ANNA'S STORY EPISODE 1

A LIFETIME OF UNCERTAINTY

 

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:
I found myself in the school nurse's office. I was in sixth grade. Complaining about chest pain. And from there, it was a really difficult day. I’d finally went home. Told my mother how I was feeling, um, and really the hallmark symptom at that point was if I laid down it hurts more, if I sit up and I lean forward it feels better. So, she called our pediatrician, who upon hearing that said, "Go to the local ER. Immediately run every red light. Get her there as soon as possible." And so, when we got to our local ER, I was actually in acute congestive heart failure. And that was my first episode of acute pericarditis.

 

Anna:
My name is Anna. I am thirty six years old. And I am living with recurrent pericarditis.

 

Edy:
So, how long have you been living with recurrent pericarditis?

 

Anna:
Twenty-five years now. But I didn't actually have a firm diagnosis, until about five years ago.

 

Edy:
Could you give me a timeline? So, you've been living with it, but you didn't know?

 

Anna:
Sure. So, I was diagnosed with systemic juvenile idiopathic arthritis when I was eleven. Right around Christmas time. And I had been discharged, sent home with some NSAIDs, wasn't really a clear care plan at that point. And unfortunately, less than a month later, I found myself in the school nurse's office. I was in sixth grade. Complaining about chest pain. And from there, it was a really difficult day. I didn't - I was, you know, pretty flatly disbelieved by the adults at my school. I finally went home. Told my mother how I was feeling, she called our pediatrician. And so, when we got to our local ER, that was my first episode of acute pericarditis, which had gone so untreated that, by the time my symptoms really manifested in a meaningful way, I was in acute congestive heart failure. So, at that point, I was medevacked and I was placed in a medically induced coma for six weeks.

 

Edy:
How did you know, or did you know, that you were going to be put into this induced coma? And, do you remember anything being explained to you through the process?

 

Anna:
No. Definitely not. It was very much, you know, to reference a ter- you know, terrible medical TV, it really is sort of, you know, what you see when the doors go crashing open in the ER and there's a million people doing a million different things. It was pretty obvious to me just by how quickly things were happening, that it was very, very serious, as far as, sort of this multi-faceted effort to bring my fever down, bring in a portable chest x-ray, get IVs in, start administering meds. it was like a parade of doctors, and med students, and interns, and fellows, and, you know, curious onlookers, just parading through my room.

 

Anna:
But I don't think anybody really knew what to do with an eleven year old in acute congestive heart failure. And I don't think that there was any effort made to explain what was happening to me. Because I really do think that, at that time, those doctors were just trying to save my life.

 

Edy:
Did you know that they were trying to save your life? Did you know of the seriousness? Did you even have time to feel your own, perhaps, fear or anxiety?

 

Anna:
I knew that it hurt to breathe. And at eleven, you know it shouldn't hurt to breathe. You know that your heart should just pump, and your lungs should expand, and you should just be able to do that. So, you know, I knew enough to be scared.

 

Edy:
Of course. Of course. At what point was there a conversation, "This is acute pericarditis."?

 

Anna:
That was afterwards. When I had been woken up from the coma, and had gotten to a point where I was, you know, coherent enough to really understand things. What had been explained to me is that there was a delay in my diagnosis, so I started becoming symptomatic with the arthritis when I was eight, but I wasn't diagnosed until I was eleven. It's difficult to diagnose. This is not an uncommon story, even twenty-five years later. And what had happened was they had focused so much on the arthritis diagnosis that nobody was really looking at anything else. So, even though I'm inpatient and I'm doing labs, and they're screening for cancer and Leukemia before they ultimately arrive at this diagnosis, nobody had done a chest x-ray or an echo. Nobody had thought to.

 

Anna:
And so, by the time, you know, I ended up in that first acute pericarditis flare, that could have been brewing for, who knows, weeks? Months? We had no idea, and I think that's really, ultimately, what got us to critical mass, is nobody knew what to look for.

 

Edy:
You had the first diagnosis of acute pericarditis and you were eleven at that time. Was there a point when you were told, "Okay. You're - you're no longer in this phase. You've had this once and that's it."?

 

Anna:
No. I really never got that kind of communication. I do think, coming out of that coma and learning everything that we did, there was the assumption, or perhaps the hope, that it would be this one-off acute episode. I had subsequent acute pericarditis episodes in high school, into college, into my early-to-mid-twenties. And, unfortunately, they were all treated as these one-off events. And it's, you know, sometimes I wonder if it's just, you know, the different care settings. You know, sometimes it would be so bad that I would wind up in the ER. Other times it was, you know, something that might come up in an appointment when I was seeing a rheumatologist. But I didn't have a - I didn't have a cardiologist. At no point did anybody step back and say, "I think that this is bigger than something we alone can manage." It was always this, focus on treat the arthritis. The pericarditis, these acute pericarditis episodes were a comorbidity of that. And it's almost like we just rolled everything up under one bucket, instead of really looking at these recurrent episodes as a stand-alone diagnosis.

 

Edy:
At what point - I'm listening to this and I’m thinking...

 

Anna:
It seems so obvious. Right? That somebody would put all the pieces together.

 

Edy:
And at what point...it was acute pericarditis. Acute. Acute. Just...that's what you were told. At what point did you get this diagnosis of recurrent pericarditis? it's like you knew, but you didn't know, and you didn't have a name for it. So, somebody named it for you. Can you take us through that process?

 

Anna:
Yeah. It was a little over five years ago, I was working to make partner at a consulting firm. So, that's 70, 80 hours a week, and I was just pushing my body to the brink. And it was really starting to affect my health, in very obvious ways. And I had been kind of avoiding going to the doctor because I knew that the reality check of like this life that you're working for is unsustainable for you and your body. And I finally went, and I saw - I started with my rheumatologist. And I had labs done, I had x-rays done. We really just did this whole holistic, like, you are not healthy, you do not feel good, your chest hurts all the time, like we've got to figure this out. And it was the first time that she said, "I think you need a cardiologist. I think that this is - this is -" I had high blood pressure at the time. I had hypertension, and I thank God I had hypertension, because I think that was the thing that she saw and she was like, "This is outside of the scope of my practice. I don't know what to do with this at this point.

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