People Living with Sickle Cell Disease Share Their Experiences

This article is part of “Innovations In: Sickle Cell Disease,” an editorially independent special report that was produced with financial support from Vertex Pharmaceuticals.

Twenty-five years ago Yvonne Carroll spent much of her time delivering difficult medical news. It fell to her to call parents and tell them their newborns had sickle cell disease. If the infants weren’t treated swiftly with prophylactic antibiotics to prevent sepsis, they could die from blood poisoning.

“People were barely taking penicillin at that point. We didn’t have newborn screening in all 50 states, so you didn’t even know who had sickle cell disease,” says Carroll, who is now programs director in the department of hematology at St. Jude Children’s Research Hospital in Memphis, Tenn. Life expectancy for an infant diagnosed with sickle cell in the U.S. was 20 years.

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In the decades since, screening has become standard practice in the U.S., and it is on the rise in deve loping nations. Life expectancy for people with sickle cell disease in the U.S. has increased to around 50 years. But these patients, most of whom are Black, still face persistent racism, stigma, and other barriers when seeking medical care. Scientific American spoke with Carroll and with people living with sickle cell about their experience. Here they speak about how the disease has affected their lives.

The interviews have been edited for length and clarity.

Juanita McClain

President, Sickle Cell Warriors of Buffalo, N.Y., and Earth Science Educator

Sometimes we know a pain crisis is coming on because of the yellowing of the eyes, or jaundice. Most of the time the pain starts in your joints because those are the places that are first affected by decreased oxygen in the blood. But you can get pain anywhere you have blood flowing through the body. And once the pain begins, there’s no telling whether it will be excruciating or something you can deal with.

The pain of a crisis is indescribable. Minor pain is a six for me because I can deal with pain up until, like, about seven. And then once you see me crying or bent over, I’m at an eight or nine or 10 on the pain scale. But if I’m still moving even when I’m in pain, then I know I can manage on my own.

When a crisis starts, if I take medicine and the pain doesn’t subside but instead continues to get worse—when I feel like I can’t walk, I can’t move, all I want to do is lie there and cry—that’s it. I know it’s time to go to the hospital.

When I arrive, it’s sometimes hard for them to believe what type of pain I’m in, even if I’m crying, even if they see the tears coming down. I bring a health-care plan with me. Medical staff know they’re supposed to give me two shots of morphine for the first round to see if that will help my pain, and if it doesn’t, they should give me another one a couple of hours later. But I’ve had experiences where they’ve given me one or two doses and then they say, “Oh, your labs look fine. We’re going to get your discharge papers ready.” So then I’m fighting this battle, trying to get the pain medication that I need because they feel like I have been treated enough.

I have been diagnosed with depression and anxiety caused by my sickle cell disease, and it’s because I find myself ready to give up when I’m in crisis. I feel like, if I have to live in this kind of pain and I can’t get anyone to understand it, and if they can’t help me, then what is the point of living? I don’t want to go through another pain crisis. I don’t want to be turned away from doctors anymore. So I’d rather just not be here. I’ve had suicidal thoughts quite often after times like that.

Jason Robert Moore

Vice President, Sickle Cell Warriors of Buffalo, N.Y.

If I went to the hospital every time I was in pain, I would be there all the time. I’m at five out of 10 on the pain scale every single day. I only go to the hospital when my pain reaches an eight and lasts longer than about three hours. I can mostly fight that until morning—I try to only go to the hospital between 7:30 and 8:00 A.M., right after shift change. I’m pretty much one of the first people seen in the morning, so I can get in and get out.

Right now I’m on ketamine, which is a high tier of pain medication. But the last time I went to the hospital I noticed that my body’s getting used to even that now. Going to the hospital four to five times a month is going to kill the efficacy of that, so I try to only go about once a month. I don’t want to push past what I’m already at because I think the only thing left for me to take would be fentanyl.

A portrait of a young man sitting on a front porch looking at the camera.

My last job, I worked at a marijuana dispensary. I didn’t use marijuana myself, because it didn’t help my pain. But I knew what it was like to be down bad. I knew what it was like to go through pain and chronic issues and being in and out of the hospital. I could always relate to the patients. The job was a match made in heaven because I could really relate to these people and have heartfelt conversations. They felt seen and heard.

That job wasn’t strenuous on my body: all I was doing was consulting with patients and cashing them out. Yet sickle cell played its part. I kept having to go back and forth to the hospital, and my body just ultimately broke down. I told my doctor, “I thought I had it this time, Doc.” And she was like, “I thought you did, too. But look, you’re still here.” And she said, “I think it’s time for you to hang it up. We’ve tried everything, and you still ended up here all the time.” So she wrote me a note recommending full disability. That was it. That was my last job. December 2019. My last day was my birthday.

Dominique Goodson

Senior Project Manager, Sickle Cell Consortium and Patient Advocate, Brooklyn, N.Y.

Imagine a really, really strong person, and they have this big sledgehammer, and they’re hitting you with the sledgehammer with all their might: maybe on your leg, your thigh, your arm, your chest, your back. Over and over again, every second or two. But it leaves no marks, and you can’t physically see it. That’s what a pain crisis feels like.

One time I had a doctor tell me it was impossible for me to be having a pain crisis because I had sickle cell beta thalassemia. And I looked at him like, “What do you mean?” He’s like, “You shouldn’t be in physical pain. Sickle cell patients with beta thalassemia don’t have crises.” He didn’t touch me, didn’t put an order in my chart or anything.

So now I tell patients, learn to speak up for yourself. And if you’re not able to, take someone to the emergency room with you. Take an advocate, a family member or a friend. Take someone with you because doctors and nurses will treat you better when they see someone else is there with you. The times I’ve taken my mentor or my best friend with me to the hospital, I was treated differently than when it was just me by myself. Even if it’s just somebody on the telephone with you. Some sickle cell patients may be scared to speak up for themselves. And I tell them, “Your first advocate should be you. If you feel that someone is doing something wrong, you speak up on it and report it. There is nothing wrong with reporting doctors and nurses.”

As far as gene therapy, I like it better than with the bone marrow transplant—because it’s my own genes, it’s less likely to be rejected. I would do gene therapy but only after having kids because the chemotherapy can cause infertility.

Yvonne Carroll

Programs Director, Hematology Department, St. Jude Children’s Research Hospital, Memphis, Tenn.

When people with sickle cell disease go to the hospital, they have difficulty getting medication precisely because they know what drugs they need and how much of them. So when they go in and say, “I need X, Y and Z,” and it’s a really high dose, people are going to look at them like, “What’s going on?”

Systemic racism exists in the health-care system. And it exists especially for a disease that affects mostly people of color in this country. When people with sickle cell disease go to the emergency room, they experience systemic racism combined with the opioid epidemic that is pushing health-care providers to not provide opioids anymore. These people have been taking opioids their entire life, so their tolerance level is high, but a lot of providers are very uncomfortable giving that level of opioids. So people wait to go to the emergency room. They don’t want to go. And when they do, the opioids relieve the pain, but they also make them feel bad. They don’t want to go, so they wait until the last minute when their pain is so high it takes multiple doses to bring them back to their normal level of chronic pain.

A portrait of a woman sitting in a chair looking at the camera.

Part of my job includes developing educational material for patients with sickle cell disease and their families in collaboration with patients and providers. Once I asked a child, “Draw what your pain feels like,” and they drew a shark biting them. I asked someone who was older, and they said, “Imagine you get your finger slammed in a car door, and then multiply that by 10.” I told him, “I can’t get past the car door thing, so I can’t even go into the factor of 10.” But what really struck me was when I talked to a mother with sickle cell trait [a condition, typically mild, that occurs when someone has one rather than two inherited sickle cell mutations]. She donated bone marrow for her child’s transplant, and after they went through the process, she had a pain crisis—something that happens to people with sickle cell trait in rare circumstances. She said she was in so much pain that she had to apologize to her 10-year-old daughter because she had never imagined her daughter’s pain was so severe.

Another part of my job has been working on a gene-therapy decision aid in conjunction with a team of patients, ethics experts, sickle cell advocates, hematologists and a genetic counselor to help patients and their families understand gene therapy and the other treatment options available.

There are a lot of logistics that go along with gene therapy: there’s isolation, there’s chemotherapy that suppresses your immune system. There’s the fact that you have to be close to the center where you’re getting the gene therapy. You won’t be able to work, so you need someone to take care of you. But beyond that, the group that needs it most is the group that doesn’t trust the medical system or clinical trials. We all know Tuskegee.

I tell people to hope and take the best care of themselves right now because 10 years from now the landscape will be different. When I started, children had pain crises all the time. Now it’s rare. As a matter of fact, when they have one, they’re like, “What the heck is going on?” It was commonplace to see sepsis, splenic sequestration and strokes in three-year-olds. It was devastating. That doesn’t happen now. Sometimes it’s still bleak, but things look much better than they did 25 years ago.

Judith McClellan

Social Worker, Salisbury, N.C., and mother of a teenager with sickle cell

If this were oncology, the carpet would be rolled out for us. But sickle cell is the forgotten, frowned-upon, unimportant disease. And because we’re Black people advocating for ourselves, people say we’re being difficult.

I’ve worked in social work since 1997. My daughter, Kyarra, was born in 2006. When she was born, I was working in a doctor’s office, and they weren’t understanding. I’ve lost jobs because I was taking care of her. We’ve been faced with homelessness on several occasions because of job losses.

As far as treatments, I left all decisions up to Kyarra. Kyarra didn’t want a bone marrow transplant. With the bone marrow transplant she said no, no, hard no. But with gene therapy she’s 1,000 percent onboard. A hospital reached out to us. It’s a clinical trial for kids aged 12 to 17. We’re at the very beginning. She just did a bone marrow biopsy. Next is her cell collection. If they can get enough cells, we’ll move forward. We don’t know how soon, but we’ll move forward. If they don’t collect enough cells, we’ll have to go back so they can collect some more. She’s 17 years old, and the only thing she’s worried about is if she’s going to lose her hair. I’m worried about the chemo. And then there’s the possibility that it just won’t take.

If it works, she would be in the hospital less because she won’t have as many pain crises. We are prayerful that she will have zero. I’m hopeful that we can have a normal life. At times, Kyarra misses 30 to 40 days of school. She just missed sickle cell camp. She wants to have a normal life as a teenager and stop missing out on school and social functions because she’s in the hospital. I could maintain a job. So that would end our risk of homelessness, that would end struggle, that would end a whole lot of things. We’re excited about it and hopeful it will work.