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I'm Aware That I'm Rare: the phaware® podcast

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Chandani Dezure, MD - phaware® interview 384 (Part 1)

Sep 13, 2022

In this episode, Chandani DeZure, MD, a board certified pediatrician and mother to a young child with idiopathic PAH,  shares the heart-wrenching journey of her son's rare disease diagnosis during the height of the COVID crisis. She details physical and emotional toll it has taking on her son and family.

My name's Dr. Chandani DeZure. I'm a Board Certified Pediatrician and a caregiver to a child with PH who just turned four years old. My son Ryan was diagnosed in July 2020 when he was two years old and COVID was in full swing. I was a frontline worker. I was five months pregnant with my second child, and he had been diagnosed with an ASD (atrial septal defect), because he had a murmur at his well-child check. So we had went in for an echocardiogram to see if his simple ASD had closed on its own. Then, maybe we'd never have to see a pediatric cardiologist again.

What happened was quite the opposite. He was incidentally found to have severe dysfunction, which would be very disproportionate to an ASD, but very concerning for pulmonary hypertension. We were admitted to do an urgent catheterization procedure, which was a little unnerving, because it was dangerous to be in hospitals at the time. Given his elevated pressures, he was officially diagnosed with Type I or idiopathic PH.

A little piece that I like to share is the consequences of COVID. A lot of routine care was delayed because COVID had other ideas. My son's echo for his ASD follow-up was delayed about three months, because the cardiology clinic had been closed to non-emergent cases due to COVID. Normally, three months for a simple ASD would not be a big deal at this age. However, in the nine total months it took to get a repeat echo, his heart function went from normal to severe. Unfortunately, we've never really been able to recover from this. I wonder if we had been able to capture it somewhere in the middle, we would've fared a little better?

Back to his diagnosis. He came out of his catheterization procedure. We met his new PH team after diagnosis at a world renowned center. It took them about 10 minutes for the doctors to relay the good news, which was that his 10 year survival at age two was 80%. I don't know which cardiologists need to hear this, but talking about survival numbers is never really good news, especially in a two year old. Also as a pediatrician, I was well aware of the trajectory and the prognosis that PH can have. It was just very difficult to hear that my toddler was going to have to deal with this diagnosis.

We got started on oral therapy, thinking it was to be fairly easy, given that we caught it early. He was young. He had no symptoms, and so that it would be easier for his heart to recover than perhaps other people who get diagnosed late. But as we followed his serial echocardiograms, they remained unchanged with severe dysfunction. Eventually, we escalated to triple therapy very quickly including Sub-Q Remodulin®. Sub-Q Remodulin® was for us the seventh circle of hell. Actually, I think Dante would've given it its own circle. My son Ryan had severe, uncontrollable, prolonged, excruciating site pain that was just unbearable.

We had to power through, as we tried to troubleshoot it. We saw a pain doctor. We did oral medications. We did topicals. We did gels. We did narcotics. We did anxiety-reducing medications. Nothing really worked. No one quite understood how awful it was. Like I mentioned, I was pregnant at the time of his diagnosis. So by the time we got on Remodulin®, which we were admitted for with a one month old postpartum in tow in COVID still. I was still breastfeeding at the time, but I had to give up breastfeeding my three or four month old at the time, because I had to hold Ryan while he screamed in 15 out of 10 pain for 72 hours straight with each site change, (which for him was about every two to three weeks). As a mom is was super awesome, because I got to fail both my children in different, but very profound ways at the same time.

Then, because it got so out of hand, nothing was working at about a year after we started it, (November 2021), we eventually discontinued it as not being able to tolerate. The one lucky piece of this is we did a catheterization before we came off of it, which actually showed that his pulmonary pressures had normalized, which would be great news. But his RV (right ventricle) had very poor function. So the thought was, “we've fixed the pulmonary hypertension. Maybe this ASD that hadn't closed was causing some problems.” So of course, most ASD's get closed in a catheterization lab, but we of course were lucky and his couldn't be, because he didn't have adequate rooms. So the things that they put in the catheterization lab to kind of latch on there didn't have anything to latch onto on one side of his ASD, so he had to be scheduled for open heart surgery at a very well-known institution.

Unfortunately, due to circumstances outside all our control, his open heart surgery was rescheduled three times for nonclinical reasons. I kind of wanted to relay the mental and physical energy that goes into prepping for your child's open heart surgery. It's just a massive undertaking, not even to mention the logistics and being told that this might be the thing that's going to fix your kid's heart when we've kind of fixed the other problem… then delaying it isn't enough to drive someone insane. Just after all of this, we felt it wasn't appropriate. We actually transferred his care just for the surgery to another institution, just so he could get his surgery, even though that meant going away from a team that knew him where he'd gotten all his previous care.

I don't blame his PH team. I don't blame the surgeons. I think this is part of why I wanted to talk about this is that this healthcare system is such a difficult system to navigate. There are things that happen that are outside our controls, but it has massive consequences on what patients and families go through. With his pressures normal, we closed his ASD. He did great. We were in the hospital about four or five days, which is wonderful. He had no complications. We were able to go home without issue.

So now the pulmonary pressures were normal. We had closed this ASD, which may have been causing problems. So we were hopeful that his heart would recover, but unfortunately he had still showed some severe dysfunction. None of it really makes sense to us or his doctors. The best cardiologists in the country can't seem to clearly articulate what is going on. Unless we're able to improve his function, he is sort of a ticking time bomb on his way to heart failure and potentially transplant is my understanding of where we are.

As a physician myself, I am well-connected, resourceful and privileged in many ways that some other families aren't. I am still constantly advocating for my son 24/7. I really don't know how other parents do it. I feel it's a full-time job on its own between the cardiologists, the surgeons, the primary care doctors, the therapists, the social workers, the IEP meetings for schools. It is absolutely exhausting as a parent. People keep remarking and how resilient my son or even I am. I just honestly wish he didn't have to live in a world that needed him to be so brave. I don't want him to be resilient. I want the world to be less traumatic for him.

I mentioned the healthcare system. It's just the miscommunications, the errors, the delays. For us, the unclear diagnosis, the frequency of appointments, the disjointedness of the system, the imaging, the procedures… it's all death by a thousand cuts. In his case, maybe even literally. But honestly my biggest fear isn't that he's going to die, though it's a very close second, but rather someday, he's going to say, “Why didn't you stop the madness when you should have?”

I think in medicine, and I know I do it too, we like to be hopeful. We have so many interventions at our disposal. But there's a difference in doing something because we can do something, and doing something because we should do something. I'm glad for people with PH who are finding ways to live their lives with meaning. I wonder if my son will do the same. I also wonder on some days, if with my son, we are simply rearranging chairs on the Titanic?  At the end of the day, as we know it, PH is incurable and potentially lethal. Don't get me wrong. My son is asymptomatic to this day and always has been, and he's only on oral meds and he is living his best life, currently. I enjoy every minute I spend with him knowing what is at stake.

I hope desperately for a cure or a way to make this a manageable chronic disease for him. But if not, and if and when that time comes for me to stop all this, I hope I have the strength and courage to make that choice for him. As bad as things are, they could always be worse. I think that's important to note. I think the silver lining is that there are moments of time where I'm able to forget that he has PH, because he's running around, he's going to school. There are plenty of moments where we're happy.

We don't live in this dismal world. I think it makes it easier we're not in the hospital every day. We're at home and he and his brother play very nicely. It's fun to watch them. As much as we say his function is really poor, severe sounds bad, but maybe for him, he can last with severe dysfunction for longer than we think he can. I mean, there's no way to predict the future. You kind of go into this rabbit hole if you keep thinking worst case scenario. But I think the Sub-Q Remodulin® really taught me there's going to be a time where we're going to have to say enough. It's not now. I hope it's never. But I don't know if it won't be never.

My name is Chandani DeZure, and I'm aware that my son, Ryan is rare.

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