Intro 0:03
Welcome to Maximal Being, a podcast devoted to ditching fad diets and using real science to get you healthy and feeling great. I’m Doc Mok, a GI and functional medicine doctor who harnesses the power of gut health to get you achieving your goals. And I'm Jacky P, a well-informed layman who challenges the experts and ask the questions that you want. Don't forget to hit the subscribe button or leave a comment, and now onto the show.
Doc Mok 0:32
What's going on Maximal Beings, Doc Mok here with maximalbeing.com. Don't forget to hit the subscribe button. Leave us a comment, it does help us to get the word out. If you have any questions, you can email us at [email protected]. Enjoy the episode.
Jacky Homme 0:48
Hello, hello, hello, Maximal Beings. It is I, Jacky P, and I am here with some new friends. I'm not here with Doc Mok. Doc Mok, my heart goes out to you. He's in Tampa. He had to get out of the way of the storm. So sorry, folks. It'll just be me today. So you won't have the big old brain that Doc Mok is. But luckily for us, I've got Dr Scott Peslak here, who is another big old brain. So we're tagging you in. As well, I've got Kyle Smith, a good friend and a fellow sickle cell warrior, and today, we're going to talk about something that is very close to me and impacts my life, obviously. I've spoken about sickle cell before on the on the show, but first I want to introduce our our wonderful guests. So first, Dr. Scott Peslak, who go by Dr. Scott. How are you today? And introduce yourself to the folks.
Dr. Scott Peslak 1:50
I'm doing great, Jacky, thank you for having me on the program. It's really exciting to be here and to be able to share this conversation with with you and Kyle. So my name is Scott Peslak. I am a physician scientist at the University of Pennsylvania. I see patients at UPenn with sickle cell disease and thalassemia, and I also study sickle cell disease in my laboratory here at Penn. So I've been really fully committed to taking care of sickle cell patients and other red blood cell disorders in my role here in Philadelphia.
Jacky Homme 2:22
Awesome. Appreciate it nice to have you. Dr. Scott and Kyle, if you'd like to introduce yourself to the wonderful listeners.
Kyle Smith 2:28
Yeah yeah, absolutely. Thanks so much for having me. Jacky. I'm excited to be here with you and Dr Scott. Kyle Smith, I am a nonprofit professional. I work for a corporate affiliated charity, but I also have my own nonprofit that is dedicated to serving individuals with sickle cell disease here in the Philadelphia area, called Crescent Foundation, which Jacky is a board member, you know, and I'm sure we'll talk more about that, but just really looking forward to talking more about sickle cell and you know, just what's happening within our community, and also how we were able to celebrate Sickle Cell Awareness Month in September.
Jacky Homme 3:11
Absolutely. And and thanks for plugging Sickle Cell wellness, awellness Awareness Month. Wow, sorry about that. It's, it's October when we're recording this. But you know, it should be Sickle Cell Awareness Month, every month, you know, I like to quote you Kyle, actually, at that event you said, you know, sickle cell should be that default. You know, I forgot what you said exactly, illness in your brain. When people say they think of a sickness, they think of something that needs research for better treatment or cure. Everyone goes to the typicals, right? The cancer, but no one really thinks about sickle cell. Sickle cell is such a, you know, impactful disease to live with, and it is something that even amongst, I would say, the medical field, not very well known. You know, I'm not too sure if you've had this experience, Kyle, where I've gone to emergency room outside of my home city, and the nurse in the emergency room say, hey, so were you born with sickle cell? Right? And then I know immediately I am in trouble, right? Because it's like, I've gotta deal with the pain, and also educate the person who's supposed to know how to take care of myself. So
Doc Mok 4:25
Are you a healthcare practitioner searching for a comprehensive solution to streamline your patient referrals and improve collaboration with other practitioners? Look no further than Rupa Health, the ultimate platform for integrative and functional medicine practitioners. As a health care practitioner who has struggled with the traditional referral process for labs, it's time-consuming, inefficient, and often lacks the necessary communication between providers and patients. But ever since I started using Rupa Health, everything has changed. That's right. Rupa Health is revolutionizing the way that practitioners connect and order lab work with their patients. Their advanced platform allows you to effortlessly send and receive patient lab referrals and secure their information, sending them the kits directly. This makes the communication directly with other patients and practitioners easy to take care of. I can't stress enough how much time and energy Rupa Health has saved me. With just a few clicks, I can easily order lab work and track their progress and then I receive the results directly into my inbox, which I can send to the patients automatically with recommendations. Absolutely, Rupa Health’s extensive network of lab testing and curated integrated and functional medicine testing allows practitioners to receive the highest quality of care and dedicate their practice to a patient-centered holistic approach which aligns perfectly with the values of health care in a functional medicine practice. Plus, Rupa Health provides you with excellent customer support. Their team is responsive, knowledgeable, and always helps with assistance in billing questions, and how kits are shipped to your patients. They are dedicated to helping practitioners like us provide the best possible care for our patients and if you're a patient listening, it allows you to seamlessly order your lab testing and receive all the necessary information. Rupa Health has been a game changer for my practice and as you know it will be for yours too. Don't miss out on this incredible opportunity to save on lab testing by heading to labs. RupaHealth that's R-U-P-A .com\store\storefront_ V as in victory, G as in grape, X 0-0-4-0-0. That's labs.rupahealth.com\store\storefront_vgx0040. I'll see you later Maximal Beings.
Jacky Homme 6:58
Let's first you know, for the folks out there who aren't aware of what sickle cell is, right, I'm going to do something different this time, just because this is a little bit unique. I want to have Dr Scott explain sickle cell right in the clinical sense, and then Kyle, I would love to hear how you explain sickle cell to folks. I know there might be some overlap, but I think it'd be interesting to see the difference. So, Dr Scott, if you want to go first,
Dr. Scott Peslak 7:27
Absolutely. Thank you. Jacky. And so sickle cell disease is a, as you mentioned, a an inherited genetic disorder, right? This is something that, if you're starting at that point where your providers don't know that that is, that is something we need to focus on, as far as helping to educate the general public and providers as to what sickle cell disease is. So this is a genetic disorder, and this is a change in what's contained inside your red blood cells. The red blood cells circulate around your whole body, and they deliver their job as a deliver oxygen to all kinds of different organs and tissues throughout your whole body, and the way that it does that is by that oxygen help is bound by a protein in the body called hemoglobin, and so normally, hemoglobin binds and delivers oxygen. In sickle cell disease, there's a single change in one of the genetic code, the genetic letters in DNA in hemoglobin, that leads to a change in the hemoglobin, at which point, if you have lower amounts of oxygen, those cells begin to form these stiff fibers in the cells themselves. And that causes changes in the shape of the red blood cell that leads to blockages in the blood vessels something called vaso occlusion, and it makes the cells a little bit more fragile and likely to break down a process called hemolysis. The combination of this vaso occlusion and hemolysis leads to really complications in all areas of the body where blood flows. And so this really is not just a blood disorder. This is a disorder that affects every organ in the entire body. And the primary complication that many people have seen, if you've ever taken care of a sickle cell patient in the hospital or talked with a sickle cell patient is pain, basically pain. But that's really not the only thing that not nearly the only thing that we see this really can affect many, many different organs of the body. It can cause chronic neurologic changes, including stroke. It can cause significant changes in kidney function, lead to chronic kidney disease and even lead to dialysis in many patients, it can lead to degeneration of bone and problems with the hip and shoulder joints, problems the lung and heart, and even problems in the eye and so, you know, regular eye exams are really critical for our sickle cell patients as well. So we really think about this as how do we, how can we help to change and reduce the not only the pain that sickle cell patients face on a daily basis, but also to prevent many of the complications that can happen in these in organ throughout the body, to help patients really live full lives and be able to, to be able to understand how they can modulate that risk through different therapies, to be able to live better, more productive lives as well.
Jacky Homme 10:25
Awesome. So for those listening, you can tell Dr Scott's been in some some medical books, right? He knows. So thank you for that, Dr Scott. And that's a very good explanation of just really the you know, the physical like, how does sickle cell impact the body? Right? So Kyle, when you're out in the world and you meet someone and they say, hey, oh, you know, you have sickle cell. What? How do you explain it to folks?
Kyle Smith 10:57
Yeah, I think that there will be some overlap, but definitely, you know, a little more grounded, you know, in Dr Scott's version. So usually I, I'll, I'll just tell people that, you know, sickle cell is a genetic disease. And you know how, you know, we all have red blood cells, you know. And there are these circular and squishy discs flowing through our bodies, and I have that too, right? We all have that. But those red blood cells sometimes, when they don't get enough of a particular protein called hemoglobin that you know, transfers oxygen to our red blood cells, they become less oxygenated. And those red, circular, squishy blood cells become hard and rigid, and, you know, crescent-shaped, and you know, block the blood flow of of the the body. So understanding that there's a blockage within, you know, our our body, there is a build up of those of other blood cells, kind of flowing, right, which causes inflammation, you know, which then proceeds to cause pain, and, you know, kind of go into the other complications that sickle cell could occur, like stroke, like, you know, organ failure, so on and so forth. So that's my kind of like go to is just kind of really trying to relate to an individual and have them understand that, you know, the genetics, you know, are the same, except it's just like a that difference in terms of we don't get that, that really crucial oxygen part, That's where things kind of go a little haywire.
Dr. Scott Peslak 12:44
If I could add to that
Jacky Homme 12:45
Yes, please go ahead.
Dr. Scott Peslak 12:46
I agree. That's a that's a much better way to phrase that. So thank you. I will say that that to add on to that point. You know, one of my, one of my mentors, always said, if, if you've seen one sickle cell patient. You've seen one sickle cell patient, right? Every single patient, even though it's the same genetic change that happens that leads to all the changes that can happen in sickle cell disease, every patient is completely different. And one of the things we're trying to understand as a field and with our patients is is how how to predict that and how to give the right therapy to the right patient at the right time, and how and the way to do that is really to come together between our patients, our community organizations like Crescent, our researchers, to really understand sickle cell disease in a much broader context, to be able to provide the best possible care to our patients.
Kyle Smith 13:40
Yeah, no. And thank you so much, Dr Scott, for adding that in. And I think it's crucial, you know. And something that we recognize, of course, is that this is such an individualistic disease, and there's no one size fits all, which I'm sure we'll recover more throughout our conversation today. So thanks for adding that.
Jacky Homme 13:59
Yeah, yeah, that's, that's also a good point, too. And also, you know, you can almost interchange sickle cell with almost any, any disease, right? When we sometimes, you know, human beings, right, we fall into the habit of, just like, recognizing patterns and attaching patterns to things. And me personally, you know, I've had issues, you know, where I go to I go to the hospital, or I reach out to doctor, and it's pain or something, and it's, oh, well, you have sickle cell. It's probably just a sick cell, right? And it's, in a way, it's not, I don't want to say it's a dismissive it's just like they feel like, oh, you've got pain in your leg. You've got pain in your joints, right and sickle cell. And interestingly enough, I've been suffering from gout for years. For four or five years, I just got diagnosed with gout like over the summer, because finally, someone at the ER said, hey, you know what this might be gout? Well, let's, excruciatingly, right, put that needle in your joint and pull out the fluid, and, you know, I've got, which is, of course, one of the many wonderful side effects, right? We deal with dealing with a sickle cell. But back to how I like to describe sickle cell to folks. Is one thing I would, I would say is one thing I really admire about Kyle is that Kyle is very educated about sickle cell, and for better or worse, when I was growing up, I had family members at sickle cell, but my mom kind of raised my brother and I to, like, think of sickle cell almost as an afterthought, like, take care of yourself, hydrate, eat well, make sure you're rested, right? So, so you don't get yourself sick. But it was almost something like I would not remember I have sickle cell until sickle cell remember that, you know that sickle cell was affecting my life, and I think one had helped from a mental standpoint, right? Because, you know, one thing that's occurs a lot with sickle cell warriors is the mental health aspect. But it took a long time for me to actually learn about sickle cell and learn all the different types of sickle cell and how it can impact your body. So when, up until recently, I used to just tell people that it's a storm, all right? And we're talking about the hurricane, alright, right? It's a storm. I'm like, I don't know when the storm is coming. I gotta stay prepared for it all the time, but the storm is going to come, it's going to stop everything, it's going to decimate everything, and when it's over, I got to clean up and hope there's just not a storm coming around the corner soon. And for me, you know, we I forget, remind me the new word. We don't say crisis anymore. We say Kyle, what's, what's the word we use now?
Kyle Smith 16:52
Oh, it was VOEs, right?
Jacky Homme 16:54
VOEs
Kyle Smith 16:55
Episodes,
Jacky Homme 16:56
Yes, episodes, vasal, occlusive, episodes. So I grew up with crisis, right, which is when you have pain. So I was moderate. You know, I'd have pain a couple times a year, four to six times a year, those would be intense, almost always hospitalized, almost always a week or more. But, you know, and we can talk about this now, the different degrees of those that fight with sickle cell. Because for my brother, my brother was on the other side of the coin. He almost just kind of always had some form of pain, light pain. He can function. He could go to work, go to school, fine. And I was opposite, you know, a lot of people, they see me, you know, and they wouldn't know until they're like, oh, Jacky, where were you the last couple weeks? And like, oh, well, I just got out of a little I used to call it hospital vacation, just because you try to find you put a foot aside on anything. So
Doc Mok 17:51
Are you looking for high-quality evidence-based supplements to support your functional medicine practice? Look no further than Fullscript, the leading platform for healthcare practitioners to prescribe and order professional-grade supplements. As a functional medicine and gastroenterology doctor, I trust Fullscript to provide me with a wide range of high-quality supplements that meet specific needs of my patients. Their extensive product catalog includes trusted brands, ensuring that I have access to the best options for my patient's health. That's right. Fullscript offers a comprehensive selection of supplements, including vitamins, minerals, botanicals, and specialty formulations, all sourced from reputable manufacturers. Plus their rigorous quality control ensures that you're getting products that meet the highest quality standards of purity and potency. And what I love most about Fullscript is the convenience it offers. With their user-friendly online platform, I can easily browse, prescribe, and manage supplement protocols for my patients. It saves me valuable time and streamlines the ordering process. Absolutely, Fullscript makes it easy to create customized protocols for your patients and track their progress. Plus they handle all the logistics from inventory management to shipping so you can focus on what matters most, providing excellent care to your patients. So if you're ready to take your functional medicine practice or wellness to the next level, visit us.fullscript.com/welcome/maximalbeing that's us.fullscript.com/welcome/maximalbeing to receive your 15% discount on customized supplements and check Maximal Being standardized protocols for gut health. Fullscript has been a game changer for my practice. And I know it will be for yours, don't miss out on this incredible resource for functional medicine practitioners and patients alike today.
Jacky Homme 19:41
I like to take this time to talk about just the different, I guess, different kinds of sickle cell, right? Because there's SS, you know, there's, it presents itself in a lot of different ways. So Dr Scott, if you can in the most layman's terms possible, right? Gotta talk about like, the broad range of sickle cell. Cell, you know, for the folks listening,
Dr. Scott Peslak 20:03
Sure. So I think, as as many, many listeners probably know, for every gene, right, everything in your body, there's two copies of everything, one from your mom and one from your dad. And so the generally, when people hear talk about sickle cell disease, the most common form of sickle cell disease is what's called sickle cell SS disease, which means you inherited one copy of the sickle hemoglobin from one parent and one from the other. And and so that is the medical term for that is homozygous. But essentially what that means is you have, you have two sickle hemoglobin copies in your DNA and all your red blood cells the there's another form of sickle cell disease. Probably the second most common form, is something called hemoglobin SC disease. So this is that you inherited one copy of sickle hemoglobin from one of your parents and from the other parent inherited another kind of a change in hemoglobin called hemoglobin C, which by itself, hemoglobin C causes the red blood cells to be a little bit dehydrated, a little bit less fluid inside. Doesn't really cause many problems, but in combination with the hemoglobin S, the sickle hemoglobin can cause either very mild or very severe forms. There's a broad range of severity in patients who have hemoglobin SC disease, much broader than in patients who have SS disease. And so there's a lot of variables beyond just these genetic changes that can affect severity. There's another subset of hemoglobin, something called fetal hemoglobin, that I study in my lab. And many, many people are trying to understand if we can develop new drugs to increase this protective form of hemoglobin that some patients, for reasons we don't really understand yet, have higher levels than others. There's and there are many social and environmental factors that can play into this as well. And as Jacky mentioned earlier, the psychological aspect of any chronic disorder is a major key factor in in determining responsiveness and therapy and and how people live at a day to day basis. And so this really does play a significant role. And you know, many programs, including ours, have embedded psychologists within the program to be able to provide mental health support, which is really, really critical. And so I think there's many different things that can play into the changes and the severity that we see in our patients, but one of the primary ones that that that we do look at is what that sub genetic change is with sickle cell disease.
Jacky Homme 22:46
Thanks for breaking that down Dr Scott, and yeah, it's and one thing that's important about sickle cell, right, is I have a friend who is a sickle cell warrior, and he wasn't diagnosed until 18. So you it doesn't always a very obvious present itself early on in life, or sometimes it just can go misdiagnosed. So it's important to understand, realize that folks can get checked, right? And we've kind of discussed this before. You know when we were when we had that panel a couple weeks back about educating the community right, educating people and and, and that's medical and non medical that you know you can have sickle cells, possibly can have sickle cell and not even know, and you're, you're dealing with the side effects, you're dealing with the pain or other other problems that come with it without knowing, like, hey, you know, without having that official diagnosis. So I like to talk a little bit about just like the care, the care side of sickle cell, because, of course, like everything, there's a spectrum. You know, you can be like myself or like Kyle, who we very well aware what we have. We're on top of it. We're lucky enough to be in a very good hospital system that takes cares of us. But that's not always the case, right? So Kyle, if you can share some of your vision, you've done a lot of this at Crescent, but what would you say are some of the I would say maybe hurdles, or, you know, obstacles that that, that you've seen in our community, that you're trying to address with, with Crescent, and how it's impacted, you know, our our wellness overall.
Kyle Smith 24:36
Yeah, sure. I think so. I want to go back to one thing you said as well, too, before addressing the care. I think that it's important for folks to know just their, as I like to call it, their status, you know, in terms of whether or not they do or don't have a carry or a carrier of sickle cell trait, right? And, you know, I think that this is super important. And especially when it comes to larger life decisions, like family planning, you know, and how that could really impact, you know, whether or not you could have a child with sickle cell, right and, and I think that, you know, it's a common belief that sickle cell is a quote, unquote, like black disease, but, you know, as we talked about, as Dr Scott said earlier, you know, this is a genetic disease. You know, there are people who, of course, here in the US, primarily it impacts people who are black and of African descent. But you know, there are Mediterranean folks with sickle cell disease who, you know, present as a white Caucasian presenting, right? And there are also a large sickle cell population in India, right? So it's just a genetic disease that impacts everyone. And I just am very passionate about folks just understanding whether or not they are carriers of the trait. You know, you can go to your primary care physician ask for a hemoglobinopathy to see whether or not you have sickle cell trait. And you know that simple, so simple blood tests to talk more about the care. There are so many directions that I could go, but I think that just understanding the history of sickle cell is super important, right when it comes to care. You know, I was born in a year when sickle cell had just been sickle cell screening had just been mandated at the hospital that I was born at in 1989 so was fortunate enough to, you know, be diagnosed at three weeks. Unfortunately, you know that's not the case for everyone, as you said with your friend who was 18 years old right during that period of time, you know physicians were still learning more about sickle cell disease and really just trying to get us to live into our adulthoods, right? And weren't really focused on what life would look like once we became adults, right? So unfortunately, you know, there was no true preparation in terms of, like, what that care could possibly look like. And unfortunately, since there are, you know, a lot of vaso occlusive episodes that are a symptom of this disease, which just causes, you know, incredible amounts of pain. The treatment, primary treatment for this symptom is opioids, right, to help with the pain, deal with the pain, and understanding that, unfortunately, a lot of patients have to enter the emergency department and to, you know, try to get care for when they are having these VOEs, knowing that opioids are the primary type of treatment. Unfortunately, there is a large stigma when it comes to individuals living with sickle cell disease, of being drug seekers, right? And so there these labels are, you know, of course, unfair, and you know bias as well. But you know, again, this is the treatment that the only treatment that we've been given for the symptom of our disease, right? So what I have been trying to do is really re envision what a new world or new care model would look like outside of sickle cell patients entering, you know, the emergency department, because when we think of emergency departments and a VOE is 100% an emergency. But that doesn't necessarily mean we need to go to the emergency room, right? You know, and we are, whenever you enter an emergency room, you are classified in terms of, like, how high your emergency is. Is it level one, two, or three, right? And you know, if you are entering a sickle cell patient is entering the emergency department, they probably will take a backseat to the gunshot wound that just came in, or the stabbing that just came in, so on and so forth. And unfortunately, this just, you know, are still they are still really suffering in pain and also not being monitored for any other complications that could occur from that crisis too, and it leads to really prolonged waits for treatment. And I'm talking 5 6 7, I've heard 20 plus hours before waiting to be seen in the emergency department. So with something that we've been working on with the medicine, is trying to really expand hours of what's called a infusion center, which is nice, where you can go in one day any time. And between. Between, excuse me, you can go in one day, Monday through Friday, from about nine to five, you know, if you're having a crisis. And you can get IV medic, IV pain medication, and also IV fluids, you know, up to three doses. If those three doses really don't touch your pain, then you they will likely admit you, right, and you will get the whole like, work up like that. But these types of, you know, infusion centers, or, I guess, kind of different routes that are really, you know, circumventing the emergency department are huge for our community, because, you know, it lessens, it lessens the weight that you, you know, you'll have to undergo. It lessens the stress that you mentally will put yourself through. Because you're thinking like, Hey, I don't want to be labeled a drug seeker. What do I have to wear to go to the emergency department? You know? What should I put on? I don't want to be, you know, labeled I'm a linger because, you know, not everybody believes My pain is real, so on and so forth. So these types of, you know, these types of, I guess, off ramps outside of the emergency department are huge. And you know, Dr Scott has been really helpful in trying to dream along with us, and, you know, looking forward to, like, trying to really expand those hours. Because, as you know, Jacky, sickle cell, and I like how you, you formed it. You said it's a storm. The storm doesn't have a set date and time, right? It doesn't just happen Monday through Friday between nine to five, right? So, so, yeah, that that's something that we're trying to address when it comes to the care. One thing also, too, is that not everyone has what, where they are, what we call a sickle cell expert, right? Dr Scott Peslak is what we call a sickle cell expert. Not everyone has that. You know, maybe they just have a hematologist. And hematologists are great. You know, they're wonderful. They know about sickle cell but they are not sickle cell experts. We need individuals to be have some type of medical home where they are seeing a sickle cell expert so that their individual disease is fully being addressed. Because, like we said, this is not a one size fits all. There are many complications that go along with this disease, and it really presents itself in so many different ways. So that's I'll pull up there. Yeah
Jacky Homme 32:33
No, I appreciate you sharing that, because I think you know, we haven't talked to ourselves, Kyle, but like, I'm sure you have story. I mean, I don't know anyone who has sickle cell or that doesn't have a story. You know, I have friends in Philly who say, yeah, when, when I'm in pain, it's 2am in the morning and I'm in like, 10 out of 10 pain. I literally get dressed up. I put on my work clothes just because they might help me get a little bit more empathy from the nurse when I need pain meds, so they don't think I'm a drug dealer, right? So, you know, these are the things that sickle cell warriors, I like to use the word warriors, because we are, have to deal with, right? No one breaks their arm or has a cold or feels ill, in most demographics of illnesses have to ever worry about what they look like when they go and look for care. They just go and look for care and expect to be taken care of. And, you know, there's a lot of things to be said about that, but you know, that's one thing I do appreciate about you, Doctor Scott, is that you understand that and you realize that. And if there's anything that you can comment on like, you know, if we're waving a magical wand. Or be like, a vision being visionaries here, like, what would you like, want to see? Or, what do you think are some actionable items that you know, maybe someone who's listening that isn't in our fine town of Philadelphia where, like, these changes are being done. But like, what do you think are some steps that can be done in those different, you know, cities and hospital systems out there.
Dr. Scott Peslak 34:04
Yeah, great question. Thanks, Jacky, yeah. And I appreciate your perspective on that Kyle. And thank you for the kind words as well. I think so, a couple things just to comment on Kyle's points, and then I'll get to that point as well. I think the emergency rooms is, I would say, you know, sick sickle cell patients do not want to come the emergency room. Nobody wants to be in the emergency room, right? It's, it's a, it's, it's a spot where it's kind of the last resort in as far as care. And so you're absolutely right in that we, we are working, I tell you, I can tell you, we're working very hard with the ER at Penn to try to improve time to care. You know, we take a lot of steps back post covid, but there's been a lot of progress. I just had a meeting with a very talented second year medical student here this morning, who's also very passionate about sickle cell patients, and we're working to do quality improvement here at Penn, across the Penn Medicine system, and this is happening throughout the country. So it's a it's a long process. But there are a lot of people that are really engaged and that are committed to improve care in the meantime, that we try to, like, try to avoid going to the emergency room. So models like day hospitals and infusion centers really are critical and are have been shown to be standards of care across the country. And I'm very proud of our team here, and I think they've really one of the most critical parts about a infusion center is that there's a consistent team who knows what sickle cell disease is, how to take care of patients. We have individualized care plans for patients, and I try to avoid the term pain plan, because it's not just pain, it's everything else, right? It is truly a comprehensive care plan that we develop, and that really can be done in many centers, but it's best done in a comprehensive sickle cell center with with the team, and we're we are looking to expand our center and many others, but the key part to do that is to get more young people and providers engaged in sickle cell research and clinical care and and so you know what? What? What you're doing, Jacky with this podcast, and what Kyle is doing with the Crescent foundation and and what we're trying to do as well, is to try to improve visibility and improve excitement about caring for our patients, and continue to do research in this field. I think that the and your analogy earlier about the storm I think, is a really apt one, and one that I use very commonly with my patients. I say that, you know, we if, if the storm surge is high, right? And you even have a little wave that comes up, that can be devastating for patients, and if you but if you can lower the overall height of that storm surge and over of the ocean, even if you have some waves, that doesn't get to a point where it's a vaso occlusive, full vaso inclusive episode. And so there's different ways to do that, but one of the main ways to think about disease modifying therapies that there's been a lot of excitement in the field, this is what I study in lab, and one of the main ones we've used for many years is a medicine called hydroxyurea, which helps to make the stiff sickle cells, as Kyle mentioned before, more full and more round and more flexible and floppy to be able to pass through the blood vessels more easily. But there's many others that I'm happy to talk about here as well, and including including gene therapy, which is very exciting and new, new therapies that are coming out of the clinic. I think, though, beyond just saying we should develop new therapies, I think the key part is understanding what sickle cell disease is at a broad level nationally. There has not been, historically, national registries of sickle cell disease to understand, in a population level, how patients do with which kinds of therapies, how we can predict who's going to do better, who's going to do worse, who needs more intense therapy and who maybe doesn't? And so there's been a lot of efforts from national organizations, including the Sickle Cell Disease Association of America and the National Association for sickle cell centers, in addition to the American Society of Hematology, all of which are working on national registries to better understand sickle cell disease in patients that now are doing great but need better and more defined care plans as they age into their 40s, 50s, 60s, 70s. That's something that is, I think, is going to be become one of the most important things to be able to solve as a sickle cell community. And we can do this by sharing, sharing knowledge and sharing information across the country and many different care settings, many different populations of patients to to better take care of to better take care of our sickle cell community.
Jacky Homme 38:33
Awesome. And thanks, thanks for sharing it. Dr Scott, yeah. And, and I think that is one thing that we should strive for, as in the sickle cell community, is just the greater just permutation into just a common knowledge of, I mean, even just the basics of the sickle cell, like existing, right? You know, I, I was, I was in Orlando, Florida, speaking of Florida, I was sick, and you know that that was the instant like, oh, sickle cell. Like, were you born? They're like, okay, the person, the nurse, may or may not have even known what sickle cell was on a very basic level. So the education and awareness is very important to make sure that we can move further the cause. But we are going to take a quick break to come back here. I've been talking with Dr Scott Peslak and Kyle Smith, and we will be back in just a second.
Doc Mok 39:31
The content included is not intended to be used as medical advice, and viewers should consult their physician or healthcare provider. Should they have additional questions? The viewers should not rely on information contained in these presentation for immediate or urgent medical needs. Additionally, if you think you have a medical emergency, call your physician or go to the emergency department or call 911 immediately. Never disregard professional medical advice or rely on seeking medical care or. Delay medical care due to information contained in this presentation.
What's going on? Maximal beings, Doc, Mok here, if you haven't done so already, leave us comment and hit the subscribe button. Let your friends and family know, that way we can get the word out and continue to bash the bro science you.
Transcribed by https://otter.ai
