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

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Cheryl Wegener - Transcript 122

Dec 22, 2017

Pediatric pulmonary hypertension caregiver, Cheryl Wegener discusses her daughter Madison’s road to a PH diagnosis and the impact living with this rare disease has had on the entire family.

Hi, my name is Cheryl Wegener, and I live in Michigan with my family. My daughter Madison is our Pher.

Madison passed out in her first-grade gym class, and that was our first indication that something was wrong. Prior to that, she was pretty much asymptomatic. We went to a doctor. Even the school nurse, when they called, they said, "Look, you don't even need to pick her up from school, she's good. She was a little freaked out. Her levels are fine, her blood pressure's fine. Did you feed her this morning?" Yes, we fed her. "Okay, you might just want to follow up."

We were new to that community, so we didn't even have a pediatrician that we knew and loved for years, but somebody said, "Okay, go see this guy." And so, we took her, just for a follow up. Everything looked good there, but thankfully he wasn't okay with the fact that the six-and-a-half-year-old passed out. And so, he's like, "You know, let's just do an EKG. We don't do a lot of them here, but let's just try it and just to be sure." And that EKG came back with a couple red flags. And again, he was like, "We don't do a lot of them here. Maybe our leads run a little off, but I'm just not okay telling you ... I want you to see a pediatric cardiologist. If I tell you to make the call, you'll be on a six-month waiting list." He goes, "I'm not okay with that. So, let me make a call, I know a friend."

He made a call. This was on a Thursday, got us in to see her on a Monday. And thankfully, we weren't one of the people that go undiagnosed for years and years and years. We went to see her. We did an echo and she knew exactly what she was looking at. And they come in and they give you, you know the pictures, they're trying to explain what this all is and there's two different versions and neither version is good. And we were basically told to go home, pack your bags, drive to Dallas, which was an hour away, and you're gonna go there for a bunch of tests. There is a team of doctors waiting to meet with you in ICU. And her pressures were probably in the 120s at that point. They couldn't believe that she hadn't turned blue, she hadn't passed out, she hadn't had all the symptoms before this period.

And so, when we did that, we went to Dallas. We were in the hospital 15 days. That was our first introduction to the PH world. It's interesting for me, when I go back and I reread our Caring Bridge page, and you watch our understanding unfold. Nobody came in that first day and said, "Your daughter has a terminal illness." Nobody gave us the severity. They told us, "Stay off the internet." So of course, at three in the morning when you're in the hospital room, you're on the internet, reading stats and hearing the good, the bad, the ugly. And as we journaled about it, I think we knew, the first thing we read, one of the first things, was that Madison wouldn't be able, or should not get pregnant. And we knew that before we knew of the implications of the disease. And I remember writing about that, and how sad I was about that.

And then fast forward a few days, and we learned how much more serious this disease was, and we learned about transplant possibilities and how, what a really serious road this was going to be. And I think as we go back, and you read our journal, you just kind of watch this unfolding of our understanding. Dallas told us that their PH team had recently went on to bigger, better things. So, they used to have a PH specialist, they no longer had a PH specialist. Ironically, what they did have was a doctor that used to work with Dr. Dunbar Ivy. He was on their transplant team. And so, they borrowed him for our ICU case, and he called the shots for us while we were in Dallas. But they also said, "You need a PH specialist and we don't have one."

And so, we're like, "Okay, where are these gods of PH?" And they started naming off places like New York and Colorado of course, and Boston. And they said, "Where are you guys from?" And we said, "We're from Michigan." And they said, "Okay, U of M. There you go." And we had only lived in Texas less than a year, and we were supposed to be here two years plus for my husband's job, and said, "Okay." You get a diagnosis like this, you're supposed to be with friends and family. And so that's where we ended up, was going back home. And so everything kind of worked out the way it was supposed to. From the early diagnosis, from Dr. Ivy's former colleague being there to direct the doctors what to do and what to test, to us getting back to our home base and having that specialist live 30 minutes from where we used to live. That was great.

Since then, I mean it's been a roller coaster. This is a disease that rocks your family to the core. On some days, it's going to bring you together and it's going to tighten your unit and tighten your relationships. And other days, it's just going to shake you and it's going to make you question everything and it's going to cause lots of stress and it's going to cause lots of strife in the relationships. And then other days are just completely normal. We're a family, and we're still a family, still a unit. But we're a family with PH, and some days that makes us dramatically different than other families, and other days we're just a normal family.

Despite the fact that we have a PH team close, when you go to ER, you're not dealing with the PH team. You're dealing with the ER people. And when we were first hospitalized with that, which was about maybe a week, two weeks after she had received her central line, so we were by no means experts on mixing. We were still learning the ropes. We weren't experts on the pump. And when we ended up back in the hospital, it became clear that maybe one person on that floor, knew how to work the pump. We weren't okay with that. And so we started advocating for, "Okay, somebody else needs to know that." And we had a great PH fellow, that really went to bat for not just us, but for the program. Saying, "You know, all these nurses on this floor need to learn how to use the pump." And from there, she started training the ER doctors. And we know, when we go to ER, it's game on. We have to have our game hat on, we need to know the program, we need to know how many clicks does it take to re-prime the pump, when you're moving from a peripheral back to the central line and all of that. You have to know, but you're also kind of doing some teaching at the same time.

Probably our biggest struggle right now, as a family unit, is with the kids. There's the normal sibling jealousy, the sibling fighting, the sibling rivalry. And then add a disease on top and it's that on steroids. So, there's days where I believe Madison is jealous of things that Matthew can do that she can no longer do, or do as well. And there's very often days, where Matthew is jealous of the seemingly amount of attention that Madison receives that he doesn't receive. Which, people are very good. If Madison ends up in the hospital and they're bringing her gifts, they almost always remember to bring him something too. People are conscious of that. But that's definitely, that's a balancing act that we haven't mastered yet. We're still juggling that one.

How do we make both kids feel that they are equally as important? Because they are. But they both have different needs, and they both have different ways of showing those needs and showing those wants, showing those emotions, those frustrations. And right now, Matthew is still young enough where he has the feelings, he has the emotions, he has the fears, but he doesn't necessarily have the words to put those appropriately into words. And so, we're seeing that come out in his actions. And as parents, that's so hard, because on the one hand, you want to discipline, you want to raise this nice, strong young man that doesn't have these behavioral issues. Then on the other hand, you're like, "Dude, I get it. It must suck to be the other kid. It must suck to be the sibling." And there's days where I am so angry and I'm frustrated or I'm sad or I have those emotions too, but I'm an adult. And how does a second grader process some of the same fears and things that I'm having to process as an adult? That's not fair to him, but yet that's not something we can change. So, it's definitely a juggling act.

From day one, we said we weren't going to put her in a bubble and not let her live. So when she, she was going to be a swimmer from early on. She was just a fish. And so it was really hard when we got a central line, maybe four months in to diagnosis. It was like, "Well, what do you mean we can't swim?" And then we heard about these dry suits. And then our doctor's position was kind of "Don't ask, don't tell. You know, we get it." And so we ordered a dry suit. So, she swims. She swims differently than she did. She can't dive, she can't do that, but she still swims. She got in to horseback riding for a while. And so that was a passion and we knew, we just made sure that as soon as you're entering that barn, you have a helmet on, because of the risk of a head injury. And we made that work.

She dabbled in to archery for a year. That became kind of really cool after the Hunger Games, for girls to be in to archery and stuff. And that was pretty cool and so she tried that out for a while and said, "You know, I'm going to try this, see if I like this." So, she tries a lot of different things. And we haven't found the one passion yet. She started piano. She likes choir at school, so we thought, "Maybe this summer we'll do a drama program." Things that she probably wouldn't have tried before PH, she's now willing to try, because it's a new path. And that's important.

I'm Cheryl Wegener, and I'm aware that I'm rare.

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