus years ago, we saw Hemorrhagic Disease, which is called bleeding disease of a newborn.

Kate Garland: If you don't have vitamin K, at least factors 2, 7, 9, and 10, if not others, can't be built properly. Without these factors, the body can't form clots, which can lead to serious bleeding.

When a baby is in the womb, it relies on the mother's body to produce these clotting factors. The mother provides the necessary nutrients through the umbilical cord. After birth, when the umbilical cord is cut, the baby's liver has to start producing these factors independently. However, the liver needs vitamin K to make them, and newborns are often deficient. Without vitamin K, the baby faces a significant risk of bleeding.

Decades ago, hemorrhagic disease of the newborn was common. It caused severe bleeding, particularly in the brain, leading to strokes and even death. To prevent this, we started giving newborns a vitamin K shot at birth.

Unfortunately, misinformation has spread from those who oppose this practice. While everyone is entitled to their opinions, as doctors, our role is to build trust with patients and promote safe healthcare choices for their babies.

Stanton Shanedling: Wow, yes, trust is so important, especially when dealing with complex blood disorders.

Kate Garland: Absolutely.

Stanton Shanedling: On a related note, how do you handle the mental health impact of these diagnoses? It must be overwhelming for families.

Kate Garland: It can be very difficult. As providers, we have to deliver life-changing news that a child has a chronic condition related to bleeding or clotting. Parents often go through denial before accepting the diagnosis. They need to understand the condition medically and commit to prevention and treatment. As the child grows, they also need to learn how to manage their health.

We're fortunate to work with patient advocates and licensed social workers who help families process these challenges. We also encourage families to seek additional support from others who have had similar experiences.

Stanton Shanedling: It sounds incredibly complex. Clarence, you had something to add?

Clarence: Yes, thank you. I was just thinking about how vast this field is. I heard there are over a thousand blood disorders.

Kate Garland: That's right. There are many.

Clarence: That seems overwhelming. How do you manage dealing with so many conditions and unique patient needs?

Kate Garland: Medicine has evolved a lot over the years. We have general practitioners like family doctors and pediatricians, but we've also developed more specialized fields. I'm a pediatric hematologist-oncologist, though I focus primarily on hematology—specifically bleeding and clotting disorders.

Patients are often referred to me for specialized care, and diagnosing blood disorders can be complex. Sometimes I consult even more specialized colleagues for rare conditions. I’m fortunate to work in a well-resourced center, but many patients in rural areas don't have the same access to care.

Stanton Shanedling: It seems like collaboration is essential, right? Working closely with primary care providers and specialists to ensure proper care?

Kate Garland: Absolutely. It's a team effort. Clarence mentioned earlier that it's a process, and that's true for both providers and families. It takes time for primary care doctors to recognize these issues and for specialists like me to reach a diagnosis. Building trust with families is essential, especially when procedures like blood draws are involved. It all takes patience and teamwork.

Sheridan: I just have been seeing a lot of stuff about Vitamin K, and I think it comes from the German word...

Kate Garland: Coagulation's vitamin. And I'm not going to pronounce that.

Sheridan: You’re correct! So all the other vitamins A through E were named in order, and then I think coagulation and K just makes sense. So next time anyone asks about the Vitamin K shot, you can think "coagulation."

Kate Garland: Thank you. I never thought that.

Stanton Shanedling: Well, listen, this has been really enlightening. I thank you very much for not only being on our show but being in our community anytime. And I always tell guests, as things come up, especially for you as a researcher, if you believe we should get some words out, just give us a call and we'll get you back onto Health Chatter.

Kate Garland: Thank you. Happy to be here.

Stanton Shanedling: So for our listening audience, great shows are coming up. Our next one, interestingly enough, will be on pharmacy deserts, which has also become more of a problem in dealing with health overall. In the meantime, everybody keep health chatting away.