Dr. Terry Gernsheimer

I think the most important thing to understand is that we’re going to manage this, and we’re going to manage this together. Most patients, we’re going to get you to a place where you’re going to be living your life and enjoying it just as you were before, it’s a matter of getting you there.

Narrator

Welcome to the PDSA podcast Bruised but Not Broken: Living with ITP. The diagnosis of a bleeding disorder like immune thrombocytopenia may leave you wondering, “How can I really live my life with ITP?” PDSA’s podcast, Bruised but Not Broken: Living with ITP, brings empowering stories, the latest research and treatment updates, lifestyle tips, and answers to the real life questions the ITP community is asking. Here’s your host for this episode, Barbara Pruitt.

Barbara Pruitt

Today's episode we're calling ITP 101. If you've been diagnosed with ITP, I know that you feel like there's an awful lot for you to learn, and it can be informational overload, and it can be very confusing. So, we are very fortunate today to have an ITP expert with us, Dr. Terry Gernsheimer. She is a professor emeritus of hematology at the University of Washington, School of Medicine. Dr. Gernsheimer's research focuses on the pathophysiology and the treatment of immune platelet disorders and pathologic immune responses associated with transfusion. She has clinical research interest in transfusion management of the hematopoietic stem cell transplant patient, and surgical bleeding in patients undergoing solid organ transplant and cardiovascular procedures. She has made significant contributions towards understanding the pathophysiology and management of ITP. Dr. Gernsheimer also shows serves on the PDSA Board of Medical Advisors. So welcome, we are happy to have you here today.

Dr. Gernsheimer

Thank you very much, Barbara. It's very good to be here.

Barbara

Well, we are thrilled. And to start off with, let me ask you, what sparked your interest in ITP and platelets?

Dr. Gernsheimer

When I was a resident, after graduating medical school, I decided to do a residency in internal medicine. I did find hematology particularly interesting. When I was a senior resident trying to decide which of the subspecialties I would go into, because I did find hematology fascinating. I had a patient who came in with a platelet count of, I think it was definitely under 5,000. She also was destroying red blood cells at the same time. It was absolutely fascinating to me. And I remember coming back from clinic to have my intern greet me with, “You are going to love this patient.” And indeed, I did, and found this completely fascinating. So, when I did decide because of that patient, it really kind of pushed me over the edge to decide to go into hematology. And I was fortunate enough to do my laboratory years because when you train in hematology, you do both clinical training and then you do laboratory research training. I was fortunate enough to hook up with Dr. Sherrill Slichter, who was one of our pioneers in platelet research and particularly in immune platelet disorders. And with that, she kind of started me on the road to studying patients who had ITP and immune platelet disorders.

Barbara

Well, we're very glad that that was the hook for you, that you got so interested in it. That's terrific. For our listening audience, let's go over some questions that I know they may have. How would you describe what ITP is?

Dr. Gernsheimer

First, I think it's important to understand what those letters stand for. And they stand for immune thrombocytopenia, or immune destruction of platelets. And particularly, autoimmune destruction of platelets. We used to, it originally was idiopathic thrombocytopenic purpura –

Barbara

Right. Right.

Dr. Gernsheimer

It's a big mouthful, meaning we don’t understand why the platelets are low and causing bruising. And it's a misnomer now, because first of all, we understand much more than we did before. So, we don't consider it idiopathic anymore. We know that it represents an autoimmune response.

Barbara

Right.

Dr. Gernsheimer

Thrombocytopenia is correct. That means that means a low platelet count, but not everybody has purpura, in other words, bruising. And so we have, we call it ITP, but it now means something a little bit different than the original ITP.

Barbara

Right. Well, I know when I was diagnosed, they just called it thrombocytopenia purpura. I don't even think they put the idiopathic in there until like the 70s.

Dr. Gernsheimer

It was, the original reason why they thought it was, quote, “idiopathic”, was because we know that platelets are made in the bone marrow by a particular type of cell called a megakaryocyte. It's a very large cell in the bone marrow. They're very, very beautiful when you look at them under the microscope. And it was assumed that there shouldn't be many of those in the bone marrow, and that's why it would be low. And when they started doing bone marrow examinations on patients with ITP, they saw lots of these megakaryocytes. And so it was, we don't know why, idiopathic, the platelet count as low. So that's how it originally came to be idiopathic.

Barbara

Well, that explains it then. So, in ITP, it's actually, you said, an immune response. So, your body is actually destroying the platelets that you're making, correct?

Dr. Gernsheimer

Right. Correct. Why people make auto antibodies is a big question. What the triggers are,  both environmental and genetic, are really not known, although we have some hints about what might lead someone to begin to do that.

Barbara

Yeah, I know that there's a lot of research going on now for genetics and to see if there are some connect-the-dots between patients and platelet disorders and ITP. Which is very interesting. How do you diagnose ITP? I know it's a diagnosis of exclusion, but what is it that you're excluding when a doctor tries to figure this out?

Dr. Gernsheimer

So, when somebody comes in with a low platelet count, we have to go through a series of questions in our mind, from the history and from other laboratory tests that are done. And as you mentioned, it is and a diagnosis of exclusion to some extent, because we'll look, first of all, are there infections that might be causing this low platelet count? Are there other autoimmune disorders that might be causing this? Is there a bone marrow problem and a problem with platelet production, specifically that the bone marrow itself is not healthy? So that's a large part of it, is seeing that there's nothing else going on that can explain the low platelet count, and we do certain laboratory tests looking for that. But the other way we actually wind up saying, a-ha, this is definitely what it is, we will sometimes, if the count is low enough that we're somewhat concerned, give drugs like prednisone, corticosteroids, to try and get the platelet count up or immune globulin, IVIG, to try and get the platelet count up. And it is indeed the significant response to those drugs that tell us, a-ha! This patient is responding to immunosuppression and that kind of clinches the diagnosis in most patients.

Barbara

Okay, okay.

Dr. Gernsheimer

The majority of patients who have ITP will respond to those drugs.

Barbara

Quickly, right?

Dr. Gernsheimer

Yes

Barbara

They respond pretty quickly. When you've figured out this diagnosis for a patient, how do you break the news to them?

Dr. Gernsheimer

So, I mean, you know, I remember very much one of my first mentors in hematology. It's a very frightening thing just to be sent to a hematologist.

Barbara

Right.

Dr. Gernsheimer

And I remember one of the things that he would do, is he would walk in the room and he would say, “I was asked to see you, I'm a hematologist,” and the first thing he said to one of these patients was, “You don't have leukemia.” Because it's terrifying –

Barbara

Right, right.

Dr. Gernsheimer

To say, there's something wrong with my blood.  And I think the first the first thing that people think about, particularly if they go to the internet, what causes low platelets, is “Do I have some terrible bone marrow disease?

Barbara

Yeah

Dr. Gernsheimer

So, I think that that's the first thing, is to allay people's fears that this is not a disease that is a mortality diagnosis. You're not going to be “There's nothing we can do.”

Barbara

Right.

Dr. Gernsheimer

“We can't fix this. We're going to put you through tons of chemotherapy.” So, that's the first thing, is to allay their fears as to, you've got something really awful that I can't fix, or we'll have a hard time fixing.

Barbara

Right. Something that hopefully is manageable.

Dr. Gernsheimer

Exactly. And an ITP for the most part is very manageable. It does mean some lifestyle changes. It does frequently mean some therapies that may or may not cause side effects, but it is not a terrible, terrible diagnosis.

Barbara

Great. So, I'm glad that you approach it with the gentleness of, listen, it's not cancer, you know let's go on from there.

Dr. Gernsheimer

We can work on this.

Barbara

Right, right. When is it that you decide to treat a patient with medication? I know that the American Society of Hematology has guidelines that they present to hematologists all over the world.

Dr. Gernsheimer

Correct.

Barbara

But when is it that decision is made by the hematologist?

Dr. Gernsheimer

I think it's very different depending where in the course of ITP you are. When someone is first diagnosed, as I said, frequently, we're not 100% sure. And if the count is, let's say, under 30 or 20,000, and we've ruled everything else out, we might at that point try something like corticosteroids. But as we go on, things become different. I am not as concerned specifically about the platelet count as I am about symptoms and how this is affecting someone's life. So, many patients can do just fine with relatively low platelet counts of less than 20,000. And so we have to make decisions about how, number one, are you having symptoms? What is your lifestyle like? Are you at a significant risk of bleeding? And have we tried therapies that will be without multiple terrible side effects, or you know are we are we starting to talk about therapies that are going to cause significant side effects and we really have to worry about, you know, we're weighing both sides, let's put it that way.

Barbara

Right.

Dr. Gernsheimer

But I think to a very large extent, as we move through the therapies, we're looking at whether or not a patient is symptomatic and at high risk of bleeding.

Barbara

Right. I had my hematologist tell me years ago if they could come up with a wind chill factor for ITP patients. In other words, not just looking at the platelet count, but looking at how the patient is doing. Are they having a lot of bruising, petechiae, bleeding? And putting those two things together in like a wind chill factor is what he said.

Dr. Gernsheimer

That's an interesting way of looking at it. Yeah. I had never, I never heard that term, but I think, you know, that is exactly what, you know, I, and one of the things I tell patients that I think is very important is not to be married to your platelet count.

Barbara

Oh, absolutely.

Dr. Gernsheimer

I'm much more concerned about what are your symptoms? Are you living the life you want to lead? And let's not be checking the platelet count all the time because I'm much more concerned about what symptoms you're having and whether or not we can safely go through life.

Barbara

Right. Oh, that's wonderful advice. That really is. What do you consider the different phases of ITP?

Dr. Gernsheimer

One of the things that's important to understand is why we call these phases, why we name these phases, these phases, okay? And a lot of it was centered around how we do our research, and being able to compare therapies and papers, journal articles that we're writing and looking into. So, I think it's important to understand that. In general, we say acute ITP is within the first three months of diagnosis.

Barbara

Okay.

Dr. Gernsheimer

That's initial presentation with ITP, at which point, usually the platelet count is low, which is why we pick it up. There may be some symptoms. And we'll try therapies like corticosteroids if the count is very, very low. We'll try immune globulin, IVIG. And we very quickly want to stop those therapies, particularly corticosteroids. We don't like to treat with corticosteroids beyond six weeks. We want people off that and see if see if the platelet count will stabilize without that.

Barbara

Right.

Dr. Gernsheimer

And see if see if the platelet count will stabilize without that. After three months, again, this is just nomenclature. After three months, we begin to call it persistent. In other words, well, it's not something that came up, because sometimes about anywhere between 5 and 15% of the time, it depends on where you read and what data have been collected, but it resolves on its own. Something triggered it, and whatever it was goes away, and the immune system corrects itself. At that point, we say, you know, this isn't going to be more of a problem. It wasn't just a short episode. And we call that persistent, with, between three months and a year. Again, just kind of what we named it.

Barbara

Okay.

Dr. Gernsheimer

Again, just kind of what we named it. And then at a year, when it's more than a year, we begin to call it chronic. Now, again, it's not like at 364 days you fall off a cliff. It's just a way for us to compare different therapies and how patients do along the way.

Barbara

Right.

Dr. Gernsheimer

It's just a way for us to compare different therapies and how patients do along the way.  But at that point, we're usually looking at something that is going to be more long-term. But many patients along the way get better. You know, we treated it and all of a sudden, the immune system, for whatever reasons, decides to correct itself, and so chronic mean necessarily that this is it for the rest of your life.

Barbara

And, well, and I know with a lot of children, you know, that are diagnosed, their ITP goes away as weirdly as it comes about. Yeah.

Dr. Gernsheimer

Particularly with children. We know when children present, it tends to be more of the acute variety, frequently correcting itself within the first two years, sometimes within the first few months. With adults, it's more, particularly in older adults, I usually look at patients and in the back of my mind, I'm saying, we're going to be friends for a while.

Barbara

Right. We're going to have a long-term relationship, which is what I tell patients when they're looking for a hematologist. I tell them, you know, you really need to be able to communicate well with the hematologist that you decide to use, because it may very well be a long-term relationship, and you need to be able to understand each other.

Dr. Gernsheimer

Find someone you're comfortable speaking with.

Barbara

Absolutely.

Dr. Gernsheimer

If you feel like you can share all of life's trials and tribulations

Barbara

Right.

Dr. Gernsheimer

And discuss comfortably how this therapy is working for me, and that you can together make decisions. You have to be able to make decisions together. It's very important.

Barbara

That's so important. And you have to feel comfortable enough to speak up for yourself and tell them what you think is going on and why you'd like or dislike this therapy or whatever. And you need to feel comfortable to be able to ask questions openly and not be afraid of  being looked down at or whatever. So that's so important. How often do you recommend patients get their platelets checked?

Dr. Gernsheimer

Well, that goes back to what I was saying about don't be married to your platelet count. Right.

Barbara

Yeah

Dr. Gernsheimer

You know, early on, or when we're starting a new therapy, we may want to check the platelet count frequently when we're trying to decide what to do or what's the course of this going to look like. You know, as later on, since I'm I know that most of the time I'm going to choose or recommend therapy, really, if someone is symptomatic or, you know, they’re - I remember one patient I had who was a hot dog skier, and he wanted to know, can I go skiing? And it's like, well, I think we better take a look at where your platelet count is going right now.

Barbara

Right.

Dr. Gernsheimer

You know, later on, there are many patients that I'll say, we only need to check your platelet count every three to six months. Let's not be doing this all the time because that's making you constantly, thinking about this when what I want you to be doing is living your life.

Barbara

Absolutely. Thank you for saying that because people do, they get, they cling on to that count and they get anxious about, then your next count. And oftentimes you've heard patients say they're on that emotional roller coaster and getting your platelet count done every week, you're going to be on that roller coaster because it's going to change week to week.

Dr. Gernsheimer

That's right.

Barbara

Whether it goes up a little down a little, up a little down a little, and there's a lot of play in a platelet count, you know? It can change day to day, but it depends on which arm. And I mean, a lot of variables, true?

Dr. Gernsheimer

Well, again, I think the most important thing are symptoms because that's what's really going to push me to treat someone. Now, again, if I'm trying a new therapy, particularly if it's an experimental therapy, if someone's on a research study, for example, if I'm trying a new therapy, I may, first of all, be required in a research study to be checking the platelet count every so often. But I may also want to see, gee, are we getting somewhere with this therapy? Do I need to change therapy? This is a new therapy. Is it working? Can I decrease the dose?

Barbara

Right.

Dr. Gernsheimer

Or when I'm trying to taper somebody off steroids, am I able to do this? Are we at a point where I can now lower the steroids more? But other than that, when we are more in kind of just a chronic management situation, I really encourage patients, let's not always be looking at the platelet count. Let's talk about how you're feeling.

Barbara

Right.

Dr. Gernsheimer

And frequently patients will tell me, I know my platelet count is dropping.  I feel really tired. I just don't feel well. And they'll say, I want to check my count because I know that that usually is associated with my count falling. And that's fine.

Barbara

Right, right.

Dr. Gernsheimer

But you know, we don't, again, don't want to be married to the platelet count.

Barbara

I know my doctor has given me a prescription to get a CBC platelet count and a chem profile. He put on it every two weeks, not that he wants me to do that, but so wherever I am, if I want to get a platelet count, if I feel like I need one, because like you said, you need to be aware of what your symptoms are, that I can go to, you know, a blood drawing place, you know, lab core or something like that, and get it done. Is that something you've ever done with patients?

Dr. Gernsheimer

Yeah, most of my patients, I indeed do exactly that. First of all, my nurse knows and the patient can call the nurse anytime and say, can you schedule me for a blood draw? I frequently will give them a prescription to go ahead because people travel, they have lives.

Barbara

Right. Absolutely.

Dr. Gernsheimer

What I frequently say is, at the end of the day, you're going home with your ITP, and I'm going home to my husband to have dinner.

Barbara

Right.

Dr. Gernsheimer

And you're living with this. You know, and although I think about my patients, I'm not living with this. And so, it's very important for people to know how to manage their own ITP, and to know, gee, I think I need a count, or no, I know I'm good right now. I also want people to feel comfortable traveling.

Barbara

Right. Yeah.

Dr. Gernsheimer

I don't want this to have the kind of effect on someone's life that they're not going to be able to do the things they want to do. And sometimes we have to kind of play around with this. I've had, I had one young woman who kept wanting to, first she wanted to go to Australia, and then she wanted to go to New Zealand, and from there she wanted to go to Spain. She was, you know, she was a student, and we managed it. And I think it's important to be able to have your physician work with you so that your life can be as normal as possible and as much of you want it to be as possible.

Barbara

That's great. That's great advice. And when a patient, talking about a physician, a hematologist, when a patient is looking for a good hematologist, I know we discussed about having a good relationship and a good rapport, but what are the questions that they need to ask when they first go to see a new hematologist? What would you consider the really important questions, to see if that person might be the right one for you?

Dr. Gernsheimer

So, I mean, the first thing, of course, is do I relate to this person? Can I find an easy, comfortable relationship, as you said? A lot of hematologists, or hematologists oncologists.

Barbara

True. True.

Dr. Gernsheimer

And, you know, that means that they trained in hematology and oncology, but most of their training is going to be in oncology

Barbara

Right.

Dr. Gernsheimer

Which means that the vast majority of their patients are going to be lung cancer, lymphoma, breast cancer, colon cancer, because that's what they're going to see. That much, all of those are much more common than ITP.

Barbara

Right

Dr. Gernsheimer

So, one of the things you want to ask is, how many patients do you see with ITP? They may or may not tell you, but I remember one patient that I saw, he asked his physician, and he was told, you're my only ITP patient. And he was like, I'm going to go find somebody else.

Barbara

Yeah, yeah.

Dr. Gernsheimer

Which doesn't mean that he's not good, he doesn't know what he's doing. I mean, part of it is, are they willing to work with an ITP expert? Because there may not be an ITP expert in your area, but that doesn't mean that it's, that it's not a physician who isn't comfortable speaking with somebody else and co-managing you with someone else. There were many patients that I've had over the years who, they had an oncologist locally who was a good doc, but knew that they themselves didn't know as much about ITP as I did. And so we would co-manage. And, you know, that to some extent, I hate to say it, depends on somebody's ego. You know?

Barbara

Yeah, no, I agree. I agree. Some doctors want you to think that they're the, that they know everything. And we can't –

Dr. Gernsheimer

Nobody knows everything.

Barbara

None of us can. Right.

Dr. Gernsheimer

You know, I was trained in oncology, okay, but I would never say I can manage lung cancer.

Barbara

Right.

Dr. Gernsheimer

I can't.

Barbara

Yeah.

Dr. Gernsheimer

There are people who are much better at that than me. When you have a rare disorder, you have to expect that whoever you see may not know an awful lot about it.

Barbara

That’s right. So you ask them, how many patients do you have? And what else would you ask?

Dr. Gernsheimer

Are you willing to work with, to let me get a second opinion with someone who is more, has more expertise in ITP? Would you be willing to co-manage my case with them?

Barbara

Great. Great advice.

Dr. Gernsheimer

Those are important questions.

Barbara

Yeah. A lot of people that live in more rural areas, there's not an ITP expert anywhere around them. They may have to travel hours to get to, you know, a medical facility that has a doctor that might be familiar with ITP. So, it would be important if you live, especially in a remote area, to have permission to co-manage. And I think possibly those doctors might be more open to that.

Dr. Gernsheimer

Yeah. Well, particularly I have co-managed ITP patients with their internist or family physician. And I think those physicians in patients who are in very rural areas, and those physicians are even more comfortable with that, because they're very comfortable admitting this is not my area and co-managing with me. So, you know, the patient comes and sees me once. And, you know, maybe once a year they come, maybe not even, but we're, you know, I was speaking to that physician and getting emailed by that physician of this is where we are right now and what do you think? And do I need to change therapies?

Barbara

Right. Well, that's great. Now, I know I've heard different doctors, maybe you also, speak about your hematologist needs to look at your blood slide in a microscope. And if they don't do that, that's kind of a red flag. Is that true?

Dr. Gernsheimer

Well, things have changed quite a bit.

Barbara

Okay.

Dr. Gernsheimer

That used to be very true. And a real, a real hematologist always had a microscope on their desk. You know we do an awful lot of stuff remotely now. And in fact, there are cameras down in the laboratory where we can actually have that sent right to our desk to be able to look around at someone's place.

Barbara

Wow!

Dr. Gernsheimer

So things –

Barbara

So it comes up on your computer, the actual picture of the cells?

Dr. Gernsheimer

Yeah.

Barbara

Oh, how?

Dr. Gernsheimer

You can actually call down to the lab and talk to one of the technicians and say, “I want to look at so-and-so's blood smear” and actually see it. So things, as I said, have changed quite a bit.

Barbara

OK, that's wonderful. That's wonderful. But do you still think it's important for your hematologist to at least look at one of your smears to make sure that –

Dr. Gernsheimer

I have to say I really like to because there are things on a blood smear that I want to see myself that help me determine that this is probably again, you know, it's very hard to make it an absolute diagnosis of ITP right off the bat.

Barbara

Right.

Dr. Gernsheimer

But I might be looking for other abnormalities on the blood smear that will give me a hint that this is something else where this is indeed ITP.

Barbara

Okay.

Dr. Gernsheimer

There are other blood cells don't look quite normal. There are hints that the platelets don't look quite normal, whether or not they tend to be large, whether other cells may have some funny look about them. So it, although we get a report about that, and some people may not have the capability to look, but at the very least, then they should be speaking to the pathologist who's looking and saying and asking the right questions. How does this look? Do you see this? Do you see that? I'd rather see it for myself. But I think all those things are important.

Barbara

Right, right. Well, and the pathology report comes with the CBC and if there is something a little unusual, hopefully the pathologist will make note of that so that, and the physician will see it and maybe lead you in a different direction, or warn, whatever.

Dr. Gernsheimer

And then it's time to have a conversation with the pathologist and say, what is it that you saw? And do we want to send this blood for more tests? There are other tests we might do that'll lead us to a completely different diagnosis. So all of those things are important.

Barbara

They all go together. I know patients, especially when first diagnosed, they suffer from a lot of emotional issues because like we said, that's this emotional roller coaster. And, you know, there's a lot of psychological effect with getting a diagnosis like this when your life has been, what you would say, normal and then all of a sudden you're hit with this. What do you suggest to patients when you realize they're having some emotional issues with this? What would you advise?

Dr. Gernsheimer

So first of all, I think it's important that we just, we have the conversation about what is this going to mean in your life? Okay, because again, as I said, as we talked about in the very beginning, over 40 years of treating these patients, I have seen, and I mean, that leads to hundreds, thousands of patients that I've seen with ITP. I have only twice seen patients die from ITP. Okay. I mean, it's a very, it's very, very rare. This is a rare disorder. Well, think about how rare that is if I've seen thousands of patients and that's only happened a couple of times. Okay.

Barbara

Right.

Dr. Gernsheimer

I think the most important thing to understand is we're going to manage this and we're going to manage this together. And let's talk about whether or not you need lifestyle changes. Again, I'll talk about that hot dog skier that I knew.

Barbara

Right, yeah.

Dr. Gernsheimer

Who we eventually got into a really solid remission and he's out there skiing again. Okay, so you know –

Barbara

Wow, I'm sure he's thrilled.

Dr. Gernsheimer

Right! You know, most patients, we're gonna get you to a place where you're gonna be living your life and enjoying it just as you were before. It's a matter of getting you there. I think it's important to talk to your friends and your family to explain it to them because I once had a patient who I had been seeing for two years and who suddenly burst into tears in my office. And when I asked her, Gloria, what's the matter? She said, my friends say that if I'm seeing a hematologist, I have cancer and you're just not telling me. Literally!

Barbara

Oh gosh.

Dr. Gernsheimer

And I was like, oh my goodness! So, I think, it's very important that as a patient with an ITP, you understand it well enough that you can say to your friends and your family, slow down. I've had a long conversation with my hematologist. I know where this is going. I know what I have. I'm not dying. And this is going to be properly managed. Because a lot of the anxiety is coming from around you as even more coming from within.

Barbara

Right. Right. Right. And a good question to ask your friend would be, “And excuse me, where did you get a medical school?” You know I mean, there's Dr. Google, which really confuses things. And you know, when people throw these things out at you, it's exasperating.

Dr. Gernsheimer

It’s out of love and concern, but you know if you found a physician you can trust, you may need to be educating your family and your friends and bring somebody with you to your appointment.

Barbara

Absolutely. I always suggest two sets of ears for your appointments and you know, it's because what you might hear might be a little different from those other sets of ears might hear and they might catch something that you missed because you were digesting that last sentence and it took you to another place.

Dr. Gernsheimer

And especially if you're anxious, right?

Barbara

Absolutely

Dr. Gernsheimer

If you're anxious, you don't, and if you can't have somebody there, you can have them on the phone. Your physician should be comfortable with having somebody either call in um or Zoom.

Barbara

Oh, terrific. Right.

Dr. Gernsheimer

Your physician should be comfortable with having somebody either call in, or Zoom. And you should be able to do that.

Barbara

And it's important to be able to have someone that is on your side that understands a bit more because so often we as a patient walk through life and people think we're absolutely normal, which I want them to think. But when you get into the discussion of, oh, I've got this blood disease. Oh, but you look so good. Yeah. Yeah, that's because I have long sleeves on and you don't see my bruises. you know.

Dr. Gernsheimer

You don't see my bruises. Exactly. And that, you know, that's actually an issue. I've heard that from a lot of women that it's embarrassing that, you know, you walk into the grocery store, if you've got short sleeves and you're all bruised, they're like looking at you and sometimes, are you okay? Is, has something bad going on at home? You know, they're thinking that maybe there's abuse and they're doing it again out of concern.

Barbara

Right. And they might not even approach you about it. They might just be talking behind your back about it. So, you know, I would wear a bathing suit, but I didn't like to wear a bathing suit in public if I had bruises. So just specifically because of that, you know, I wanted to avoid having those conversations behind my back. But, well, what are you optimistic about when it comes to the future of ITP and treatment and research? That's, I think, an important question to ask.

Dr. Gernsheimer

You know, we're learning more all the time. Just thinking about my own path of research through this, when we originally thought this was a disorder of only platelet destruction, I'm going way back now, and we did some research that taught us, there's more than that going on. There's actually a problem with platelet production in this disorder. And, completely changed the face of what we know about ITP and how we address it. And now, and that changed our approach to therapy quite a bit of, oh, we have something else to work on. It used to be, let's just suppress the immune system, and now we can do things like make platelets actually increase by making the megakaryocyte, that cell that makes the platelets in the bone marrow, make more platelets. So we learned about that balance. As we're going forward now, because that works for a lot of patients, there's a lot of patients who still, we're not quite managing things the way we'd like to. And so we're learning more about the immune system, how we can control it better and control the side effects of suppressing immune regulation. And we've learned more that this is a disorder of immune dysregulation, if it will. The immune system is out of balance, if you will, and how we might be able to better balance it. And as we go forward with this, with more treatments that are balancing the immune system or more regulating the immune system, we will develop more therapies that may be important for patients who haven't responded to the previous therapies and more therapies that have fewer side effects or side effects that we can handle better. What we're really hoping and looking for now is can we put people into more prolonged remissions without having to have chronic therapy or constantly have to renew therapy. And I think that's where we're going now is let's find some therapies that we can treat ITP, particularly when it first presents and maybe get it into more prolonged remissions so that patients can go off and again, live the life they want to live.

Barbara

Right. Absolutely. Well, that's great. I mean, I keep looking forward to see what's coming down the line. What other tools are you going to have in your toolbox? You know, what is helpful for us as ITP patients as far as treatments in the future? So, we did have a couple of questions from social media that I wanted to ask you. Sage Schultz asked, “Hi, I was recently diagnosed with ITP. Any tips you can give a newbie?” I know we've covered a lot of things in this podcast already, but anything in specific, you know?

Dr. Gernsheimer

I think, again, the first thing is develop a good rapport with your doctor. Okay. Talk to your family and friends so that they understand what's going on and decide what are the things that are important to you in terms of how to live your life and what therapies you're willing to accept. And read. Don't go necessarily to the internet and start googling everything. I tell patients right off the bat, I want you to go to PDSA.org. The information there has been curated, it's been looked at by experts, both experts in the medical field and experts in patients who have had ITP for long terms and know more about what it is like to live with this. So that, those are the things I specifically do right off the bat.

Barbara

That's great. And I refer people to the PDSA, of course. But I tell them that you can depend on the information you get there, whereas it may not be as up to date if you're reading it elsewhere or someplace else that you know you can't be as confident in.

Dr. Gernsheimer

It may not even be correct. And that's what I really worry about is, you know, people put all kinds of things on the web that are not necessarily true. And so I like the PDSA because the information they put out is curated.

Barbara

Yeah, I agree completely. There's another question here from Debra 2012. “My count was 1,000 in December of 2023. As of August, my count is 75,000. Am I considered chronic or acute?” Well, I think we covered part of that. It's more than three months of not acute, but persistent or chronic?

Dr. Gernsheimer

Yeah. You know, if it's less than a year, again, it's very important to understand that these are not hard and fast. Well, 90 days just passed, you're now persistent. 365 days have passed. Again, there's not a cliff with this. It's just a way of us making definitions that we can work together as researchers. It's persistent, but that still doesn't mean that it's not going to go into a remission, where I don't know whether or not this particular patient is on therapy, whether or not this has, without knowing that we can't really say, gee, can we look forward to you getting off therapy or, you know, it takes sometimes years to really understand what the ups and downs of someone's course is going to be. So we just have to follow it.

Barbara

Right. And at this point for this person, really, the label is not that important. You know, is it?

Dr. Gernsheimer

Yeah. Put those definitions, again, yeah, they were put together for us to do our research and for us to have some kind of understanding when we talk to one another. But it doesn't necessarily tell us, other than acute, when the first few months when they say, this might get better all on its own, go away and, you know, and I'll wave to you, you know, have a great future, you know, and I want to see you at first every three months, then every six months, then once a year, and then I'll, you know, every few years.

Barbara

Say, bye!

Dr. Gernsheimer

Yeah, exactly. You know, come back to me if you ever need to. You know, so which is my hope for every patient that I see is, I'd like you to forget I ever existed.

Barbara

Right.

Dr. Gernsheimer

That's my hope.

Barbara

I'd rather you see me in the grocery store and say, hey, how are you doing? Not how's my platelet count.

Dr. Gernsheimer

Exactly.

Barbara

Well, I can't tell you how much we've appreciated this because this is such valuable information for anyone going through ITP, and their family, friends that want to understand a bit more about it. So, you have been so terrific. We really appreciate it. And if you want to see Dr. Gernsheimer in person, you've got to come to one of our PDSA conferences because we have these fabulous medical advisors and they come out of the goodness of their heart and they speak at our conferences on different topics, but these are real people. They're approachable. They'll talk to you. They're just, I can't tell you how fabulous they are. So, keep in touch with our website, the pdsa.org. You'll find out when our next conference is. They're always in late July. So, thank you again, Dr. Gernsheimer. We really appreciate you.

Dr. Gernsheimer

Thank you, Barbara. It's really been my pleasure. Take good care.

Barbara

Thank you.

Narrator

How do you live your life with a bleeding disorder like ITP? From working in the kitchen with knives, to navigating sharp corners in your house, going out to eat in a restaurant, traveling on a plane, attending a sporting event, even dancing at a wedding. ITP patient, Barbara Pruitt, shares her tips and tricks for moving through life with ITP for more than 60 years. Here's her lifestyle lesson for the day.

Barbara

Have you had bruises where you don’t know where you got them and you start wondering if they’re happening all by themselves or if you’ve actually hit yourself or injured yourself. Well life gets busy, and we tend to move a little too quickly sometimes, and when we’re in a rush to go from here to there, we’re busy doing five things at the same time. And we don’t stop to think that when we’re walking through that doorway, we hit our shoulder on the molding around the doorway, or, you know, we bump into stuff. And because it doesn’t necessarily hurt when you bump yourself, you can have a bruise later on that you’ll say, what did I do? Because you’re not going to remember it because you’re moving too quickly through life. Well, my advice is to slow down, and move more cautiously. Stay away from anything that would impede your movement. Look for wide open spaces when you’re out walking in a crowded area like an airport or a mall, look for the open spaces so people don’t bump into you. If you’re at a party and there’s a dance floor, for heaven’s sake, dance! Have a good time. But make sure that people aren’t going to be ramming into you, because then, you know, who knows, somebody’s going to be stepping on your foot with their high heel, you’re going to have a big bruise on your foot. So, be cautious, be very aware and alert of your surroundings as you move through life. It’s something that you actually can train yourself to do. You kind of feel like you have this bubble around you, so if you picture kind of a bubble around you, how are you going to move through life, and get that bubble through the doorway and get that bubble in and out of places. If you kind of set that up in your mind, and you’ll find that you move along a little more carefully, you’ll save yourself a lot of headaches, and a lot of bruises in the future. So, that’s my lifestyle lesson of the day.

Narrator:

Thanks for listening to the PDSA podcast, Bruised but Not Broken, Living with ITP. Made possible by our presenting sponsor, Amgen. Special thanks to Gus Mayorga for composing our theme music. To see what's coming up, visit our website at pdsa.org, and subscribe wherever you get your podcasts. Please share this podcast through social media with anyone who you think might benefit from the information and stories we share with the ITP and other platelet disorders community. As always, please speak with a healthcare professional before making any treatment decisions. But know that pdsa.org is a wealth of information and resources to help you navigate life with ITP and other platelet disorders. Remember, you are not alone.