Courtney shares what it’s like living with sickle cell disease

Ruma: Hi, my name is Ruma Kumar. I write stories about patients and families at St. Jude Children’s Research Hospital. I also get to talk to doctors and researchers who work at St. Jude.

I’ve learned and written about blood disorders. One of the patients I’ve met along the way is Courtney. She has sickle cell disease. A person with sickle cell disease faces a lifetime of potential health complications like heart disease, kidney disease, stroke, and frequent bouts of intense pain.

Courtney is 24 now, but she spent her whole childhood coming to St. Jude. Recently, she visited the Memphis campus. And I got the chance to talk with her, and I wanted to share that conversation with you. Before we sat down together, she spent some time with younger sickle cell patients.

Courtney: Today was the first day that I got to do artwork with kids 10 years younger than me, with (sickle cell) disease. And we talked about how we managed it, what made us feel strong, what made us feel happy. And a kid drew a picture of a Transformer, and we were saying that it symbolized something to us, and he said, "Because I'm strong." He is strong. He is so strong. And we, everyone in that room, we’re so strong. And I had never gotten to see that from my perspective, as a bit older. And so it was really cool to see.

Ruma: I have to tell you, the joy on Courtney’s face when she told me about this opportunity to connect, to find community, it gave me chills. And that’s part of why I wanted to share this behind-the-scenes conversation – because she’s SO driven to talk about what she – and others like her – have learned, the difficulties they’ve faced and overcome, and what they continue to endure.

She’s also optimistic about the work scientists and doctors at St. Jude are leading.

OK, let’s listen.

Courtney: My health is wonderful. I'm doing very well. I am proud that I graduated on par with my peers. So that was always something that was a big conversation that my mom was really worried about. I couldn't read too well when I was about to kind of hit those benchmarks that kids expect. And it was a really big concern for her. The fact that I was in and out of the hospital, I missed a lot of school. When you're really focused on quality of life and making sure that you turn your next age, my reading level was something that was able to fall back on the back burner. But once St. Jude got my health back, I was very quickly able to kind of like regain all of those footsteps that I kind of had missed with my peers.

And so I'm incredibly grateful to feel well. And with that, that kind of gives me the energy, the joy to be able to do a lot of the things that I really hope to be able to do. And so I don't live with my parents. At 24, I'm able to live with my roommates, who I went to college with, like every other 24-year-old. Definitely do not live alone. And life is good.

Ruma: How do you spend your time?

Courtney: That's a great question. I spend my time working a little bit more than I would like to, but I think every 24-year-old would say that. I live in New York. And funny enough, someone reminded me today that when I was younger, people used to ask me what I wanted to be when I grew up. And I said, "A fashion designer in New York." And while I didn't hit that fashion designer piece, I do live in New York. And so I still love going out, seeing art, seeing theater, hanging out with all my friends. I'm really grateful that so many of my friends from undergrad moved to New York. And so I'm having fun.

Ruma: Sounds like a great life and a full life.

Courtney: Thank you.

Ruma: I'm sure that when you were first diagnosed, your parents probably were so worried you wouldn't be able to have this. Tell me a little bit about your experience with sickle cell when you were young.

Courtney: Of course.

Ruma: And then we can talk about how you're managing symptoms and so forth now.

Courtney: So, managing my care and my health when I was young was always exceptionally difficult because we had a benchmark. My mother, her eldest sister had a son, who would have been my eldest cousin, who died before he could turn the age of 3. And so those first couple of years really mirrored my late cousin. Thankfully, I was so young that I don't have very many memories. But I can tell, in the way that my mom recounts these stories, how truly scary it must have been. And I have an older sister. So you're managing one child who's just now like 3, 4, and another who is incredibly sick and mirroring a lot of these really scary memories you just witnessed.

But my doctor said, at the age of 3, that I would be eligible for a study. It was a drug called hydroxyurea, something that I'm still on today, 21 years later. And so I say that to say I am fully a product of all the incredible work that's happened in these walls. And just recognizing that's a hereditary disease, I have so much respect and understanding of what this disease affects, and the family that came before me, and family that might come after me who might be affected. And so it was definitely scary early on and I’m so incredibly thankful to know that that’s really not at all my outcome now.

Ruma: What were some of the symptoms that you faced early on from sickle cell disease?

Courtney: So the most front-of-mind symptom of sickle cell disease, and maybe I'll walk back for just a moment and speak about the disease as a whole. So it's a blood disorder. And recognizing it's a blood disorder, it truly affects every single organ of your body. And I took that for granted as a kid. I can remember, because it's a children's hospital, they were wonderful in every way. They explained things to me in a way that I could understand, right? So they explained that your blood usually is shaped like a full moon. It's flexible, it's malleable, it can navigate through the body, through vessels, properly and easily. But mine happened to be a crescent moon, and it wasn't as flexible. It was a bit more rigid and so it could get stuck. And when it gets stuck, you're going to feel something. And I remembered being like, "What? What am I going to feel in there?" And they were like, "It's really not going to be comfortable." And I knew, I definitively knew I had experienced it. But it was the fact that someone desired not only to speak to my mother about my care, but also to me. I was an agent in my care.

But pain crises were, and still are, such a huge aspect of my life. And it's hard because they can come out of the blue. But I think there is a degree of composure I can have because I've been told the same kind of regimen of how to take care of myself for a really long time. The fact that that was started so young, like, I know what to do. And so in times where it's scary, in times when my life is busy, it's very easy to recall something that I've heard hundreds and hundreds of times that never changed from people who I knew cared about me and taught me what my disease was. Asked me every single time that they came into my room what medications I took, not asking my mother, but ensuring that I knew that information so that if I'm in the ER a million miles from home, I have the composure to share my story because I've been enabled to since I was 3 years old.

People will say this very silly thing when I'm in the hospital, which is that you seem very well spoken and have a strong knowledge of your care. And they say that as if that's a compliment to me. And I will take any compliment someone gives me. But I'm not owed that compliment. St. Jude is. The fact that St. Jude had the care and discernment to teach me from such a young age, knowing your care and your health history well, in my opinion, is not something that you were born with, but something you were taught. And I had the best instructor. And that's something that I will always be grateful for. And so even in very scary times, I'm able to really have a lot of agency about my care and kind of have strong expectations of what kind of needs to be done in very routine health complications that I've had. And that's because I was taught a regimen that I feel a lot of confidence in from St. Jude.

Ruma: What is one piece of advice you would give to someone who's recently been diagnosed with sickle cell disease?

Courtney: That's a great question. I would say find community. And I think that community is explained as someone who has this exact same ailment. But community can also be someone who you just know really cares for you. And community can be your healthcare practitioners and really thinking of it as a partnership and a relationship, which is always something I had at St. Jude, so something that I now expect. Like, I expect my doctors to look me in my eyes. I expect my doctors to know my name without having to look at my chart. And that's because I never questioned that at St. Jude.

And so I think when it comes down to advice, I think it's having confidence in your care and your body and also having some patience for yourself.

I'd hinted at before the fact that I wasn't always aligned with my peers. And you would have never known, I was as happy as could be. And I think it's because there is a certain degree that you must have appreciation for what your body has already taken you through. And I think that that was also taught. Like, St. Jude was not a place that only provided me medication and said, "This is what is causing you to feel better." They also cared about my rest, and my diet, and larger picture things that fed in and was so much more holistic. And that was very forefront and cutting edge at the time. No one really cared to ask you what you had eaten that day. It was much more programmatic and to the point.

Ruma: So wait, did they bring up those things as a way to, "Hey, all of this matters”

Courtney: Yeah.

Ruma: “... in your health?" Okay. So, what are some tips that they gave you to say this is how you manage your symptoms?

Courtney: I definitely think tips that I would provide to someone else, because they were given to me, were that you have to get your rest. You have to wear appropriate attire in terms of cold and warmth, and just like changing of climates.

And I happened to go to a school where it was very cold, and that was something – the moment I shared that I was interested in going to that college, my mom thought, and I'll never forget this, my mom was so confident that they were going to be like, "Are you sure that's a good idea? It's so far away. It's so cold." And they weren't. They turned their computer around to me, and they were looking up the weather. They were looking up all these things, and we researched together. I'll never forget this practitioner. He was so proud of me and so excited for me. And so they just cared about the whole person.

My advice is that you have to care about your whole person. There's only so much that can be done by reactive care. And so, that proactive care was really important at St. Jude, and that's like continued to be really important to me and something that I could not recommend more.

Ruma: Can you describe a pain crisis? How does it feel?

Courtney: So it was described to my mother for the first time as someone taking a small hammer and hitting it at a joint. And the importance of this is that when you think about blood cells and them flowing through your body, very often it happens in like points of axes. And my places that are most frequent are in the joints of my arms and in my back. And it's always been that way. And so, it's this pounding and aching that's somehow also sharp, that can vary for such drastic durations.

I think that's the part that sometimes gets me. It could be an hour. It could be days. At worst, weeks. And that's just a lot of unknown. And I think that that is something that is really common to sickle cell is that the unknown is hard.

Ruma: I was just going to say you've just described how hard it is grappling with an unknown. What things helped give you some measure of control? What things did you do/say to yourself to give you some measure of control?

Courtney: I think there were constants. And that's not even something I necessarily had to say to myself because I saw them. My mom was a constant. St. Jude was a constant. Even the medications I took were a constant. And so with that, of course, there are things that were so out of my control and that I very well knew were out of my control, even to the degree of how long I'd be in a hospital.

There were points in time that we kept a bag packed because it was so frequent that, after family travel, I would be so run down, no matter what we did, like, as precautions, that I might have a pain crisis or I might need to seek immediate medical attention. And for that, we would have a bag packed by our door. There was so much unknown. But knowing where I was headed and knowing that those same caretakers had been taking care of me for quite some time was a constant. Having my mother, who also cared so much and worked so hard to ensure that she was knowledgeable, after seeing such a difficult outcome of a family member that she loved, the worst case was always present to our family. And so, there was no unknown in that degree.

But there was a lot of unknown on the upside, on the positive outcome. And that was really exciting and hopeful. And hey, I'm still surprising myself, and I hope to keep doing so. And I hope that St. Jude keeps surprising us, and I hope that so many sickle cell patients keep on surprising us because there's a lot of unknown. But the worst case is always the worst case. So the least you can do is hope and have the support. I think that's the biggest thing. St. Jude gave us the support and the hope to focus on a positive unknown.

Ruma: Do you feel like people talk about sickle cell disease enough?

Courtney: Oh, now we're getting to the nitty-gritty. I do not. And something that I will always say is that education is power and that knowledge is power. And the best way to solicit that from people is by talking about it and about sharing and talking about disease and what pain looks like.

Pain is such an uncomfortable thing to talk about, especially pediatric pain. No one is writing campaigns about it. Like, I wouldn't want to read campaigns about it, but it's incredibly real. So, no, I do not think that sickle cell is talked about enough. But I think it is so special to know that I have never felt that way, that it wasn't a priority at St. Jude.

And so I'm really, really grateful that I've been given opportunities to talk about my experiences, and I really hope to never stop. So I hope more people know about sickle cell. I hope more people know that it is not the prettiest disease, has a lot of pain, has a lot of unknown.

So many people ask me this. They're like, "Oh, so you're cured. You're so healthy. You work a full-time job. You are able to live on your own. By all definitions, you are, for lack of better words, like a functioning adult." And while that is a compliment, that is not to say that I'm cured. I have never received a cure for sickle cell disease. I live with it day in and day out. But I was equipped with tools to make it manageable.

But there is a lot of hope. Advancements are being made. And so, I hope that if we ever get to sit down again in a couple of years and you ask me that same question of "Do you think that sickle cell is well spoken to, and that there's a ton of awareness?" I hope to have a super, super, super different answer for you. Because I do have a lot of hope. But I also think that there's a lot more that we can all do. And I think that St. Jude has always made it clear that one of their true missions was to help individuals with sickle cell disease.

Ruma: Where do you go from here? What does your future look like? I mean, it's already very bright.

Courtney: You're incredibly kind.

Ruma: Just tell me what your plans are. I don't know. Where do you see yourself in three years, five years?

Courtney: I'll take three. I'll take three because that's a little bit easier. I'm 24 years old, so I'll be 27. Twenty-seven always felt like a very adult age.

I hope to love what I'm doing. I hope to know someone who's been cured of sickle cell. Know someone. Doesn't have to be me, but it has to be someone that I know. And I hope that they are flourishing. I hope that they feel so good. Because something that people always talk about is that sickle cell is a very draining disease. You are tired. And as someone who I'd say has pretty good energy, I would love to get to see that. I'd love to get to see someone I've known get a burst of energy from being cured from a really devastating and pervasive disease.

Where else do I go from here?

Ruma: Are there places you want to – you have a bucket list of travels?

Courtney: I do. I have a lot of travels, so maybe I'll go back to Europe. I'll go back to Spain and then some new places. I've never been to Greece. I've never been to Cape Town. I've never been to anywhere on the African continent. I've never been to Thailand or Southeast Asia. So that's definitely something on my bucket list.

And maybe even later down the line, I really want to have a family. I'm old enough now that I can say that and not sound crazy. One day, I want to have a family. And I want to talk to them about sickle cell. Like, I really, really want to talk to them about sickle cell. I'd talk to them about the fact that it's hereditary. I want to talk to them because they'll have trait, because that's the only gene I can give. So it will be a conversation. But I kind of want it to have a different ending. And I want it to still have so much hope, as I do right now. Hopefully, by then, when I have a family, so, like, years down the line, I hope to know more than just one person who's been cured. I hope to know a couple, maybe even a lot. And I definitely want them to know about St. Jude.

Ruma: Well, I hope that for you, too. Children who know and understand what you've endured with sickle cell disease, who have agency over their own health, and know more about it thanks to what you've shared. So it's wonderful. Thank you so much, Courtney.

Courtney: Of course.

Ruma: Hi, it’s Ruma again. I wanted to give you a little more context about the future of treatment for sickle cell disease.

St. Jude is one of the largest sickle cell disease programs in the country. In 1983, a St. Jude patient was the first person to be cured of sickle cell disease through a bone marrow transplant. Today, St. Jude is participating and leading several clinical trials studying the use of hydroxyurea in children and studying gene therapy as a potential cure as well as techniques to make stem cell transplantation less risky and more broadly accessible.

That hope Courtney talked about, wanting to know a lot of people who have been cured, that’s exactly what these advances are aiming for.

There’s more information about sickle cell research and the patients and families who find care at St. Jude in the show notes.

My conversation with Courtney was recorded by Dan Yohey and John Phelps. It was edited by Geoffrey Redick and Micah McGee.

Thank you for listening.

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