Jill Vorst

We went almost a year with her platelets being in, like, critical range and it was just so taxing on all of our mental health and our bodies and how we just lived. It just felt like we were in this, just such severe isolation for so long. Being able to talk to other people and reach out to other people and have that support has been just so life-giving. I think finding that is so important.

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

As we all know, there's many aspects and issues to having ITP. In today's episode, I'm calling, “A Mother's Story”. So, if you have a child with ITP, I'm sure there's a lot in today's episode that you can relate to. And if you are an adult with ITP or a caregiver, you know that we can always learn from others' experiences. So let me introduce to you Jill Vorst. Welcome to Bruised but not Broken.

Jill

Thanks for having me.

Barbara

I'm glad to have you here, Jill. I know I've met you at two of the PDSA conferences briefly, but specifically, I met your daughter. And your daughter is Elizabeth, and she's the one with ITP. So we'd like to hear about your journey with this. How did all of this start? How old was Elizabeth and what made you proceed to find out what was wrong?

Jill

Elizabeth was two when she was first diagnosed. We noticed she had a lot of bruising on her legs and one bruise in particular was about three inches long and probably three inches wide but it stood up in height, probably a good half an inch, and it was just on like, just below her knee on the top of her shin, and I really was concerned about it. It wasn't going away. So, I made her an appointment with her pediatrician on, for a Monday morning. Incidentally, we had been seen the week previously for a pre-op appointment. She was going to have a dental procedure. She needed to go under anesthesia and have some dental work done. She had had that dental work done on Friday before this appointment was scheduled. They didn't do labs that are pre-op. So we had no way of knowing what was coming, which is pretty typical. They don't always do labs at, you know, for pediatric patients pre-op. So she had the dental work, and we had so much bleeding over the weekend. I called on-call dentists over and over and they were like, it's okay. You don't, you know, blood, it looks like it's significantly more when it's mixed with saliva and in water. And I was like, I have a nursing background, so I was like, thinking I knew this was a lot of blood. So I was watching her very closely. We were able to get that bleeding stopped eventually, but it just really, really was more than anticipated. I don't think I adequately was able to explain to the dentist how much bleeding we were seeing. And I think they're probably used to hearing frantic parents call and assumed it was the normal amount of blood. So, on Monday morning, we woke up and I was so thankful I had this appointment already scheduled because she woke up and her face and neck were covered in petechiae, which I didn't know really what that was or the word for it at the time. I was like, I remember seeing this in nursing school. I remember seeing it, but I wasn't fully versed in that. She looked almost like she had been strangled and like every blood vessel had popped. It just looked so bad. So we brought her in and the doctor was like, you know, at first when I saw a toddler with bruising on my schedule, she's like, I was kind of laughing. She's like, then I saw which family it was. And I was like, oh well, Jill knows. So, this must be something serious. She took one look at Elizabeth and sent us straight down to lab and then told us to come back and wait in the room. And I was so afraid. I was like, oh my gosh, she's going to call CPS on me. The police are going to be coming. I'm texting my husband. Like, I don't know what happened. And he, he's the one who put her to bed the night before. And he's like sitting at work, just panicking. He's like, I didn't hurt her, I swear. Like she had bruises everywhere. So, we waited about 30 minutes. The doctor came back in. She told us that Elizabeth's platelets were really low and that I needed to take her home, have her just rest and be calm for the day, that she had made an appointment for us at the university hospital for the next day, to just go home and stay off of Google.

Barbara

Oh, okay. Good advice. Cause that can be very scary. Dr. Google. Yeah.

Jill

I didn't stay off of Google, which was probably not the wisest idea cause low platelets can be a symptom of some other big scary things too.

Barbara

Well, so then you went to see, I'm assuming a hematologist at the university.

Jill

We did. So we saw the hematologist the next day who did a great job of explaining what ITP was and all of the labs and stuff they did that could exclude all the big scary options. They asked us a lot of health history questions. They were really under the assumption that this was going to be a quick little blurb for Elizabeth and that, you know, most kids who get ITP have it after a virus and that it's a quick blurb, we just kind of wait and see, and that she would recover on her own.

Barbara

Right. This is a very familiar story, yeah, with children especially. And in all honesty, that's usually what happens. But there is that percentage where the ITP remains persistent. And is that what happened with Elizabeth?

Jill

Yeah, her ITP did not go away. Her first bout of ITP lasted about three years and then we finally got remission and then she relapsed again. That first time we waited and saw for a while, we tried steroids, we tried some different medications. Nothing really worked well and she has lots and lots of side effects. So, we were kind of left in this really scary place where her platelets are low, she's young, toddlers fall a lot. I felt like we lived in the ER for a long time, just so many head bumps with really low platelets. And so, it's just a lot of like anxiety and trauma that you're constantly living in this state of fear and just uncertainty. You know it's very, you can't wrap them in bubble wrap, which is what everybody jokes. They're like, oh, just put her in bubble wrap. And it doesn't work that way, you know? So you're always trying to find like this balance of how I can let her be a kid and how can I let her play, how can we have peace and calm in our house, but also keep her safe, which is not easy.

Barbara

Now, how was she handling this? I mean, was there anger? I mean, did she know the difference? As she got older, I'm sure she had issues with dealing with life, maybe she couldn't do certain things.

Jill

Yeah, when she was little, she kind of rolled with it. You know, it wasn't as big a deal. She really struggled with not being able to go to the splash pad with our friends, not being able to play on the park equipment with family. She really had, she had just learned to ride her bike and couldn't ride her bike anymore. That was a really tough thing. Those were probably the biggest ones for her. She also just loves to climb and be really active. And so the restrictions were really tough. And then I think the fatigue was also really, really hard. And she didn't understand that at the time. She didn't have the capability to process why I feel so tired all the time. Why is it hard to walk? Why am I sleepy? And why does everything hurt? You know, she really had a lot of like body aches and pains. After we tried steroids, we tried IVIG. She ended up with aseptic meningitis from that, which was absolutely terrifying. Yeah. She woke up screaming. She was projectile vomiting, and then she'd lose consciousness. So, it was a pretty terrifying ride to the ER trying to get her some help, you know. They did CT scans and then I think they had to poke her like 13 times that first time to try to get an IV in so they could get her steroids and morphine. And it was pretty, pretty traumatic. She remembers that pretty clearly, you know, and the doctors are, they're pretty honest with her about that, like, this is really serious and that, you know, we need to get this under control and she knows that, you know, she's had multiple life-threatening side effects from the medication. And she also knows that the illness she has is also life-threatening. And I think that's really hard for a kid. I mean, it's hard for an adult and it's hard for me.

Barbara

Right. Well, as a mother, I think that it can be amplified, the fear, you know, because your, your job is to take care of your children and this is something you can't fix. So it's it weighs heavy on your heart, I'm sure. And she probably actually doesn't know the difference as far as she's grown up with this now. My own experience was I didn't know the difference. I felt like I had had it my whole life. So, how old is Elizabeth now?

Jill

She's 10 now.

Barbara

Okay.

Jill

We did rituximab when she was three and it took about a year and it fully, and it finally worked. And she was actually able to go into remission and had, so it was from three to four and then about four and a half until age seven. She was, had normal platelets, which was pretty exciting. She really enjoyed climbing trees and playing on the zip line. We got a ninja line, she drove her four wheeler, riding her bike again, all the things.

Barbara

Having fun like a child should be, right?

Jill

Yeah. I think the hardest part during that was she still had some kind of PTSD and trauma from ITP in the first place. And so we worked really hard on going to therapy and trying to be proactive in healing this trauma and giving her the skills that she needed during that time. And Rituximab really takes out your immune system. And so we spend a lot of time trying to be safe as well, which was difficult when the global pandemic hit.

Barbara

I was going to say, that does make it very difficult. Yeah. Because you're so worried about your child getting another virus. And COVID was not an easy thing for all of us to deal with. So how long did she stay in remission? Or did she relapse at some point or again?

Jill

Yeah, she relapsed at seven and we were kind of, we're like, oh man, we had been at the Children's Museum. She had been up and climbing and playing and doing all sorts of stuff. And she was playing with clay and like, rolling the clay out with her forearm. And the next morning her whole arm was covered in petechia. So I came downstairs and she was like, mom, I have this weird rash. And dad looked at me, my husband, he was just like, he knew. And I was like, all right, well, we need to go to the doctor and get some labs. I think this is petechiae. And she immediately started crying. And she was just so heartbroken. And she just laid on the floor crying and our German Shepherd laid on top of her. And they just laid there for like a good hour where she just cried and the dog just laid on top of her. And it was so heartbreaking, but just so like, I don't know, nice. I was so happy he was there for her. Pets are just amazing things.

Barbara

That's true. That's true. They, they are intuitive and they know when something's wrong. They know when you're hurting and they want to be there for you. It's, it's remarkable. It is. So, what did you do at that point?

Jill

We went in and got her labs and her platelets were, I want to say a seven the time or 7,000. So she was pretty low. We were able to get in with the doctor the next week. And it was so different. I feel like the first time when she had ITP, we went in and it was like, oh, this is a blip, blah, blah, blah, you know? And then her journey went on. you know It took two years. And so we knew when they had discharged us from care, you know she discharged us with standing orders for labs. And it was kind of like, this will probably reoccur. We don't know if or when, but you know, statistically kids who have chronic ITP will end up relapsing. So, we went in and the doc was like, just really solemn, really sad. You know, she was happy to see Elizabeth because they just love each other, but she was also really sad to see Elizabeth. We went over our options and realized that there's not as many options your second time around. Elizabeth had failed so many medications already. But thankfully, like in those years, the science had changed and there were some new medications. So that was pretty exciting for us going in.

Barbara

Were they available for pediatrics?

Jill

Yeah, there were quite a few new things that we could try. We tried Promacta, which helped us a little bit, not much. We tried that for a while. We tried NPlate. That was really traumatic. Elizabeth was really struggling with blood draws at the time. She's a difficult poke. And so then, you know, to get labs and have two, three, sometimes four pokes, and then to have another shot on top of that. So there were times that she would be crying before we even got into the hospital. Crying and hiding underneath the exam table, just really, really traumatic and sad. And then it didn't work. We tried rituximab again, and that didn't work. And she's allergic to rituximab, and so it ends up being long appointments. She has to have lots of steroids. Steroids have some pretty yucky side effects for her. You know, she just feels like crap. The rest of us have to deal with the extreme hyperactivity and just inability to control herself at all, which is really hard on our whole family. Then she tried, we did some IVIG because her immune system tanked a little bit more, which she got aseptic meningitis again. That's happened, I think we've had it four times for different reasons and they've tried different versions of IVIG and different pretreats. Sometimes her platelets have been so low for so long that we need to do some IVIG despite the side effects. She tried 6MP and ended up in liver failure, which was terrible. We were in the hospital for weeks for that. She was in so much pain and you can't have Tylenol in liver failure. You can't have ibuprofen with ITP. They refused to give her any narcotics because they were like, your, her stomach was so bad at the time. That led to her having some major GI issues and her esophagus became inflamed and she couldn't eat and she lost a lot of weight.

Barbara

Oh goodness.

Jill

And it's just led to, just a lot of pretty severe side effects. Her mental health has deteriorated pretty significantly throughout this last to ITP journey.

Barbara

I saw her, what, a month ago. She looked pretty good. Is she in remission?

Jill

She is not quite in remission, but her platelets are stable. Our journey led us to doing a drug trial of Doptelet, or avatrombopag. And yeah, so she did the drug trial last year when we were at the PDSA conference, we had just started the drug trial. It wasn't working yet. She was, her mental health was in the tank, and we were desperate. She met you and met some other kids and we left with so much hope because she was just, I think fully convinced that she was going to die soon and that, you know, she was just so terrified of that and so traumatized from all of the sticks and all of the side effects and everything. You know, we had, we had a few other meds that we tried as well that didn't work and gave bad side effects and, she's the kid that gets those. In the last year since then, you know, she has a new therapist. She has a new psychiatrist. Her anxiety got so bad, she wouldn't leave the house. And so we ended up having to do an inpatient mental health program for her, which she, she thrived. She did really well. She's learned some great skills. So her, she has pretty severe anxiety that stems from ITP. She has a lot of PTSD and trauma around that, but she's really working very hard, and our whole family is working hard to help her overcome and succeed. I think the biggest success was right before the PDSA conference this year. She went to a camp called Camp Not-A-Clot, sponsored by our local hospitals and the Hemophilia Association. They work on really empowering kids with bleeding disorders to help them take control. So I feel like there's so much that we don't have control over and I think that's what's, one of the really hard things with ITP is like there's so lack of any control you're just left, you know? And she earned a Big Stick Award. She actually poked herself this year and the nurses from the hospital emailed me and they were all so excited and the Child Life Specialists were so excited and were just fully celebrating. A year ago, she was hiding under the table and this year she did so much work that she could poke herself. Like, that was really exciting.

Barbara

My goodness, that's quite a transformation and kudos to you and your, kudos to you and your family for being able to give her all that support that was really needed for her mental health and her wellbeing, which was terrific. So, is she going to school? Is she homeschooled? What's your school situation?

Jill

We homeschool. We found that the anxiety with ITP and being in school was just too much for her. We worked with our therapist to come up with a better plan and so now she's doing an online public school program. This year she'll be starting to be with some, a smaller group of kids one day a week and I think it'll be a good step for her.

Barbara

Okay, I was wondering if you're familiar with the Poke-R Club that the PDSA has to offer.

Jill

Yeah, Elizabeth joined the Poke-R Club about two years ago. We hadn't really known about the PDSA our first time around. And then I was able to find it on Facebook and joined. The Poke-R Club has been really nice. She loves being able to get prizes for her pokes. I think it makes it feel like what she's doing is worth something.

Barbara

That's terrific. Explain for those of that are listening that have no idea what that is. Could you explain a little bit about the Poke-R Club and it's Poke P-O-K-E dash R, not the card game. It's Poke-R Club.

Jill

You're right. But you do get poker chips. So the kids get a package that comes with a card that they can mark off each time they get a poke. And then you, as the parent, give them a chip. And then when they reach 10 pokes, you send that into PDSA and then they send your kid a prize.

Barbara

That's terrific. And does she have the Buzzy bee thing also with that?

Jill

Yeah, that was amazing. I think when we first signed up, we got the Buzzy bee. She takes that with her to all of her appointments. We use the Buzzy bee. We have lidocaine, some essential oils, and then we also have that shot blocker. It's like a little plastic wing-shaped piece that has little tiny spikes kind of in it and you put that gently on the skin and it kind of, the body senses those little spikes and then getting the poke doesn't, the kid doesn't feel it as well.

Barbara

It's like a distraction, like a distraction, and the buzzy bee is like a distraction

Jill

Yep. It works pretty similar to the Buzzy bee. So we, we really love them. We started bringing the Buzzy bee to her clinic and her clinic actually hadn't heard of it yet. And they bought a couple for their clinic after seeing how amazing it worked for Elizabeth.

Barbara

Well, that's great. That's wonderful. I'm glad to hear that she's on a stable path right now. And hopefully that will be a prolonged period of time. And that she's doing so well because she looks like a healthy, normal 10-year-old, you know, she really does. And she's very bright, very interested in, in ITP, I think, and seeing her at the conference. I know for myself to see the children with ITP together, connecting, learning about each other. It's, it's a chance for them to feel like they're normal. Because the other kids that are around them are going through the same things. And it just warms my heart. I just think it's a wonderful part of the annual conference that they're able to give the children their space and support the parents during that time too with their own, you know, sessions that are, that are geared towards the parents and geared towards the children. You've had quite an experience with this. Do you have any thoughts that you would like to share to maybe other parents that are going through similar hard times with their child?

Jill

I think finding some people who get it is really important. We have a few close friends that kind of get it, but not like the other parents of kids with ITP. Going to the conference was pretty life-changing for all of us. We were really able to meet people in person that made those connections. Often we would talk about ITP and people like, oh yeah, my, you know, cousin's kid had that. And, you know, most of these people who have had it are the people who just have the little blips. And so they don't really understand what life is like living with ITP long-term, how much it affects the entire family. You know, our, we have five kids and each of them live with ITP just as much as Elizabeth does. When we went to the conference meeting other parents and other kids that had similar journeys, we felt like, you know, Elizabeth is in this super small percentage of kids that, you know, not only continue to have ITP, but have, don't respond to treatment and have ITP long-term and have so many side effects. And so, you start to feel just really isolated and hopeless and it feels really dark and heavy. We went almost a year with her platelets being in like critical range and it was just so taxing on all of our mental health and our bodies and how we just lived. It just felt like we were in this, just such severe isolation for so long. Being able to talk to other people and reach out to other people and have that support has been just so life giving. I think finding that is so important.

Barbara

I agree. I agree wholeheartedly. I think you obviously had a wonderful hematologist that, you're very fortunate in that way because when I talk to patients that are struggling, you know I often ask them, what is your relationship with your hematologist? And you have to look at it as a long-term relationship. And finding the right hematologist is really so critical. And finding one that understands ITP and that actually treats ITP and is aware of the different choices and treatments is a big hurdle for a lot of people. And fortunately, you've had wonderful care that way. And I agree that the support from other families and other parents is just essential for you and your husband, I'm sure, going through this journey with Elizabeth. So, well, I'm so happy to have you share your story with us. And it's, you're a tough lady. You're hanging in there. And I commend you for that. This is not an easy thing and not an easy journey. And it looks like you're, you and Elizabeth, you're on the right path. And hopefully the future will become brighter and brighter for her. I know when I met her, I said, well, look at me. I'm an old lady and I've had ITP since I was four. I think that surprised her, but hopefully it gave her some hope.

Jill

It definitely did. We left the conference, or that first night after she met you, she was like, mom, I met an old lady who has ITP. And I was like, that's pretty cool. She was, no mom, She's old. She's old and she has ITP! And I was like, it took me a minute to realize that she didn't think of the possibility that she could be old. And that was so heartbreaking to me and I, you know, we held her and we were like, you will get to me an old lady too, Elizabeth. It's going to be okay.

Barbara

And have a full life, you know, and have a full life. We just have parameters that we learn as we go through life, things we can and can't do. When you don't get sad over what you can't do, you get happy over what you can do.

Jill

This is so true. I think that would be my other piece of advice is that we really focus on all the things we can do and not what we can't do. And one of the things our family does, we have another child that's really medically complex and is on oxygen, has a feeding tube and stuff. And so, our family motto is we don't say we can't, we try to figure out how we can. And so often times with Elizabeth's ITP, we figured out ways we could do all sorts of things safely. And so there's a handful of things we can't do, but there's a world full of things we can do. We try to focus on that.

Barbara

That's right. You find those opportunities. Well, thank you again, Jill. This has been great. I know that your voice and your stories will be helping other parents and other people with ITP. And I wish you and your family and Elizabeth good luck. And I hope to see you at future PDSA conferences. So, thank you again and give my best to your daughter.

Jill

I certainly will. Thank you so much. Thank you for having me and for doing this podcast.

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

The sun. I'm sure everybody knows how important it is to wear sunscreen. But aside from sunscreen and the sun damage that the sun can do to your skin, if you suffer from petechiae, you need to be concerned about the heat that the sun can present on you. Let me explain. The blood vessels that you have that are very close to the surface of your skin are little capillaries. And what petechiae is, is when those capillaries break or leak blood, that is what presents as petechiae. They're little blood spots, little tiny dots on your skin. Now, if you remember your science classes, you know that heat expands things and cold contracts things. So, when your body is exposed to excessive heat, meaning your skin gets hot, the capillaries under your skin have the opportunity to expand. And when they expand, they are more likely to leak blood, especially if your platelet count is low. This also can happen when you're exposed to heat, not just from the sun, but think about how hot is the hot tub and how hot is the showers that you take. If you are one that suffers from frequent petechiae on your body and you don't know why you have it or where it's coming from, think about heat exposure. It doesn't apply to everybody, but it might apply to you. So that's just my little lifestyle hack for the day. And that’s it for today, until next time, I hope you have high healthy platelets and stay safe.

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.