A Patient and Her Daughter’s Personal Journey With TTP

HELEN: Welcome to Talking About Blood. I’m Helen Osborne, host of this podcast series and a member of the advisory board for the Blood Project. I also produce and host my own podcast series about health communication, and that’s called Health Literacy Out Loud. Today, I’m talking with three guests about a rare but life-threatening blood disorder that is commonly referred to as TTP. One of the guests is Lisa Ferguson. She’s a business development coordinator who has worked in the mortgage lending industry for over 20 years. Lisa was also diagnosed with TTP in December 2024. Now in remission, Lisa is passionate about raising awareness about TTP and its impact on patients and their support systems. Cara Ferguson is a prevention coordinator whose work focuses on prevention education about substance abuse and problem gambling. This builds on her undergraduate degree in American government and public policy. Cara is Lisa’s daughter. Brian Carney is a hematologist and apheresis physician at Beth Israel Deaconess Medical Center and an assistant professor of medicine at Harvard Medical School. Brian has a strong clinical and research interest in TTP. He’s also part of the medical team treating Lisa. Welcome to, all of you, to Talking About Blood.

LISA: Thank you.

DR. CARNEY: Thank you.

CARA: Thank you very much, Helen.

HELEN: It is unusual, a treat to be hearing about a disease, a medical situation from different perspectives. Lisa, you bring the perspective of someone who is living with this condition all the time. Cara, you’re part of the caregiving group. You’re your mom’s daughter. You bring the family perspective. And, Brian, you as a physician looking over all of this. How wonderful to come to terms and have some kind of a shared understanding about what this condition is like from three quite different perspectives. So let’s start from the beginning. What is TTP?

BRIAN: So I can start. TTP is an acronym for thrombotic thrombocytopenic purpura.

HELEN: Okay.

BRIAN: And it’s a disease where the body’s immune system attacks one of the key proteins for blood clotting, which results in increased bleeding risk, increased clotting risk, and a very dangerous situation if treatment isn’t started pretty much immediately.

HELEN: Okay. So you know it’s serious. You can say this big, long word of what this condition is. Lisa, it’s you. Tell us your story about how this situation unfolded.

LISA: Well, unfortunately, I’m not quite sure. I knew I was having some issues, some health issues. I had been to my primary. They ran some blood tests. Nothing really stood out. I then was just lethargic and just felt terrible. I mean, just, just was in bed sleeping all the time. My daughter actually was the one to pick up on the fact that when she came to check on me one day, she saw that I was jaundiced.

HELEN: Jaundice?

LISA: Jaundice, very yellow, and I wasn’t coherent.

HELEN: Huh.

LISA: And, um, she immediately took me to our local hospital. From there, I basically collapsed in the garage, and they took me in and immediately started to give me blood transfusions as they saw that, after they ran their blood test, that my red blood count was extremely low.

HELEN: Okay. I’m gonna just put a pause on that one-

LISA: Sure.

HELEN: … because I want to be looking at kind of that moment of going from you’re fine or you’re, you know, you deal with life’s issues, I guess, to all of a sudden you’re p- not feeling well, kind of like a little worse than usual and then much worse than usual. Is that correct?

LISA: Yes.

HELEN: Okay. And then, Brian, you haven’t yet entered the picture on this one. Cara, you notice something that’s particularly alarming. What’s it like for you from your family perspective?

CARA: Absolutely. So one of the things that I first thought when I saw my mom was I truly thought she was having a stroke because all of the signs and symptoms that she was presenting were speech abnormalities. She couldn’t hold a coherent conversation, like she said earlier. We couldn’t engage in a lot of dialogue. She was very jaundiced. Her face was just not normal. So I, for some reason, you know, had been taught the F.A.S.T. acronym, and all of that was lining up.

HELEN: Okay. The F.A.S.T. for stroke, okay.

CARA: Yes. But it wasn’t until we got to the hospital that a physician came into the room and said, “We’re talking to the Beth Israel because we suspect it could be TTP because…” And I didn’t realize what a normal platelet count range was, but it wasn’t until I got there that they told us her platelet count was three when typically it ranges anywhere from 150 to 400 for the average person.

HELEN: Oh my goodness. Okay. So you’re in this emergency situation. You’re at home or something, and then everything starts swirling all around you. And somehow, Lisa, you get to a hospital. I don’t know, Cara, are you with your mom? I assume that you are. So now you’re in some medical setting. And then, Brian, when did you enter the picture? What happened now that you’re in the medical situation?

DR. CARNEY: I entered the, the picture several days later, so I’ll defer this part of it to, uh, Lisa and Cara.

HELEN: Okay. So let’s build along the story. So you’re in the hospital. Something is definitely wrong. Lisa, are you so sick that you are kind of not aware of what’s going on?

LISA: Correct.

HELEN: Okay. So, Cara, take the story from there. You are observing, you’re part of this and it’s your mom. So tell us what is that like now that you’re in this medical setting?

CARA: It definitely was scary at first just because obviously with anyone’s parent or loved one, you know them at their normal and she was definitely not at that. I will say that the hospital staff, when we first went to the emergency room, they were very reassuring and they were very quick to initiate communication. But it took me a while to even understand the severity of TTP and how much of in a crisis position we were in. So when they realized that her platelet count was at three, they started blood transfusions, but her platelet count did not increase in the way that they wanted to see it progress. So that’s when they said, “We might be transferring her to the Beth Israel just because they have the machine, the technology, and also the staff training to administer a process called plasmapheresis that they wouldn’t have been able to at the hospital where we went initially.”

HELEN: Okay. I’m aligned with you. I mean, we’re all faced with these awful situations. You went from your mom being okay to your mom seriously not being okay. Now you’re in one place. Certainly, the validity is she’s really not okay. But now they’re throwing words at you: TTP, Apheresis. I mean, gigantic words. Maybe she needs to go somewhere else. How quickly did this happen?

CARA: It snowballed within a 24-hour period. We went in on a Friday afternoon, and we were at the Beth Israel being transferred that next day, and then that is when we met Dr. Aird. He was the first, you know, professional at the Beth Israel that we made contact with, and he explained to us what the plasmapheresis process entailed. So, the way it was initially explained to me was it’s on this almost dialysis-like machine, and it’s like a huge oil change for your blood. So, for me, that created a very strong visual to understand that there was something in her bloodstream that needed to get out, and they needed to do it in a very, on a fast timeline. But the only hiccup was, for TTP, they have to send this test out to a very specific facility. So, even though her initial admission date was on a Friday, we didn’t get an official diagnosis until that Wednesday. What was very critical was the fact that Dr. Carney, Dr. Aird’s team, was very proactive and started the plasmapheresis as soon as they suspected it, because it was my understanding that it would only benefit her, and even if it wasn’t TTP, if it was another autoimmune blood disorder that was similar to it, it still would be beneficial to a certain capacity.

HELEN: Did you have to give consent? I don’t know if, Lisa, if you were in a situa- able to give consent. Or, Cara, were you the one who needed to do this for your mom?

CARA: I was the one who, um, had to give consent. I mean, she was present in the room, but, Mom, you want to speak to that?

LISA: I mean, they may have asked me. I do remember them asking me to, that they needed to put ports in my neck. But that, as far as I remember, that’s the only thing I consented to. Cara really handled it from there, because I… Even if I was coherent, it was only for seconds, and I wasn’t able to understand what was happening around me.

HELEN: And, Cara, for you to give consent, it sounds like this is a very dramatic situation that you’re dealing with. There’s, you talk about these big machines that are coming in. You’re being told it’s an emergency. You’re being thrown at by numbers. This doesn’t sound like your everyday world up until this moment. It all is unfolding so quickly, and you don’t even know for sure that this is what’s going on. Can you explain what that was like to give consent, and then what it was like seeing your mom with all these scary things happening so quickly?

CARA: Absolutely. So, I will say, the saving grace was all of the team members, from the doctors to the nurses to anyone that really came into the room, they were so thorough at understanding, you know, what the process was and explaining that to me. The one takeaway that I had was, you know, there are some risks that are involved with putting in a central line, of course, but this was the gold standard treatment, that if, if this was TTP, time was of the essence. And it really wasn’t until I got to the Beth Israel that I realized that time really was of the essence. So, while it was obviously scary to see, um, you know, the lines being put in and seeing my mom for, you know, almost three weeks going through this process, I knew that it was very much something that we had to take day by day. And she had the best team, who had, you know, the highest level of training, so they really provided that comfort and that support and were very thorough in explaining the whole process. They were even showing me slides of her red blood cells, and I said to Dr. Carney one time that, you know, initially it looked to me like her red blood cells looked like Pac-Man had came along and bit them and ate them apart. So, I knew that obviously that’s not what a red blood cell is supposed to look like, so I left it to the professionals, and I knew my role was just to support them in any way that I could, to try to walk into that room every day with, you know, the highest level of understanding, and, you know, to look out for my mom, and to, you know, be the expert of her body and her normal whereas they could be the experts of TTP.

HELEN: I love the way you phrased that, “Being the expert of her body and her normal.” Wow. Okay, Brian. Somehow, now you enter the picture. Tell us about that.

DR. CARNEY: Yeah, so I entered the picture a couple of days later. We still didn’t know that it was TTP. We were still waiting on the lab result, which actually we have to send to Wisconsin. We send this lab, and they run it in Wisconsin, so it’s a several-day turnaround. In the meantime, we need to continue, because just with the suspicion of this disease, the risks are so high, we treat it empirically. We treat before we know about the diagnosis. So, we continued for a couple more days, and then I think, Cara, as you were saying, by Wednesday, uh, I was able to tell you that TTP was confirmed.

HELEN: So, now, it’s Wednesday. We’re nearly a week into this. Share from all your perspectives, please, what’s happening now, now that you are being treated. Lisa, are you better immediately? Does this take a long time? Cara, can you catch your breath from all this? And, Brian, is this something you do routinely? So, I, I have a feeling that you all have, each have very different perspectives about what’s like when that acute emergency is being handled.

LISA: Well, for me, from my perspective, the pheresis team came in every day and gave me a treatment, and that happened for 19 sessions. So, I could feel by probably the second week that I had, like, turned the corner, that I felt much better. My platelets, you know, had gone up, not a lot, but they were doing so much better. And I just needed to stay with thetreatments and just be patient, and I could feel myself each day getting better.

HELEN: Good.

LISA: The team was… Uh, I mean, I can’t speak enough for the team that they came in every single day and updated me. You know, it, you just have to be patient.

HELEN: Well, you are a patient – … and you are a patient patient. For those of us who are not familiar with TTP and its treatment, what happened during this treatment? You said you had it 19 times. What  was that like?

LISA: A team came in. I, I had a, a fabulous apheresis team. They would come in every day, and they would use the ports that were in my, uh-

HELEN: A port?

LISA: I think that’s what it’s called, right, Dr. Carney?

HELEN: Oh, you’re pointing to something in your neck.

LISA: Yes.

HELEN: So, they would insert something into your body through this port that had been connected already.

LISA: Correct.

HELEN: Okay, so they put something in. Is this uncomfortable? Is this okay? You’re just lying there? Does it… What’s it like?

LISA: This whole process from start to discharge, I have not felt one ounce of pain. It was seamless.

HELEN: Okay.

LISA: They would come in. They would start the plasma. So, they would, so the plasma would start to enter my body. It took between an hour and a half and two hours for the entire treatment, but at the same time, it was taking out the bad plasma. Like I said, each day, you felt a little better. Um, it wasn’t right away, but it, definitely took some time. But again, you know, that’s what you have to do when, you know, you want to get better. You just have to be patient and, and deal with the treatment.

HELEN: So, from this point, from the time you had your medical emergency to starting to feel better, it was starting to work. And each day, I mean, you were so much farther along from when you described yourself as nearly incoherent, so-

LISA: Yes.

HELEN: … yes, it’s working. Cara, you’re watching this.

CARA: Yes.

HELEN: And it’s your mom.

CARA: Yes.

HELEN: So, tell us what it’s like from your perspective.

CARA: Um, I would say probably those first couple of days until we got the diagnosis, those were the hardest, because I think it’s one thing when you have a diagnosis and you know for sure that the treatment path you’re on is the correct one to be on. It’s a whole other ball game when it could be TTP or it could be a myriad of other things.

HELEN: Okay.

CARA: Um, so I would be leaving the hospital and then going home, doing research to try to come in the next morning and be well-equipped. I would sit by her side as long as the visiting hours would allow me for. She was hooked up to the plasmapheresis machine for several hours a day, in addition to other medication she was being administered through an IV. Um, I will say that we don’t know when it happened, but at some point during this whole process, she did have a stroke, so that was another thing-

HELEN: Ooh.

CARA: … to be mindful of, just because that was something that I learned about is when you have TTP, you are at an elevated risk for having a stroke. So, that was another thing was also working with her to, you know, try to keep her feeling safe and secure while all this was happening, but also, you know, wrestling with the fact that her memory was not as sharp as it maybe was prior to this. There were a couple of times where we’d be having a conversation, then we’d have to repeat the whole conversation. So really just, you know, also having patience with her and trying to explain everything in as much plain language as possible. But like she said, it wasn’t really until two weeks that I felt like she was starting to come back into her normal. But it was very interesting because you’re sitting in the room, and you can see the bag of the bad plasma coming out. So, it was-

HELEN: Wow.

CARA: … a very hands-on experience, and it, it was just very fascinating to watch everyone doing their job.

HELEN: Cara, I’m fascinated listening to you. You’re doing your volunteer job because nobody would choose to be doing this if they didn’t have to. You talked about you have to make decisions, so you have to be the voice of your mom and go along with what you knew would be her desires when she wasn’t able to. You had to start doing your learning, and you talked about you appreciated when the team explained to you, but you also would go home and do your own homework or research into this. And you’re a caring, loving daughter throughout, and you didn’t ask for any of this. It just kind of unfolded. And then, your mom has another medical situation that, in itself, is scary. What’s it like being the, a family member, a loving family member, perhaps a caregiver? What’s it like for you?

CARA: I mean, it definitely, uh, I, I keep saying it, but it was very scary because, and again, it’s going to be different for every person, but at least for me, my mom is typically the person I go to when a situation presents itself, and she’s the person I go to for comfort or advice or whatever it is that I’m seeking. So, for her to be at the center of it and having to, you know, have the roles flip. Typically, it’s the parent being the caregiver to the child, not the other way around. Um, so definitely a lot of pressure to make decisions that I felt like would honor her wishes, you know, in those moments where maybe she wasn’t able to speak for herself. But my mom and I have a very close relationship, so I do feel like, you know, after the fact, when we would debrief, she agreed that every decision that was made was aligned with her wishes. But again, it was very helpful because, you know, I had Dr. Carney and Dr. Aird even refer me to the Blood Project as a starting place for resources on what TTP is. There was a couple of videos and podcasts that I had found online. So, really anything that I could get my hands on in the virtual realm that would help me better understand both what my mom was going through, but what it, what I could expect not only during her hospital stay, but what were the long-term ramifications of this? Just hearing from former patients of TTP and understanding that even once she was discharged, she was going to have her good days, and she might also have her bad days. So, just having patience with that process because even, m- my understanding is once a patient is in remission, you know, there is a risk for a potential flare-up again in the future, or there is the potential that they’re going to have days where they’re very fatigued and they’re not their normal. So, just trying to best understand what was to come.

HELEN: I’m just awed. You, you have your degree in political things, or, and you do your advocacy work in another sphere, but here you’ve become a scholar on this disease you hadn’t even heard about a few weeks before. Brian, you’re part of this picture now of the treatment, the inpatient treatment, and I don’t know what happens. Tell us a little bit about from your doctor perspective.

DR. BRIAN CARNEY: Yeah. So, uh, you know, this is, uh, a rare disease. At my hospital, we’re considered a center of excellence for TTP, but we only see about 10 patients a year. So, that gives you-

HELEN: Oh my goodness. Really rare.

DR. CARNEY: … a sense just how uncommon this is. But, you know, it’s something that on the inpatient side, we really need to institute therapy emergently because time to starting plasmapheresis, that procedure we’ve been talking about, is really important to get that time as short as possible. And then, um, yeah, it’s, it’s pretty much plasma exchange until the patient’s feeling better and the labs look better, and then after that, there are additional therapies to suppress the immune system and reduce production of the bad antibody that’s causing this disease. And then afterward, it’s just regular follow-up in clinic, and as time goes by, we space that out more and more and more, which, you know, we’re, we’re all kind of experiencing now.

HELEN: I’m so glad all of you are sharing all your perspectives through the, the illness, the crisis part, and the ongoing treatment. Let me tell you a little bit about the listeners to Talking About Blood. They may be seasoned professionals or hematologists like Brian, like Bill Aird, people who have been doing this work for a while. Listeners may also be those newer to either medicine or the science or even thinking about going into such careers. And listeners can be like me, maybe like you, you two. I mean, we’re just curious about the world around us, including about the role of blood. I welcome hearing from each of you what would you want others to know in any of those groups? The seasoned professionals, the people newer in the sciences and medicine, and everyday people like you and me. So, that’s a, that’s a broad question. I’m throwing it out. Who wants to, uh, yeah, start answering that?

LISA: I guess, it’s hard.

HELEN: So Lisa, from your perspective as the patient?

LISA: Yeah. It’s hard because, again, there’s no blood test that you could do proactively to have determined what was going on with me. So, I could have gone to my primary care, you know, over and over and over, but they would not have been able to determine this. They would just have seen that my red blood count was significantly low and probably referred me to a hospital. So, that’s the difficulty with this disease. The treatment, which was the ritux-, Rituximab, I never say it right, I did that four times, and, you know, we now follow up. You know, I get a blood test done. I started doing that weekly, and now it’s biweekly, and I meet with Dr. Carney. It’s probably going to be, like, once a month now, but that was a progression. We were meeting and blood testing weekly, you know, in the beginning. But I think I now would know the signs if I was, you know, to have a relapse. I think I, I think I would know the signs, but I would go, obviously. I’m heightened to any kind of change in my body.

HELEN: Thank you, Lisa. Thank you for sharing that. Thank you for sharing all of this. Overall, how are you feeling?

LISA: I feel excellent. I’m planning on going back to work in, in a few weeks, um, which, which is encouraging. And, um, I, I don’t know if I don’t know if we hadn’t met with Dr. Carney and the team that I, I would be even here. I mean, I, I have a second chance. And my daughter, you know, knowing to take me when she did was just a miracle.

HELEN: I also think it’s kind of maybe a good news/bad news situation. The bad news is you had this problem, and the good news is we all live in Massachusetts around Boston somewhere. We are at the heart of a lot of medical excellence, where even Brian, who’s an expert on this situation, only sees it rarely. So, the good fortune everyone did what they needed to do in a timely way, but we had access to care. Cara, can you briefly just… What are your recommendations or tips?

CARA: Absolutely. So I will say, you know, starting at the beginning, for the caregivers or anyone in the support system, I really want to emphasize listen to your gut. Because even though my mom was not coherent, the one thing she was able to be coherent about was she was very adamant that there was nothing wrong with her. There was a serious disconnect between what I was seeing and hearing versus what she understood to be how she was. So, if you feel like something’s wrong, if your gut is telling you this is not right, follow up on that. Also, I know that I, you know, had the privilege to have the time and the energy to be able to try to do as much research or be with my mom, and I recognize that’s not everyone’s situation. But for those times you are able to be in those rooms with the medical professionals, you know, ask the questions. Don’t be afraid to ask something that you don’t know or you feel like might be a dumb question. Um, I, you know, I was working with a team that was very patient with me and was very good about re-explaining anything that I needed re-explained. But the more that I understood what was happening to her, the more I could be more effective as a part of her support system. But even for the medical professionals, like you were saying, you know, we live in Boston where, you know, there’s a full specialized team at Beth Israel, but that’s not the reality for other states or other counties in this country, or even internationally. So definitely the more that medical professionals can try to understand the signs and symptoms of TTP, just because it does present like a lot of other diseases, it can be hard to miss. And again, like at the local emergency room, I don’t know who was trained then to, you know, get tipped off that this could be TTP. But if it wasn’t for them, we could have been wasting very much precious time. So definitely, you know, trying to refresh yourself as much as you can. Like the more that you understand, the easier you are going to be able to detect it when that one in 10 patients that you might see every year walks through your door.

HELEN: Wow. What lessons learned. Thank you. Brian?

DR. CARNEY: Yeah. I mean, I think echoing a couple of things that have already been said, but for patients and family members, if something seems wrong, a- as was clearly the case with Lisa, seek help. Most of the time, it won’t be TTP, but regardless, seek help from a medical professional, um, you know, as was done in this case thankfully. And for medical professionals at kind of all levels, training, nurses, doctors, I would say learn the signs and symptoms of TTP and have high suspicion, uh, for this disease in patients who look like they might have it, because it, it’s a diagnosis that we can never miss and it’s a disease that we need to get treatment started emergently. And if we do that, we can get patients better and cure them. So, really, it’s something that needs to happen.

HELEN: I’m going to ask myself that question. What did I learn about this? As a podcaster, someone trying to give out this information, talking to the three of you, I learned the value of hearing about a condition from all these perspectives: the perspective of the person who has this condition, the perspective of a caregiver or a loving family member, and the perspective of the physician. We are all in this one together, and I really, truly feel that coming to this shared understanding will move us all forward. Thank you all so much for your candor, your wisdom, your treat in sharing all this story. Thank you for being guests on Talking About Blood.

DR. CARNEY: Thank you.

LISA: Thank you.

CARA: Thank you, Helen.

HELEN: As we just heard from Lisa and Cara Ferguson, and also Dr. Brian Carney, it is so important to value and hear each other’s perspective about diagnosis, treatment, and care when it comes to medical conditions. In this case, it’s about a blood disorder called TTP. To learn more about The Blood Project and explore its many resources for professionals and trainees and patients, go to thebloodproject.com. I invite you to also listen to my other podcast series about health communication at healthliteracyoutloud.com. Please help spread the word about this podcast series and The Blood Project. Thank you for listening. Until next time, I’m Helen Osborne.