ANNA'S STORY EPISODE 3

LIVING WITH RECURRENT PERICARDITIS

 

The views and opinions expressed in these interviews are those of the individuals and do not necessarily reflect the views, opinions, policies, or positions of Kiniksa Pharmaceuticals.

 

Anna:
When I was a kid, I used to say it felt like an elephant was sitting on my chest. As an adult, it's just like I feel like I'm being squeezed. But it's like a very constant squeeze.

 

Anna:
Like a vice. And you feel like you can't fully expand your chest. So, there's like this instinct you have. You want to take this, like, deep cleansing breath that will hopefully relax some of this pain...and you can't. You honestly feel like you can't take that breath to fill up your lungs. It's just this sharp, unrelenting squeeze of like not being able to fully take a breath. And also, sort of this panic-inducing, no matter how many times you've gone through it, of like you might not ever catch your breath ever again.

 

Edy:
I’m speaking with Anna, a 36-year-old woman living with recurrent pericarditis. Anna, in the previous episodes we’ve talked a lot about your diagnosis journey and some of the incredible struggles you had to overcome along the way, including acute congestive heart failure and then being placed in a medically induced coma, all at such a young age. Once you came out of the coma, what was the treatment route at that time?

 

Anna:
So, starting from coming out of the coma, and we looked at this idea of acute pericarditis...I think, even then, the medical team was looking at it as a very one-off thing. Even with that first bout of acute pericarditis, before a lot of the therapeutics that we have on the market today were available. So, you were really just treating symptoms. NSAIDs and high dose steroids. Which is just...in an eleven-year-old when your face — you get that nice moon face that other kids are just so kind and understanding about. But that was really all we, sort of, had in the arsenal at that point.

 

Edy:
Were you told by your support team, what the effects would be of these meds?

 

Anna:
When one of your treatment goals is to reduce inflammation around a patient's heart, it's very easy to be dismissive of the side effects. Right? Because that's so serious that you can almost justify any side effect. So, I always felt as though the response that I got was more of just the, "Well, this is what's available...”

 

Anna:
“You're not having, you know, an acute pericarditis flare. So, that's a win. So...kind of what else do you want from us?”

 

Edy:
How often are your flare ups? And what's that in between time like for you?

 

Anna:
Every, you know, three or four months, I call it crashing and burning. I probably just pushed myself to the brink. It certainly used to be far more frequent than that. Once a month, every other month, I'd find myself, you know, sort of down for a couple days with a recurrent pericarditis flare. But one of the reasons that I...I think that I feel like I've pushed myself to the brink is that when you live with recurrent pericarditis, when you live with something that affects your heart, affects your ability to breathe, and feels like it has the potential to affect your ability to live a long and healthy and happy life...I have what borders on like a compulsion to make every other day that I feel good as full, and rich, and meaningful, and busy, and productive as is possible. Which sounds like a great approach to live life, but it isn't. I...And I'm very aware of this. And, it's hard for me to give myself permission to rest from anything. Because I have created this little, it's like this chip in my brain, or like voice, that's saying like, "This day might be a waste. You won't get this day back. You're gonna regret laying on the couch six months from now when you literally can't move." So, there's a good part of that. Right? That I really do want to squeeze so much out of this life that I...probably better than most people understand how fragile it can be. But, you know, the harder part of that is just not really ever giving myself that space to just rest, and to just be. To even just sit with some of the emotion of what it means to have a chronic illness that permeates every aspect of your life.

 

Edy:
So, you're in perpetual motion. And then, you're in a cycle of recurrent pericarditis...You can't live the way you want to live.

 

Anna:
Oh, it's like running headlong into a brick wall. I can always feel it coming. It starts as like a dull roar, and it's not that intense like acute pressure. It'll be like a little low-grade, maybe feel like I can't take a deep breath, maybe catches up with me during a walk or run. And I just know. It's like instantaneously, I'm like, "Oh, God. Okay."

 

Edy:
What happens in that moment of knowing? Is there...is there fear? Is there anxiety? Is there resignation?

 

Anna:
Well, it kind of goes back to that anger. Right? It's like, "Oh, God. Not this again. Like, really? We're doing this again?" And it always feels very ill-timed with everything else that I have. But I wouldn't say it's resignation. I feel like it's I'm the exact opposite, where I like fight it tooth and nail. And I think a lot of that is, when it comes to an acute pericarditis flare, the only tool we have in our box right now, for me at least, that gets it back under control, is a short burst of high-dose steroids. And I hate them. It like takes me right back to being that eleven-year-old kid, and my face is swollen and I'm retaining water.

 

Edy:
And how long does an episode usually last?

 

Anna:
I tend to go down hard for a week when it happens. I mean, I'll be sitting at, like dinners with friends. Friends who would understand me canceling, and I full-on can't breathe, can't enjoy a meal. But I'd rather do that than cancel. But I think that if I were more appreciative of the warning signs in the first three days that the flare is setting in, that the subsequent four to five wouldn't be as miserable as I inevitably make them for myself.

 

Edy:
Could you go over those warning signs?

 

Anna:
For me, it starts as a very dull ache. Like, in my chest. And it doesn't quite get to like pressure or squeezing right away. It's just this very dull ache. And the way that I would explain it is it almost like arrives and then just settles in and makes itself comfortable. And then it's usually, you know, I'd say it's usually like day three or four of me trying to ignore this that that's when it starts to get much more acute. And it's like, if we were talking about that vice around your chest, the vice is getting tighter and tighter and tighter. And that's when you're starting to feel like, I can't inhale fully. I can't take that deep cleansing breath that I want. Now I'm getting tired. I'm having a hard time focusing. And I think it's the radiation up into the shoulder is usually the last for me, and that's usually right around the time that I start like running out of breath at the end of sentences.

 

Edy:
And is that the point when you call the doctor?

 

Anna:
Yep. When I can't finish a sentence, when I'm exhausted by talking, that's when I know that I'm past the point where I can just like rest for a few days and this will resolve on its own.

 

Edy:
So, in addition to having to deal with these symptoms and the side effects that come with the treatments in this, sort of, limited toolbox, I’m curious how having recurrent pericarditis has affected you, just in terms of your life overall.

 

Anna:
Recurrent pericarditis takes up a lot of space in my life that I wish I had available for other things. And not just the physical aspect of managing the symptoms, and having to sort of give in to those days when I do have an active flare, and I know that I'm going to be down for the count, and really need to stop and get some rest. Not being in an active flare is not enough for me. I need to be able to run after my little nieces, and go hiking, and walk my dog a million miles, and to do all those things that, to me, makes life so much richer and more beautiful. But it's also like the mental energy of just having to sort of confront that unknown over and over and over again. I don't really feel like I have a firm grasp on how recurrent pericarditis will or won't affect my life expectancy. I have no idea, if I'm being honest. I don't know where I am in, sort of, the trajectory of life compared to my peers. And, of course, we all know that we could step out of our houses and get hit by a bus tomorrow. We all accept that there's a certain amount of uncertainty in life. It's very, very different when you live with something that effects an organ as vital as your heart. And I feel like confronting that unknown over and over again in my life is...it's a lot.

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