Dr. Donald Arnold

I felt that this was the clear gap in the literature, was a clear gap for guidance and for guidelines, and there needed to be something, at least some sort of roadmap that either emergency room docs or hematologists who may not be fairly well versed or comfortable with ITP management could lean on.

 

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

Greetings. I'm glad you could join me today. Today's podcast is about the emergency management of ITP. This is something I hope you will never need, but as they say, it's best to be prepared. Today's guest is Dr. Donald Arnold. He is a professor of medicine at McMaster University and associate chair of research in the Department of Medicine. He is the co-director of the Michael G. DeGroote Center for Transfusion Research at McMaster and co-chair of the Canadian Transfusion Trials Group. Dr. Arnold is a hematologist. with a clinical and research focus on platelet disorders, bleeding, and best practices in blood transfusion management. A translational researcher, Dr. Arnold integrates clinical research, basic research, and methodology to advance the knowledge in blood diseases such as immune thrombocytopenia. Through the years, Dr. Arnold has fostered the interest of many internal medicine and hematology residents in research. which he considers one of his most important accomplishments.

Well, welcome, Dr. Arnold. As our listeners can hear, you are a very busy man. But before we get into the emergency treatment of ITP, I want to acknowledge that your work is recognized worldwide. The International Society of Thrombosis and Hemostasis, known as ISTH, will be honoring you this year in Paris at their 26th annual Congress with the Harold R. Roberts Medal. This medal is given for your service in advancing the mission of the ISTH, Scientific and Standardization Committee. So, I wanna give you a big congratulations.

Dr. Donald Arnold

Thanks so much, Barbara. That's really that's really kind of you.

Barbara Pruitt

I know that that this will be given in July and that some of our PDSA staff and our president and CEO, Caroline Kruse, will be attending the award ceremony. They will also be attending many of the educational sessions and engage with um clinicians and researchers because the PDSA has a booth at their exhibit hall. So, I know this medal is quite an honor honor for you. So, congratulations again.

Dr. Donald Arnold

Yeah. Thanks so much.

Barbara Pruitt

Now, let's get into the emergency management of ITP guidelines. Because recently, the emergency management of ITP guidelines were published. And I know this took years to compile and complete but they were badly needed. What is it that provoked you to do this?

Dr. Donald Arnold

Thanks. I, you know, this was, um, out of pure necessity, I think, and it comes from, you know, experiencing this firsthand when a patient with ITP would show up to the emergency room with a bleeding event. It inevitably would be a Friday night or Friday evening when, you know, people have gone home and the hematologist on call, which was me, I happened to be, you know, 20 minutes away or 15 minutes, but whatever. So, it's, it, in, in essence, it's like pandemonium and that happens at the emergency room and lots of phone calls being made and who's this. And even when, in fact, I was not on call that night, I remember. But I was still getting the call because the hematologist that they were speaking to wasn't comfortable with that.

And so, yeah, I remember it was actually it was not just a Friday night. It was New Year's Eve. And I was an hour away in Toronto something. And I'm taking a call over the phone. Anyway, it was you know that's just an example and unfortunately not the exception to the rule when these urgent emergency events happen to patients. And so I felt that this was the clear gap in the literature, was a clear gap for guidance and for guidelines, and there needed to be something, at least some sort of roadmap that either emergency room docs or hematologists who may not be fairly well versed or comfortable with ITP management could lean on and say, here's what we can defer to. And so we know what to do at least right in the immediate, immediate term.

Barbara Pruitt

Great. Great. Well, like I said, they were badly needed, you know. Now, when we say emergency and ITP, what do you consider that? How do you explain that? Because there's different types of bleeding symptoms. And how does somebody know when they're supposed to seek emergency treatment?

Dr. Donald Arnold

Yeah, this is a really important question and is the crux really of this whole research program because the risk of doing this is that it could almost get overused, right? And then when things aren't necessarily in the emergency, um patients are ending up getting over treated with a lot of things. So, we were pretty careful even before doing the guidelines, like step number one was to define what is emergency bleeding in ITP? And where we landed with that was First of all, the assumption was that this was a person with known ITP who had a very low platelet count, call it less than 20. So it was, it was, that was definitely the important contributing factor that their ITP was really active. Second was they were having a bleeding event, which we would call severe or emergent. Um, and that was carefully kind of categorized as our first step. So included things like an intracranial hemorrhage. right So you might not know that right away, but someone might have the worst headache of their lives, or they might even be unconscious. They go to the emergency room and there's a CT scan that shows an intracranial hemorrhage. or, um a bleed that might be internal. So, a gastrointestinal bleed that's causing significant blood loss to the point where the blood pressure is low or their pulse is too high. Their vital signs are off or a bleed. Let's say even nosebleed that's causing so much trouble that it's causing them trouble to, to breathe. They can't breathe properly. It's obstructing airways. Like things are critical. We, in fact in fact, use that word, a critical bleed. And it's meant to really differentiate a major bleed, which is pretty bad, but it's not critical. And, you know, a non-major bleed, which is way more common, like bruising, petechiae, even blood blisters, where you're starting to get a little bit more severe. This is the tip of the iceberg, worst bleeds ever. And when they happen, it's like all hands on deck. So that was an important first piece is to really define what we're talking about. And in this case, it's the worst types of bleeds, those critical bleeds.

Barbara Pruitt

Right. So, what you've been describing is actually very enlightening as an ITP patient, um because sometimes you just don't think about, oh, is this the worst headache in the world that I've ever had? Or is this nosebleed really going to stop? Is it, you know, is this worse than the previous nosebleeds? Am I having a hard time you know, breathing or am I swallowing an awful lot of blood? So those are symptoms I think that patients should be quite aware of.

Dr. Donald Arnold

Yeah.

Barbara Pruitt

Now, lots of people with ITP live with a very low platelet count. I know that's myself and lots of people. What kind of advice would you give those patients as far as distinguishing, like, okay, I can wait another day before I, you know, I can call me hematologist in the morning. Or is there anything in particular that you would recommend that they really need to get to the emergency room? Are there any specifications that you would think of?

Dr. Donald Arnold

Yeah, this is a great question too. So first, let me just say that the critical bleeds that we were targeting in this guideline are very rare, like very rare. It's very unusual for that to happen, but when it does, which maybe you know, once every two or three years in our hospital, which is a big catchment period. So very unusual, but when it does happen, we wanted to make sure that whoever's taking care of that person right off the bat is prepared.

Okay. Having said that, your question is more about when should a patient with ITP, proceed to the emergency room or make sure they're getting urgent care right away? Now it's not generally going to be that critical bleed because that's, you know, obviously they're calling 911 or their family member. This is much more of a common situation where let's say a person has known ITP and they, as you say, live with a low platelet count and that's just their day to day. Most of those patients are fairly accustomed to their bodies, okay? And they have a pretty good sense of what's okay and what's not. Now, usually what that means is oftentimes patients with the low platelet count all the time may have bruising. They may have little red dots called petechiae. That's kind of, you know, important, but not dangerous.

Barbara Pruitt

True, true.

Dr. Donald Arnold

The next step up would be what we call mucosal bleeding or wet purpura in some textbooks. And those are things like blood blisters in one's mouth or nosebleed that won't quit or you know GI bleed sometimes that you know looks like hemorrhoidal bleeding or things like that. That especially the blood blisters, that might be just a notch up in terms of severity from blood, uh, bruising and petechia. But even then, if someone knows their bodies and they've had this before and they know it's going to go away and it kind of ebbs and flows. You know, those are signals that they should say, oh I, I may be in trouble here. Like things are going out off the rails a little bit. might, if this is unfamiliar territory to me, I should be speaking to my doctor. If I'm very comfortable with this, I might wait till the morning and give them a call, you know? So, there's a lot of knowing your body and then knowing kind of where things are outside of the normal for you. And that's a process for your doctors also to really understand. It really is hard to answer that kind of as a statement that applies to everybody. It almost is an individual.

Barbara Pruitt

Absolutely. Yeah. And I always am preaching that you really need to know your body and you need to know what is out of the norm. But you've put that very succinctly. And like the blood blisters, the mucosal bleeding, you bleeding is kind of a step up from maybe what you would expect to see on a day-to-day basis other than the bruising and petechiae. And I can't help but think that a lot of this, that what we're talking about, the emergency treatment, would occur maybe after you fell or injured yourself or something out of the ordinary could happen would kind of be a red flag like, wait a minute, this this could be a serious situation. I don't know if something's happening, but if I'm feeling something a little different, maybe this is the time I need to go to the emergency room. Would that be correct?

Dr. Donald Arnold

Yeah, I think that's fair. So, you know, a trauma or some sort of head trauma or whatever would be a danger signal for sure. um you know, there are times when, you know, these things do happen even without trauma. And so, a spontaneous, we call it a spontaneous intracranial hemorrhage or a bleed in the brain. It usually happened almost always. There's something proceeding that though, the petite guy might come first and the bruising might be more just diffuse than the blood blisters. And then maybe even there's blood in the urine or something like that. This is like, this is not right. I mean, now it's time for me to speak to my doctor or go seek help right away before it gets to that. And again, getting to that is pretty unusual. There's usually a lot of warning signs, but you kind of want to heed them when they happen outside of the ordinary.

Barbara Pruitt

Absolutely. I can see what you're saying. So, if you're already kind of in a; you know that you're a little more fragile than normal, and then you start to get more symptoms. That's a red flag. That should be a red flag. And that's why patients need to be so aware of themselves. I know life gets busy and we don't examine our bodies every day, but you really need to kind of keep tabs on, is the petechiae worse? Am I bruising more? You know, as a patient, that's really our responsibility to do that.

Dr. Donald Arnold

You know, also there's the new patient who's not accustomed to having ITP. And when, when it develops for the first time or they're, you know, their doctors meeting them for the first time, we're as physicians anyway, are going to be tend to be more aggressive. So if this is someone who has new ITP for the first time and we're seeing their platelet count is 20 or 19, we're probably going to intervene at that point because we don't have any familiarity with how this is going to go for that particular individual, you know, and, and sometimes, you know, treatments upfront or helpful long-term. So, it does depend on how long ITP has been around. If it's brand new, we're still unknown. If it's been around for years and years, and you've been living with the low platelet count and you're fine. Then you know the bar is set a little higher.

Barbara Pruitt

Well, I've kind of noticed that patients that have had ITP a longer time don't get as hysterical and as concerned as newly diagnosed patients. And in some ways, I've kind of thought, and maybe this is wrong, I've thought of a newly diagnosed patient as being more fragile than a person that has had it for a longer length of time. But I don't know that the word fragile is really appropriate. Would it be better to say that um a new patient with ITP is going to be treated more aggressively because it's kind of unknown what their course is or going to be?

Dr. Donald Arnold

I think that's fair. I would use the word uncertain, maybe not fragile, but uncertain because we just don't know how that's going to turn out for that particular individual. And you're right that the ones who have had ITP for a long time are usually very well versed in their bodies. And in fact, sometimes what happens is, you know, they might get a new family doctor or something and They send that family doctor sends them to the emergency room right away.

And then they show up there. And meanwhile, they're the ones that patient is teaching the emergency room docs kind of what to do and what not to do. They say things like, right.

Barbara Pruitt

Oh, I've been in that situation a number of times, you know?

Dr. Donald Arnold

They're like, calm down. I'm going to be okay. Don't worry.

Barbara Pruitt

I have actually said that and my husband knows what to say too. Listen, you're going to be hysterical when you see her platelet count come back from the lab, but believe me, This is her normal. We're here because she has a sore throat or something else.

Dr. Donald Arnold

That's right. Exactly.

Barbara Pruitt

That's why I'm excited about these guidelines that you've developed. But I have a question. When we go to the emergency room, should we feel confident that the emergency departments have these guidelines? Are they available or how do they get them? Or do they look for them? Or how does that all happen?

Dr. Donald Arnold

David Price- You know, this we're struggling with this part right now actually our team of you know doctors and researchers, we're trying to address that question right now, which is okay. David Price- Good on us we've made these guidelines we've published them there was a ton of work that went into making them. Now we need to get them into the hands of the people who are seeing these patients. And the challenge there is that it's very rare. Um, as I say, like the critical bleeds and patients with ITP happen once every two years in our hospital. And we're a catchment of 3 million plus patients in this region. So how do you get this information into the hands of people who need it and only see it very rarely? So, we're working on that. I think it's a combined effort where, you know, the emergency room doc or emergency department is, is one person who's going to see this person, but there is no world where that physician is not calling a hematologist as soon as they see the blood counts come back in that situation. So, we need to get this in the hands of hematologists also who are perhaps a little bit more comfortable, but as, but still may not be very comfortable in these situations. And there's a whole science around, you know, knowledge translation and how to how to make sure the work that you do is meaningful and gets taken up in practice. This takes on even another layer because of the rarity of it, but we're trying to work through that. So, I'm optimistic, but I don't know the answer to your question just yet.

Barbara Pruitt

Well, that's good you're optimistic, you know, because the guidelines, I know that that the American Society of Hematology has published them, but are there emergency department journals also that do that? I don't know. I don't know at all. Is that something that I would expect to happen?

Dr. Donald Arnold

I mean, you know, in, in today's day and age, um, a doctor who's in that situation is not going to pull up a journal. They're going to pull up their phone or their open AI or whatever, you know, database that they go to. So, we kind of got, have to meet them where they're at.

And so, I think that's part of our challenge is how do we get this into the right media medium so that people can access it quickly. and I don't know the answer, but we're working on

Barbara Pruitt

Okay. But like you said, you're optimistic, which I am too. At least they're there and they've been established.

Dr. Donald Arnold

Yeah. And the other piece of this, of course, is that PDSA does a great job of raising awareness about these guidelines for patients. And so, it is conceivable to me that it could happen that a patient comes into the emergency room, again, teaching their emergency doc about a lot of things, including please access these guidelines that are really helpful and they're very succinct. The first page tells you what to do. So, you know, those types of scenarios may happen, but I would not want all of the onus to be on the patient. So. Right.

Barbara Pruitt

True. True. I get that. And emergency room doctors generally know how to treat ITP patients. And we have to realize that, you know, the situations that they're confronted with in the emergency room are variable. I mean, so varied, there's thousands and thousands of different situations that could come in. So, it's hard to say, oh, you should know all this. You should know all this.

Dr. Donald Arnold

Yeah. Right. Yeah.

Barbara Pruitt

But it's good to know that nowadays with the internet and everything, they can get information quite quickly. It does put us in a difficult situation as a patient, but again, knowledge is key. And, you know, like we talked about, oftentimes patients are educating the doctors. One thing I want to talk to you about is the surprising situation about platelet transfusions. I know that generally speaking, we don't get platelet transfusions. It is not this type of treatment that is given for standard care. Explain when it is needed or when it is used and why should it not be used.

Dr. Donald Arnold

Right. When I think about treating ITP, it all comes down to how urgent is it that I get the platelet count up even a little bit. If it's really urgent, that means within the next few minutes, I really want the platelet count to come up. Even a little bit. The only thing that's going to do that is a platelet transfusion. Now a platelet transfusion, you're right. You typically; you give it to someone with ITP, you measure their platelet count an hour later. It's exactly the same as it was before. It had no meaningful increment to their platelet count.

But in that short period of time, when platelets were infusing that the platelet count was higher. And if you give enough, sometimes it's like two or three or even four units, you can boost the platelet count temporarily, but Just for a brief period of time and hopefully that required a time is all it takes to get the bleeding to stop okay so that'd be kind of urgent thing number one if time was absolutely critical and you wanted something to happen within minutes, if you want something to happen within hours. been like 12, 24 hours.

IVIG is probably the next best thing because it's going to work fast. But again, 12, 24 hours later, it may not last very long, but it could last days or even weeks in some patients. If you only need to get the platelet count up in the next few days, you may use something like steroids and even high dose steroids. Or you may use something like a thromboquedin receptor agonist. We think about end plate because it's injectable or some of the oral pills, they take a few days to a week or so to really kick in. So, If you think about it that way, almost like a time-based treatment approach that was a lot of the rationale that went into, you know, kind of coming up with guidelines for emergency management.

Barbara Pruitt

Okay. And in regular treatment of ITP, the reason you don't give platelets is why? Could you explain that? Not in an emergency treatment, but in the regular treatment.

Dr. Donald Arnold

Because they just don't last. Whatever's happening in the ITP patient body, which includes antibodies that are binding to platelets and clearing them right out of circulation. All of that is happening to the transfused platelet as well. So as soon as you give it something is just clearing it out of circulation. And as I say, even if you were to give a platelet transfusion, And then one hour later in a person with ITP who's otherwise fine measure it the platelet count one hour later, it probably would not have budged at all.

Barbara Pruitt

Wow.

Dr. Donald Arnold

So, it's not going to make any significant difference over the span of time, except maybe in the first few minutes or hour. And if, if there's a situation where you just need the platelet count to be up a little bit in that first hour, that's when platelet transfusions may be useful, but otherwise they're, they're just, they don't work.

Barbara Pruitt

Otherwise not. Yeah.

Dr. Donald Arnold

They just don't work.

Barbara Pruitt

Does it put your immune system into overdrive because they're getting all these platelets boosted you know into your system? I guess in some ways it does if the platelet count's really staying the same.

Dr. Donald Arnold

I'm not sure if it makes ITP worse. Like, I'm not sure if it makes the immune response worse, but it can stimulate the immune system for sure. And I don't think there's any benefit to that. There's downside risks to play with transfusions too. Like these are products from other donors. There's potentially an infectious risk, et cetera. So, there's really no utility to doing it outside of that emergency, critical bleeding.

Barbara Pruitt

Okay. I understand that. Now, the guidelines kind of walk the doctors through what they need to do first or second or third, depending on the situation. Is that what the guidelines really kind of help with?

Dr. Donald Arnold

Yeah, we took the approach of, okay let's level the playing field and say, this is a critical bleed in an ITP patient. The point here is you're doing everything you possibly can as soon as possible. And the guidelines went through the literature on this, but at the end of the day, came up with a bit of a recipe to say, do all of these things at once. Right. Then there may be a bit of a pause to say, is this working or not working? And if it's not working, I think about kind of phase two. But if it is working, maybe you're okay. So, it's almost the worst-case scenario and it's giving doctors, some approach to the worst case scenario.

Barbara Pruitt

Good. And there are there's testing done in the interim, depending on what is happening as far as CAT scans and such, correct?

Dr. Donald Arnold

For sure.

Barbara Pruitt

When they're indicated, when they're needed.

Dr. Donald Arnold

Yeah.

Barbara Pruitt

A doctor I saw once told me that I should have an emergency plan in place before a crisis occurs. How do you feel about that? And what do you envision that to be?

Dr. Donald Arnold

Is that like having a rope ladder outside your window in case there's a fire or something?

Barbara Pruitt

Yeah, I guess so. She compared it to um a patient that has asthma and is having an asthma attack that they should have something on their refrigerator so that when they call 911, that information is available. And she suggested that maybe that's something I should do.

Dr. Donald Arnold

I think that's ah that's a big ask to have patients come up with an emergency plan. um Most of the treatments that we're referring to in the guideline for critical bleeds are, are medicines that are held at the hospital. Patients generally don't have those, you know, may, might have a tipo drug at home, but If this does require kind of a blood bank and a coordinated effort, I think it's what's more important, I think, than having an emergency plan ready is being aware of the signs and symptoms that would say, I need to get urgent care right now. And, you know, we talked about some of those before, but if the bleeding is more than just your usual, or the blood blisters are getting worse, or there's, you know, bleeding in the urine or a nosebleed that won't quit. Like those types of things say, okay, now I got to get some help right now. So, the important kind of safety net is for patients to know when are those things, what are those things and when they have to kind of seek urgent care.

Barbara Pruitt

That's true. I agree with you on that. And I also have to say that um sometimes we don't always see what's going on and ah in ourselves, whereas your caregiver, your partner, um other people around you might notice things are different. So, we need we need to depend on their input also. um as far as caregivers looking out for these signs or symptoms that we've been talking about. Don't discount their voice if they're seeing something, you know, like if it's a bad nosebleed, well, wait, are you having difficulty?

Dr. Donald Arnold

Absolutely. Yeah.

Barbara Pruitt

Noticing that they're having difficulty breathing or it's obstructing their airway or something like that, which is good. Since the guidelines have been developed, what is your hope? I know you said you're optimistic, but do you think things are going to change? Hopefully in the emergency care for ITP, I guess my hope is that it, reaches not just the big hospital emergency departments, but that it can reach all of the rural ones also.

Dr. Donald Arnold

Yeah, I am certain things will change for the better now that these guidelines are out. So, at very least, if I or someone else were to get that call on New Year's Eve, and we're not on call, but we kind of know a little bit about ITP, now the answer could be, go to this website. you know, here's the resource. Look up this piece of paper. It's going to tell you exactly what to do. And it doesn't have to be you know someone who's well-versed in ITP. It could be anybody who's finding the guidelines. Now, that's the hard part is finding them in that moment, but at least we could direct people to it. The bigger challenge where we were talking about the beginning is how do we avoid them having to find it? Like, let's put it right in their pockets or they just have to type a couple of key words and up pops this guideline.

So, it's there right now. It takes a little bit of work to pull it out. If we're really good Sometimes, we could push it to them if, you know, we just get the signal that that's what they're looking for. So, to answer your question, I think this has already had an impact. And I'm very optimistic about that and pleased about that. Do we have more work to do to make this more accessible? Yes, of course we do. But this has been an important advance.

Barbara Pruitt

And it's important that ITP patients are listening to our conversation here because it's giving them information on, you know, hey, there are guidelines out there for emergency treatment. And I know for myself, it makes me feel better that they're at least there.

Dr. Donald Arnold

Right. Right.

Barbara Pruitt

Hopefully I'll never have them used, but it does make me feel better that they're there. um Now I carry in my wallet in, you know, a card that says that I've got ITP. Are there things that you recommend for patients that they need to do to help protect themselves if they run into a situation? Let's say that they're unconscious and you know people don't know.

Dr. Donald Arnold

I'm of two minds about the medical alert or those types of bracelets. Sometimes, you know, in one way, I think it is helpful to have a medical alert that says I've got ITP. On the other hand, sometimes having a medical alert bracelet um has a psychological consequence to the patient. Also, it says, oh gosh, i I'm kind of chained to this thing and it's a part of me. And you know now this takes on a whole new meaning because most, you know when let's say someone is unconscious, they come to the hospital. The first thing that's gonna happen to them is they have what's called a CVC or a complete blood count. And the doc's going to know right away that their platelet count is two. And that will clue them into a path, you know, that goes to treating ITP or all the other things that come with a low platelet count.

So, there's not going to be much mystery about the platelet count being low for very long.

So, to just say getting a people should get a medical alert, I think is a bigger conversation. It's not just go ahead and do it. It carries some psychological consequences too, that may not always be necessary. So, I, again, I think that's a discussion that someone should have with their doctors, but it's not a blanket statement.

Barbara Pruitt

You know, you brought up an interesting topic because I've never really considered the psychological consequences of that, but it does kind of make you feel like, oh, darn, I've got something weird. You know, I mean, and I have felt for years, you know, like I was dealing with ITP on an island because I never had met anybody else with it. And you do suffer kind of psychologically, right?

Dr. Donald Arnold

Yeah.

Barbara Pruitt

And people in a rural area with ITP, they might never ever run into somebody else with it because we are a rare disease. So, I hadn't considered the psychological aspect of it. I've always thought, oh, I've got to be proactive, proactive, make sure I have, you know, I don't always wear a bracelet um or a necklace, but if I know I'm going to be alone by myself or something, I'll do that. I'll wear it.

Dr. Donald Arnold

We encountered this in a previous study that we had done a while ago around you know patients' attitudes and beliefs, I guess, around splenectomy. Because splenectomy is such a charged issue for some people. And it ends up being a lot of discussion. It's not like doctors; They follow guidelines. We'll say, now it's time for your obstinate to me. It's more of a, well, it's an option. What do you think? You know, like it's the onus ends up being on the patient a lot about making that decision. And what we learned just from speaking to people who were faced with that decision was a lot of them did not have the supports that you were mentioning at home. They'd go to their families and say, I have ITP and their family says, what's that? And then they'd say, well, then the doctor wants me to take my spleen out and they'd say, what does that have to do with it? It just seems so there's not a familiarity. It's not like saying I have breast cancer and I need a lumpectomy. Everyone understands what that means and there's twelve different support groups that you can go to that'll help you with that. ITP is not the same. You're kind of on your own. And that's why, you know, PDSA has been so important for patients because it's a place where you can find people who are going through the same things.

Barbara Pruitt

And it's actually a wonderful feeling. And it sometimes I think it's hard to explain because people think, oh, I'm strong. I can handle this myself. I can get through this. I'm reading up on it. I'm learning things. But when you meet another person that has actually been there and it makes such a difference personally. And I tell people that you know when you go to the PDSA conference, you feel normal because all these people around you have the same thing. And even though our journeys are different, there's so many similarities in our journey and you don't feel alone anymore. So, I agree with you. I think a support group like that and association is really quite important for a patient to reach out to. Now, when we're talking about children, I know I think our conversation has predominantly been about adults that are aware of their condition, but when we're talking about a child with ITP, are there certain considerations that a parent needs to think about? Because sometimes they're not verbal enough to inform you what's going on.

Dr. Donald Arnold

Well, I'll just say that the guidelines that we developed apply to both adults and children. So that's good news because the child has a critical bleed, which again, very unusual, but at least there's some guidance on what to do in that rare situation. Kids, uh, you know, tend to rebound from these things, uh, even better than adults do their platelet counts tend to get better. They tend not to bleed now. I've used that word, tend, a lot. That means there are exceptions to the rule. There are some kids or children with ITP who end up with very low platelet counts that stick around for a long time and they have bleeding symptoms and they need treatments. So again, really hard to say any blanket statements about this, but parents are really good at knowing their kids. Kids are pretty good at knowing themselves too. And between the two of them, usually you can say, this is outside of normal for us and we need to go get this checked out. And, and there again, there's some advocacy that's happening at the emergency room level. And even with hematologists that might meet the parents and the child for the first time say, you know what, we can just wait on this. And the parents might have to say things like, you know, we've been through this before and this is different like there's something unusual about this nosebleed or there's something unusual about all these blood blisters that they don't usually happen like this. And so those types of statements, even though they're pretty soft, there's nothing you can measure necessarily about them. They are very important for doctors to hear and it does make a difference sometimes. So, you know, it's a bit of a gestalt, but we rely on parents and kids tell us.

Barbara Pruitt

Yeah, and I think you're right. An observant parent, whether it's a mother or father or even a caregiver, does know these little nuances that might be a little different and relaying that information to the emergency department would be, like you're saying, very important for them to hear. Is there anything else you can think of that that you want to share regarding this discussion for people living with ITP and going to the emergency room? I think we've covered an awful lot of material here.

Dr. Donald Arnold

Well, I do also want to acknowledge you, Barbara, and a few others who were on this research program with us investigators and doctors. And it was very enlightening and really important for us in formulating these recommendations to hear from the patients and the parents about, you know, what, what is important? How should these things be prioritized?

What are the outcomes that we really need to be paying attention to? And that, um, I would encourage as many other investigators do, but I encourage other investigators to make sure that they are including kind of the patient perspective and in their research, it makes a very big difference and it makes taking it up in practice a lot easier because you've got the buy-in already from at least some, you know, patient partners might not be everybody, but at least you've got that perspective there. So, I'm very grateful for your help and others who have put help us put this together.

Barbara Pruitt

Well, we're appreciative of the fact that you want to hear the patient perspective because after all, it's we're the ones that are going through it. And you know I think that's been a wonderful thing that's been included in the more recent research; more recently is to have the patient perspective and the patient voice heard. So, I thank you for including me and the others on the committee that did that.

I have to say this information has been great. For any of our listening audience, I hope I hope this has given them a feeling of ah of assurance that things are people are out there looking out for our welfare, and we appreciate that completely. um And I feel better knowing that there's these guidelines available. So, thank you for all the work you did in creating these. I know it was a labor of love, and it took years to put together.

But one thing before we go, I would like you to tell us about you, you hold an ITP Summit every two years. Can you tell me about that? What, what does this involve? This is big pat on the back for you, but what, what is it that you do every two years?

Dr. Donald Arnold

Right. We, you know, we're trying to raise awareness about some of the mysteries of ITP. And, you know, if you've been following the literature on ITP for the last; it's gotta be 50 plus years, maybe more. One of the themes that emerges is that we simply do not know what causes this. We have lots of theories and you know, some good postulates about what's causing the platelets to be low in most patients. But we don't we haven't really gotten there yet. I think we're missing something. And there's and you know the more I look into this and the more we research this, the closer I think we're getting to something that maybe at the end of the day, maybe it's a diagnostic test that eventually we could say, you know what, this person has ITP and this one doesn't, or this person is more likely to bleed and this one isn't, you know we are, we are behind on figuring that out yet. And so what keeps me going is to try to stimulate our group and others to think about um What is it that we're missing in ITP and what's parallel to it happening in other disciplines that maybe we could learn from?

So, for example, we had one conference on and what's the difference between primary ITP and secondary ITP? Maybe we have something to learn from that. What's the most recent one was let's compare and contrast ITP and warm autoimmune hemolytic anemia, which is the same kind of disease just happening in red blood cells, but lots of differences with the anemia disease versus ITP. And maybe we can learn from that. And so, the theme of the ITP Summit is to try to understand what it is that we're missing, maybe by learning from other examples. It's meant to stimulate researchers thinking it's meant to engage patients. It's meant to engage even um industry partners who have, you know, a lot more resources than us academic folks and getting them to think about maybe we should be pursuing this line of research so that we could make things better for patients at the end of the day. That's essentially the crux of it. And it's been a really rewarding experience for sure.

Barbara Pruitt

It sounds like a think tank where researchers are physicians come with their ideas or possibilities. or um it sounds great. I mean, it sounds like there's a lot of growth possibilities with all that consideration.

Dr. Donald Arnold

Yeah. I think it's led to some interesting ideas. I hope it leads to more. We haven't quite cracked ITP yet. Maybe when we do, we'll stop holding the Summits. But are now but for now, I think we've got our work cut out for us.

Barbara Pruitt

That would be great. How long have you been doing this, the ITP Summit?

Dr. Donald Arnold

We just had our third one. Well, that was last fall. So, every two years we're doing one. So next fall would be the next one. I don't quite know what that topic is going to be yet, but we're planning on doing it again.

Barbara Pruitt

Well, that's terrific. Well, I want to thank you for being here today, for participating in this. Like I said, we've got lots of information here, and it's so wonderful to know that you're also on our medical advisory board at the PDSA, and we're blessed with such brilliant minds and doctors that really care about us as patients, and we're very thankful for that.

And thank you. Thank you for joining us today.

Dr. Donald Arnold

Yeah. Well, thanks so much. It was a real pleasure talking to you and I'm happy to continue the conversation at a time too.

Barbara Pruitt

Thanks.

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 Pruitt

You know yourself better than anyone. You know your body better than anyone. That is why it's important to speak up when something changes or doesn't feel quite right. If you have a spouse, partner, or friend that may notice something about you, listen to them. Maybe it's information that you need to share with your doctor. If you have a child with ITP, I'm sure you watch them like a hawk. You are their advocate. You need to share any nuance that you may see in your child. It could actually be something quite significant. Your voice needs to be heard. What you share with your doctor could affect the course of your treatment. So don't be shy. Speak up. Well, that's it for today. Thanks for listening to Bruised but Not Broken, Living with ITP. I hope you will join us next time. Until then, I'm wishing you lots of happy, healthy platelets.

Narrator

Thanks for listening to the PDSA podcast, Bruised but Not Broken, Living with ITP. Made possible by our presenting sponsors, Amgen and Sanofi. 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 care 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.