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Hope Charities
Honest conversations about bleeding disorders, rare diseases, and chronic illnesses.
Hope Charities
Empowering Women: Dr. Tamuella Singleton on Bleeding Disorders and Healthcare Equity
In this podcast episode interview, our host Jonathan James discusses the challenges and critical changes needed to improve the diagnosis and access to treatment for women with bleeding disorders with Dr. Tamuella Singleton.
In this conversation, we discuss important questions such as:
"What steps are being taken to improve equity and access to healthcare for women and underserved populations?",
"What role does genetic testing play in diagnosing and managing bleeding disorders?"
"How can patients and healthcare providers better advocate for comprehensive care and education?"
"What are your hopes for future research and legislative changes impacting people with rare bleeding disorders?"
#hemophilia
#bleedingdisorders
#vwd
#vonwillebranddisease
#womenbleedtoo
#podcast
#chornicillness
#rarediseases
Welcome to the Hope Podcast. My name is Jonathan James and it is my pleasure to be with you today to talk more about women with bleeding disorders on this episode, and we want to say a big thank you to our episode sponsor, Genentech, for providing support to us through Educational Grant to make these moments like this possible. So today I'm super excited to do a deep dive into really the history of where all we've came from but, more importantly, truly an expert in the field, Dr Tamuella Singleton.
Jonathan James:It is great to have you with me today.
Dr. Singelton:Thanks, so much for joining for the Hope Podcast. Yes, I'm super excited to be here. Yeah well.
Jonathan James:I just absolutely love every moment that I get to spend with you. You're just such a hero in my eyes. Thank you very much. I know so many people in the community just have so appreciated, not only from the patients you've treated we've heard a lot of that and about so much great feedback from that point but also just your leadership, because you've provided so much leadership for the community as a whole. But maybe somebody's listening right now and has no idea who, who you are.
Jonathan James:And so tell us a little bit about where you're from and then you're kind of you're journey about becoming a Hintologist.
Dr. Singelton:First of all I have to start by saying I feel the same right Like that um just kind of intense respect, admiration, you know, love for care for everyone in the community I feel. I feel the exact same you know so it's, it's.
Dr. Singelton:It's nice to to kind of have that relationship, I think as a as a physician. I don't know that I expected that it would quite be like that, but it's, it's. It's really um, a once in a lifetime kind of experience and journey, so so I'm so grateful. So with that, well, we are sitting here now in Louisiana, right and so, born and raised I am from Louisiana and usually when I travel, and especially when I start talking and I'm traveling, I get the head, you know, turn like where are you from?
Dr. Singelton:So, what are you, what are you from? So, um, and I usually say Louisiana and they go yeah, yeah, yeah. But I mean originally like where are your parents from? And I would go. Well, I'm actually from Louisiana for probably about 300 years, on both sides of my family you know, probably about 300 years. A few people came across on a boat some voluntarily, some involuntarily.
Dr. Singelton:So yeah, it's a little bit of a mix, but I'm originally from Baton Rouge. I was born and raised here, my parents too, born and raised here, and when I grew up in Baton Rouge we didn't have a hundred thousand people here and I can remember my dad saying you know how it was going to grow and what some of the plans were, and I'm like yeah, yeah, yeah, like whatever, yeah, but it was a much smaller place and I'm the daughter of two educators. My father was military in education, retired from the Army and was actually my elementary school principal, to get that together. So I was at home all of my formulative, very early years, before school, with my grandmother who was a teacher, and my mother was a kindergarten teacher in East Baton Rouge, parish for 40 plus years. Wow, parish for 40 plus years. So lots of education, right, kind of going on and opportunities to read.
Dr. Singelton:I was told I was reading. When I was three I went to McKinley Middle, magnet, baton Rouge High. Ultimately graduated from Lehigh School. I grew up kind of right down the street Stratford Place, concord, stratford Place, college Drive, perkins Row area.
Dr. Singelton:Lehigh school I grew up kind of right down the street stratford place, concord, stratford place wow um, college drive, perkins row area, yeah and um, I think being a physician was always in there. When I look back on it. Um, I was glued to the tv as a very young kid when, when mash was on yeah I didn't understand what they were saying, but I wanted, wanted to see the OR scenes.
Dr. Singelton:You know, I'm like yeah, I was like wait, what is what is happening here? So you know all of those kind of old movies. Um, I didn't grow up with any physicians, Um, and I certainly would never have imagined that I was going to be a hematologist, oncologist, that I was going to be a hematologist, oncologist. I don't think that I solidified yes, I'm going to go to medical school, but I was very driven, for some reason, to go to Xavier University in New Orleans.
Dr. Singelton:I was determined and very driven, and I think it was because this is sort of what I was supposed to do yeah. And that place is just a miraculous place For me. It was a place where it's a historical black college but Catholic. Oh, wow. And it was a place where and it was important to me, ultimately, where God was present. Come on, and I had an amazing education where my instructors, they knew who I was, you know, they knew me, yeah, yeah.
Dr. Singelton:So they had expectations. Yeah, the support there, the way we supported each other and every school has a personality and I think Xavier had a very academic-like personality. Yeah, at the same time, it was a very nurturing place and when my parents dropped me off, they loved the fact that we didn't have co-ed dorms, there was no visitation and there was a curfew and if I broke it they got a letter. You know it was very serious. So, anyway, I said all that to say I think my steps were kind of ordered. Yeah, I love that.
Jonathan James:I love that it's destined all the way from the beginning. Yep, yep. You were such a I know you to be such a science nerd in a million ways, and so I always love that about you. But you know, we've talked about this before about how you know you have such a great people connection, which is not always the case.
Jonathan James:Yeah, sometimes the science nerd and the people skills don't go hand in hand, but how did you at some point during your journey? How did you come into hematology specifically? Because, generally speaking, hematology and oncology both are very research-oriented and you find a lot of people that love to be in labs and you know, not with people. So how did that?
Dr. Singelton:I know part kind of happen yeah, well, I think at my core I'm a bit of a geek nerd. Yeah, um, love science, yeah, kind of thing. I think the people loving kind of person and mean that's my mother.
Dr. Singelton:Yeah, it is 200 my mother yeah um, everyone at the albersons down the street, they know miss chris and terry, everybody the whole store, like they know. You know exactly who she is. She talks to everybody in the whole store. I take her to a restaurant we go to par. Right now she's gonna stop and try to talk to everybody. You know, from the time we walk in I'm like, oh, my god, like come on, like so I think I got that from her.
Dr. Singelton:Yeah, but the hematologist thing is a little bit of a long. It's a little bit of a long story. I'll try to abbreviate it for you. Um, I knew I wanted to go to medical school. Um, especially once I got to Xavier that was really I was. You know, I knew that that was going to happen. I was a chemistry major. And I remember I told my, I called my dad and I said you know, I think I've really, truly committed that I'm going to go to med school. And he says, yeah, you're a chemistry major. You know chemists make great money, tammy. And I said I didn't tell you I wanted to be a chemist. I told you I'm going go to medical school, which I think he probably found kind of hard to conceptualize.
Dr. Singelton:You know, his baby girl, you know, going to med school, but I was very determined um. I had an opportunity to go to many schools across the country and um decided really honestly to stay at LSU because it was very inexpensive.
Jonathan James:In-state. It was in-state.
Dr. Singelton:I had little to no tuition to pay, wow. But when I tell you, the education I got at Louisiana State University School of Medicine in New Orleans and Charity Hospital is, in my opinion, second to none, tried and true tested. I was interested in research, though very early on and sort of long before I really knew about the hematology thing. So I was attracted to residency programs in Miami. I went for a summer program and I was determined I was going to train in Miami. I was like this is like New Orleans and Charity Hospital with palm trees, like I love this. This is great. Their medical center was phenomenal and so I knew I wanted to go there.
Dr. Singelton:So I had opportunities as a med student to apply for kind of research programs, research grants. So I did, and in doing that, in receiving those grants, I called up the director of the pediatrics program, which I thought I was really interested in pediatrics, and he just so happened to be a hematologist and he was treating. His area of interest was sickle cell disease at the time, and so I said, well, hey, his name is Charles Pegelow. I said, hey, dr Pegelow said well, hey, his name is Charles Pegelow. I said, hey, dr Pegelow, this is cold call. My name is Tammy Christentary and I'm a medical student in New Orleans. I have a grant. I would love to have an opportunity to work with you for the summer. Is it possible for me to meet you to come? Blah, blah, blah. He said absolutely. And I spent 12 weeks with him and as a result of that one, I really started to like hematology and two, my schedule was altered at LSU kind of because of that.
Dr. Singelton:So I sort of got the last pickings for something called a sub internship and as a sub I most people want to be on the floor with the younger babies. Um, the adolescent floor was last and the peds hematology, oncology floor. I was told no one wanted that and I, she said, um, the lady who was giving out the rotation, she said, well, no one wants that. That's just so sad, those little children suffering in their cute little ball heads. And I said that is terrible, you know. To myself I said why would you say that? Right right.
Dr. Singelton:In my head I'm saying this right. And I said you know what I want? That one.
Jonathan James:Because nobody else does. I was like I'm not this right and I said you know what I want that one because nobody else does.
Dr. Singelton:I was like I'm not afraid to have it and you think it's horrible, and I think it's probably not and I want that one wow and so I went. And to make this long story short, um, I did not expect to love it. I did not expect to fall in love with it, but I did. The residents I was with during that rotation, they hated it and I was so excited to be there. Every time the attending would ask questions on rounds, I'm like raise my hand.
Dr. Singelton:I know, I know, I know I can totally see you doing this and eventually she would go not you, but I loved everything about it. But what I found most inspiring about it, honestly, were the patients and the families. I love the science and how interesting it was and it was both hematology and oncology. It just seemed like in like the opportunities were endless. It was so interesting from, again, a scientific standpoint, but the people I mean it was just amazing.
Dr. Singelton:And so I was really upset one day about you know, some pair of shoes that got messed up and you know I spent my whole allowance on the shoes and I got really angry and I went to work that day, went to the hospital that day thinking about that and all I had in my mind I'm in a bad mood.
Dr. Singelton:You know, my favorite suede shoes got so messed up. I'm so upset about this. And I walked into a patient room. I opened the door Good morning and they turned and looked at me and it was a mom and a daughter and the child life people. They were in there painting her bald head green with yellow polka dots and they looked at me and beamed and just smiled with this, you know, the most pleasant welcoming. Oh, hey, dr Tammy, you know, come on in. And I looked at them and I went. I feel small. I'm so upset right about my shoes and this trivial thing. And this mom and daughter and the fight of their lives the fight of their lives, but yet they can manage to be positive and try to give something back to me that morning and I just said you know what?
Dr. Singelton:this is the space that I want to be in every day. I want to be surrounded by this kind of hope, by this kind of determination, this kind of love. I just I want to be in this space and from that day to this one I kind of never looked back. I wanted to be a pediatric hematologist, oncologist. I trained at the University of Miami for PEDS. I trained at Johns Hopkins and the National Cancer Institute for PEDS-HEMOC. I mean, the doors just kind of unfolded.
Dr. Singelton:Now the coagulation thing happened in a similar way. I was on maternity leave. I had two children in fellowship. I was probably the pregnant fellow and I don't want to put my program director on blast, but kind of right. So he said oh well, you know you're going to be on track, you know you're going to finish everything on time. You shouldn't have a problem. He said you know you have this four weeks that you needed to make up in the COAG lab, but you can do something else if you want to. You don't really have to do that. And I was like I said to myself I don't have to do that. What do you mean?
Dr. Singelton:that same response that I had with the lady right with that and I'm like you're telling me I don't have to do that, like that sounds insane. Of course I have to do that. I'm going to do my rotation and I insisted on doing my co-ed rotation and because of that, again the geek in me comes out and I had a ball, wow. So, um, my husband and I talked about potentially going home after fellowship and, um, I said, okay, well, let me call and see my old attendings at LSU. I'll call Tulane, I'll call Oxner and say, hey, I'm a fellow at Hopkins, I'm from new Orleans. Um, I think I'm coming home. Yeah, so, as I'm training, are there any needs in the state in the city? Like, where are the holes? Could I possibly focus on something here? Right to serve a purpose?
Dr. Singelton:and bringing it back home yeah, and the first thing all of them said was COAG. What Hemostasis, thrombosis, coag? And I said, how about that? I said, well, that's not a problem, because I newly discovered that I liked it. So, I tried to do everything I can to go to the hemophilia clinic. Like when I could.
Dr. Singelton:To spend more time in the COAG lab, and one thing led to another and I can tell you other stories about Katrina and how, you know, I met Cindy Lessinger and she took me under her wing, as I mean from the moment I met her, dr Lessinger.
Jonathan James:She's wonderful.
Dr. Singelton:Oh my God, she became, you know, my mentor and friend and didn't hesitate. And then, you know, I learned from Karen hesitate. And then, you know, I learned from karen wolf. And you know that entire team and, sue my gosh, like those guys were amazing. Yeah and um, all of the patients that I've had over the years at tulane, that's who really taught me right, all of the patients, the complicated patients, patients coming from Alabama and Mississippi and Louisiana, and although I was training Peds, I saw adult patients and I saw kids. Now we're talking about women today and what I can say is I've had an opportunity in that journey to see the transition but to also recognize, like, where we got it wrong and how we're starting to get it right. And I never talked about women or girls. We never thought about a woman or a girl, which seems crazy to me now. Now right.
Dr. Singelton:I mean, it wasn't a thought.
Jonathan James:Yeah, because traditionally it was always contemplated. Even in the literature it was always contemplated.
Dr. Singelton:It was predominantly a male disease, right disease, right but what's so interesting is um, for those of us who are scientists, right, we do understand the concept of lionization. It is a thing, wow. So if you have two x chromosomes, right one of them may not make up for what's absent on the other, right right.
Speaker 5:It's not strong enough in other words, you could be affected.
Dr. Singelton:Right, exactly, right. So just because you have an X chromosome doesn't mean that you cannot and will not be affected by the X-linked disorder. Right, right Again. These are scientific.
Speaker 2:That seems so obvious now, but these are scientific concepts that we did not apply.
Jonathan James:Interesting.
Dr. Singelton:Right Directly to women and I guess, in all fairness, as we learn more about DNA, yeah, and learn more about genetics, right, this, it became a thing. But when I think back, we just never. It was never a thing. We never talked about it. We didn't treat them.
Jonathan James:Right. Well, and I have to say too like I mean it presents differently for women as it does men, but in essence, the root cause, or the root problems are, are really still there, like either way. And so the fact that we're unfolding some of this more and and science is catching up, I don't know if maybe science, the science is always there, but it's like we're starting to realize, oh my gosh, like there are people out there that could benefit, and uh, there's a whole lot of things that I want to unpack in that I feel like we have a lot to cover there.
Dr. Singelton:Yeah, we have a lot to talk about it.
Jonathan James:But one of the things that I think is a driver too, is understanding. And it's not just this and I'm learning a lot in this because I don't come from a science background, but, having seen the crippling effects that I experienced as a child, you generally don't see women having crippling effects at five years old. There are some that have severe hemophilia. We've met a few in the community but but generally and they do have crippling effects early on but but.
Jonathan James:But that doesn't mean that it's not present. And I think you know we always kind of and I know with a lot of the patients I talk to, it's like we always wish we kind of had one of those like diabetic, you know things that could tell you what your factor level is all the time.
Jonathan James:But you know that doesn't exist and so it's kind of this idea of like, how do we manage that? And so questions that I don't think I've ever thought, or most of the people that I know have not thought about. That we're starting to think about now is can factor levels change? Do they go up, Do they go down? Does that present differently? For women as it does for men.
Jonathan James:You know there's, there's so many.
Jonathan James:What's really neat is to see to see this being embraced both from and it takes kind of both right. I mean, the patients need to ask the right questions in some ways to move the needle along so that you know those who are making the decisions also can respond to those questions. And then and then simultaneously, there's this aspect of okay, you know we've got to be educated on both sides, but it's. But there's another measure of it. It's like okay, well, if, if there are problems maybe with longer than normal administration cycles I can't tell you how many women I've talked to that it's common for them to go two weeks and they just thought that was their normal, so just getting educated to the point and go oh, this could be connected because your son or maybe some relative had hemophilia. There's a reason why you're going two weeks instead of just eight days or whatever, and so I just think that it's almost like the learnings and the understanding has kind of been there, but we didn't see the forest from the trees For a long time, would you agree?
Dr. Singelton:with that. Well, yeah, and let's unpack that for a minute.
Jonathan James:Yeah.
Dr. Singelton:Right and think about that.
Speaker 5:So when you're managing a crisis, yeah Right, totally different set of rules when you're managing a crisis.
Dr. Singelton:Yeah, your concentration is on the crisis. That's right, absolutely. So if a house is burning, you're concentrating of anyone who's standing around the house. Right right. Even if the people around the house are also suffering or maybe even burned. You're trying to save the lives of the people who are in imminent danger. Right, right, right, right, right right. Right, right right right, right.
Dr. Singelton:So I think that to kind of maybe give all of us a break the physicians, physician scientists, the community, you know everyone that for a long time, in hemophilia care in particular, which is the center of bleeding disorders right, because the bleeding was so severe. Right, which is the center of bleeding disorders, right, because the bleeding was so severe. So if we almost have a bullseye and we're thinking about bleeding disorders, hemophilia would have been at the center, with the number of patients, the severity of what was happening, right, and the center of the crisis, right. So you focus on the crisis, which is the most severe patients first, right? So even though someone who had 5% circulating factor VIII or factor IX may have been suffering, it may have been pale in comparison to someone who had none. Right right, right right right, that makes sense.
Dr. Singelton:So, with few to little treatment options too, the hustle and the struggle is real, right, because you're focusing on managing the crisis, right.
Jonathan James:And we had two of those. It wasn't just the fact that we identify. Oh, there's a protein deficiency. This is a genetic mutation we're going to get more protein. It was the one-two punch, it was HIV.
Speaker 2:That's right and and honestly I mean, yeah, late 80s, 90s, hiv, but I mean all the way up into what?
Jonathan James:1995. Yeah, hepatitis c is still pervasive, so so it's almost like we had a one three punch where there was like you're dealing with multiple crises that are happening within a generation.
Dr. Singelton:That's correct.
Jonathan James:That's correct, and so it's all focused on how do we stop the bleeding?
Dr. Singelton:Pun intended Correct, yeah, it was focusing on that crisis. So I think, as therapies have evolved right as treatment options for HIV, have evolved right. We put the fire out Right, and so when you put the fire out now you can turn around and start kind of surveying the damage.
Jonathan James:Who else is in trouble, yeah?
Dr. Singelton:The relatives that may also be in trouble. Right. What happened to the neighbor's house? That didn't quite burn, but there's still an issue there, but they don't have electricity. They don't have electricity. Right, correct, correct, correct, right. They don't have water, they don't have electricity.
Jonathan James:In hurricane land.
Dr. Singelton:I have a lot of connections to this analogy yeah, yeah, right, there's the window. There are no windows. Right. Yeah, they can't stay in the house, right. So With the improvement of therapies and I can certainly say, as a provider, I certainly have more time to reflect on and to take a look at just speaking of me independently my patients that have moderate mild hemophilia and other bleeding disorders Because I spend a lot of time managing people and families of people living with hemophilia, especially with inhibitors.
Dr. Singelton:I mean, not only did I have a lot of severe patients that were struggling and we were struggling to help to manage, like constant right Trying to manage that Patients who were living with inhibitors, it was daily, many times during the day there was a lot of intensity there. So once therapy's improved, then you really again kind of have the time to look around.
Dr. Singelton:And collectively, we had the time to look around, and collectively, we had the time to look around, and, collectively, we had the time to look around, and collectively, we had the time to ask additional questions and to not only look at men living with factor levels that are in that moderate to mild range. You now also cannot ignore the women who fall into that same category right, right and for every severe male.
Jonathan James:I mean, I know what the statistics are, I think, macek, just the women who fall into that same category, right, right and for every severe male. I mean, I know what the statistics are I think Maysak just put out a note about this too that there should be like 50% of you know could potentially have the females in the same bloodline could be, affected, but I mean between mom, daughter, granddaughter, cousin. I mean you could easily have three females for every severe male, oh, absolutely. That are surrounding that person that just don't even know.
Dr. Singelton:Absolutely.
Jonathan James:And dealing with. Maybe it's not just extended menstruation cycles, but maybe it's also like having trouble in childbearing. Maybe it's having difficulty with joint, you know, microbleeds, all kinds of things. We've seen so many women that had like joint damage at age 35 and you go, oh, you probably just have some arthritis why would you have arthritis at 35?
Dr. Singelton:years old and you're an otherwise healthy female who doesn't have autoimmune disease, like well, why would you have that? So there's so many things and, thank goodness, right. The therapies evolved and allowed us to have an opportunity now to ask these questions. But it's linked to other things, right? So you brought up childbearing right and childbirth, so all by itself, that's associated with significant morbidity and mortality.
Jonathan James:Right for women right right and so it's shocking, it still is I look at the numbers of that, sometimes, like on the world health organization puts out it's pretty, it's it is unbelievable how many people still are impacted by that and it's actually um, for for women of color and women of kind of marginalized groups from a socioeconomic status.
Dr. Singelton:yeah, apparently it's gotten much worse. Actually, believe it or not? Oh my gosh, but when you have something like that, that already exists is the problem. Right, right, then women with bleeding disorders would kind of get lumped in and hidden within that group for the larger medical community where you're like, yeah, that's a problem. Yeah. But within that problem are hiding women who have that problem because they have a bleeding disorder. Right.
Dr. Singelton:But it has become almost like an accepted norm and accepted fact. Yes, that exists. In medicine, they would say anemia is the fifth vital sign, the vital sign that we really don't pay attention to, the vital sign that we should pay attention to because it can have such a significant impact systemically. So when you're anemic, your heart works over time. Wow. So that puts you at risk from a cardiovascular standpoint If you have decreased oxygen carrying capacity. Well, what is that going to do to your brain? Right, what is that going to do for you from a memory perspective? Are you going to be able to function optimally if you're walking around chronically anemic? But again, that's one of those things that, in medicine, for us, has been accepted for so many women, because, oh, women have heavy periods and oh yeah, women can be anemic and oh yeah, just take iron. Just take this birth control pill with iron.
Jonathan James:Just, it has been an accepted we just have to give some extra. Women have to have hysterectomies because they just bleed Right. We just do. That's just what we have to do. It's just a part of what we do. Right.
Dr. Singelton:So again it was kind of lumped into things that were accepted. Right.
Dr. Singelton:So even now, with the advancements it's hard to kind of tease that out of what has become like an accepted norm In addition to you know this about me, but all the folks listening probably maybe don't know about me. Know this about me. I'll just speak my version of the truth, even if it doesn't sound right, girl you got to say it. I'll just speak my version of the truth. That's what I love about you yeah, coagulation is not one of those things in medical school that most people like.
Speaker 5:Yeah, well, probably because of all those people telling you nobody's gonna like it first. I'm sure that has something to do with it.
Dr. Singelton:We gotta go right, we gotta go train these people that are like managing the fellowship I think that's where we start most people don't, most people don't like it, like you're not you're not gonna like this, yeah, you spend only the time that you have to spend, you learn it and you move on. Yeah, right.
Dr. Singelton:It's a blip on the radar for most doctors. You know a little something about bleeding, yeah, but you don't have that intensive training. Even most hematologists, oncologists, do they know about it? Sure, did they know enough about it to pass the board exam? Sure, could they treat a patient with it? Sure, because they're qualified to do so. But they don't necessarily have, like, the experience or the interest right, right, right or the repetition.
Dr. Singelton:They're not a part of that community you know right right, so it's a little different so coagulation for most doctors is not something they like, it's not something they want to do and again, even my hemat colleagues they would prefer that you do it. You know you doing that coag thing Great. Can you see these patients for me? Can you handle that for me? So, because it's something that has kind of been pushed out and marginalized.
Jonathan James:Even within the practice of medicine, Even within the medical community.
Dr. Singelton:it becomes a little bit of a problem now when you have to interface with physicians for care.
Jonathan James:Do they treat and I'm just asking because I don't know but do you feel sometimes like other physicians treat you as like a second class citizen, almost as a physician, where they're just going well, that's your thing, We'll just kind of give it to you and we're going to move on? Do you feel that?
Dr. Singelton:It kind of runs the spectrum. So there are some physicians especially like some surgeons, like when they're doing procedures who are like oh, you're the coag doc, you're the hemophilia doc, what do I need to do and where are you going to be and are you going to help me do this? Are you going to tell me what I need to do? Are you going to take care of such and such Like? They ask a lot of questions and they want the participation. They don't want anything to go wrong. They're open to whatever you need to do. At Tulane we would stand in the OR Most of the time it was the nurses. If I was available occasionally, it would be me, right. But then the other side of the spectrum is I don't need you to tell me anything. I know what I'm doing. I've operated on patients who have this before. I've seen this before. You don't need to tell me how to do this. So there's a spectrum. Yeah.
Dr. Singelton:Right, right. And then there are the people who fall in the middle, and so there are so many variables and factors right there we're facing when we're talking about people who are living with mild to moderate hemophilia, who still bleed significantly, who are living with mild to moderate hemophilia, who still bleed significantly. There's a risk of life or limb, depending on the situation, and those people include women. They also include, which you're not even going to touch necessarily today, people who are living with other rare bleeding disorders they're.
Dr. Singelton:They're a part of this right so there's so many factors and variables that contribute. So you threw out a question earlier about factor levels and do they change and can they fluctuate with women? And the answer is yes, because in people who actually produce factor eight or factor nine, that can change with stress or time or age, because it's what we call an acute phase reactant. So depending on when you're testing it, depending on where that person falls, it may be higher one time, lower another time. So there is some variability. Some of them change with age, but will it ever be completely normal? Quote unquote normal no, I don't believe that part. And then there's that whole question of and this is tough and even for someone, I think, who's very seasoned in terms of my experience with people bleeding disorder patients when I have a patient and a factor level is drawn and is documented as normal, I absolutely understand why there would be a challenge for some people. So even right now there are several women I'm thinking about who I know they have. They carry the gene. The factor levels are normal.
Dr. Singelton:What do I do with that? I don't need to treat them to prevent anything. If they're not symptomatic, I don't need to address it. There may be conditions and times, depending on what it is, that I do have to address it. We would probably have to have an ongoing plan, like, depending on what it is. But I empathize and sympathize with the provider who doesn't have the experience to kind of ask those questions and may just check off the box and say this woman's factor levels are normal, I don't need to do anything. I, on the other hand, may have a conversation where I'm looking at, let's say, if there's a surgeon involved and I'm like, listen, she doesn't have a very strong history of bleeding, but there has been some, especially from a menstrual standpoint, and her factor levels fall within the normal range. But I don't necessarily want that to be a false sense of security. So we have to have, I think, a crisis management plan.
Speaker 5:That's good. Yeah, so we have to have, I think, a crisis management plan.
Dr. Singelton:That's good yeah. But we would have to be sort of in agreement, like with this and explaining if you have unexpected bleeding in addition to what you would normally do. Here's plan A, plan B and plan C.
Jonathan James:And for the patients that are involved. I think about so many guys that they, and for the you know patients that are involved. I think about so many guys that they've. You know it again. Their mom, or maybe it's their daughter now may not really see it the same. Cause it again. You're, you're putting that fire out of the house trying to all the time, but for the men.
Jonathan James:But I think it's important that we give the people the language to even ask the questions, because how does that conversation even come up? I mean, I think a lot of treatment centers now are starting to look at hey, let's just go and do blood work for the family. So we understand where some of them, but not all are, and a lot of that's driven, obviously, by payers and insurance and all that kind of stuff.
Jonathan James:But but I mean, I do think, like at some point, if the mom, if the daughter is coming and saying, hey, the, the, maybe they don't understand why, but they're saying, hey, these are the things that I'm honestly having trouble with. Or I had a tooth extracted and it just bled and bled and bled and bled Not just, I think, within our own family context. Sometimes we can just push those things aside and say, oh yeah, you know, it's probably, it's probably like a little, you know.
Jonathan James:But if we bring that to you, and we say hey, how do you feel about then? Then you know that that I mean, it is really. That's the impetus that starts the conversation so that you can create a crisis plan because you can follow that person, that child, maybe that mom, maybe whoever for many, they're probably going to have lots of medical things that happen throughout their life and having that plan develop, and so I think it's imperative that we as a patient population and community also formulize this understanding like this could be a thing, and we probably should at least just know like uh.
Jonathan James:I remember um recently my wife went to go get some uh blood work done or whatever and the or as a uh ekg they were doing on her heart and I said we just need a baseline to where you're normal, like, like, just where you're regular and not in a crisis, so that later on in life we just can come back and go. Okay, this is what this is what it was, and now, five years later, this is what we know it to be now that's right and I think.
Jonathan James:But that all starts with at some level, like if my wife didn't go to the doctor at all to just get a normal checkup. That conversation probably would never have happened and there would be no baseline to measure it against right. So I think at some level, like we need as a patient community, we also need to be asking the right questions, because it's important that we do get a crisis plan because if you're in a car accident or something else, that could be the you can't diagnose it.
Jonathan James:I mean you shouldn't be trying to diagnose in the middle of a total disaster. Trauma like that, Right?
Dr. Singelton:So I think you know we could probably take that back a little bit and, um, you know, let's say what are the, what are the, what are the tangible things, right, that can be helpful, that we can kind of concentrate on. And you know, let's start with, if we're talking to people kind of within the bleeding disorders community, right, someone you love know related to right, has a bleeding disorder. I'm a mom, I have two sons you know with with hemophilia. I'm a mom, I have two sons you know with hemophilia.
Dr. Singelton:You have to understand that, even if no one has ever asked you that it is possible that you are at risk from a bleeding standpoint, you have to first understand that it is absolutely possible. So, because it's possible, there are two additional steps that you need to take, starting off with yes, it's possible Recognizing that you probably should be tested to determine where you fall. But then it can't end there. You have to recognize that maybe, in spite of or no matter what happened with that test, even if you don't have genetic testing, do you bleed and what does it mean to bleed? So there are so many women and just people in general who don't understand, like I don't know what that means. What do?
Dr. Singelton:you mean bleed. So do you mean when I get up and brush my teeth every day and you know I'm spitting out blood the whole time I'm brushing my teeth that that's not okay? Not okay, right? If I have a nosebleed every morning before I go to work, you know? Or in the middle of work, right? You mean like, well, that's not, that's not really okay. Or, you know, when I was 12 years old and I started having periods, I couldn't go to school for a week because I had to wear pads, tampons and a diaper, or you know, there are so many. So first, I think we have to recognize that, yes, it's possible that you could have a significant bleeding disorder, and then, too, it's important that you understand well what is bleeding for me as a female.
Dr. Singelton:So not only do providers have to be educated about that, but the community, patients, women we have to understand. What does it mean to bleed? The third thing would probably be the third thing would probably be, sadly that there are not always going to be my colleagues or other providers who will understand how to help you. Sure, it's not that they don't want to help, it's not that they are doing something deliberate. I think it's just so many people don't know they don't know how.
Dr. Singelton:So, first, understanding that it's possible that you could have a bleeding disorder. Second, understanding it's important for you to learn or know, or understand what is bleeding for me For me, right, you know if I have frequent ankle sprains Right.
Dr. Singelton:Right, right, am I really having a joint that's being injured and now I'm at risk and I need to pursue that and push that and then finding someone who can help you? You and I'm going to quote my husband real quick and say we were talking about doctors and, um, sometimes, the limitations that you face as a patient if there's something your doctor doesn't know. Some of them are smart, some of us are smart enough to say, well, you know, I don't know that, but I'm going to explore it and sometimes not, and my husband said well, so I'm just at the mercy of that because I don't know, and that's right.
Dr. Singelton:So this is where I think organizations like yours and when communities come together that you can talk about what some of the barriers are, what some of the challenges are and maybe how you could overcome some of them. You could address the education.
Jonathan James:Language right To think about it, to chew on it, to say what are the commonalities we're experiencing? I've been amazed the more that I've learned about you know just modern medicine in general. I've really been amazed at how much anecdotal you know things are experienced, things that we go through that are what really push medicine along. I think in this modern age we think, well, everything's known and you know we just everything's got a lab and everything's got a test result, and you know we just go to some specialists and they tell us what to do and then we just follow those instructions.
Jonathan James:But, truly there's so much that's still not known. I mean, I think about what you know and a lot of it's just because we're continuing to grow and understand. It's the forest from the trees thing. Sometimes we were putting the fire out in the house and then we get to assess okay, wait a minute, this person has a burn that could lead to an infection, that could lead to really, really traumatic you know amputation because they didn't get it treated properly or within time, so it could be eventually severe to be down the road, and I feel like that even within the bleeding spore space. I've been talking about this a lot. You just brought it to mind while you're talking about defining what a bleed is for you. You've probably heard me talk about this, but I talk a lot about we have leaned so much on this concept of abr or annual bleed rate, but if I ask 10 severe guys that are, like you know, live they've lived with fully metastasized bleeds that are just extreme if I ask 10 guys what a bleed is, I'm gonna get 10 different answers.
Jonathan James:Yeah, still Now, some of that is because the advancement of medicine has grown so much and there's so much good treatment out there, that's really doing a great job at managing all that preventatively. But at the same time, I hear guys I mean literally it's surprising to me. Sometimes we'll be in a room and there's 30 people in there. They're all affected and you go okay, describe to me what a bleed is, and it'll be crickets for a little while, cause everybody's on treatment of some kind, which is wonderful. That's progress. It's amazing progress compared to what it was when I was eight, nine, 10, 11, well, even 20 years old you know, there was a.
Jonathan James:there was a different day where that was not the case and we all could tell you I can tell you, I can't walk.
Jonathan James:I can't you know whatever, but but at the same time, like knowing what that is for you is so critical even now, even for, even for the extreme end of the spectrum. But how much more so is that the problem in moderate or mild hemophilia, where men, women, whatever it still could be a problem in a lot of different ways? I've said for a long time that I think in mild hemophilia it's probably like the Wild West right now it's the most dangerous place to be because you could be in a car accident. We just had an amazing meeting with a lot of legislators recently in Washington DC where we were meeting with one of our friends. That was a patient who had been infected. Her dad passed away from a completely solvable problem because he had moderate hemophilia he didn't have factor with it instead of a wristband.
Jonathan James:He actually had a thing in his wallet and he was in a car accident, taken to the emergency room they call the family and by the time they went through his wallet he had passed away from a brain bleed. Yeah, that kind of thing is still going on every day and most often cause of death is not listed as hemophilia.
Dr. Singelton:No.
Jonathan James:Cause of death was head trauma. Brain bleed so even in the records it's not being truly unpacked. Does that ever get down to the school level where we're? Training physicians for the next generation?
Jonathan James:Probably not school level where we're training physicians for the next generation? Probably not. And and and if we as patients don't have, if we're not able to raise a flag, I always say you know, there's a fine line between advocating and being adversarial. And sometimes I think a lot of people there, when they don't feel like they're getting answers, they go to the physician appointment or the clinic visit and they just go in with their Dukes up and they're like defensive and all this stuff. And I, just as I've gotten to know you more and so many other physician friends that we all know and love, it's like I've just grown to realize man, you guys are human just as much as I'm human and I can't go into this being like I have an opinion and you're trying to be a gatekeeper. I got to go into this like we're a team and how do we work together?
Jonathan James:and let me bring up everything that I'm thoughtful of and then you can just help me pick and choose which one is better or worse, we had a situation with a female that had had a really borderline factor level borderline, and it was not a black and white thing and found out through ultrasound that there was joint damage as a teenager and through that process got diagnosed on preventative treatment. Total life change. I mean total life change. Gave her her life back and she didn't even know she was struggling. She had soreness every day. Didn't realize all this stuff was going on.
Jonathan James:And even for my own. I mean here I am preaching on the stage and whatever doing my rah, rah, rah and I had my clinic visit with my physician recently, at the beginning of this year. We sat down and looked at it and went through the things and said we can do better. Let's go to this. Let's correct to 100%, let's not just correct to 60, 70%.
Jonathan James:I thought I was a totally new person. And look, I, I kind of know a few questions to ask. I'm not the most expert, but I kind of know a little bit about what I'm talking about. And so I. You would think I would have thought to ask that question but, I, didn't think to ask that question. I didn't think to push the envelope because that wasn't my. Again it goes. I love the the house burning down. Analogy um, because it wasn't the biggest problem in my life.
Jonathan James:But, by going through hey, here's the list of challenges that I'm experiencing and then collaborating with that as a team has been. I know it's been life-changing for me. I know it's been life-changing for many other patients and sometimes providers aren't always the most empathetic. But at the same time I've've found at least in hematology seems to be there's probably more that have a lot more empathy than there are maybe and yeah, I had an orthopedic surgeon one time.
Jonathan James:That was like military, like no expression and and you know it was like I walked out of there thinking that guy, I'm not going to see that guy yeah yeah, but he saved my life, yeah, and I didn't know that I needed him. So, sometimes they're the most you know, but it seems like a lot of our treatment centers, at least a lot of hematologists in this space, are really, you know, more empathetic.
Jonathan James:But I think we got to give grace to each other and we got to give grace to level we're learning, but I think it starts with language it's, and then it starts with just investigating and realize that really, if we're experiencing this, the onus is on us, it's, it's really on us to to, to really start to explore and discover and and process with and especially if you have someone in your family.
Jonathan James:You know, I think this, this phrase, one that is keeps coming back to mind and I feel like has been very effective in, in, in a lot of circles, and that is keeps coming back to mind and I feel like has been very effective, and in a lot of circles, and that is bleeding disorders runs in my family. I think we should investigate this and just by saying that all of a sudden, it seems to unlock a switch whether it's a PCP or whatever.
Jonathan James:Whatever that is OBGYN like, whatever that is. I have a history of this in my family. I think I need to look into this yeah and that has been a great way to sort of unlock conversations and see an investigation.
Dr. Singelton:Yeah, it's just so complex, right? Because at the end of the day too, it's about feeling confident in a space. Yeah.
Dr. Singelton:And it's also about having a relationship with someone, and so a lot of those things are much easier for us who have a relationship with a provider Like you, of communicate, you know, with that provider, and if you're also fortunate enough to have a provider who is inquisitive enough or knowledgeable enough or confident enough to or a science nerd enough, or a science nerd enough. Or a science nerd enough, yeah, yeah, to really push the envelope and ask the additional questions.
Dr. Singelton:Yeah, um that can be, that can be tough to kind of, you know, put together, and it's also another piece to this too, like if you're not getting the answers, you're not getting to the solutions. Don't stop. That's good. Don't stop. Go back to that provider again. Right, you know, give them the opportunity. Hey, listen, I'm really concerned about this, and is the workup that we've done here like really enough? Can you help me to really explore and find out, like, is it, is it really enough because something's not right?
Dr. Singelton:mm-hmm and can I follow up, you know, in a shorter time frame because something isn't right now. That's easy to say, the way I said it, when I don't have a crisis going on. And so even recently for me, my daughter was in a car accident and inside I was almost screaming at these people that no, under these circumstances, you need to do a CT.
Dr. Singelton:I was almost screaming but I was with it enough to control myself and not let the inside screaming come to the outside, because then people match energy and it becomes about like my behavior and we can't have a conversation. So I tried to as calmly as possible and swallowed real hard right and said so there's, there was a significant car accident. The car is decimated. Here's a picture of the car. She can't tell me all the airbags deployed. She can't really even tell me exactly what happened.
Speaker 4:You can't really even tell me exactly what happened. Under what conditions would you want to look?
Dr. Singelton:at someone's brain when there's been that kind of trauma. If something happened and it wasn't witnessed, you know, or if the person who was engaged in the accident again can't really tell you anything, under what conditions would you investigate? So do you think that this would call for like a CT to be done? I had to very try to, very calmly, you know, pull that together and ultimately the answer was yes. But had the answer been no, my response would have been I don't really feel comfortable with that. Can we discuss this a little further, and is there anything that you can do to kind of, you know, to really explore that?
Jonathan James:yeah, I don't feel, I don't feel comfortable with that I want to peel that back a little further too, because I think there's a deeper thing here that it's crazy, unfortunate and it's a complete reality that insurance, unfortunately, I had a bad week with insurance.
Dr. Singelton:Yeah, I had a bad week with insurance. Sorry, I had a bad week with insurance. I don't mean to create PTSD or anything. My trauma triggers, I'm sorry, I don't mean to trigger.
Jonathan James:Yeah, but we got to also understand that sometimes the barriers to doing more investigation in moments like that, whether it's trauma or whether it's, you know, whatever, labs, whatever that there's a barrier in the sense that and again I'm asking cause I don't. I haven't sat in your shoes or in your seat.
Jonathan James:So I don't know, but I'm just curious. Like when somebody asks you a question, are you going through the checklist in the back of your mind, like basically to answer the question can I, is insurance going to even allow me to do these tests? Because at some level it's not just about what the patient wants. At some level it's like we could want all the things in the world. We could even be right about it.
Jonathan James:And our suspicions could be absolutely accurate. But if you can't get it funded, if you can't get it paid for, then none of us really have a.
Dr. Singelton:you know, I call it the silent provider in the room.
Jonathan James:Yeah.
Dr. Singelton:Right paid for, then none of us really have a. You know, I call it the silent provider in the room. Yeah, right and so yeah there's a silent provider, yeah, right in the room, and for the most part, I'm. Most of us are pretty well versed with what our limitations are in terms of like insurance, like what can?
Dr. Singelton:we do, what can we not do? Right, um, I know what I can do in an emergency. I know what I need to describe adequately. Right, if a patient has something, I, over the years, I know how to make sure that I'm explaining myself so that they have enough information. Right, right, that they need to authorize, right, right, the test. To authorize the test. Sometimes, even with me going through all of those steps, is still not authorized. And then it requires me to have what's called a peer-to-peer, and now I'm having a conversation. Those things are barriers right.
Dr. Singelton:Some doctors don't get to do them. Sometimes they're very hard to set up. The barriers are just there.
Jonathan James:So is that part of the checklist you're going through when somebody brings to you like, hey, I think I need?
Dr. Singelton:a chromogenic test.
Jonathan James:It can be, and I'm a female and I don't have like a confident history, but I really believe.
Dr. Singelton:Yeah, no, it can be, but it becomes. For me it becomes a part of the conversation. Right no-transcript, this genetic testing. So then, what do we do, right, I? I have a choice. Either you're going to pay for it at, you know, a thousand dollars a pop, $2,000 a pop, or I'm going to say we can't afford to get that. I'm going to explain why, but it's going to be a conversation and shared decision making between me and the patient. Now that's me.
Dr. Singelton:Again that's with my level, experience and the kind of provider I am. You may not have the same conversation right with another provider.
Jonathan James:Right. But I found, sometimes like from the patient seat, sometimes like just going and saying, like that surface level answer, if you feel like that's what you did with your daughter's accident, just asking more questions is a great way to sort of get, maybe to take them out of the instant no or the instant yes, probably not going to get.
Dr. Singelton:And you're not challenging them, you're not putting the person on the defense. I love.
Jonathan James:What you said is that, like, when you give a certain energy, they're going to match that.
Dr. Singelton:They're going to match that. People match energy, I match energy. Yeah, people match energy.
Jonathan James:But by asking questions and staying inquisitive and saying but, if this were the but, if this was your daughter, what would you? And you saw these symptoms. Would there be anything else? That we should be considering. I mean, I've gotten to the point now where I like asking this. This has worked well for me recently. It's just not maybe in this exact setting, but in a lot of settings you're going okay. Is there any other questions? I didn't bring up that I should be asking.
Jonathan James:Because sometimes I found, I mean, you know, some of these people are really, I mean, way smarter than I am. And, and it's like when I asked that question, it gets there, you know their lab brain go on and they go well. I wish you'd asked me this question you know or? Whatever, they start thinking of it and they start feeding you the very questions you can ask. So getting them to the point of like let's collaborate, can be a little bit of a skill sometimes.
Dr. Singelton:but it is so critical. Right, it is critical, but again, it's one of those barriers when you're not the person who knows how to do that. I'll give you another kind of personal example. My mom is older. She fell, she broke her wrist. It was not witnessed. Right. She has dementia. She fell. It was not witnessed. Okay.
Dr. Singelton:We don't know what was associated with the fall. We don't know if she was having a heart or cardiac event that made her fall. We don't know if she tripped Like we did't witnessed right, right, all we have. We're just looking at an elderly woman who has two broken wrists as a result of a fall. So she's at the hospital with my brother. My brother's an educated guy. The doctors did whatever they did and they came and said well, your mom has two broken wrists, we're gonna do such and such and such and such, and you know, blah, blah, blah, blah, blah. I hear your instructions. And what does my brother say? He goes, okay, and what are we supposed to do? And okay, and blah, blah, blah.
Dr. Singelton:And okay, and you're going to discharge us, and okay, two seconds later I roll up. Right, I'm coming in hot from New.
Dr. Singelton:Orleans. I roll up and I go what's happening? Well, they're about to discharge mom. Ok, and what did they do? Well, they did some x-rays and and they said she has two broken wrists. And I said and that's all they did was some x-rays. And they said yeah, and I said OK, so the nurse comes and I said I didn't introduce myself as a doctor, I tried to. You know, again, I'm not calm inside, but I tried to remain calm and I said well, sir, you're her nurse. He said yes, and I said she's being discharged. I said well, I do have some questions for the physician who's caring for her. So if you could let him know, we'll wait when he has time, but I don't think that she should be discharged right now and I have some additional questions. So can you just please point out to him, just in case it wasn't pointed out when he's on his way over or her way over, that this is an 80 year old female With end stage dementia who had a fall that was not witnessed, and we'll wait.
Dr. Singelton:And so when he went back to, maybe they didn't necessarily have right All of those facts like maybe you know it's busy in the ER, just an 80 year old who fell and they didn't really get to the bottom of blah, blah, blah like whatever it was. But when I said I'm sorry, we do have some additional questions and here's some additional facts all of a sudden she had an ekg, a chest x-ray, a head ct, because those were the appropriate things, right, right to do. But there's nothing wrong with my brother he just didn't know. He just didn't know to ask those types of questions.
Dr. Singelton:And so that's where I think we're getting to the sort of meat of this, for women and girls with bleeding disorders.
Dr. Singelton:It's not one answer. Are you expected to always have all the answers for yourself? Absolutely not. But it does help if we could try to empower the community with even what you are capable of doing. So, even if it's only to start by saying I have a family history of a bleeding disorder, and so that means that I might have one too, and I don't really know what it means to bleed, let's just even even if we're starting there right.
Dr. Singelton:The other complexities of knowing how to talk to a provider or knowing what provider to go to. Well, maybe that's when, again, amazing organizations like yours and feeding back into the community and providing support and providing education, you can help to uplift people you know who face all of those barriers because there's so many barriers right so.
Jonathan James:I think it's we're in a wonderful position. It's just giving giving us a unified language to be able to say like, okay, let's, let's think out just knowing what questions to ask can be everything. It's not about you know, being combative or or trying to say that you, you know everything as a patient, but it's, it is about just continuing the conversation through.
Dr. Singelton:you know you can track more bees with honey than by vinegar, but sometimes you are met with some combative energy. That's true. Like you are. Right. And that's not always fair Right. And it's not always right. Yeah. But you can't stop when that happens. That's true.
Dr. Singelton:Like if, even if you, when you feel very strongly and you're concerned that something is going on, if you need to leave one emergency room and go to another one. Get a second opinion, get a third opinion, get a fourth opinion, if you need to you absolutely need to, or even if it's, I don't really feel comfortable with this. I don't mean to challenge you, I really don't, but can we talk about this collectively? Is there anyone else here that we can have a conversation?
Jonathan James:yeah, just bring in de-escalating right, like is it.
Dr. Singelton:But again, I I'm, I may have the skills to do that. You know, deep down inside I'm like, no, go get your supervisor, you know. No, I'm like, no, you're gonna get like, but I'm not Better get all New Orleans on them?
Jonathan James:Yeah?
Dr. Singelton:yeah, you're going to get your supervisor baby. I'm suppressing that person, but that's not easy for you know what I mean. For everyone, for everyone to do, and Lord have mercy. Don't add in the other preconceived notions about sometimes what people look like or how they're dressed or how they talk, or anything else when you start adding in all those other factors there can be issues. There are many things to fight. I think we have to take them sort of one at a time. We need new diagnosis codes.
Dr. Singelton:We need new ways to identify bleeding in women. We need more providers who understand how to manage women and girls. Yes. We need more evidence-based things to go to, so we need more.
Jonathan James:Women in trials.
Dr. Singelton:Yes, we need more women in trials, we need more research. We need things like ATHEN. I'm now the chief science officer for ATHEN. Yeah. We need people in the community to understand you like Athens. Who? What is Athens? You need to understand what Athens? Yeah, is that what is?
Dr. Singelton:that the American Thrombosis and Hemostasis Network. You need to understand by participating in that, how you're contributing to the community and then ask questions. What's happening with this data Like? Is there anything that's really going towards women? Ask your doctor are you participating in research for women? Yes, you know like what can we do? So there are so many boxes that we need to check off and we have to start somewhere.
Jonathan James:That's so good Right. We have to start somewhere I know that there's been. You know you bring up Athens and I just recently, you know, was able to sit in on the MESAC meeting which was so.
Dr. Singelton:I'm so glad you had that chance. And I have to say thank you to you, for really pushing.
Jonathan James:You've told me about coming numerous times and I've just always been like well, I got this, or that or this or that. It's information is power, oh my gosh, I was just mind blown, you know. And for all the advancement that we do in all of society, right, there's.
Jonathan James:I know there has been a lot of fingers sometimes pointed at MESAC at times and, and and and I you know I've been one of them. At times I've kind of been frustrated with the lack of product. But after participating and seeing and being involved in that I for a minute, I just realized like oh my gosh. The time there's a methodical the thought Genuine.
Dr. Singelton:That's right Intricate process that's going through that.
Jonathan James:And in order to be thorough, there is a longer timeline. Yes, and then you just got to put it out there and then see how it interacts with the other providers and the treatment and all the other things going on, and so then you got to wait for that response. It may be three, four or five years before that gets revisited in in another thorough fashion. So you know, anyway, I just wanted to say I just you know.
Jonathan James:Thank you for inviting me to that but also thank you for for investing in it, cause you've been a part of that for a long time. But I guess one of the things that's really exciting because at that meeting in particular was voted on this new document 286, which is specific recommendations for the management of inherited bleeding disorders for girls and women with personal and family history of bleeding.
Dr. Singelton:That's right.
Jonathan James:It's a really lot of words there, but there's really no other way to shorten that, because it really is important.
Dr. Singelton:You have to be inclusive, right? You want to make sure you hit all the buttons.
Jonathan James:But that document, if I understand it correctly, I don't think it goes as far as saying these are the new protocols, but I do think it's a recommendation to say, hey, we've evaluated this with a great deal of thorough measure and this is kind of the conclusion that we came up with the best minds in the business, if you will, and this is what we kind of came to as a conclusion.
Jonathan James:And it was interesting. For the first time I felt like I really heard what we've all been kind of discovering in the patient community as well as what some treatment centers and providers have also embraced through the years. There seemed to be this resonance within this document. That said, yeah, I actually had recently a conversation with the CDC on this, about the discrepancy between NIH, saying that it's anybody 40% or lower.
Dr. Singelton:Oh yeah, 40 and 50%, yeah and then the 50% from CDC.
Jonathan James:And I'm hopeful that in the next few years we're in conversations, hearing a lot of things but, I'm hopeful that NIH you know we'll go that number will kind of get washed out of the system because I think there is there's a lot of reinforcement behind this 50% number. But at the end of the day, arguably I heard a physician say this one time one of our mutual friends it's a hematologist in another area said arguably, if you have anything less than 100%, first of all the range is not zero to 100, the range is zero to really like what 270 or something like that.
Jonathan James:So it's this idea that there's a pretty broad range of that has been observed in society, and and so the question is like but if you're less than a hundred percent, there may be moments in your life where you could benefit from some supplement, and that doesn't mean if you have 99% or 90%, whatever, but the bottom line is anything lower than a hundred percent.
Dr. Singelton:Let me throw something in there that kind of justifies that too, right? So let's say for me um, I don't, I don't have or carry a gene for hemophilia in your heart in my heart. I carry all of it in my heart. Yeah, not in my genes, but in my heart yeah yeah.
Dr. Singelton:So because I don't have that. If my factor level right now, if you test it, and it's 87%, but when my body is stressed and I need to have more factor eight or factor nine available, my factor level might be 187%, but a woman who carries the gene for hemophilia may not have the ability to do that or generate that. So there's a dynamic here, right. There's something that could be different about that. There's something that could be different about that.
Dr. Singelton:So these are some of the things when the Medical and Scientific Advisory Council for the National Bleeding Disorders Foundation I almost said NHL For- NBDO, formerly known as it's like Prince, yeah, like formerly known have available to us.
Dr. Singelton:We're talking about the physiology right of a condition what happens with your body like the changes and what may or may not happen of a condition. What happens with your body, like the changes and what may or may not happen if you have a bleeding disorder or a family history of a bleeding disorder. So we understand it's not necessarily just about the factor level that you're looking at. It's about everything that surrounds that right. So there's a lot that goes into that.
Jonathan James:Yeah, there's a lot still not known, like how do hormones interact with that? How does enzymes interact with that? How does there's a? There's a plethora of other things that we still are, like iron deficiency, all of these things there are. So many.
Dr. Singelton:there are so many things, but what I think you and you pointing out the MACE-ED guidelines right and these recommendations is so important even that it's there for women.
Jonathan James:It's a baseline.
Dr. Singelton:It's a baseline.
Jonathan James:Right.
Dr. Singelton:It's there and the thing about guidelines and recommendations, so that really provides a place that, if you really don't know or don't understand, in addition to the literature, this is a place where you can start to sort of try to understand. Well, what are the people in the general community kind of recommending and what's done? The references are there. You can go back and use those references.
Jonathan James:Even for insurance approvals.
Dr. Singelton:Now the danger To sort of substantiate right the argument we can use it for insurance approval, and so there's some power in that that we really have to take seriously.
Jonathan James:Right right.
Dr. Singelton:Right.
Jonathan James:And what I love about this particular document which, again, I just I have to say I felt like.
Dr. Singelton:I felt like Well, you got to see how the sausage was made. Yeah, and it was.
Jonathan James:It was just, it was beautiful the parts of it that also were. I think that I I just got more context of why there's frustration sometimes, because it's like it's just a long process. This is how the sausage runs it. It doesn't happen overnight.
Jonathan James:And and and and, but for the sake of it being thorough and accurate and intentional. There's a need for this, but one of the things I really did appreciate that I felt like I heard the voice of the community and in unlike maybe some of what the past thoughts have been on this, is this last sentence here says I'm just going to read it because there's no other way to say it abnormal bleeding symptoms may occur with normal factor levels in up to 70 percent of genetic carriers of hemophilia.
Jonathan James:so one of the challenges I think we've heard a lot of is is that, like there's this you talk about diagnosis codes I think still most women, the code itself is going to say symptomatic carrier, even though we okay, the predominant line of thinking now is if you have a 30% level, you have hemophilia. Like there's not, it's not really, but the diagnosis code, if I'm not mistaken correct me if I'm wrong here but I think it still is going to read on that MIB report as symptomatic carrier because it's a female which so we need another code for that, we need another diagnosis. But the but the bottom line is, is that the fact that there has been observed and that in this document to say if there's as a baseline, as a, as a way to sort of a starting point, this document 286 really said something that I thought was really interesting. It says it spoke to the fact that it's not just about above or below 50%.
Jonathan James:It's not a hard line. You've got to look at the symptoms and, by the way, this could occur in normal levels. I interpret that to mean which, again novice at this but I feel like what I'm hearing in that is that normal factor levels would be above 50%. So if you have a 60% or a 70%, that there's some percentage of time that they will have potential bleeding symptoms, even if they're over 50%.
Dr. Singelton:Absolutely. And that goes back to the explanation that I kind of gave you earlier, right, who carries the gene? You know that causes, you know, factor eight deficiency or factor nine deficiency, that woman, even if that factor level is measured at 65%, may not be able to mount the appropriate physiological response. So again, it goes beyond just checking off the box right for the factor level. That is what masac is saying, right? Masac is saying yes, that this is something that can occur. We know that it occurs, we're aware of the fact that it occurs. And just because you have a normal factor level does not necessarily mean that that woman is protected. And that also goes back to the example that I gave earlier. What do I do when I have a woman who I know carries the gene that causes hemophilia A or hemophilia B and the factor levels are normal and there's a big surgery that's planned?
Jonathan James:That's so good and so necessary. It goes back to beyond crisis management. It's also about you know. It's one thing that I wanted to mention here, though, that I think is worth touching on briefly is, you know, we seem to be and again I have my pulse on like what's happening within the patient population across the country, right across the country, right, but what we seem to be seeing is more and more women getting the diagnosis but not getting treatment.
Jonathan James:I guess what I wanted to ask you is do you feel like in the future and I mean I know there's tensions right now, and a lot of it, I believe, has to do with and I want you to speak to this because I may be seeing it wrong, but I feel like what I'm observing is there's tensions on two places. One is many of the treatment centers are at capacity and they just logistically can't bring in another hundred patients. That's one aspect of it is insurance is pushing back a lot, and it's it's there's so many problems right now within the insurance space in the us in general, from afps to pbms to you know all all the things there's like a million problems going on in that space.
Jonathan James:So so maybe those have more to do with this than it is just just the science. I mean, I think we'd like to lean on and just say, just okay, the science is here, and then Masek says it, therefore it should be so and whatever. But there's still a lot of other things we need to problems to be solved. But I guess the question is is do you see a future for the community where more women may be on a once a month or maybe a twice a month?
Jonathan James:preventative treatment where they're having extended periods Definitely.
Dr. Singelton:Yeah, I mean, I'm already doing that.
Jonathan James:I know, but you're a one-eyed unicorn under a rainbow right.
Dr. Singelton:Yeah, so that's one of those things. You know what I mean, yeah.
Jonathan James:Do you think that that will become eventually the predominant line of thinking within?
Dr. Singelton:treaters across the country. I think eventually it will be dominant line of thinking within treaters across the country. I think eventually it will be. I think that, again, just real talk right. Just you know, being really honest, I think that it probably should almost be the expectation that, even if you jump over all the other hurdles, as a woman you understand that you could have a bleeding disorder, you understand what the bleeding is, you understand that you need to be treated. But then, connecting with a provider who has both the experience but then connecting with a provider who has both the experience and the understanding of what happens, like with women.
Dr. Singelton:That is where the challenge may be. That's the real truth right now. That may be the challenge.
Jonathan James:Yeah, I mean case in point for me. I have four kids, two of them are girls.
Speaker 4:They went through a period of time where both of them did not really have extended menstrual cycles.
Jonathan James:other symptoms they were playing sports had problems and retrospectively we look back at those years and go, oh my gosh, like. In some ways it's devastating, because you realize it was right in front of my face the whole time. I wish I would have spoken up. I wish I would have said something.
Dr. Singelton:But remember. You have to remember, though we were all in the same boat, I know.
Jonathan James:I know Right, the house was on fire. We gotta give ourselves grace. They gotta give us grace.
Dr. Singelton:We've got to give ourselves grace. They've got to give us grace.
Jonathan James:The house is on fire, right, but nonetheless you want to talk. Why is this? Conversation so important for. Jonathan, that's a driver for me, right? I?
Jonathan James:don't want to see anybody else go through unnecessary suffering because of a lack of understanding. So, that's what drives me. But in that I also simultaneously saw that there was a place where, even when we did ask questions, the predominant line of thinking was like, well, do they have extended minstrel asylum? No, well, then they're probably okay. I mean like no investigation, and I'm not pointing fingers at anybody. Again, like you said, we were all in the same boat, boat.
Jonathan James:But, but I, I do think that I, at least I hope that enough progress has been made and observations have been made that not only will that start to invest, let's just, you know, I was just with a geneticist, actually from Tulane, not hemophilia related. We were part of the rare disease advisory council for the state legislature here and uh, I was talking with Hans and he said you, know, I love him.
Dr. Singelton:Oh, he's amazing Love him.
Jonathan James:But he said specifically he said which, again, I'm just quoting him in this, I don't I haven't read it myself, which I'm sure he's. I trust everything he says, so I would trust this too is that is that genetic testing is now on formulary for every insurance provider across the board, which is great news. So maybe there's a day coming that we can get to that where it's just like you know what you're in the family, we're just going to test everybody see where everybody is, and if you have the chromogenic or the dispensation towards this genetic mutation, then we know the markers are there.
Jonathan James:Therefore the levels almost become matter of fact. I think levels have been kind of a primary form of looking at it. Now I do think phenotyping and understanding that there could be a positioning for that, just because of the gene mutation actually could be the way that we're looking at that in the future and maybe that's just done. It's just automatic, hopefully.
Dr. Singelton:I mean, I don't know. We have this dream project with Athens and I know there are a lot of treaters who are very interested in this too that every female born with a family history of hemophilia, of course, when we know there's spontaneous mutation. So not discounting for that, but every female with a family history that just as a male is tested at birth that the female will also have a cord blood sample scent and a genetic sample scent. Amazing, would that not?
Jonathan James:what that's like our dream.
Dr. Singelton:That's like our dream project, our dream project is, yeah, every female right, and we want to collect that data right and follow those females, kind of over time follow factor levels. You know who wants people, hey, bleed logs. But we need, like you know, a bleed log to kind of track like what's happening. What if we could even follow joints of those women longitudinally? To then have that information to say well, this is what has happened like over time.
Dr. Singelton:Now those types of things take people who are willing to do it. It takes, you know, financial support, but that's the kind of information like that we really need to to move the needle Again. This is a war that has to be fought on multiple fronts. We have made tremendous progress right over time. We do have resources. You know Tulane, the HTC there. They've dedicated like an entire clinic, like they're really trying to focus on, you know, women and girls. I know that LSU like has an initiative, but they're really also like trying to. They've been involved with the foundation. So even here in the state there are people who are trying, like they. You know they care. I already drank that Kool-Aid a long time ago.
Dr. Singelton:You know I now have my clinic at Ochsner. You know we have voices like within this state, but as we look at people like across the country, again using your resources and the people you know and hope, right as a tool to say if I want to find a provider, you know and I'm in New Jersey. If I want to find a provider and I'm in Iowa, you know, or I'm in Washington state, iowa, you know, or I'm in Washington state. Like where? Like where do I go to have someone who's at least willing to listen and have this conversation with me? Cause it's not a one and done?
Dr. Singelton:you know, with the with the woman or a girl.
Dr. Singelton:And so very early on. Even when I have those conversations, I'm like listen, I don't want you to think it's the one and done. We may need to follow this right out over time. I can't say that prophylaxis or anything has to start right now, but it doesn't mean that it doesn't need to be at some point. But we need to follow this out and understand what's happening. It's about having a provider, I think, who is knowledgeable and willing, you know, to evaluate, but empowering yourself too with the information that you need to have. So it's so good, you know, you know.
Speaker 4:I evaluate, but empowering yourself, too, with the information that you need to have.
Jonathan James:So it's so good. You know, you know I do a lot of work on in in advocacy in particular, just both with Louisiana Rare Disease Advisory Council, but also with federally and in in a lot of different projects there. One of the things that I'm really excited about about the future is this idea that we could get a policy change within the Medicaid Each state. We're working on one here in Louisiana Accelerating Kids Access to Care Act to be where a child that has been identified as either having a rare disease or has a propensity of genetic disposition for a rare disease could actually be reimbursed for a specialist outside of the state limitations.
Jonathan James:It's a huge ask, it's something that's been pushed back on over and over again, but I actually feel like I see the beginnings of a glimmer of hope that there could be some of this legislative change from a policy point of view. And what we've told legislators is and I think that this is a really big need and it's not just for hemophilia, it's like a lot of rare diseases obviously have this issue, but when you have a specialist that's across state lines and you're on Medicaid, you cannot go to a specialist across state lines. Even many insurance policies won't allow you to do that. So you know it starts to me, it starts within that medicaid framework and then maybe medicare, but then once you do, I think the downstream happens in commercial as well. But at the end of the day that there's this, what we've told legislators legislators is especially in the in in the state of louisiana is if we can even show that they're having to go to Houston or they have to go to wherever to get a specialized grant.
Jonathan James:it actually could help identify the number of patients that live in the state that have this need, and it could attract those same talented physicians from other states to come here and so that's exciting about Louisiana, but we're fighting for that federally as well in a way to see that maybe that from a CMS change that could actually free up the system to where people could go to the right provider across state lines. I know there's a million other issues that could run into there, but that's something we're advocating for.
Dr. Singelton:I'm excited about the future. It's so important, it's so, so, incredibly important, and I think that that's one of those issues that you know when you start throwing out those DEI like terms and you say diversity, equity and inclusion, people automatically kind of go to race. Right.
Dr. Singelton:And many times it has absolutely nothing to do with that. So what you just mentioned, right, it's like an equity issue. Yes, so if I have great healthcare, yeah, right. Because of my job or my husband's job, right. And if there's no one here in the state that treats the the condition that my husband has, well, we can hop on a plane and go and go to New York or Texas or California or wherever we need to go. Right, right.
Dr. Singelton:Right. Someone who's on Medicaid Right Should have a similar opportunity. Right, that's right. Right Now, I'm not in any way suggesting that it shouldn't it should happen like with no restrictions, right? Or shouldn't it should just be just kind of carte blanche? I think you have to establish that is actually needed Right First, Right, that is actually needed right first. Actually, Medicaid in Louisiana will allow you to have a consultation. You can go across state lines to have a consultation. It takes an act of God.
Dr. Singelton:I've done it a few times when I tell you it's like an act of God. It is incredibly difficult to do and it's incredibly difficult from a Medicaid standpoint to get that consultation approved, and it's incredibly difficult to get the receiving state to agree to it too. It is almost impossible. But that continued care, that's the part that can't happen. So when I have access to that and you don't, that's an equity issue.
Jonathan James:And so when we're talking about things like rare diseases right and women and girls, um, it's about having access to the same health care yes so especially if we say this has got the best Healthcare in the world In the US and unfortunately, because of these types Of barriers, there's a vast diversity, that's right Of people who are living Right next door to something that could save their lives and they can't get to it Because they're just In whatever category.
Dr. Singelton:Again, yes, wrong, it's wrong To toot that horn and say again, when you hear diversity, equity and inclusion, those are the types of things, yes, right, that we have to zero in on right because that's what really matters we deal with this a lot with rural health.
Jonathan James:We've been working with uh hersa a lot on this, because rural health, rural health with with um within the context of why are people who are three hours away from a human affiliated treatment center not participating in trials?
Dr. Singelton:They can't get to it. They can't get to it.
Jonathan James:It's not, yeah, so it's both equity and there's also this idea that there's missing pieces in a trial that need to be present in a trial. That's right, so that we can have that best information. You're not including all the people that you need to include. You don't have the diversity of people.
Dr. Singelton:That's right, so that we can have that best information, so you're not including all the people that you need to include. You don't have the diversity of people.
Jonathan James:That's exactly right Right.
Dr. Singelton:You don't have people who again, there are different types of people, Right. So people who live rurally actually may have a different state of health. That's right In general in comparison to someone who lives in the city. That's right. Right, that's a certain kind of diversity that we need to know about and kind of have as we're looking for different therapeutic options. When I was just 45 minutes outside of New Orleans, I was the only hematologist on that side of the lake that would see patients with Medicaid.
Speaker 5:Wow, wow, wow. That would see patients with Medicaid, wow, wow.
Jonathan James:Wow, and you and I know that there's a ton of people On that side of the lake that don't ever go To the other side of the lake because they can't. There's no bus route, there's no way to get there, or they can't take off from work. They have a sick wife, a sick mother. They just can't get there.
Dr. Singelton:I was the only, the only one. Wow, right. And so when I was again just in the center of New Orleans because you know, we had Medicaid transportation and we did have a couple of satellite clinics and places that we would go, right, I thought that we were killing it Right, I thought that we were providing care. I thought that we were killing it Right, I thought that we were providing care. And when I went there, I mean, it was heartbreaking and sobering to know that there's so many people that couldn't get there.
Dr. Singelton:So I acted almost like a triage center. So I said send me all, all of the adults, whether it was hematology or oncology related, and I would try to see them and go OK, ma'am sir, you really need to get over there and do a warm transfer, right? I would pick up the phone and call the colleague and say I have Mrs Brown here. She has anemia, that's why she came to me, but it really looks like she has stage four colon cancer. She needs to come today. Right, right, right, right, right, and we would send her.
Dr. Singelton:Now did that lady have to have stage four? You know, colon cancer? Wow, right. So it's those types of things. And again, this extrapolates and applies to people living with rare bleeding disorders and it certainly applies to people who are living with mild and moderate hemophilia, including women and girls, like these same types of of issues. So when we're fighting for things for people living with rare diseases, there's so many people right that we're trying to include when we're fighting to have care for people who live rurally. Right.
Dr. Singelton:I mean, that could really change the face of America, right, but I didn't know that that existed. Right, right right. I thought we were killing it Right, like it wasn't a part of my stratosphere.
Jonathan James:Well, you only know what you know and, honestly, that's so progressive comparative to a lot of other states. It is, it's so progressive. You are better than a certain number of folks it is.
Jonathan James:But at the same time does that mean you're meeting every need? No one can meet every need, I think that this is. The thing, too, is that this is part of having grace for all of us, because we're all on a journey. And while we know a lot, we still don't know everything we don't know everything, and the reality is is that there are probably, you know, many things that we need to change and do and it is about the satisfying.
Jonathan James:The fire really in the house is burning down first and then prioritizing. I actually have a the chairman of our board used to work for an nonprofit organization that did disaster response and relief and one of the things they did through especially through Hurricane Katrina, was sort of disseminating resources to these really tragically, you know difficult damaged areas. One of the factors that he used to say was, he said, one of the problems that you know everybody was mad at FEMA for a while and it was, like he said, one of the problems that he realized was that FEMA was very methodical about what they did and their mission was to manage a crisis. And he said the problem is you can't manage a crisis. He said you have to just respond. When there's a crisis going on, you just respond, but it is once that crisis is now settled that then you can sort of manage some of the ancillary phase. Two phase three of the recovery.
Jonathan James:And I feel like in American healthcare, like in North America, for our healthcare system, you know, there are so many people, whether it be cancer, whether it be hemophilia, whether it be all of these things we were so focused on just having any solution and some of those solutions created their own sense of challenges and and and yet now we're getting to a phase where some of those things can come down a little bit. I think right now we still have quite a few things that need to be addressed from a policy point of view as it pertains to the insurance and all of that. But we can't just go from one crisis to the next. I do feel like, overall, we are seeing some progress.
Jonathan James:The problem is many of us feel like it's too slow and it's not fast enough and all that stuff. But if we can give each other grace and still speak up for ourselves and speak up for others and really keep looking until we find that compatibility with a provider, when we find that compatibility with a, and sometimes, look, people will drive forever to go find you know, to go find you know the right person, and with telemedicine, that's an element that sort of opened up a window. I don't think that that's the long-term solution, but there are ways that those types of tools, I think that we can gain. We can gain, just like this conversation today is being streamed and broadcast to thousands of people all over the place, and that was never even possible, that's right.
Jonathan James:Even just 10 years ago.
Dr. Singelton:This wasn't a normal thing. It was not a thing.
Jonathan James:Yes, there's probably people listening to this today that are going oh my gosh, I didn't even know that there was somebody out there who really understood what I was going through that there was somebody out there who really understood what I was going through. So, anyway, I just want to say that I'm applaud seems like too small of a word, but I'm so grateful for all the work that you've done and all of the empathy that you've had.
Dr. Singelton:I appreciate that and it starts with just caring for one.
Jonathan James:It does and it starts with just embracing your calling what you've done.
Jonathan James:And simultaneously, I think what many people miss and I'm going to say this to all the patients that are listening right now I think what oftentimes we miss is that in a crowd of difficulty, sometimes it just takes a spark of one person to champion it that makes progress for the whole.
Jonathan James:You know rising tides of all ships. If you can, just if you can just rise above it for just a minute in the emergency room in that one-on-one conversation, you think it's just you and that, but today you and I are having just a one-on-one conversation, but there's thousands of people. It could maybe potentially elevate the conversation for the whole community and this is what I want to say to all the patients who are listening to this. It's so important that you get this is that your diligence to stay on task, to continue seeking answers for the challenges that you've had and looking for the right partners and the people to join forces with in your healthcare team is not just going to fix a problem for you, but it could fix a problem for generations to come in your family, but for the whole community.
Jonathan James:You've done that. You are lift. I mean people are going. Oh, we better pay attention. This team is going to tell us how it is, you know, but really every one of us, as people living with it, are also this, you know. We were talking about just before we started the recording about how some of us are trained for this.
Jonathan James:And some of us are trained by this, and what's really magical is when you have people like you in the corner that have been both You've been trained for it and by the community that you served, and so thank you so much, really from the bottom of my heart.
Speaker 2:And I know I say that on behalf of thousands of people that have said the same thing, so we appreciate all you do and keep it up, because we need more of it, and we'll be following you closely to see what we can do next.
Dr. Singelton:So thank you so much. Thanks, thanks for having me.
Jonathan James:Thanks so much for joining us with this podcast today. I certainly hope that you enjoyed this. I know I learned a lot and. I think that you did too Fascinating enough. I actually took notes. Today I have some things that I took some notes on and.
Jonathan James:I don't always do that, so this is really helpful and inspiring to me, but I hope it was helpful and inspiring to you too. And if you have any questions or if any thoughts come to mind that you go man, who do I go to? Where do I go? I don't even know what my next step should be Don't hesitate to reach out to us. We're real people with a real office, with a real team of people. There's 15 of us around here trying to help people every day, and one of the things we do is love to help you as you are on your journey. So if you need that help, you can always call us 888-529-8023. Or you can email us at info at hope-charitiesorg. We would love to be able to serve you in any way that we can. Thanks for listening in today and I look forward to seeing you on the next one. So take care.
