Hemophilia is a rare bleeding disorder caused by a deficiency in clotting factors in the blood, which can cause permanent damage to joints and in some cases, life threatening bleeding, both externally and internally. Today, people with hemophilia can live generally long, healthy lives, but in previous generations, the future wasn’t so bright. In fact, less than a century ago the life expectancy for someone with hemophilia hovered around just 10 years. It doesn’t receive much attention, and when it does, what you hear might not be all that accurate. In today’s episode, we cover the history of hemophilia and the science behind treatments over the decades, including the devastating impact of the HIV/AIDS epidemic on hemophilia patients. We’ll also clear up a number of misconceptions, including one that seems to show up a lot in popular culture: that hemophilia is a quote “royal disease.”�
Transcript of this Episode
Ben Marcus: My parents found out that I had hemophilia probably in the worst way you can imagine. When I was born, I appeared healthy, I was discharged, I went home. But then a few days later, I was laying on my mother's chest. She could feel my heartbeat, and she noticed it was beating irregularly � you know, mother's intuition. So my parents rushed me to the emergency room. They ran tests, and they found that I had a blood clot pressing against my brainstem.
Sam Jones: That’s Ben Marcus, a science communicator and media relations specialist.
Ben Marcus: So they ran me into surgery. They installed a shunt into my brain to drain some of the fluids and relieve pressure. And that's when they diagnosed me with hemophilia, and they determined that I must have sustained a bleed during labor, which had clotted and caused the problem in the first place. And one of the doctors actually told my parents that I might never walk or talk. And I was six days old at this point. Can you imagine?
Sam: Hemophilia is a bleeding disorder caused by a deficiency in clotting factors in the blood, which can cause permanent damage to joints and in some cases, life threatening bleeding, both externally and internally. As soon as Ben was diagnosed with hemophilia, he started receiving treatment.
Ben Marcus: Fifteen years later I had an appointment with my hematologist. I ran into one of the nurses who was there at the very beginning in the NICU and she recognized my mother, and then she turned and looked at me and she was shocked. I mean, I had never elicited a reaction like that before in someone. I think she was just amazed that I was alive, first of all, and that I was stable and, you know, relatively healthy. I was talking, I was walking and all that jazz. It gave her an answer to that question that must've been in her mind of “how was I going to turn out?�
Sam: But in previous generations, the future wasn’t so bright for kids diagnosed with hemophilia. In fact, less than a century ago the life expectancy for someone with hemophilia hovered around just 10 years.
Welcome to Tiny Matters, a science podcast about the little things that have a big impact on our society, past and present. I’m Sam Jones and I’m joined by my co-host Deboki Chakravarti.
Deboki: Hey Sam, I’ve really been looking forward to diving into this topic. Hemophilia is a rare disease that doesn’t receive much attention, and when it does, what you hear might not be all that accurate.
Ben Marcus: A lot of people assume that if I get a cut, my blood's going to gush out like water from a fire hose, and I'm in mortal danger, but it's really not the case. I don't bleed faster than anyone else. My blood just takes longer to clot. So as long as the injury or the bleed is handled appropriately it’s really not that big of a deal.
Deboki: In today’s episode, we’ll talk about some of the history of hemophilia and the science behind treatments over the decades, including the devastating impact of the HIV/AIDS epidemic on hemophilia patients. We’ll start by clearing up another misconception, one that seems to show up a lot in popular culture: that hemophilia is a quote “royal disease.”�
The reality is that hemophilia has been around for thousands of years and you can find patients with hemophilia all over the globe. But it is true that hemophilia affected many European royal families in the 19th and 20th centuries. And it all seems to have started with Queen Victoria of England, who ruled from 1837 to 1901.
There are three types of hemophilia: hemophilia A, B, and C, though hemophilia C is much more rare compared to the other two. What sets these different types of hemophilia apart from each other are clotting factors, which are proteins involved in the process of blood clotting. A person with hemophilia A has low levels of factor VIII, a person with hemophilia B has low levels of factor IX, and a person with hemophilia C is low on factor XI.
Sam: Queen Victoria of England is believed to have been a carrier of hemophilia B or factor IX deficiency. About one third of babies who are diagnosed with hemophilia are the first in their families with the disorder, which was the case for Ben. The other two thirds of kids will inherit the disorder from a family member. Hemophilia is X-linked, meaning it’s passed down through the X chromosome. Because males receive one X chromosome from mom and one Y chromosome from dad, if the X chromosome they get from mom has a clotting factor mutation, they’ll have hemophilia. If a female receives an X chromosome from mom with a clotting factor mutation, there’s still another X chromosome from dad that will hopefully help compensate.
This is a simplified version of things � we now know it can be far more complicated but, for a very long time, it was just assumed that women could not get hemophilia. That’s false, but severe hemophilia is far more common in males, meaning they produce less than 1% of the normal amount of factor VIII or IX. In the United States, it is estimated that around 33,000 males live with the disorder. In Queen Victoria’s case, she passed the trait on to three of her nine children � her son Leopold who died of a brain hemorrhage after a fall at age 30 � and her daughters Alice and Beatrice. Alice’s daughter Alexandra married Tsar Nicholas of Russia. And this is where I think many of us, myself included, started to think of hemophilia as solely a quote “royal disease.”�
Tsar Nicholas and Alexandra’s son, named Alexei, had hemophilia. As a young kid I kind of became obsessed with the Romanov family, mainly because of the lore surrounding Alexei’s sister Anastasia. We’re not going to cover how the family hired Rasputin to try to heal Alexei, which likely played into the Bolshevik Revolution of 1917 which ended their dynasty and ultimately their lives� but it is fascinating and 100% where I got the wrong idea about hemophilia.
Deboki: In the past people often didn’t know someone had hemophilia until they had died from an injury � sometimes as minor as a bump to the head. Today there are a number of blood screening tests that can be done as soon as a baby is born that will measure how long it takes for their blood to clot. There are also clotting factor tests that look at the activity of factors VIII and IX in the blood. And, in some cases, prenatal genetic testing can diagnose hemophilia before a baby is born. About 15 years ago, Ben decided to find out the exact mutation that led him to produce so little of factor IX. Turned out it was an incredibly rare single nucleotide change in the factor IX gene.
Ben Marcus: Which has led me to only have 5% of the normal factor in my body. And now the two joints with arthritis and all the stress of medical insurance and everything caused by a one letter change in my DNA.
Deboki: A big concern for people with hemophilia is that any kind of normal, every day activity can lead to bleeding. Maybe they twist their elbow the wrong way or step a little weird off a curb. If you don’t have hemophilia, that injury could heal on its own and you might not even notice it. But for someone who has hemophilia, it could lead to painful swelling. And surgery can be dangerous, especially if a person doesn’t know they’re a hemophiliac. That was the case for Robin Monin.
Robin Monin: I found out that I had a bleeding disorder in third grade. I’d had an appendicitis, and so I had to go in to get my appendix removed and everything was fine, but then they couldn't stop the bleeding for it. So I ended up having to stay in the hospital quite a bit longer than normal for that procedure. It was not a huge surprise. My little brother has hemophilia and it was in our family.
Sam: Not even 10 years ago Robin, now the program manager for the Hemophilia Association of the Capital Area, was talking with an oncologist who told her, “Oh, women can't get hemophilia.� But Robin, and Queen Victoria, and many other women are living proof that they can. Although things are improving, women with hemophilia have been consistently overlooked.
Robin Monin: I think one big reason is definitely the money � research gets put into where the money is. And if you talk broad strokes, men are more likely to be severe. And if you're severe, you have to go on prophylactics. If you go on prophylactics, that means millions of dollars for the pharmaceutical companies. I don't want to sound too jaded, but they get millions of dollars off these people. My two kids alone, I think it's like half a million dollars a year, and they're nine. They're so little.
Sam: When Robin was pregnant with her first son, she and her doctors made a plan for the distinct possibility that her son would also have hemophilia, which would mean a very cautious delivery.
Robin Monin: I went in, got induced, everything was going beautifully well, and then halfway through a midwife who I've never met before in my life, I'm still mad at her, even though she was right. She came in and she's like, we're doing this all wrong, we have to change the whole entire plan. And I'm like, what are you talking about? We have a plan. And she's like, first of all, we can't give you an epidural because it can paralyze you because you can start bleeding into your spinal column, all this kind of stuff. I'm like, that's fine. I just won't have an epidural. And then she's like, and we can't do a natural birth because if anything goes wrong at all, it's like spectacularly wrong. It's not just a little wrong like they’ve been telling you, it's spectacularly wrong.
Sam: Robin underwent a last minute emergency C-section, fully under anesthesia.
Robin Monin: They were really worried about brain bleeds, so that's what they were worried about. So they don't want to do anything that might compress the head. So we're going to do a huge incision under anesthesia. They're like, but we have 60 seconds to do it because after 60 seconds, the anesthesia goes to the baby, they stop breathing.
So what we're going to do is we're going to count to three. They're going to push the medicine in you to make you go to sleep, and I'm going to cut at the same time because we only have 60 seconds. All I remember was her going 1, 2, 3, push and me screaming, “don't cut, I'm not asleep.� That's all I remember. And then, yeah, I didn't see him until the next day. I woke up, but I lost so much blood. My blood pressure dropped really low, and so I couldn't see anything. I was completely blind. I was shaking really badly. So that's when I learned I need to get everyone on board with everything before big events happen.
Deboki: As it turned out, Robin’s son, Josh, did have hemophilia. She told us that, initially, figuring out his medication was very challenging but now he’s doing great.
Robin Monin: He's in tons of sports. He's very active. I can count on both my hands how many joint bleeds he's had. He's been doing really, really well.
Deboki: Robin and her husband adopted their second son, Patrick. He’s a severe hemophiliac, like Josh, but is also very active and doing incredibly well.
Once Robin was diagnosed with hemophilia, her relatives began looking back at their family tree and even did some testing with relatives who were still alive. They discovered that Robin’s grandmother was a carrier. Her grandmother’s brother � so Robin’s great uncle � also likely had hemophilia. He died unexpectedly when he was only a teenager from falling off his bike. Robin’s uncle also had hemophilia, and he sadly died during the AIDS epidemic, because he was given a factor treatment contaminated with the virus.
So let’s backtrack a bit.
Randall Curtis: As I was growing up, what I can remember was mostly plasma. I was getting plasma.
Sam: That’s Randall Curtis, the president of the Hemophilia Foundation of Northern California. Randall was born in 1954. It was around that time that fresh, frozen plasma was becoming the primary treatment for hemophilia. Plasma is the fluid part of the blood, and it has clotting factors that can help hemophilia patients. Before that, patients were treated with a number of different things, from diluted snake venom that causes blood to clot to whole blood transfusions. Plasma was a big step forward, because it contains a much higher concentration of clotting factors than whole blood does, but it still wasn’t all that efficient.
Randall Curtis: The problem with plasma is that there's a very small amount of the factor VIII, which I need, so I had to get a lot of plasma. So if I bled bad enough to have to go into the hospital and get treated, it was like a week of just having this plasma going into me. And it took a long time to resolve the bleed.
Sam: Randall was diagnosed with hemophilia in 1955, when he was about a year old. After his dad was playfully tossing him up in the air and catching him, he noticed that Randall had developed bruises under his arms.
Randall Curtis: And they took me into the hospital and when the doctor came out and they said, your son has hemophilia, my mom burst into tears because her brother had died from hemophilia and they knew that it kind of ran in the family kind of thing, but they weren't real clear on the genetics at that time, but they knew that they were in for a fairly rocky road.
Sam: When Randall was young, most kids with hemophilia went to special schools where they could be more carefully monitored and treated in a fragile manner to prevent bleeding episodes. Randall’s dad was a teacher and convinced the district to let Randall attend one of the public schools with kids who didn’t have hemophilia. Out of an abundance of caution, though, Randall had to use a wheelchair.
Randall Curtis: At the time, most of my bleeding episodes were in my ankles. And so by putting me in the wheelchair, that reduced the potential for me to have bleeds in my ankles. I had braces on my elbows and braces on my ankles, and they told my parents I wouldn’t live much past 13.
Sam: But today, Randall is in his 70s. And the treatments available have progressed far beyond plasma.
Randall Curtis: In 1964, there was a physician at Stanford. Her name was Judith Graham Pool, and Judith Graham Pool was looking at coagulation, and she noticed that when you froze plasma and thawed it, there was a deposit of kind of gunky proteins in the bottom of the bag. And she looked at that and realized that that part of the plasma was actually very rich in the factor VIII that people with hemophilia needed. So they developed a technique of freezing the plasma and letting it thaw slowly, and then draining off the fluid and keeping this gunky stuff at the bottom, and they called it cryoprecipitate.
Deboki: When Randall started using cryoprecipitate in 1966, it completely changed his life. Instead of being hospitalized for a week, he could receive this cryoprecipitate in around 45 minutes.
Randall Curtis: And my bleeding was fixed, and I was able to get out of the wheelchair, get rid of my braces. It was a huge liberating event.
Deboki: Then, in 1968, pharmaceutical companies figured out how to take that cryoprecipitate and freeze dry it. That was the beginning of clotting factor concentrates. By the early 1970s, it was being used throughout the country.
Randall Curtis: And this was the beginning of the golden age of hemophilia. We started summer camps. We could actually take these kids and put them in summer camps and give them clotting factor and they could swim and they could hike, and they could do all that stuff that they weren't able to do before. People were going to college, people were getting jobs, and we weren't dying.
Deboki: But these plasma donations still came from people across the country. And many times donors were drug users and people considered high-risk for infection.
Randall Curtis: And so there was a lot of hepatitis in these things because the way they produced clotting factor was they would take all this plasma and they would put it into a huge vat, and they would process it that way, which was cost effective. But what had happened was anybody that had, let's say, a viral infection, if you throw that one bag of plasma into that vat, then the whole vat was contaminated. And every time I used a bottle of clotting factor, I was exposed to hundreds of donors that were used in that batch of clotting factor.
Deboki: And then, in June, 1981, the U.S. Centers for Disease Control and Prevention released the first report of a rare form of pneumonia in young, previously healthy men. By 1982, that disease had a name: Acquired Immunodeficiency Syndrome, or AIDS. It took until May, 1983, for the cause of the disease � human immunodeficiency virus, or HIV � to be identified. By then, so many people had been infected, including a huge percentage of people with hemophilia. Of the 10,000 hemophiliacs in the United States at the time, around 5,000 were infected with HIV and over 4,000 of those patients are estimated to have died of AIDS.
Randall Curtis: So there was a long period of death and just horrific reactions to HIV and AIDS and people with HIV were stigmatized in ways that we can't hardly imagine now.
Sam: One awful case was that of the Ray brothers � Ricky, Robert, and Randall. All three were hemophiliacs who contracted HIV from contaminated factor. They were banned from school in Arcadia, Florida and their home was set ablaze by people in the town. These boys were just 7, 8 and 9 years old.
Randall Curtis: I can tell you that I was a counselor at hemophilia summer camp in the early nineties, and there were kids coming to the camp with IV poles, and we all knew that they only had a few months to live, but they wanted one last camp experience before they died. And it was just horrific to see all these young boys die just because the stuff that was supposed to keep them alive was killing them.
It was horrific. But what it did is it really caused the hemophilia community to come together in a way that it never was before. And to march on Washington and to demand changes and to sit on these blood advisory boards where they had never had patients on them before. And the hemophilia community became the watchdogs of the blood supply, and we became extremely militant and active. And we still have that role today.
Deboki: As it turned out, Randall was born with a mutation on his T cells that makes it so HIV can’t bind and infect the cells, which was likely the reason he was one of very few survivors of his generation. He did, however, get hepatitis C from the factor treatment available at the time. And hepatitis C is really, really hard on your liver.
Randall Curtis: My liver started to fail in the early two thousands, and by 2012, I was fairly close to making an exit, let's say.
Deboki: Randall was put on medication that killed the virus but a lot of the damage had been done. He knew he would need a new liver. By 2020, things were dire. Fortunately, soon there was a liver available.
Randall Curtis: I got the liver, and by the time they finished sewing me up, my hemophilia was gone.
Sam: Which is amazing.
Deboki: I know, It really is. And the reason it happened is because clotting factors are produced by liver cells, and the new liver Randall received was producing enough clotting factor to take care of things. Pretty remarkable.
So, going back to the 1980s: By the mid to late 80s, clotting factor products had become a lot safer. They were heat inactivated to kill any viruses. And by the early 90s, the products could be tested for specific viruses including hepatitis C and HIV.
Sam: In 1992, the first recombinant factor VIII product was approved by the FDA � recombinant meaning it's produced in bacteria or cell lines in a lab instead of people. And then in 1997, the FDA approved the first recombinant factor IX product.
A range of recombinant products and synthetic drugs have entered the market since then. There are now products that can be delivered subcutaneously, meaning a patient can inject into a fatty area of the skin as opposed to needing to locate and inject into a vein, which is much more difficult and can be painful. And these products don’t provide clotting factor but instead inhibit anticoagulants, increasing the ability of your blood to clot.
For Ben, what seems to work best is clotting factor that arrives as a freeze dried powder. He reconstitutes it in saline, puts it in a syringe, and then gives himself an intravenous injection.
Ben Marcus: Right now, I am on what's called an extended half-life product because the certain therapies today, they are manufactured in a way where the factor is conjugated to an antibody that is less susceptible to degradation in the body. So it lasts longer. And that means that I don't have to worry about treating myself as often, and right now I get to only have to treat myself once a week.
Deboki: In June, 2023, hemophilia treatment took a big step forward when the FDA approved the first gene therapy for adults with severe hemophilia A. Now there are two additional FDA-approved gene therapies for hemophilia, which address hemophilia B.
These gene therapies use a virus, specifically a non-pathogenic one called adeno-associated virus. The virus’s natural genetic material has been removed and replaced with new DNA containing the factor VIII or IX gene.
Ben Marcus: When a patient comes to the clinic to receive a gene therapy, they get an IV infusion of this virus, billions and billions of copies of this virus. It goes into their blood and it has a little tag on it, like an address label, that directs it to the liver, to those cells that normally produce factor VIII and factor IX.
Deboki: The viruses then inject the new DNA into the patient’s liver cells and soon they begin to produce the factor VIII or IX protein that then makes its way back into the blood.
Ben Marcus: The gene therapies available today are a single infusion. So once the virus gets into your body, delivers the factor proteins into your liver, and the liver starts pumping out factor, you should be good to go, theoretically for years. They're optimistic that it'll last for a long time. And the thing is, once you receive this treatment, your body becomes immune to the virus that you injected into your body. So you can't get a second shot because your body will just destroy it as soon as it enters your body. So it's really a one-shot deal.
Sam: We asked Ben if he would ever get this gene therapy and he said, at this point, no.
Ben Marcus: It really comes down to the cost benefit ratio. Right now I feel like my hemophilia is well managed. Gene therapies are so new that they're still doing surveillance on these treatments with every new patient. So that means I would have to go back and get lab work done every one to two weeks for the first few months. And then those appointments carry on periodically for the first couple years. And that's just to make sure that the factor level is staying up to where the level is supposed to be, and also to make sure that my liver is not freaking out because there's been some concern that these treatments could cause an immune reaction that could hurt your liver. But I don't want to suggest that anyone should or should not get gene therapy because I can understand if someone's hemophilia is harder to manage or they're more severe and their treatment regimen is more disruptive to their daily lives, then it might be worth it to undergo gene therapy. But for me at this time, it's just not the route I want to take.
Sam: For Ben, the biggest hemophilia-related problem he currently has to deal with is joint pain. When blood enters the joint space it can irritate the joint lining, causing inflammation and eventually damaging the cartilage, leading to arthropathy or joint disease, specifically arthritis.
Ben Marcus: My main issue right now is chronic pain in the joints where historically I've bled a lot, and the bleeding in two of my joints has caused arthritis. So I take daily anti-inflammatories, and they're not over the counter NSAIDs. I can't take aspirin or ibuprofen because they're blood thinners, right? They deactivate your platelets. So instead, there's a prescription NSAID that works a little bit differently. So it still helps reduce the pain, but it doesn't inhibit my platelets.
Annette Von Drygalski: Especially for those who are already somewhat older and have not had access to clotting factor or any of these new treatments before, they have quite a significant burden of arthropathy with early joint replacements, sometimes in their twenties and thirties.
Sam: That’s Annette Von Drygalski, the director of the Center for Bleeding and Clotting Disorders at the University of California San Diego. One of Annette’s major research focuses is finding better, more accessible ways of detecting patient joint bleeds.
Annette Von Drygalski: Previously, 20 years ago, it was all based on patient perception, right? There was pain and the patient thought, okay, I'm probably having a bleed. I need another dose of clotting factor where that was sort of the go-to treatment. Now we have developed over the past 15 years, ultrasound and a rapid bleed protocol with ultrasound detection.
Deboki: The Center for Bleeding and Clotting Disorders has led that effort worldwide. And now that smaller, portable ultrasounds are available in clinics, it makes detecting bleeds simpler than ever.
Annette Von Drygalski: It's very accessible and very cost effective and cheap, relatively speaking to large ultrasound equipment, which is not needed to say, is there a bleed or not? Or is the pain associated with bleeding or not? Or is there something else that may need other diagnostic measures in a complete different treatment?
And there is now sort of the possibility to perhaps thinking with pilot studies, giving a little handheld ultrasound to patients, they might easily learn, especially with teleguidance. We had a pilot study to hold the transducer to see if there's bleeding or not. And maybe later on there can be something like artificial intelligence added that would give a diagnosis without even physician involvement. So that's on the horizon.
Deboki: Hemophilia research and advocacy has come a long, long way. But still, particularly globally, there is a long road ahead.
Randall Curtis: The rough estimate is that 75% of the people with hemophilia in the world get no treatment at all. And they have an average life expectancy of maybe 20. And so to address this, my colleagues at the World Federation of Hemophilia, which is the global organization, they have negotiated with the producers of clotting factor concentrates to donate some product to the WFH, and they have a humanitarian aid program that distributes this material to these lower middle income countries. And they are now up to billions of units of these products that they are sending out to developing countries.
Sam: A very hopeful note to end on. There is so much more we could say about hemophilia advocacy and ongoing research. We’re going to provide a bunch of great resources in the show notes if you’d like to learn more. OK, shall we Tiny Show and Tell?
Deboki: Yeah, let's do it.
Sam: Okay. So my Tiny Show and Tell would've been excellent around Valentine's Day. This is going to come out long after Valentine's Day, but that's okay. So actually my friend, who is a physician-scientist texted me last night and said, “so a lot of bacteria have undergone name changes and the bacteria that causes acne has been renamed Cutibacterium, which feels like a Tiny Matters episode.� And then said, "Too bad Valentine's Day is over." But I said, "No, no, no, I'm definitely going to talk about this." So I guess this bacterium, it's normal flora on the skin. So it can also, unfortunately, cause surgery site infections. In particular, my friend said, shoulder prosthetic joint infections.
Deboki: Interesting.
Sam: But most commonly it causes acne vulgaris. And so I guess it used to be called Propionibacterium acnes, but it was renamed Cutibacterium acnes, which A, is easier to pronounce. And B, sounds fun, but it's acne, so it's actually not. But the bacteria sounds like it'd be cute. I mean, I looked it up. It looks like a bacterium. I don't know.
Deboki: So adorable.
Sam: Yeah. So this change happened, I guess back in 2016, but she just texted me about it, but then she said, "So everyone's got some cuti on their skin."
Deboki: Aw, that is cute.
Sam: Yeah, it is kind of cute. So as long as it's not causing acne or an infection during surgery, Cutibacterium, I'm into it.
Deboki: I like rebranding acne so that when you get it, you're like, "No, I'm actually being really cute right now."
Sam: Yeah, I have a lot of cuti on my face. So thank you, Jen for writing in.
Deboki: Do we know why they rebranded it? Or not rebranded it, they renamed it.
Sam: They reclassified it, I guess, based on genomic evidence. So I guess there was better understanding of the species habitat, something about the peptidoglycan composition that you're seeing on the surface of these bacteria. And I guess that that led to it needing to be renamed from what it was. Why they chose Cutibacterium, I'm not entirely sure. So I guess it was really to differentiate it from the Propionibacterium species.
Deboki: Cool. That's really fun. I also just love that your friends are texting you, like "This should be your tiny show and tell."
Sam: Me too.
Deboki: If any of my friends are listening, you can also text me.
Sam: Deboki wants some texts.
Deboki: Well, thank you. I don't have something to pull out for my texts, but I do have one of my classic kind of, this is an article that I think y'all should be reading because it's just kind of a helpful read. This is an article by Ivan Oransky and Adam Marcus in The Atlantic titled The Scientific Literature Can't Save Us Now. The title is a little bit nihilistic, just a little bit, but I think it's summed up by its subheading, which is "You can cite peer reviewed research in support of almost any claim, no matter how absurd."
So I think for me, really what I took from this article is more about thinking about why we need to approach even scientific literature with a degree of critical reading skills. Ivan Oransky and Adam Marcus, for people who don't know, they're the co-founders of a great resource called Retraction Watch, which monitors journals for papers that have been retracted.
Sam: I didn't know those were the founders, but I am a fan of Retraction Watch.
Deboki: It's a great, great source for people who are less familiar with the publication process. Usually research goes through a whole peer review process. You send your results out to a journal, they will send it out to other scientists or experts in the field to review it and then decide whether or not it's going to be published. Sometimes unfortunately, things don't get cut in the peer review process, and so people will find it later on and papers will get retracted for that. And so Retraction Watch keeps really great track of that.
So yeah, given that background, you can tell these authors are really coming from a place of wanting to make sure the scientific literature is a place that is trustworthy. And so I think that's really underpinning this article, which is how we've actually gotten to a place where scientific publishing for a number of different reasons is maybe not this thing that we can all just be like, "Oh, yeah, I found this in a journal. So that means that this thing is proven. That means science says that this is doing this."
There are things on the academic side, there are things in the publication industry, and then just kind of the general state of the internet and all those things have come to this place where you can really find research that supports many contradictory arguments. And so I think it's just an important paper to read at this point in time. Again, without the mindset of being nihilistic or cynical about it, but just thinking about how you approach using scientific literature to understand the world.
Sam: Yeah, I love that. I mean, the majority of the science research out there is great and the peer review process a lot of times is really wonderful. The reason that we have medical advancements and other advancements in science is because there is a lot of really good legitimate research. But I definitely think we live in an environment, people call it a publish or perish environment, where if you're not publishing pretty much all the time, you are at risk of losing your job or losing funding or whatever it may be.
And so there is this really intense pressure, and sometimes that can lead to sloppier science than you should be doing because you're rushing and you're freaking out about getting something out. And not to say that that's acceptable either, but we definitely have created an environment that unfortunately this is one of the effects of that kind of environment. If someone wasn't expected to publish every year or more than that, they might take more time. They might say, "Actually, let's do that experiment again," or "That's not super compelling," or whatever it may be.
Deboki: And the existence of places like Retraction Watch are also a sign of a system that is working in some ways because it is about the fact that science is supposed to be constructed in a way that corrects these issues. But also, if our underlying foundation is pushing people to publish in this way, there is still something else that retractions alone can't fix.
Sam: Well, thank you, Deboki. That's really fascinating.
Deboki: Thanks for tuning in to this week’s episode of Tiny Matters, a podcast brought to you by the American Chemical й365betĹ and produced by Multitude. This week’s script was written by Sam, who is also our executive producer, and edited by me and by Michael David. It was fact-checked by Michelle Boucher. Our audio editor was Jeremy Barr. The Tiny Matters theme and episode sound design is by Michael Simonelli and the Charts & Leisure team.
Sam: Thanks so much to Ben Marcus, Robin Monin, Randall Curtis and Annette Von Drygalski for joining us. Go rate and review us wherever you listen, we super duper appreciate it. And we haven’t asked for that in a while so� help us out! We’ll see ya next time.
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