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Conan Gasque: I'm Conan Gasque, host of the Inside Pediatrics Podcast at Children's of Alabama. Our topic in this episode is sickle cell disease, and we have a unique way of sharing it with you. Recently, a former sickle cell disease patient of ours, Chris Ruffin, Jr., interviewed his former children's doctor, Dr. Lee Hilliard. Chris is now a senior news producer at a TV station in Memphis. We recorded this interview on Zoom as they recollected on Chris's battle with sickle cell disease and how Dr. Hilliard empowered Chris to advocate for his care and create his own success story. Here are Chris and Dr. Hilliard with more on sickle cell disease and how patients can manage it.

Chris Ruffins: Welcome to Inside Pediatrics, a podcast brought to you by Children's Hospital of Alabama in Birmingham. I'm Chris Ruffins, Jr., a guest on the podcast today. But I'm also joined by a legend in her own right, Dr. Hilliard of Children's of Alabama. Dr. Hilliard, how's it going?

Dr. Lee Hilliard: Oh, it's going great, Chris. I thank you for the awesome introduction. I'll have to remind everybody around here that I'm a legend.

Chris Ruffins: Yeah, you are. You are. I think anytime that I've needed a question asked, no matter how old I've been, you've been quick to respond. So that's a legend to me.

Dr. Lee Hilliard: Well, you know you're family.

Chris Ruffins: So look, Dr. Hilliard, before we get started, I definitely want to thank Childrens of Alabama for this opportunity to get both of us on. Our history goes back, I guess, 25 years ago when you first started caring for me when I was a toddler. So do you just want to give people a quick history about us?

Dr. Lee Hilliard: Sure. Yeah. So that pretty much sums it up, is literally your family brought you in to see us. You probably weren't even walking when I first met you. And then, took care of you all through younger life until you graduated, and we finally had to release you out into the world.

Chris Ruffins: And so for people who don't know, they brought me to you all because of my sickle cell. And so you specialize in sickle cell, taking care of sickle cell patients, so that is how you and I became family. And like you said, it's been years and years and we still haven't had a chance to let go of each other.

Dr. Lee Hilliard: Right. Yes, exactly. I'm a pediatric hematologist and I've been here since 1993. So I think, when you say I'm a legend, you really just mean I'm old.

Chris Ruffins: No, no, no, no. Not at all. So we're talking about sickle cell today. And World Sickle Cell Day recently passed by. And I know there's a lot of things going on with sickle cell right now, these conversations. But can you just let me know, like the first question I have for you, for those who want to know, like what type of crisis-- Well, first, can we define a crisis. And then, can you tell us what type of crisis can stem from sickle cell?

Dr. Lee Hilliard: Sure. When most people hear the word crisis, they're thinking about pain and probably that's the thing that most people think about when they hear the word sickle cell. But unfortunately, sickle cell affects your whole body because as, you know, it's a disease of red blood cells where there's an abnormal protein that makes an abnormal shape in the red blood cells. And red blood cells go all over your body, you know, to carry oxygen to your whole body, so sickle cell disease literally affects your whole body.

I think about it in kind of two categories for the problems that patients can have. And the first category that would be thought of more as "crises" are acute things, you know, things that happen all the sudden out of the blue. So that would be pain where those abnormal red cells float around and all of a sudden they get stuck in different bones in your body. And then, there's not enough blood flow and it starts to hurt. They can also get stuck in your lungs. And then, you have a sickle crisis in your lungs. That's called a chest syndrome. They can get stuck in your spleen and that can clog up your spleen and that's called a splenic sequestration. So those are, you know, the three main sudden problems. And from your experience, a stroke is a sudden clogging up of the blood vessels in the brain so that you, you know, have weakness or some sort of impairment of function of your brain.

The other category of problems though, and this is the thing that people don't think about as much with sickle cell, is more of the chronic problems, so the long-term problems, because every day, you're walking around with these abnormal blood cells going all over your body. And even if they're not causing a crisis that day, those abnormal cells are going through your kidneys and your lungs and your brain, essentially scraping the blood vessels and, over time, that makes your organs wear out earlier. Well, if you think about it, like all of us are going to die because our organs eventually give out, you know, unless you're hit by a bus or something. But unfortunately, for patients with sickle cell disease, the organ damage happens sooner because of those abnormal red blood cells.

Chris Ruffins: Yeah. I know for me, whenever I've experienced this sickle cell crisis, this last time that I had was probably the most severe that I've had since I've had a stroke when I was six. But in the past couple years, I've only experienced tightness in my arms and my legs. And usually, with that, I'm able to stretch, drink some water, rest a little bit, and then, you know, it'll pretty much be gone, but I know like a lot of people who I know who have sickle cell, they deal with those other experiences that you said, like the acute chest syndrome and things like that. So I know that's a little bit more severe than what I've dealt with. But yeah, I felt all of those, but I've only dealt with the lesser pain the most.

So speaking of the stroke, do you remember when I had my stroke? I know it's been a while, but can you remember back when it happened, like how you reacted to it, you know, how'd you diagnosed me and everything like that?

Dr. Lee Hilliard: Yeah. So you're going to have to help me with this, because my memory may not be totally accurate, but I'll tell you what my memory is. Remind me this, what year did you have a stroke?

Chris Ruffins: It was when I was six, so I believe it was 2000.

Dr. Lee Hilliard: Yeah, that sounds right. And the reason I'm saying that is we were starting a program to do screening for stroke prevention, but you had a stroke right before we got that functional, you know. One of the main things I think about for you is could I have picked up your stroke risk sooner if that program had been in place? And the other thing that I remember is when you were younger, I think you did have a chest syndrome. I think you were playing flag football and you may have overdone it a little bit. Knowing you, it's possible. You might have overdone it a little bit. And that was before you were on any treatment for your sickle cell. And I don't remember the timing between the chest syndrome and the stroke, but yeah, just always for you, I think about, "Man, could we have now--" You know, if you were here now, you would've gotten screening starting at age two to look at your stroke risk. But the symptoms of a stroke are usually not too hard to recognize, because it's usually weakness on one part of your body and your parents were, you know, smart to recognize that something wasn't right and get you into the hospital where we could get it diagnosed and get you treated.

Chris Ruffins: Yeah. My mom, she recently told me this story again. And she was like, "Yeah, you were just crying all night. I asked you what was wrong and you said you couldn't stop crying." And she said from that she just started Googling. I don't even know if it was Google back then. I think it was probably like ask.com, but like I said, she started trying to figure out what was going on. And she realized that the symptoms were that I pretty much had a stroke, so she brought me in from there.

And I know we're just speaking from my perspective, but once somebody has that crisis or somebody has that stroke, how do you help them bounce back, you know, to be fully functional as a sickle cell patient?

Dr. Lee Hilliard: Yeah. So a stroke can be pretty complicated because a stroke is injury to your brain. So the short answer to that is patients need lots of support. You know, you need support from your family. You need support from the medical team and you need goals. You know, try to set some finite goals and work towards those each day as your condition allows. So it depends on what the problem is, right? So if your crisis is you're in the hospital with pain, then at first you may not feel like getting up out of bed and doing anything, but just having people there with you to say, "Let's try this today and just keep trying to move forward," I think that's the most helpful thing.

Chris Ruffins: Yeah. And I think that support system is probably the most crucial thing when you're dealing with sickle cell at least for me. And you know my mom, she's in your face, like, "Hey, you need to get this done," anything that I don't want to do is not up for discussion. You know, it's kind of just like, "Hey, we're trying to reach this goal of getting better. And if you don't want to get better, then you really don't have a choice. You just got to do it."

Dr. Lee Hilliard: Your mom is the real legend.

Chris Ruffins: Yeah, she is. And I think that's the real thing though, and I talk to a lot of families who, you know, ask me questions about their younger children having sickle cell. And, like I said, the crucial thing that I say is you really have to be able to support that child, you know, mentally, because there are a lot of times as a sickle cell patient where you're just, like you said, "I don't want to get up out of bed today. It is hard. I'm tired. You know, I'm weak." And you just need that person there to help you understand, like, you know, this is the goal at stake and this is what we need to do to reach it. So I think you're definitely right when it comes to that support.

So, I have another question for you, what do you think as far as when you think of like a patient, your sickle cell patients now, how do you categorize them? Like what are the characteristics of a good sickle cell patient? And then, what are the characteristics of a sickle cell patient who's not doing so good.

Dr. Lee Hilliard: Yeah. So, I mean, that's the really interesting medically thing about sickle cell, is that it does affect everybody differently, even though patients with sickle cell disease have the same changes in their red blood cell, you know, why did you have a stroke? But you know, eight out of 10 other patients didn't. That's the frustrating thing for doctors, that we can't figure, you know, that part out. So by that, do you mean what are there things that patients can do?

Chris Ruffins: Yeah. So like, you know, taking your medicine, making sure you eat right, those type of things.

Dr. Lee Hilliard: Yeah. So all of that definitely helps mental and physical part, right? Like we talked about the mental piece and I think that needs to start early. I mean, we try to do that in clinic to say to families, "Okay, you have this disease and there's certain things we need to do differently, but don't let this define you. Go on and live your life, right? So mental part. And then physically, you don't want to add any stress to your body. So get enough, sleep, you know, eat as well as you can. And now, we're learning more about exercise and sickle cell disease. And we admittedly do not have the exact recipe for that because you know, going out in the Alabama heat and trying to run five miles is not a good choice. But there are different types of exercise that we think can be helpful. And one of my adult colleagues, Dr. Ogunsile, at UAB is actually doing research on exercise and sickle cell disease. So I hope we'll learn more, that we can, you know, give more precise recommendations for that.

Chris Ruffins: Yeah. I'll tell a quick story. You brought up the Alabama heat. And I remember I was about 15, 16 at the time and I was playing summer basketball and I practiced the day before I had a red blood cell exchange. And I don't know if you remember, but while I was at the hospital, I had finished my red blood cell exchange, but while I was getting something to eat out in the lobby, I passed out.

Dr. Lee Hilliard: I definitely remember that.

Chris Ruffins: Yeah. And I remember telling you and my mom, like, "Yeah, they didn't have air conditioner in the, gym that we were practicing in." And I guess it kind of just like, you know, hit me at all at once the next day when I, you know, pretty much got done, getting my exchange. But I thought that that's kind of like one of those things where you're like, "Hey, you need to make sure you stay hydrated". I think that was one of the things that you all preached to me the most, was like, "Hey, you need to make sure you're drinking as much water as possible."

The second thing I bring up is when I was recently in the hospital for my crisis, that's what I told the doctor how I pretty much stay away from hospitals as much. I work out at least four to five times a week. And I think that's like the key, so I think the research that y'all are doing is, very good. And I think you'll learn a lot that exercise, no matter what type of exercise it is, that it helps sickle cell patients function properly and well, you know, throughout their day.

Dr. Lee Hilliard: Yeah. Now, tell me, are you doing like weights when you say workout, you mean you're lifting weights? And then, are you doing anything aerobic?

Chris Ruffins: Weights, cardio. I don't know if meditation is considered as...

Dr. Lee Hilliard: Yeah. Now, there's all this research on that too, right? Like for any diseases or just for sports training that meditation and visualization clearly helps.

Chris Ruffins: Yeah. So, those are the three things I mainly do. And lifting weights is usually about 40 to 50 minutes. Cardio is usually about 20 to 30. And then, meditation is usually, I would say, 10 to 15. But, you know, it's not all in one day. You know, I kind of do it sporadically, but it is within a week, I do most of.

Dr. Lee Hilliard: Great. And the trickiest part of that, honestly, is the cardio part. And that's what Dr. Ogunsile is trying to understand a little bit better because you obviously don't mind sharing things about your medical treatment, so you were getting blood transfusion, right?

Chris Ruffins: Right.

Dr. Lee Hilliard: So you are less anemic than the average patient, so that helps you be able to do the cardio part better. So that's what we're trying to understand for, you know, a broader range of, you know, levels of anemia, what do we need to do for exercise and taking into account more severe anemia.

Chris Ruffins: Yeah. And I think the other part to that is with the cardio, you have to be careful because you can't overexert yourself.

Dr. Lee Hilliard: Exactly.

Chris Ruffins: So that's the tricky part of finding that balance of, you know, should I run or should I just walk, you know, those type of things to take into consideration.

Dr. Lee Hilliard: And walking is beneficial.

Chris Ruffins: Yeah. I usually walk. I hate running. I'd rather bike.

Dr. Lee Hilliard: I hear you.

Chris Ruffins: So yeah, that's pretty much how I usually do it.

Dr. Lee Hilliard: Yeah, I knew you and I are totally connected here because we love the basketball and biking.

Chris Ruffins: Yeah, we are. We are connected. I know we talked about the mental state of sickle cell, but like mentally, how can it have an impact on patients?

Dr. Lee Hilliard: Well, the first thing that pops into my head is just not knowing when your health is going to change. I mean, obviously, I don't have sickle cell, but just in seeing so many patients, just for the patient and for their family, right? Like, if you have a child and you don't know if they're going to get sick, are you going to be able to go to work? How am I going to be able to go to school? And just the unpredictability of the acute problems. And then, I'm sure for the patients that have more severe disease, they probably think, "Am I going to be able to do what I want to do with my life?" And that weighs on you.

And then, you know, we can't have a podcast about sickle cell disease and not acknowledge some of the healthcare disparities in this country. So the other thing I think about when you ask me that question is, you know, as a black person in America, unfortunately, you may have been in situations where you did not get the same care or the care that you should have simply because you're black. And that's a mental strain too, where you may go into some situations thinking you're going to have to, you know, be battling to get the care that you need, and that doesn't happen all the time, but you know, it's not just my opinion. There are research studies now that clearly show that that's the case. So that's the added factor, unfortunately, with this disease that we've just got to do better.

Chris Ruffins: Better, yeah. I think the thing that I've seen when we talk about the mental state, when I was in Charlotte, the hospital that I was attending there, they had an in-house therapist that you could see. And I thought that was really, really good. Of course, Children's of Alabama is the best hospital for pediatric care and for children. But this hospital, Levine Cancer Center in Charlotte, they had a really good system of having that therapist there. I'm just talking about my recent visit to the hospital, being in the hospital for so long and, you know, just being in that bed and having to take, you know, all types of medicine, it definitely takes a toll on you mentally. And I never realized how much of a toll it takes on you until this last time. And it was like, "Yo, I need to go see a therapist," you know, and being able to have a person in-house, you know, when you're done with your blood exchange or something like that. To be able to talk to them then and there, I think that's, you know, a great way to help patients. And I think a lot of hospitals should try to look into that to help the patients out.

Dr. Lee Hilliard: Yeah. Yeah. So we do have, you know, similar services like that here. There's always room for expansion of those things. But, you know, the last few years, we're trying to be a lot more cognizant about the mental side of recovery. And even simple things like when you have pain, we have some virtual reality goggles, things like that just to try to to help, you know, de-stress. But the fact that you can recognize that you needed some extra help and that's part of your medical care. I mean, when people think sometimes when you suggest that, that you're saying that they're crazy and that's obviously not the case that, you know, we've talked this whole podcast about the connection between the mental and the physical, so I think that's going to be an expanding area to help patients with sickle cell disease.

Chris Ruffins: Yeah, definitely. I definitely agree with that. Have you seen the symptoms of having sickle cell? Do they get worse as a person gets older? Or have you seen people not really have any problems as they got older dealing with sickle cell?

Dr. Lee Hilliard: Yeah. So, I mean, admittedly, I'm not an adult doctor, but just general, the things that get a little more difficult as you get older are going to be be some of the organ function problems, you know, like we talked about at the beginning where you can get organ injury over time. So some patients, for example, might get where they get short of breath, they might need oxygen, not everybody, but just as an example. And then, some patients, if they've had a lot of problems with the sickle cell clogging up their bones may have more trouble with their joints as they get older. So those are kind of the main categories of changes as you age.

Conan Gasque: Once again, that was Chris Ruffin, Jr., and Dr. Lee Hilliard. For more on sickle cell disease, visit childrensal.org and search sickle cell. Thanks for listening to Inside Pediatrics.