Joey Wahler (Host): They go hand in hand. So, we're discussing sickle cell disease and blood donation. Our guests are Dr. Jennie Law, a hematologist-oncologist at the University of Maryland, Greenebaum Comprehensive Cancer Center, an Associate Professor of Medicine at the University of Maryland School of Medicine; Dazja Marshall is with us, a sickle cell patient working with Dr. Law for five years now, who was a blood donor recipient this past year; and also joined by Sherman Roy, Division Diversity Program Manager for American Red Cross Blood Services, Central Atlantic Division.

Welcome to the Live Greater podcast series, information for a Healthier You from the University of Maryland Medical System. Thanks so much for joining us. I'm Joey Wahler. Hi there, everyone. Welcome.

Jennie Law, MD: Hi.

Dazja Marshall: Hi.

Sherman Roy: So good to be here.

Host: Hello. Hello. Great to have you all aboard. So first for you, Dr. Law, for those not as familiar, what exactly is sickle cell disease in a nutshell? And who is it that's most affected?

Jennie Law, MD: Great. Yeah. Thank you so much for having us on this podcast. This is such an important topic. Sickle cell disease is a group of inherited red blood cell disorders with red blood cells abnormally shaped and rigid like a crescent or a sickled shape. That sickled cell does not live very long in circulation, can result in hemolysis or breakdown of the red blood cells resulting in anemia.

In addition, these sickled cells can get stuck in small blood vessels, which can result in significant pain and other organ toxicities for our patients.

Host: So, tell us a little bit more there about how sickle cell impacts the body, especially over time.

Jennie Law, MD: Over time, patients with sickle cell disease can be prone to recurrent, acute, painful events called vaso-occlusive pain crises. These can result in frequent admissions to the hospital for pain control. In addition, there are a host of head-to-toe complications that affect individuals living with sickle cell disease.

These can include strokes, significant retinopathy, blindness; avascular necrosis where their bones are not getting enough oxygen leading to chronic pain from joint damage and early joint replacements; acute chest syndrome, which can be a very scary complication in which there is not enough oxygen getting to the patient and it necessitates emergency therapies; damage to the kidneys, which can result in spillage of protein as well as potentially complications such as needing dialysis therapy. Our male patients can be impacted by priapism, and there can be potential impacts on fertility for our patients.

Host: So Dazja, when were you first diagnosed with sickle cell and what was it like getting that diagnosis?

Dazja Marshall: My first realization, I guess, of having it is my mom telling me that, in the womb, they tested for it and that they knew at that I was going to be born with it.

Host: Obviously, that was young as can be. And so, when you were first aware that you had this, do you remember what your reaction was? I know you were probably quite young.

Dazja Marshall: Yes. I want to say 10 or 11. So, about that late elementary school era going into middle school. When you start to be a lot more active, kids want to go outside, you get to go to sleepovers, that's when I realized I wasn't, I guess, normal like everyone else. I wasn't able to just jump in the swimming pool when it's 50 degrees outside or go camping in the woods and spend the night and all of the different triggers that come along with having it and not understanding it at such a young age can be very terrifying.

Host: I'm sure, I'm sure. Did there come a point when you were able to, if not accept it, at least understand it and deal with it better?

Dazja Marshall: I want to say as I got older in high school, when the next phase of life, when you're getting older, that stage of feeling grown, you're able to work a job now, because you might be 16, 17, you might be able to do things on your own now without your parents. And I started to also realize or trying to realize: "Why do I have it? How did this happen?" I need a little bit more explaining, because I don't have much family that have it. So, it was kind of a lot trying to process it at the most in that era of my life, from 16 to about 18 years old, I'll say.

Host: I can imagine. So Sherman, for you, how do these blood transfusions help patients like Dajun? Why is it so important that the blood is closely matched in this case?

Sherman Roy: So, the blood transfusions do provide a life saving treatment patients like Dazja, when suffering a sickle cell crisis, primarily by increasing the number of normal red blood cells that are in the body, which in turn help deliver that oxygen through the body and unblock those blood vessels from those cells that have been hard and crescent shaped. Closely matched blood is critical in preventing patients from developing immune responses to blood that may have come from a donor that was not necessarily a compatible match. It's also critical in preventing patients with sickle cell disease from experiencing any transfusion-related complications. we do know that there is over 300 known antigens running through our blood, and most of those antigens are unique and specific to our racial and ethnic background. So, it is very important that that blood is closely matched.

Host: So Dazja, what's your experience with blood transfusions been like in affecting your daily life and now your long-term health as well?

Dazja Marshall: I would say great, honestly. I haven't had any complications ever since I can remember my first blood transfusion. They always made me feel better. Yeah, I want to say, as I say to everyone in the world that asked about the side effects and everything that comes with the stereotype of getting blood transfusions and feel like you're injecting someone else's blood, it definitely helps a lot with sickle cell patients and it's able to recoup energy that we tend to lose when we have crises and admitted in the hospital for a long time. So, I am grateful that we came up with the blood transfusion whenever it was created, because it definitely feels like a lifesaver. It helped me a lot this past December when I got my last one. So, love transfusions. Nothing bad to say about them. I think they're amazing.

Host: Great to hear. And I'm guessing, just having met you a couple of minutes ago, you strike me-- you mentioned energy-- as someone that has a lot of it when you're healthy, right?

Dazja Marshall: Yes, love to. If I could be way more active and bungee jump out the sky, I would love to--

Host: Okay. I guess that's more a question for you, Dr. Law, about bungee jumping and such. We'll save that for a little bit later on. But first, doctor, you ran down some of the more common symptoms of sickle cell earlier. How about as the result of these blood transfusions, some of the more common symptoms and complications that you see in sickle cell patients, and how are they managed and treated?

Jennie Law, MD: Yeah. So for many of our outpatients, chronic exchange-based transfusion therapy is the mainstay of their therapy. So, monthly, patients will come to the hospital as an outpatient, undergo what's called red blood cell exchange, where we transfuse about anywhere from two to maybe eight units of blood at one time, while also removing sickled blood from the patient.

So, the idea is that we're exchanging out the sickled blood for non-sickled blood in an effort to keep the hemoglobin S or sickled hemoglobin at a very low percentage on a chronic basis for patients. In the inpatient setting, when someone's admitted for an emergent complication like a stroke or acute chest syndrome, we will do red blood cell exchange in an emergency setting to really quickly try to get sickle under control or take sickle out of the picture by lowering the hemoglobin S as fast as we can.

Host: Now, Sherman, you work with, of course, many families affected by sickle cell. And so, everything the doctor mentioned there, that can take place of course until you first get the blood. And so, what are the emotional or logistical challenges that patients and families face when trying to find that right donor match, and how do you walk them through that and help them deal with that, because that can be challenging in and of itself, right?

Sherman Roy: Absolutely. Absolutely. I would say the most often that I encounter and that I hear when interacting with patients is the fear of going into a sickle cell crisis and arriving to the hospital and being told that they don't have that proper match or the blood that is necessarily needed. Oftentimes, I hear that patients have to wait hours, sometimes even days for the hospital to locate and receive that blood.

Limited treatment options is another one. So, we know that there are curative treatments out there, such as gene therapy and bone marrow transplant, but these are not widely accessible or approved for all sickle cell patients, which leaves many patients having to manage their pain with pain medications or blood transfusions and so forth. These help to manage those symptoms, but unfortunately these are not cures. Another would be finding the right provider. Many patients who live in rural areas report having to travel far out to find the proper physicians and facilities to help treat and manage the disease.

And finally, and to me, the most impactful is hearing of the mental and psychological drain that comes with dealing with the disease. A lot of patients report dealing with depression and anxiety as it relates to coping with some of those day-to-day challenges. And I think the biggest, for me, being an advocate for educating the community on the awareness and the importance of donating blood. I think that that's what we can do to let these patients understand that the Red Cross is here to support. The Red Cross has several initiatives and programs in place to continue to create that awareness and increase our blood donations to, in turn, help sickle cell patients.

Host: The Red Cross always there when you need them in so many different ways. Dazja, back to you for a moment. You mentioned, of course, that you're doing great now, which is awesome to hear naturally, but what are some of the biggest challenges you faced with sickle cell care, be it physically, emotionally, logistically? And what gives you hope or comfort when you've been struggling?

Dazja Marshall: So first, some of the biggest challenges with dealing with sickle cell care, I would say, number one, is being believed, having people believe in invisible illness to them that they can't see and seem to understand is a really big struggle mentally and physically.

I would say another one is what Mr. Sherman touched on in regards to finding providers that are in your area, in the rural areas that generally care about you more than just a patient on the paper. So, those are my top two, I would say, in regards to the first question. The second thing-- I'm sorry, if you could repeat the second question if you don't mind.

Host: Sure. When you're going through some of the challenging times, what's gotten you through that?

Dazja Marshall: I kind of have a little obsession with dancing, as Dr. Law know. I love to dance, so that's kind of my escape place. Music, movies actually being active, I genuinely love working out. If I could work out way more, I totally would. And family, I have a big, big, super gigantic supportive family that I genuinely can say, if it wasn't for them, I don't know if I would've gotten through some of the crisis and some of the hospital visits I've had numerous times in one year because it can be, as everyone said, very mentally draining. It can be very physically draining. It can be very just draining in all aspects when it comes to you working, going to school, very challenging. So, you do have to find whatever works for each person for their outlet of escaping from the reality of having sickle cell and dealing with it on a daily basis. Everyone thinks it's just dealing with symptom of when you're sick. That's the only time we deal with sickle cell crisis, only when we are having one in the moment. No, that's a 24/7 job we have to do around the clock from dusk until dawn, 24 hours a day, seven days a week, that you don't get a break from because you're born with it. And it's just something you have to learn to deal with and incorporate in your life.

But I'm very happy, as Dr. Law has said and Mr. Sherman, about the new treatments that we have. So, I think that is very-- how can I say it?-- It helps a lot in the mental aspect now to know, like we can look forward to something as sickle cell patients like, "Oh, there's something out there that can potentially take away this such painful illness." So, those are the things I would say that helps.

Host: Very well said indeed. And you led me beautifully, Dazja, into my next question for Dr. Law. Any new sickle cell therapies that show the most promise right now?

Jennie Law, MD: Yeah. So for a long time, one of the mainstays of therapy has been hydroxyurea, which is an oral medication that raises fetal hemoglobin in an effort to counteract some of the damage from sickled hemoglobin on the body. Looking on the horizon, and in 2023, there was a new approval by the FDA for two gene therapies. One is called Lovo-cel, which is currently being offered at the University of Maryland. And the other is a CRISPR-based technology called exa-cel. So, I think these are very exciting therapies. It's wonderful to know that there is an option available for a potentially transformative therapy. They don't come without risks obviously. And as of right now, only a small number of patients have gone through this process. So, I think over the next few years, we will know more and more about potential complications, efficacy, and how really to apply these new therapies to our patients.

Host: Sounds very promising. Couple of other things before we let you all go, Sherman, what can people do to help recruit more donors, more diverse donors, and how can our audience get involved?

Sherman Roy: Great question. So, I think the first and the most easiest answer to that is what can people do? Donate blood. If you are able and eligible to donate blood, that is the most impactful thing that you can do. You've heard several times throughout this call that blood transfusions are currently the most widely available treatment. And we, myself, Dr. Law and the providers out there cannot complete those transfusions without receiving the blood donations from donors like yourself.

I would say the second is if you're connected to any community organizations, educational facilities, host your own blood drive. If you're able to host a blood drive, please do so. That is the way that we receive our blood transfusions. The Red Cross relies on what we call mobile blood drives, and the way that we host those mobile blood drives is relying on community organizations and partners to help us do that.

And final, and most important is assisting us in creating awareness. Take some of the information that you've heard here on this podcast and share it within your community. Create awareness within the community on the importance of donating blood to help treat sickle cell patients. And I do encourage everyone I have to say this, if you're able to donate blood, please visit redcrossblood.org. You can locate your local blood donation area to make an appointment. You can also find information there on how to host your own blood drive if interested.

Host: All right. Thank you for that information as well. How about for each of you, if you could give us one quick thought on busting one myth, one misconception about sickle cell disease or blood donation, what would it be? How about you, Dazja?

Dazja Marshall: Number one, I would say believe us whenever we are telling anyone, whether it's a provider, a nurse, a therapist, a family member, friend, coworker. If we are telling someone that we're in pain and we need help, we're not just saying that for a medicine.

Host: How about you, Dr. Law?

Jennie Law, MD: I think the one myth that I would want addressed is that there's really no confirmation-- just to piggyback off what Ms. Marshall said-- no confirmation about whether or not a patient is truly in pain. So, it's very important to have individualized care plans. So, the patient comes to the hospital, and we know right from the get go what's going to be implemented to help with access to care, and expeditious access to care.

And on top of that, being connected with the comprehensive Sickle Cell Center, I think all patients should be seen by someone who has some expertise in sickle cell to help connect them with available treatments, provide adequate support so that their hospital admissions can be as smooth as possible, and then let them know about emerging therapies that they may be candidates for.

Host: And Sherman, what's one fallacy about blood donation that you want people to understand isn't true?

Sherman Roy: Blood cannot be manufactured. The only way that we can again get and receive the blood is by the help of you all going out, rolling up your sleeve and donating blood.

Host: If someone could invent blood to be manufactured, it would solve a lot of problems, wouldn't it? In summary here, Dazja, let's hear from you one last time. What would you say to someone either newly diagnosed with sickle cell disease or to someone considering becoming a blood donor? What's your advice?

Dazja Marshall: My advice. Great question. Oh, that's a big one because I feel like I have so many things. I guess, I would say try to be as supportive as possible in regards to the blood transfusion one. And then, in regards to someone that's just finding out they're diagnosed with it, pray. Just pray and make sure you find the right providers and keep your family and loved ones close to you. Unity is very important when you're going through illnesses, whether it's sickle cell, lupus, cancer. Number one thing we all need in these trying moments with these illnesses is family, loved ones, providers that love us, believe in us, are going to actually want to take care of us and not just be there just as a number or a patient on a piece of paper.

So, those would be my two different things I would say for each, because they're kind of different to me. So, those would be my answers for each one individually.

Host: It all works for all of the above. Great wisdom indeed from you. We appreciate it. And a final question, going back to something we touched on briefly earlier, you mentioned bungee jumping, Dazja. Is that on the table for you?

Dazja Marshall: Yeah, let's not tell Dr. Law, guys.

Host: Shhh!

Dazja Marshall: Yes, bungee jumping, a little rock climbing, maybe some hiking, all those super adventurous things that my provider would tell me, "Uh, we don't know about that."

Jennie Law, MD: We want you to, you know, live your best life.

Host: Well, let's talk to the provider one last time, that being you, Dr. Law. Your whole face is lit up hearing that. How rewarding is it for you just to know that she feels well enough to want to do that?

Jennie Law, MD: Yeah, I think that's fantastic. And we wish that for all of our patients, that they're connected to care, that they feel supported in whatever their life decisions are, and that we are able to get them on some disease-modifying therapy that will allow them to live their life to the fullest.

Host: That's terrific. Well, folks, we trust you are now more familiar with sickle cell disease and blood donation. Dr. Law, Dazja, Sherman, thanks so much again for all sharing your thoughts.

Sherman Roy: Thank you.

Jennie Law, MD: Thank you.

Dazja Marshall: Thank you.

Host: And you can find more shows just like this one at umms.org/podcast, as well as on YouTube and on your favorite podcast platform as well. If you found this podcast helpful, please do share it on your social media. I'm Joey Wahler. And thanks so much again for being part of Live Greater, a Health and Wellness podcast from the University of Maryland Medical System.