In This Episode
Sickle Cell Disease is a debilitating genetic disease that almost exclusively affects Black folks. While genetic breakthroughs have made a cure possible, the high price tag may keep them away from people who need them. Abdul reflects on the way that society shapes the scientific questions we ask — and whose diseases we take seriously. Then he interviews Dr. Titilope Fasipe, a pediatric hematologist who treats sickle cell disease — and has lived with the disease her whole life.
[AD BREAK] [music break]
Dr. Abdul El-Sayed, narrating: Senators grilled the CEOs of major social media companies over the health risk their products pose to children. Europe is in the midst of a massive measles outbreak, and the U.K. intends to ban disposable vapes to combat rising teen vaping rates. This is America Dissected. I’m your host, Doctor Abdul El-Sayed. [music break] There are lots of podcasts about science. There are lots of podcasts about society. This podcast is about neither of those alone, but about both of them, about the way they interact with each other, shaping each other in critical ways centered around our health. Now, let me call out a discrepancy that many of you are probably thinking right now. Abdul, I get how science shapes society. That’s clear enough. We’re watching it every single day. How Covid fundamentally rocked us, or how AI is changing the structure of the economy. But how does society shape science? After all science is a rigorous process by which we try to answer questions. But science is nothing if not a human process. And what is society but the emergent outcome of humans coming together? And society has its fingerprints all over the scientific process. It’s most obvious in two ways. First, in the questions we ask in the first place, the diseases we count as worthy of our scientific funding. The questions that capture our collective attention as, quote, “important,” but it’s also in the ways that we interpret the findings of those studies, the ways we build policy or design interventions based on what we learn. Today’s conversation is about sickle cell disease, one of those diseases that demonstrates exactly the way that society’s interests and foci can shape the questions we choose to take seriously, and the ones we don’t. Sickle cell disease is a deeply debilitating genetic disease that causes red blood cells to be deformed, rather than being shaped like inner tubes measly making their way through our bloodstream, they’re shaped like sickles clumping to each other and to vessel walls, causing severe organ damage, high rates of infections and debilitating pain crises. People with sickle cell disease die an average of 20 years younger than their counterparts. The science of sickle cell is clear. That life expectancy difference isn’t just about science. It’s also about a society that’s been laggard in asking questions about how to protect people with sickle cell in the first place. Sickle cell disease is uniquely a disease of Black folks. We’ve discussed the deep structural racism that robs Black folks of their lives and livelihoods in our society. The way that it creates inequities in health outcomes across the board, from infant and maternal mortality to hypertension to diabetes, to stroke, to cardiovascular disease, and ultimately to death rates. Each of these inequities arises from the ways that Black folks are treated in our society. Whether overt racism and the anxiety and stress it creates, or more deeply, the ways that racism shapes access to good schools, healthy communities, good food, clean air and water, or quality health care. But one of the ways that racism shapes health outcomes goes deeper than that. It’s in the scientific questions we choose to take up in the first place. For decades, our progress on sickle cell was held up by a failure to fully invest in the research we’ve needed to address it. I say this at a time when we’ve finally seen important breakthroughs in the treatment of sickle cell disease. As we discussed a few weeks back. Two new gene therapies have the potential to fully cure sickle cell disease. Of course, at millions of dollars a pop. But how much faster should they have come? How much cheaper would they have been had we taken this disease seriously? If the folks who suffered it weren’t Black. My guest today, she’s dedicated her life to answering these questions as an advocate and a clinician. Dr. Titilope Fasipe is a pediatric hematologist. She’s also been living with sickle cell disease her whole life. She joined me to describe her experiences with sickle cell as a patient, a friend, a family member, and a physician. And to reflect on what sickle cell disease can teach us about racial health inequities more broadly. Here’s my conversation with Dr. Fasipe.
Dr. Abdul El-Sayed: Can you introduce yourself for the tape?
Dr. Titilope Fasipe My name is Dr. Titilope Fasipe.
Dr. Abdul El-Sayed: Dr. Fasipe, you’re an expert in sickle cell disease. Can you tell us a little bit about what the disease is?
Dr. Titilope Fasipe Yeah. So sickle cell disease is a red blood cell disorder. Um. But at heart, it’s a hemoglobin disorder. And hemoglobin is the molecule inside red blood cells that carry oxygen. And so if you have a genetic mutation, you can get all sorts of red blood cell disorders. Sickle cell is when the hemoglobin um has an abnormal attraction to itself. So it’s almost like connect sticks and it forms these long rigid chains. And it causes the red blood cell to not have that nice round shape, but instead turn into a C shape. Um. It got its name, actually, because sickle was in a farming instrument that has that C shape. And so that’s where sickle cell disease um got its name. But yes, it’s a red blood cell disorder. So blood flows throughout the whole body. So you can see complications of sickle cell disease throughout the entire body.
Dr. Abdul El-Sayed: You know, one of the ways I’ve heard, um red blood cells that are not affected by this disease described is as Bialy bagels. And it might be because I went to med school in New York. Um. [laughter] For folks who don’t know what a Bialy bagel is, imagine a Boston cream donut that’s a little bit squished out. Right? And so you’ve got this, like, round pillow that should have a hole in the middle, but it doesn’t. Right and it’s just um, it a, it’s a little bit concave. And, you know, these red blood cells, um then flow through your uh, your arteries and veins and venules and arterials and all that good stuff um with no problem. But you can imagine what happens when they start being shaped like a, a C and um, and and the consequences that that can have. And then the thing we sometimes take for granted, you know, unless you cut yourself, is that your whole entire body is full of blood. And um, and so when you think about the impact of a blood disorder, it literally can be anywhere. And um that I think characteristic when you think about, you know, zooming out from the uh the pathophysiology of the disease, the lived experience is that this, this single disease can cause you pain and discomfort and dysfunction anywhere in your body. Um. And you never really know when it’s going to strike. And I can imagine that that causes just immense pain and fear and anxiety for people who are living with the disease.
Dr. Titilope Fasipe Exactly. So let’s go back to that C shape. Right. It has two big problems with it. One, it makes the red blood cell fragile so it doesn’t live as long. A typical red blood cell will last about three months, well this one will only last a couple of days or few weeks at best. And so that fragility leads to um most individuals with the disease having anemia. The other part of that fragility is that and that uh shape is that it gets stuck in places where we don’t want it, as you said. And that can lead to several um organ uh damage and dysfunction. The most famous complication, the one that sickle cell is known for the most is pain. And that’s because when you block blood flow to a certain area, it’ll trigger pain, uh nerves. Um. And that will send the pain signal. However, pain is not just the only complication. Um. It can cause us spleen damage, liver, brain, unfortunately. Eye, kidney, again, you name it, and sickle cell can impact it.
Dr. Abdul El-Sayed: Hmm. I want to step back. I want to talk a little bit more about um sickle cell disease. But, you know, you dedicated your whole life to, to sickle cell disease. Can you tell us a bit about why?
Dr. Titilope Fasipe Yeah. So I was born in Nigeria. So I consider myself Nigerian American, and I, I had a childhood where part of it was in Nigeria, part of it was in America, kind of went back and forth and I didn’t know it at the time. But Nigeria is, you would call it the capital of sickle cell disease. It has the most sickle cell disease in the world, population wise. And I was one of those people that was born with the disease. Now, in my country, we don’t have a newborn screening program for sickle cell disease. So my parents found out when I started having my first complications, and that was when I was about one years old. So I was um having painful episodes, um they would take me to the doctor and that’s when they did the tests that determined that I had sickle cell disease. Now, because it was something that I was diagnosed with when I was young. For me, memory wise, it’s always been a part of my life. Um. My parents came to the United States for education, and so I spent my elementary education, here in the United States. And I had this experience of always being the only person with sickle cell disease. I never knew anybody else with it. In my family, I have three siblings as well as my peer group at church or at school. But then after my parents graduated, we moved back to Nigeria and that’s when I met several other children with sickle cell disease. And it was um a common experience. Some of those people were friends that we would go to school with, friends that I would go to church with. And then I was really inspired when I met my first family member with the disease, my maternal cousin, and she was a few years older than me, and I looked up to her. I was so happy that she was like, what I considered a healthy, vibrant person. Um. So it was, a shock and um highly devastating to me when this same cousin passed away at the age of 17. When my dad told me about my cousin, it was like, that was the first death that I had the experience of um you know, the emotional reaction where food literally tasted like sawdust. And I was um I had multiple thoughts. One, how could she have passed away? She was what I considered a healthy person with this disease. She was doing so well in school. She was smart, intelligent. I just couldn’t understand that component of it. And then the other part was um, an internal struggle where I wondered about myself. I um, I thought maybe this is just what happens to people with the disease. I guess this fact that I thought um I created in my mind was supported by the fact that my mom’s nursing textbooks said that people with sickle cell disease die by the time they’re 18. And so her uh death marked this kind of um pause in the way I viewed my disease, where I realized that time was running out. And some time before I turned 18, I too would um likely die. Now, to answer your direct question, I think a lot of people think assume I go into my field and study sickle cell disease because I had the disease, but it was really because of my cousin. I was like, people should not die at the age of 17. Um. You know, they should have the ability to live vibrant lives. I will say that my naiveness, I assumed her death was because of Nigeria, meaning, you know, poor health care system, didn’t have access to high quality medicine and this is what led to her, her death. Um. I’m going to jump ahead to when I finally got to practice medicine in the United States medical school, and I met an individual with sickle cell disease. I noticed that there were barriers in the health care system in the United States that also put people at high risk for early mortality. And so what started as this kind of quest to make it better for sickle cell, in fact I think my medical school essay, I said I wanted to cure sickle cell disease. I realized that it was actually this public health burden that required advocacy. And so it was encounters with people like my cousin and other people with sickle cell disease and the experiences that they had that inspired my career and shaped the the way that it panned out.
Dr. Abdul El-Sayed: First, I really appreciate you sharing your own story as painful as it is, and I’m sorry for your loss. I can’t imagine what it’s like to lose somebody who you see as a mentor and in whom you see yourself. And I’m I’m grateful that you’ve um, out of that pain uh launched yourself on a path to, to to building out a better world for people with, with sickle cell. So thank you for for that and for sharing your, your, insights and your perspective with us. I want to ask you, you know, as you think about um, how much of the consequence that the death and the the disability and and um pain that comes with sickle cell is a function of the disease? And how much of it is a function of our failure to build the best possible health care system uh for people with the disease?
Dr. Titilope Fasipe The question itself is almost like the answer. I um, no matter where you live in this world. If you have sickle cell disease, you face a disparity. You face an inequity of some kind. And, sickle cell is a very old disease. So it has been around for thousands of years. We were talking earlier about Africa, and um, I will say that years and years ago, before there was a Sahara desert, the area that we call the Sahara Desert now used to be like a tropical land um you know, streams, rivers, lakes. And that came with malaria. And so, scientists have determined that their earliest findings of somebody having the mutation that can cause sickle cell disease, um was a child that had one copy of the disease and that led to sickle cell trait. Sickle cell trait allows you to be somewhat protected from malaria. And so there was a beneficial reason for this mutation to persist in places that were ravaged by the devastation of malaria. And so it grew, and we found various haplotypes over the years. There’s four main ones, but they all arrived from this one mutation, this one child and I call it the origin story of sickle cell, where you have this superhero that was born, um that was able to fight against malaria. But in this twist of a paradox, if you will, the same mutation that allows you to have the superpower to fight against malaria or to live in spite of malaria, if you inherit it in a double dose, it has the horrible effect of messing up your red blood cells in the way that causes sickle cell disease. And so you would wonder, why would so many people around the world have sickle cell disease? And there’s millions of people impacted, and it’s because malaria was such a big problem, and it was beneficial to have sickle cell trait or one copy of this mutation. So I start that far back because now the mutation has spread throughout the world. For America’s purposes, it came first through trans Atlantic slave trade. So the first diaspora for Africans in this continent of North America and South America come from those slave ships. And so with the, culture, with the people, you also brought the sickle cell mutation. And so sickle cell has been around in America for years. However, it was quite invisible because it impacted a population that was invisible, that was not seen as um you know, as equal to the men and women of that day. And so the first reports of sickle cell disease for the United States purposes that is, came in 1910. And in fact, I always point out that they did not um discover it in an American. It was actually an immigrant, a young man who had come from the West Indies to study dental school. So he was Black, but he was not an American um Black individual. And so this young man was educated. He started having these pains that he couldn’t understand. His dental school was in Chicago. And it turns out he had sickle cell disease but we didn’t have a name for it back then, but that’s what he had. And so it wouldn’t be until decades later where they actually started realizing that this same condition that they saw in this dental student actually existed in Americans, um several Americans of African descent. And so decades would pass. And um, I always say, I thank God that sickle cell was a red blood cell disorder. Otherwise we probably wouldn’t even have made the progress that was made. But it’s easy to study blood. You can take some of it, look at it under the microscope. And so it was also a very um, a disease that sparked a lot of scientific curiosity. And so you have the likes of Linus Pauling contributing to the work of sickle cell disease and, and so forth. So with every um understanding of the disease brought us closer to solving this puzzle of why it did what it did. However, with all that scientific progress, it still did not erase the fact that the population that was impacted is a population that did not have rights and faced several inequities um on the social side, um as well as the side of health care. [music break]
Dr. Abdul El-Sayed: I really appreciate you sharing that that history. Right. And it really does speak to um, the origin of sickle cell at the advent of a public health failure. Right. You have a situation where you can’t control malaria. And in that situation, in that setting, you have this evolutionary enrichment of sickle cell trait. And when you have two people with sickle cell trait, then a quarter of uh their progeny are going to statistically have sickle cell disease. Um. And so you have this advantageous trait driven by a public health circumstance that then leads to um the advent of of sickle cell disease. And the point that you’re making, I think, is a really important one. Right. When you have a disease that predominantly affects Black folk, whether you’re talking about Black folk in America, in a society marred by racism, where diseases that Black folk disproportionately suffer are given less credence and less importance both to the research and to the clinical community. But then also in the context of our global focus, right where you have, you know, an African populace that tends to have still to this day, less attention paid to diseases disproportionately suffered by them. I mean, the fact that, you know, we don’t have um uh real investment in things like, you know, river blindness and uh other diseases speaks specifically to the way that racism plays out on a global scale in terms of the diseases that we focus on and then we treat. And, you know, it even shows up in clinics and hospitals today. And one of the things that um really struck me when I was in medical school was the way that um people with sickle cell disease tend to be uh stigmatized around their pain crises. And, I’d like you to, to tell us a little bit about, you know, the experience of having sickle cell disease, that the pain crises and then what tends to happen in clinical encounters when uh people come in in severe pain?
Dr. Titilope Fasipe I first, I have to say, I loved the way you summarized that. The way your public health brain works is fascinating to me. Um. And I appreciate that because you even hit on some things I never fully appreciate, you know, thought about and reflected on. Um. So I have now been saying this, um since that time in medical school when I met my first patient with sickle cell disease. Me being the student doctor, I’ve started to say to myself, and now I say it publicly, that sickle cell disease is the medical representation of the Black experience in America. And because I, I just, I couldn’t understand. I just couldn’t understand. And it’s because I was um, protected by my um, my, my two part heritage. When I was um in Nigeria. Nigeria has a a different way of addressing sickle cell disease. It’s not from the lens of racism. There are disparities, as you mentioned, and there’s social stigmas as well. Um. But not because of the color of my skin. So I come to America again, college, med school. And I didn’t realize it at the time, but I realized I had been put in a category by doctors as having good sickle cell. And first of all, there’s no such thing as that. But, um I surprised them, right? Why is this young lady in college? Okay. Surprise. Why is this young lady in medical school? Surprise! Um. And I didn’t realize the reason for the surprise until I got to medical school and met somebody with sickle cell disease who did not have my experiences. She was um, a woman of Jamaican uh heritage descent who happened um to also be homeless. And she also happened to have a family of young children. And I remember when she came into the emergency department, I realized um a sensation of the doctors not believing her. And I didn’t understand why I had I had I did not understand the relationship um between the pain and um the stigmatizing at that time. But it came to life, um front and center with this encounter. I was told by um the senior doctors that she is faking it because she’s a drug addict and she’s looking for pain medicine because she, you know, she’s a drug addict. And um, I couldn’t uh reconcile that because I had the disease, and I knew the pain was real. So I would talk to her privately, and I would ask her some questions and I was convinced her pain was real. I didn’t understand all the reasons why the doctors were convinced she was a drug addict. But I just knew her pain was real, and I didn’t know how to distinguish and explain all of those things at the time. So I mentioned that my medical school essay said, I wanted to cure sickle cell disease. But by the time I wrote my residency essay, um to enter Pediatrics, I realized I had to fix this, this problem that I couldn’t fully understand. The problem of why is sickle cell ugly in America? Why aren’t people believed? And why don’t people like the disease? I would talk to doctors telling them that, oh, I’m thinking of going into hematology to study sickle cell. And they’d be like, no, don’t do that. They’re drug addicts. It’s horrible. I just couldn’t imagine doctors talking this way about any other patient population. Um. And it took um some years later, after some research, I realized I had to leave medical research, and I looked into the field of public health and sociology. I read books by Doctor Keith Wailoo, and then I started to realize this relationship, this historical uh narrative of sickle cell disease and that that burden of the structural racism and the implicit and explicit bias that the patients face. So where does this come about? I talked, we mentioned earlier that one of the most common features of sickle cell disease is pain. And when we talk about managing pain, there’s two ways to manage it. You can manage the root cause of the pain. So let’s say you take a medicine that tries to control inflammation, something like um ibuprofen or acetaminophen. Okay. But then what about the suffering component of pain? Sometimes those medicines help with that. Acetaminophen can help bring down your headache. Ibuprofen might help that backache. But what if they’re not enough. For our um medical world, opioids enter into the picture for managing that suffering component of pain. And um, most people first encounter opioids maybe after a dental procedure. Sadly, some people have encountered it because of cancer pain and other diseases that cause significant pain. Opioids treat the suffering component of pain. However, opioids are also related to drugs that can cause addiction or to illicit drugs as well. They have um they affect the same receptors. And so this is where those links come across. But if I come into a hospital and I tell you that I have a migraine, or I tell you that um I’m about, you know, I’m pregnant and I’m having labor pains, my pain is respected and it’s managed um with compassion. But if I tell you I’m coming with sickle cell pain, something happens in in the the receipt of that knowledge, and it becomes embroiled into the belief that this person must be deceiving me. This patient is either a drug addict and they’re using sickle cell um as a way to get their drugs. This is highly problematic.[laugh] Um. And I um, I, I, I’m not trying to say um the medical field is bad or that uh doctors are bad, but what has happened here is that inaccurate beliefs and biases have seeped their way into a field that is seen as a pure field. You know, doctors go into medicine to help people, right? Compassionate care, all of that. But yet we are not um immune to having bias, and we’re not immune to having stigma. And so if you grew up thinking that a Black person’s skin is tougher than a white person’s skin, and this is, this is, been published that doctors, young doctors and other doctors believe this, you will manage a person that’s Black, their pain you’ll manage it differently. If you also um believe that every person with sickle cell is has a drug addiction problem, you are going to be hesitant when they describe their pain to you, because you don’t want to add to the problem of their presumed addiction.
Dr. Abdul El-Sayed: I really appreciate um the way you broke that down and and and the the extremely poignant uh quote you had about about sickle cell almost being the, the manifestation of, of Blackness in health care in America. I’ve been around clinics and hospitals enough to know that bias is real, and sometimes the worst bias is the bias that’s held by people who think that somehow they’re immune to bias. And I think the medical community, um the scientific community, uh tend to commit that kind of bias in ways that are, you know, so profound. And, you know, there’s a simple way, a simple thought experiment that, that you could ask, which is to say, if sickle cell was a disease specifically of upper income white people, how do we think we’d treat it? And I think it’s pretty clear that a lot of the ways that it gets intertwined, as you talked about with uh substance use disorder has a lot to do with the color of the skin of the people who have it, and the stereotypes that people hold about who these people are and why they might be coming in. And I, you know, I want to ask you, in your experience, both as a patient, as a provider and as someone who’s just an observer of the medical space. If you ran that thought experiment through, how how do you think we would treat sickle cell if was a disease predominantly of upper income white people?
Dr. Titilope Fasipe I suspect it would have been cured a long time ago. [laugh] Um. So I’m going to go back to, Doctor Pauling. So when Linus Pauling um started looking at sickle cell disease and he wrote this beautiful paper, you know, Nobel laureate, this guy is the scientist, a scientist. He had been interviewed on sickle cell disease, and he’s also been interviewed on another genetic disease, called cystic fibrosis, which predominantly impacts Caucasians, not necessarily rich Caucasians, but um Caucasians. And if you see the way his response was to how do we help cystic fibrosis and, you know, get it to where it needs to be from a health care perspective, get them better care and cure the disease, the response was um compassionate. It was very strongly for the fact that we need to figure out how to fix cystic fibrosis and help the people impacted at all cost, which was beautiful. But when you contrast that to the response he gave for sickle cell disease, um he basically said that um individuals with sickle cell trait need to have uh tattoos on their forehead so that they can recognize each other and not marry each other and stop the disease. So in this case, the solution to fixing sickle cell disease is to stop having babies with sickle cell disease, which is, you know, for any genetic disease. That’s that you can say that this is a plausible solution. But um it removed the the compassionate tone and the seeing the humanity behind the disease. And um I picked on Doctor Pauling because his words were public. Right. You can go to his uh, his nice, beautiful website. They have all of his recordings there, but every day there’s people that have that same um concept. We value um the pain of people that we value is the best way uh to say it. And unfortunately, the person that has the most value in our society appears to be a person that is white. But, um and you emphasized a double component there um the wealth component, wealth and whiteness do buy access. They get you access, I should say, not buy access. I mean sometimes they buy it. And so I think we would have had a different society. We may not hear of sickle cell disease in the same ways that we hear about it now. People would understand why the pain medicine is necessary. Maybe we would even find better pain medicines. The research dollars would have poured in a long time ago. When did we start even paying attention to sickle cell disease? Well, it dates back to the 1970s. President Nixon actually signed the first sickle cell act, and he did it after getting lots of pressure from advocates. So I mentioned sickle cell disease was written about first in 1910, but it wasn’t until 1970, so 60 years later, that people acknowledged it as a problem. And it would take a few decades later from that 1970 for people to actually call it a public health problem. So the second Sickle Cell Act was in 2003. So I, I um, I am not a cystic fibrosis expert, and I don’t like pitting diseases against each other, but cystic fibrosis is a disease that um and I will also add hemophilia to this list. They’re diseases that have largely, predominantly affected Caucasian uh populations. And you end up seeing, um the advocacy and the support for that advocacy, uh have a huge, uh benefit of return. So, for instance, the Cystic Fibrosis Foundation has done beautiful work that has been highly funded. And, several people quote these numbers that well, if you compare um funding for sickle cell versus funding for cystic fibrosis, it’s like seven times or more higher, you know, things like that. The Hemophilia Foundation has also been able to do impressive work, and it’s because it’s been supported financially and with advocacy at the highest of levels. Each of those diseases have multiple drugs and multiple research projects associated with them. Sickle cell disease, pales in comparison. And so, I mean, if it was a disease that impacted rich white people, I don’t know if we’d even be having this, we would not be having this conversation today. And um, we would probably be talking about how everybody with sickle cell gets a cure as soon as they turn one years old or something like that, you know, um because right now it is possible to cure sickle cell disease. Um. However, access is part of the issue.
Dr. Abdul El-Sayed: And I want to ask you about that because we have these new Crispr mediated uh treatments or even there’s there’s two of them. One is Crispr mediated, and one uses an adjacent uh platform. But the treatment is is profoundly onerous, and it’s extremely expensive. And, I want to ask you, A.) What do you think about these treatments? Um. And uh, and and the kind of hope that they offer versus the kind of cost that they incur. And how do we do better to actually get them to the people who need them?
Dr. Titilope Fasipe So we’re going to we’ve already been on a dark road and talking about heavy things. We’re going to still be on this topic, but I want to remind people that I am a pediatric hematologist and I am a person full of hope. So but I will um say that sickle cell is a disease that has been um blessed by accidents. So how did we even learn we could cure sickle cell disease? Well, in the 1980s, a young girl had sickle cell disease. And then, sadly, she also got leukemia. In efforts to cure her leukemia, she underwent a bone marrow transplant. And you might hear this term stem cell transplant. And her leukemia was cured. And lo and behold, her sickle cell was also cured. So that led to us understanding that there is a way to cure a disease like sickle cell. You can transplant them. But there’s some um caveats to this. I call transplant a complicated cure. And even in the day and age of gene therapy, it’s still very, very complicated. Why is it complicated? So first, if we stick with just transplant before we get to gene therapy, a transplant required that you had a donor that matches you. And for sickle cell, it was best that that donor be from your family. This also has to do with the uh, the lack of ethnic diversity in our bone marrow donor pool. And so even if a person with sickle cell disease found a match in the donor pool, first of all, it was very hard to. It was not as perfect as somebody in their own family. So the barrier of having a donor was there. And then transplant therapies already are um very expensive. But thankfully, if you have a donor for children, at least uh, you it’s something insurance could cover. So what about the majority of people who do not have a donor? And for context, I would say about 1 in 10 people would have a match in their family. So the majority of people with sickle cell disease, transplant was never on their radar list because they didn’t have a match. So that’s why there was such a um, a high interest in gene therapies, genetic therapies, as they stand right now, they are still transplant based therapies. So the two ways that were recently approved by the FDA. One is uh, I’m going to put them in categories. One is called the gene addition category. And it uses a vector to help add that gene into your cells, into your um, your genetic makeup. And then the cells will start making healthy hemoglobin. And then there’s another way that is gene editing. And that’s the Crispr based method and the editing that it did, it kind of went and did some um, editing of your, of your genes to remove the thing that um keeps you from making something called fetal hemoglobin. Fetal hemoglobin is a protective hemoglobin that all of us know how to make, but we stop making once we’re born. So the two types of ways, the end goal is give the person healthy hemoglobin. But how do you get this back into their body? So if you took out their cells you fix them genetically. How in the world does it get back? Well, that’s why gene therapies are still a transplant therapy. You still have to give some chemotherapy, what we call conditioning, to prepare the body in order to get the new stem cells. So that means you have to get rid of the old stem cells. And then you can infuse the new ones. And so it requires the same medical rigorous-ness as stem cell therapy. And then there’s that added step of the gene um therapy, the fixing of the gene. So the price tag differences. So you have all the cost of regular stem cell transplant. But then that fancy step, the step to fix your genes adds an additional cost. And so I know folks that have been following this in the media have seen um the cost range from two million to three million dollars. [laugh] So um, so yes, there’s that. Now, nobody is going to pay two million to three million dollars um for this therapy. Maybe there are some people who are in um an income bracket that could afford this, but the majority of people um cannot. And so even as the gene therapies were being u researched, there were several cost um models and economists would be pulled in, health economists to try to see how do we make this a feasible treatment? How do we say that this, the value of a life of somebody who has sickle cell disease, it’s worth investing this amount of money and also how do we make it achievable for an insurance company to cover it? These are conversations that are ongoing. And I will say um, there’s several expensive therapies that have uh price tags this high. And it goes back to what we say, how we value. For some of those therapies I don’t think we blink an eye to say it makes sense to pay two million dollars. Um. But with sickle cell, there is a lot of um [laugh] hemming and hawing, if you will. Um. Because they the public burden is great. And yet we still are arguing about whether it makes sense to pay this much money to actually cure somebody.
Dr. Abdul El-Sayed: I want to I want to ask you, because, you know, this, the whole idea of a debate over the value of a life. This is something economists spend a lot of time thinking about. But implicit in those models is that they value people based on their economic output. And I remember learning about this in my PhD program and just feeling kind of nauseous about it. Because it’s such a crazy way of thinking about how we value one another. And, you know, I think if it was my kid, right, I I the two million, three million, whatever it was, it’s my kid. Everybody’s somebody’s kid. And we don’t we don’t value uh folks in that way. Plus, right there is this added effect of asking, you know, who are we if we can’t take care of people who are our most vulnerable? And a lot of this kind of goes back to the point that you made earlier, which is how do you stereotype people with this disease? And so you have this, this stereotype that um stigmatizes and implicitly devalues folks’ lives. And we now have a treatment that can buy folks out of all of those pain crises, out of all of those visits to the E.R. and we’re debating, uh the costs and and whether or not it’s worth it. Now, you talked about being a pediatric hematologist, which, you know, you see a lot in your practice, um suffered by people who, by definition, have no agency in any of what they’re suffering. These are children. And um, and in order to do that kind of work, I know that you got to have a certain kind of metal and a certain kind of hope. So as you think about, you know, where we’re headed, uh when it comes to um sickle cell disease, what what gives you the most hope right now?
Dr. Titilope Fasipe So when I mentioned that I I’ve been trying to understand sickle cell disease, I’ve really become a student of understanding all these um components of what it means to have sickle cell disease, not only in this country, but just what does it mean to have sickle cell disease? And there’s this question that keeps on rolling in my mind. I I show it in every presentation I give, and it’s how do we solve a problem like sickle cell disease? And the solution is redefining the problem. I will say that redefining the disease means do not just see it as a medical red blood cell disorder, but embrace every single public health component of the disease, embrace every negative, the good, bad, the ugly, and really work that into your definition of how to solve the problem. And then we’re going to get ourselves somewhere. The first step to it is defining the problem, right? And so um I mentioned the 1970s. I mentioned 2003. Well, over time, there have been these waves of progress in sickle cell disease. And the most recent wave I will attribute to about 2015 when several groups got together and it was almost like this enough is enough moment. And that was interestingly timed with some of these research companies getting more vested in sickle cell disease. But I feel like this wave is not coming crashing down. I and I used to um there was a term I picked up in one of Doctor Wailoo’s books called Roller Coaster Therapeutics. But I feel like we’re at a situation where we’re no longer on a roller coaster, and we are maybe on a train and we’re cruising forward. We have a destination, and that destination is equitable, high quality care for individuals with sickle cell disease. One of the things I left out when I was talking about gene therapy and transplant is that most people in this country do not have access [laugh] to quality care for their sickle cell disease. So even if I stood out in the street and said, hey, free gene therapy, they wouldn’t even have had their basic needs met before they even talk about getting such a cure. And so where the hope comes from is that I am glad we are calling out the problem. And it’s been at the highest levels, the highest levels, and it’s been the entire world. It’s not just a piece of the world. And as long as we’re calling out the problem, we’re holding ourselves accountable to addressing the problem. And so I am happy we’re in 2024. And when I see children, families come in with newborns with sickle cell disease, I keep on telling them, I’m so happy your child was born now, because if your child was born in the ’80s, if your child was born in the ’70s, like maybe the uncle you told me about or the grandma you heard about, um it wouldn’t have been a happy story. But now I can prevent organ damage with good medications. I can prevent infection with good medications. It’s it’s more about access. If you can get the access, I can help prevent some things until we can get you that cure. And that it feels incredible to say when I I can look somebody in the eye and say, I think your child is going to be okay, and we’re going to work hard to ensure that that’s the case. Um. I, I say this, like I said confidently. I look them in the eye and part of my brain is like, Lord, um I hope it’s before this child becomes an adult. My teenagers, I have the hard talks with them that the adult side is not pretty. Um. But the babies, I, I’m like, I think we can do this. We can do this. We got to do this. [laughing]
Dr. Abdul El-Sayed: Well, I, I deeply, deeply appreciate that you’re the one doing it. And, um we really appreciate you taking the time to share your experiences, uh your insights and and your wisdom and hope with us. Our guest today was, Dr. Titilope Fasipe and uh she is a pediatric hematologist, an expert and advocate on sickle cell disease. Thank you so much for taking the time.
Dr. Titilope Fasipe Thank you so much. Really appreciate being here. [music break]
Dr. Abdul El-Sayed, narrating: As usual, here’s what I’m watching right now. The CEOs of Meta, TikTok, Snap, X and Discord were on Capitol Hill like IRL last week for a Senate Judiciary Committee hearing where they were absolutely grilled.
[clip of Senator Josh Hawley] So let me ask you this. There’s families of victims here today. Have you apologized to the victims?
[clip of Senator Lindsey Graham] These are bastards by any known definition. Uh. Mr. Zuckerberg, I know you don’t mean it to be so, but you have blood on your hands.
Dr. Abdul El-Sayed, narrating: And you know what? They’re right. It’s almost impossible to overstate the way that social media has fundamentally reframed our society. It’s gutted so many of our social institutions, lying to us that the online simulacrum might be better than the real thing. It’s polarized us, feeding the worst of ourselves to one another. It’s created whole realities that people, particularly young people, can’t escape. And that’s the thing that I think those of us who remember a time before get wrong. We don’t even appreciate that if you came up in the social media era, leaving social media means literally leaving the social milieu that most of your generation occupies as a matter of course. The impacts on young people have been profound. And the thing about it is that these companies have known what they’re doing. They even studied it themselves. But they’ve put the pursuit of the ability to market to young people over what the consequences of that might be. That said, I worry a lot about two things here. First, too often public policy is shaped by what politicians signal then what actually makes a difference. That means that rather than focus on the humdrum, mundane aspects of the way that social media leaves young users lonelier, sadder, and more anxious, they’re focused on those salacious stories that are awful on their own terms, but not the main reason why these companies need regulation. The second is that the train’s already left the station. And worse, there are new trains coming to take its place. Time for regulation would have been in the early 2010’s, when senators were baffled by the basic business model of these companies, let alone had wholly baked perspectives on how to regulate them. And while senators are busy trying to fight yesterday’s war, I worry that they’re missing today’s on AI and the ways that it’s going to accelerate so much of what’s rotten about social media. Europe is experiencing a major measles outbreak. Just to offer some context, at the end of 2022 there were just under 1000 measles cases reported. By the end of 2023, just about a month ago. There had been 42,000. And if you’re doing the math, that’s more than a 4,000% increase. The outbreak represents a confluence of factors that should give us all here across the pond a bit of a pause. Measles is one of the most infectious diseases known to man. If every case of Covid in an immune naive population will go on to create another four cases, measles will go on to create 18. Talk about going viral. So it’s really, really good at exploiting crevices in our immunity. And guess what? There are a lot more of those crevices. The Covid pandemic left a lot of public health destruction in its wake. And one of those was kindergarten immunization rates. In some context, that was because during Covid, there just wasn’t enough infrastructure left to assure that it happened. But in places like the US or Western Europe where this massive outbreak is happening, more of that has been idealogical. The number of Americans who believe that pre-kindergarten vaccines for things like measles should be required fell from 82% before the pandemic to 71%, and actual uptake rates of the MMR vaccine, which stands for measles, mumps and rubella. They’ve been slipping down from 95% pre-pandemic to 93% now. That 2% may not sound like a big drop. But when you’re talking about measles with a reproductive rate of 18, it can make the difference between a few cases and a few hundred. Measles is a serious disease, but with good health care, it has a very low mortality rate, less than 1 in 1000. But it has some scary consequences. One of them, the rare, is a serious, nearly universally fatal neurological complication that emerges in teenagers who survived measles. And when the number of cases starts to climb, the risk of subacute sclerosing panencephalitis, that starts to climb too. It turns out that the vaccine debate has real life consequences. Who knew? Teen vaping is on the rise again, and one country is doing something about it. In the UK, more than 20% of young people between 11 and 17 reported trying a vape last year. That’s up 5% year on year. The government in that country is clamping down. They unveiled a bill package that would ban single use vapes and heavily restrict both packaging and flavoring. That follows a report from an independent public health nonprofit that found that most underage use was driven by flavored, single use vapes. This is a smart move, one that follows a call by the WHO director general to do just that. Vaping was originally created to provide a healthier alternative to actual tobacco smoking. But too often it’s done the exact opposite. Hooking a whole new generation of young people to nicotine. A large proportion of whom then go on to smoke the real thing. Here in the US, Congress amended the Pact act to target flavored e-cigarettes in 2021. But the policy has a notable disposable vape sized loophole, which, you guessed it, the policy doesn’t explicitly prohibit flavored, disposable vapes. While it’s hard to imagine Congress doing much of anything right now, fixing this would be a great place to start. That’s it for today. On your way out. Don’t forget to rate and review. It really does go a long way. Also, if you love the show and want to rep us, please drop by the Crooked Store for some America Dissected merch. And don’t forget to follow us at @CrookedMedia and me at @AbdulElSayed no dash on Instagram, TikTok and X. [music break] America Dissected is a product of Crooked Media. Our producer is Austin Fisher. Our associate producers are Tara Terpstra and Emma Illick-Frank. Charlotte Landes mixes and masters the show. Production support from Ari Schwartz. Our theme song is by Taka Yasuzawa and Alex Sugiura. Our executive producers are Leo Duran, Sarah Geismer and me. Doctor Abdul El-Sayed, your host. Thanks for listening. [music break] This show is for general information and entertainment purposes only. It’s not intended to provide specific health care or medical advice, and should not be construed as providing health care or medical advice. Please consult your physician with any questions related to your own health. The views expressed in this podcast reflect those of the host and guests, and do not necessarily represent the views and opinions of Wayne County, Michigan, or its Department of Health, Human and Veteran Services.