Sickle Cell Disease and COVID-19

[MUSIC PLAYING] Welcome, everyone. I'm Dr. John Whyte, the Chief Medical Officer at WebMD, and you're watching Coronavirus in Context. We know when we talk about COVID there are certain groups of people that are at greater risk based on their underlying health conditions. Sickle cell disease is one of those health conditions. September is Sickle Cell Disease Awareness Month, so it provides a perfect opportunity to talk about sickle cell disease, why it's so important for people who have sickle cell disease to get vaccinated, maybe even the boosters, and how we reach populations that are disproportionately impacted by COVID.

So to help unpack it, joining me are two experts. The first is Dr. Wanda Shurney, she's the CEO and Medical Director of the Michigan Chapter of the Sickle Cell Disease Association of America, and Mr. Clifton R. Kirkman II. He is a sickle cell disease warrior, a person who has sickle cell disease. He's chosen to receive the vaccine, and he's going to share his story. Thank you both for joining me today.

Thanks for having me.

It's great to be here.

Dr. Shurney, I want to start with you. You're a pediatrician. You've been treating people with sickle cell disease for over 30 years. Can you remind our audience what we're talking about when we mention sickle cell disease? Sometimes people get confused. They have heard sickle cell trait. Remind us what sickle cell disease is.

OK. So sickle cell disease is a blood disease. I always say it's a disease of the red blood cells, but it actually affects a lot more than the red blood cells. But basically, the red blood cells take on an unusual shape. They're shape like a farmer's sickle.

And that carries with it two problems. Number one, the red blood cells are sticky. And number two, the red blood cells are fragile. So when they stick to each other, they block blood vessels. Now, if you block a blood vessel, you know red blood cells carry oxygen to all the different parts of the body, so if you block the flow of oxygen, then that causes pain. So the hallmark of sickle cell disease is pain.

But what is even a bigger problem is that when you block the flow of oxygen, you have damage to your organs. So organ damage is a big issue. Now, when the red blood cells break down rapidly, then that's what causes the anemia, or the low blood. And that's what causes people to be fatigued, or to tire easily.

So you get sickle cell disease from your parents. So if you get one sickle gene, then you have sickle cell trait. And that's a carrier state. And usually people with sickle cell trait don't have any problems. But if you get a sickle gene from both parents, then you have sickle cell anemia. And that's when the problems happen.

Sickle cell disease disproportionately impacts people of color, is that right?

That's exactly right. And the reason is because having sickle cell trait, getting that one sickle gene, protects you from malaria. So you really see sickle cell disease wherever you see malaria. And then you might say, well, we don't have malaria in the United States, so why do we have sickle cell disease?

And whenever you see sickle cell disease where you don't see malaria, it's because of migration. And so in the case of African Americans, that migration is the transatlantic slave trade. So we brought the sickle gene with us when we were shipped here to help build this country.

And what's the relationship between sickle cell disease and COVID? I mentioned at the beginning people with underlying health conditions, such as sickle cell disease, are at greater risk. Help explain the relationship for us.

Well, one of the problems with sickle cell disease is that your immune system doesn't work as well, and particularly a part of the immune system called the spleen where you make antibodies. And actually, if you do a spleen scan on a patient with sickle cell disease by the time they're six months old, you can't see that spleen anymore. So we know that's not working well. So we say that individuals living with sickle cell disease are immunocompromised. And so that's the reason why they're at bigger risk for getting COVID, and, if they get it, maybe having a more severe course.

Tell us Mr. Kirkman's background.

WANDA WHITTEN-SHURNEY: In Michigan, we coordinate the newborn hemoglobinopathy screening program. So all the blood diseases that are diagnosed at birth are sent to the Sickle Cell Disease Association of America Michigan Chapter. And so I was the one that called Clifton's mother to tell her that Clifton had sickle cell disease.

And then he started getting his care at the Comprehensive Sickle Cell Clinic at Children's Hospital, so I basically was a pediatrician there so I literally watched him grow up. And all of my patients are special, but some are particularly special. And Clifton, his whole family, I got to know his sister, his mom, his dad, and when you have a family that wants to learn, that gets all the information and then acts on the information-- like we have to give penicillin twice a day until you turn five years old, and knowing that she knew the importance of that and did that and did everything that she needed to do to make sure that Clifton would have the highest quality of life that he could have.

And I think she's done a remarkable job. His dad passed away, but he was very active too, so I got to know him well. So that's Clifton. I feel like he's like a son to me.

Wow. What an intro. Clifton.

Thanks so much. Yes, this is such an amazing opportunity. Dr. Shurney and I experience have been over 34 years. And so who I am, as of now, is attributed to her. It's something that she saw me as a child who was very adamant about speaking out and living with sickle cell and making the decision that my voice could be used to share my story with others. And so thanks so much for having me today. It's an honor.

And sickle cell disease is considered a chronic disease. I want to ask you, how has the COVID pandemic affected how you manage sickle cell disease?

CLIFTON R. KIRKMAN II: So the pandemic for me personally was and is still very scary. Having a compromised immune system is something that we all face. And it's something that we have to be very careful about. And so when COVID came along, it was like here's this unknown illness, this unknown virus, and what do I need to do in order to be safe?

So many of us as sickle cell warriors, we face the likelihood of death. It's just part of having sickle cell. And so here's a disease or here is a virus that's come into play, and now to have to think of the possibility of passing away from COVID, it's very scary.

It is absolutely scary. And when this first came out, I didn't know what to do. I honestly didn't have any clue, didn't know if this would be a death sentence or whatnot. But I made sure I contacted my doctors to get insight on the steps that I should take.

Dr. Shurney, what have you found are the barriers in getting patients with sickle cell disease good care during the pandemic? We've been telling people for a long time not to come in. People are still hesitant to come in. You mentioned issues of pain control, infection control. What have you seen over the past 18 months?

Well, I think their biggest barrier is fear. And one of the issues is that individuals living with sickle cell disease already have so many barriers to care. And then adding the pandemic was just another layer. One of the problems is that there are not enough physicians who are, number one, capable of, and number two, willing, to care for warriors.

Part of it is because of how complicated the disease is, both medically and psychosocially. So when you turn 18 or 21 or wherever the transition point is in your particular clinic with sickle cell disease, there is basically nowhere for you to go. And we actually see a peak in the mortality in sickle cell disease at the point of transition to adult care.

And so what happens is a lot of patients are receiving their care in the emergency room, and that's not the best place. Of course, it's the most expensive, and you don't get continuity of care. And the other problem is that frequently, even though the hallmark of sickle cell disease is excruciating, unpredictable pain, our warriors get treated as drug-seeking or drug addicts. And so there are a lot of myths and there are a lot of issues with that.

So patients basically don't want to go to the emergency room. So they're going to try everything they can try to stay home. So they're going to maximize their pain medication. They're going to do relaxation techniques. They're going to use heat and meditate or whatever, because they don't want to go to the emergency room because they're afraid that they're not going to be treated well.

Dr. Lakiea Bailey from the Sickle Cell Community Consortium talks about going to the emergency room is making a choice between dignity and death. And if you think you're going to die, then you have to swallow your pride so that you can go to the emergency room and get this treatment that's not compassionate. So then we add COVID on top of that, so it's more difficult to go because you're afraid when you go you're going to get exposed to COVID.

A fever is an emergency in a person with sickle cell disease because we're worried because they're immunocompromised, and we have to make sure that they don't have a bacteria in their bloodstream. So they get this fever, and it might be COVID. But no, I don't want to go to the emergency room. And it might be sepsis. So there are just so many issues that kind of get messed up in terms of getting care for patients.

Clifton, I mentioned early on you chose to get vaccinated. Help walk us through what that decision was for you. Were you hesitant at all? Did someone have to convince you? Or did you say, sign me up from the beginning, I'm ready for the shot?

Well, it was absolutely concerning for me when it comes to taking something new. I mean, it was absolutely something that I didn't feel had been around or had enough research as it relates to sickle cell and how that would affect me down the road. Was I skeptical? Absolutely. Was I on the bandwagon at first? No.

But I ended up having surgery this past January. And from that surgery, it was one of the doctors in the ER who actually told me, like, hey, you've got sickle cell. You're already in here with acute chest. You need to consider getting the COVID shot. And it was upon that conversation that I had, and an in-depth conversation, that I make the decision once I got home from the hospital that I would actually go and get the vaccination.

But I think that's the biggest thing. The biggest thing is that even with us as sickle cell patients have these concerns. The only thing that you can really do is take it upon yourself to reach out to your health care to get those questions answered and really dig deep and don't hold anything back as it relates to your concerns and your fears. And make an informed decision. And so because of that, I was able to talk it out with the doctor then, and that led me to going ahead and getting the COVID vaccine.

So you had hesitancy even though you knew, in many ways, you're at greater risk?

So, yes. I mean, I absolutely had hesitancies. As a sickle cell patient and a sickle cell warrior, it's always contemplating in the back of your head, like, hey, this illness could potentially end my life in some form or fashion. I could catch the flu. It can create an issue. I could catch pneumonia. It can create an issue.

I can be on transfusion programs for a number of years and that creates an issue. So with this new vaccine that was out, it was just frightening not knowing the research behind it. And of course, as consumers, we don't know all of the intricate details as a physician does. And so I had to lean on the fact that I trust my doctors and that they would not lead me to something that would be detrimental for me.

Dr. Shurney, has that been your experience in talking to people with sickle cell disease still a lot of hesitancy even though they're a population at greater risk? If they get COVID, they may have a worse outcome.

WANDA WHITTEN-SHURNEY: Absolutely. I think that I knew that it was going to be a huge challenge to get individuals living with sickle cell disease to take the vaccine, because in clinic I had trouble getting people to take the flu vaccine. So this is something new. This is something that was fast-tracked. And then they started talking about messenger RNA.

And people, if they remember from biology that helps you synthesize proteins, and you're going to float this around in my body? And yeah, I knew that it was going to be a huge challenge. And I think that people feel like, well, I'm just going to keep myself safe from COVID, so I'm not going to leave the house or I'm going to have on a mask, I'm going to wash my hands, I'm going to do everything, and I'm going to protect myself from COVID.

How'd you change their mind?

Well, in a lot of cases, I didn't. And that's what's so concerning to me. And that's why this kind of interview is so important. I can't tell you the number of calls that I got from patients asking me, should I get the vaccine? And it's like absolutely, yes, you should get it. Which one should I get? The first one you can get your hands on.

And so then they would tell me about their fears. And a lot of times they would tell me, even after they talked to me, that they still didn't want to get the vaccine. One of the things that I would tell them is that I'm more afraid of COVID than I am of the vaccine. But that didn't really-- because of all the myths that are out there and they're injecting things into your body and people having all these weird things.

And one of the things that I would talk to them about is true, true, and unrelated. So maybe you got the vaccine and then you had this problem, but it wasn't necessarily related to the vaccine because different problems come about all the time. And then talking about the fact that most people just have mild side effects. I talked about the fact that my 92-year-old mother got the vaccine and did not have a single side effect, because sometimes real-life stories help people to understand. But the other thing is pointing to other warriors who have gotten the vaccine, because they trust people who are living the same life experience.

Clifton, I want to ask you, what is your message for people with sickle cell disease living in the setting of COVID? Let's acknowledge COVID may be around for a while. What's your advice?

Dr. Whyte, my advice simply is this. Every patient has to do what's best for them, one. Take the time, do the research for yourself. Take the time to talk to your health care provider and ask questions. Ask those hard questions that will help to get you to the place that will ease those fears and those concerns.

We know COVID is probably here to stay for a while. And so as sickle cell warriors and sickle cell patients, we've got to take our time and we've got to take what we know how to do and that's to survive. And surviving, that's in all capacities. We've got to make sure that our health is at the peak in which it can be.

So whether that's taking your vitamins and taking your medicine and making sure you're making to your appointments, as sickle cell patients, we have to do what's best for us. Now, I can't tell someone which decision is best for them. But I can tell you all to make the best decision for you. Take the time to do the research. Take the time to talk to a doctor. And make that decision that best suits you and your family.

Dr. Shurney, you've been involved with sickle cell disease awareness and outreach for more than 30 years. Your father, the late Dr. Charles Whitten, was also involved in sickle cell disease. He was one of the co-founders of the National Sickle Cell Disease Association of America. What's the future of sickle cell disease? What are we going to be talking about five years from now?

WANDA WHITTEN-SHURNEY: So the future of sickle cell disease is very bright. I think that things are getting better and better. The primary mission of our organization is to maximize the quality of life of individuals living with sickle cell disease. So we don't just want patients to live longer and longer. We want patients to live better and better.

So the NIH has the Cure Sickle Cell disease-- period-- Initiative. And they're looking at a genetic cure for sickle cell disease, which would be bone marrow transplant. So we have bone marrow transplant using donor marrow from a different person. But now the genetic cure has to do with a bone marrow transplant using the patient's own bone marrow that has been genetically modified. And so you actually cure the bone marrow, and then give it back to the patient.

Now, I was at Howard University College of Medicine, and we were talking about how sickle cell disease is just this tiny point mutation, just a single amino acid difference between sickle hemoglobin and normal hemoglobin. And they say, we're going to have a genetic cure in five years. Well, I graduated from medical school in 1983 and it's 2021, and we still don't have that genetic cure. But I think that is coming.

But in the meantime, what for me makes the future seem bright is the number of new medications. For 20 years, we only had one medication. That was hydroxyurea. And people were afraid to take it, because it's a repurpose cancer drug.

But now we have three other medications that were developed specifically for sickle cell disease and lots of clinical trials, so lots of medications in the pipeline. So what we have to do is we have to get doctors interested in taking care of our patients, because if they don't have a doctor outside of the emergency room, how are they going to take advantage of all these advances? So I'm cautiously hopeful about the future in sickle cell disease.

I want to thank you both for joining me today and helping raise awareness of sickle cell disease for Sickle Cell Disease Awareness Month here in September, and also for the relationship between sickle cell disease and COVID and the need to get vaccinated.

It's been our pleasure.

Thanks for having me.

[MUSIC PLAYING]