Shannin Strom-Henry - phaware® interview 274

Aug 20, 2019

Pediatric PH Caregiver Shannin Strom-Henry discusses her daughter Zoe's congenital diaphragmatic hernia and pulmonary hypertension diagnosis. She describes how being a rabid researcher, collaborating with her PH care team and the power of parent online support is helping Zoe (11) lead as normal a life as possible.

 My name is Shannin Strom-Henry. I live on Bainbridge Island, Washington, an island just off of Seattle, and I have a daughter who will turn 11 on Monday who has pulmonary hypertension.

Zoe's story goes way back. When I was 20 weeks pregnant, I was told that she had a congenital diaphragmatic hernia, and our world sort of came to a screeching halt for a little while. That diagnosis in itself means there's a hole in the diaphragm and her stomach and intestines and spleen were up in her chest cavity, so her lungs didn't form properly. That condition has a 50% chance of survival to begin with, so we knew we were in for a long haul on that.

We ended up down in Portland instead of at Seattle Children's Hospital because the ECMO beds were all occupied at the hospital at the time. They sent us down to Portland and we spent three months in the NICU down there. And I lived down there at the Ronald McDonald House, and Jason, my husband, would commute back and forth from Seattle to down there.

That was sort of the first thing we were dealing with, but part of congenital diaphragmatic hernia is pulmonary hypertension, a lot of kids that have a CDH have pulmonary hypertension as a result of that. It was a word we heard sort of bantered about when she was little, but it was never something that was the immediate thing we were dealing with, we were more just dealing with trying to get her to survive and the day-to-day of all of that.

Her repair on her hernia was done at about a month old, and as she sort of stabilized a little bit, she was still super frail, but as she was stabilized, they started talking about her pulmonary pressures more and talking about us getting back up to Seattle since they didn't have a clinic down there to deal with pulmonary hypertension. It became sort of a thing then that that was going to be the focus, along with all of the other, getting her to eat and do all of the things that she wasn't able to do at the time.

The adjustment was pretty crazy. We went from living in the hospital down there to being transferred up to Seattle Children's Hospital, and we went from the NICU to the general floor, because I think they weren't quite sure where to put us. The nurse at the time didn't even know how to suck the stuff out of her nose, which was crazy. I was like, "Oh my gosh, give me this thing." Because they were all learning. It was very much of a shock for us to go from nine people in her room all the time to just sort of being on our own.

After that, they soon got us into the cardiology clinic at Seattle Children's, and we started focusing on those aspects of it. She was immediately started on Sildenafil, some diuretics, and some other things like that at the time. She got stabile enough to go home. We ended up going home after three months in the hospital. She was on oxygen, she was tube fed, she needed a million meds all day long. I stayed home with her. I quit working as soon as I had her, and went from working full-time to being basically a NICU mom hanging at home.

We learned as we went. It was a lot of work. Tube feeds and medicines and stuff, and three, four months, five months into it, started to realize she wasn't going to get any better very quickly. And so, that's when we started talking about doing an IV Flolan.

At nine months old, she had a central line put in and we started IV Flolan. She also had a feeding tube placed at that point in time, because we weren't getting anywhere with that either, we knew that was going to be a long haul. And lots of monitoring. I mean, I think Zoe got stronger very slowly.

She was 2 and a half when she started eating. That was sort of a crazy thing. We did a rapid ween on her and basically stopped feeding her for about two weeks to make her hungry and get her to eat, and we did all this with the cardiologist at Seattle Children's, of course. They're very progressive there, and great with some ideas that we bring to them.

This was based off of a German therapist that had done this program. The Flolan helped her a lot, it stabilized her pressures. When she first went on an IV, she was at 140 and she dropped around 85 to 90, which is where she's been ever since then with her pressures.

Our thing with Zoe was to live as normal a life as we can, but make sure quality of life is good. I would still take her out and we'd go shopping and do things, and have birthday parties, and do as much as we can with her. She was a happy little kid.

The first central line that she had, she would scratch at it like crazy to the point where she was gouging herself. We couldn't figure out how or why she was doing this for the longest time. I started doing some creative things, because I work in costuming, so I know how to sew, and I started making these basically little crop-top, bra-tops for a baby basically, that would cover her site just to try to keep them out of her fingers. I would make them in all these different patterns.

It helped a little bit, but she would still get in there. We finally found out she was allergic to the dressing and to the chlorhexidine. I don't remember how we figured that out other than maybe we stopped using it to try to let it heal or something. Since then, she hasn't had any issues with that.

She's always been very aware of her pump, but she's had it since she was nine months old, so she doesn't really know any difference. Since then, I've done other creative things with it, like right now she wears her pump in a little sock on a tank top, so it goes under all her clothes.

When she was younger, it used to be in one of those backpacks, the animal backpacks that have the leashes on them that people use. I would take those and make the pocket deeper in them, because at that time, she was on the CADD Legacy Pump, the big pump, so the pump was the size of her back at the time because she was so little.

But she just would get used to it and people got used to it. I think at first it was hard for me to see people watch her in public or look at her. There used to be a woman that would come up to me at the grocery store and be like, "Something's wrong with your kid, her face is really red." I mean, I think we've all heard these stories over the years of things, awful things people have said.

But you kind of get used to it, and Zoe has adapted really well. I think she doesn't let any of it phase her. I think we keep her pump under wraps as much as we can, more for safety than anything, but she's not phased by it. She's never been embarrassed by it.

She started preschool at three in the school district, and they have been amazing, the school district's been amazing working with us on what she needs, and having a nurse on staff, and letting us talk as little or as much about it as we want to. So, a lot of kids she goes to school with have known her since she was three, four years old, and are used to her and know her limitations, know that she's sometimes on oxygen.

Nobody thinks it's weird. They know she physically has limitations, where she knows she can't run as fast as they can or all that. But they're really good with helping her when she needs it, and she's sort of shockingly not embarrassed by it at all. We will see how that changes when she gets older, but for right now, she's pretty chill about it.

Zoe's been through a series of pumps and medicines. She started on IV Flolan, we then switched to IV Veletri when she was a couple years old, which was great and we didn't need icepacks. Then we switched to Remodulin, which was great. Eventually, we were able to get on the Crono Five Pump, which is now been discontinued, that little tiny pump, which was a game changer for us.

That probably happened when she was in fifth or sixth grade, which was really nice to have that pump not be so front and center anymore. And now she's on SubQ Remodulin, which we've been doing for about three months now, so we're figuring all that out.

Earlier this year, she had sort of like a Potts shunt procedure. She had her PDA reopened. It had been closed surgically when she was about a year and a half old, and the doctor here in Seattle was able to go back in and put a stint on the side of it, from her PDA into the descending aorta, and basically do a Potts shunt without having to go in and do open-heart surgery.

We're still sort of waiting to see what the progress that helps, or if it helps at all, but she certainly seems to have more energy from then. She's still oxygenating really well and that has been great.

I have just always been a rabid researcher of all things related to Zoe. I mean, I think it started when she was diagnosed with the congenital diaphragmatic hernia. I never knew what that was, and you just start digging into the world of the internet.

There's different ways of looking at things that are different than what the medical staff in the United States, what insurance lets you do, all that sort of thing. But there's a world of information out there, so between the PH support groups, the family support group, and going back to when Zoe was tube fed, finding this German psychologist who did this rapid ween things, there's a lot of information out there and new ways of thinking about stuff.

I'm kind of one of those people that digs in and something, I'll read it and be like, "Oh my God, that's it, that's what we have to do. This sounds like it'll work." We'll try all the ways that the doctors normally recommend and if you don't get anywhere, then there's got to be other things.

We're super fortunate, Seattle Children's Hospital and Dr. Young, her cardiologists there, they're really progressive, and they like to hear our ideas, and they love that we talk to the other parents in the groups and bring them ideas. Sometimes they work and sometimes they don't, but they're always willing to hear them and potentially try them.

I think the parent groups are a life changer. I mean, we've made so much progress with Zoe because of things other people have gone through and learned on that. That probably was actually where I figured out why she was scratching and that she was allergic to chlorhexidine, because somebody had mentioned that that's what they figured out on their kid.

I think we are all our children's best advocates, and us sharing information as parents is what helps our kids, and doesn't always work for every kid, but there's some great information out there. Strength in numbers, right?

Our take with Zoe is we want her to have the best quality of life ever. We want her to be able to live life and experience life and do all the things. And so, yeah, we just did a three week trip to France. A week in Paris, and then in Strasbourg, and Loire Valley, and Normandy. It was a trip Zoe's been talking about for a couple years, she wanted to go see the Eiffel Tower.

Jason and I, my husband and I, we both used to travel a ton for work, and we haven't really done any since Zoe was born other than going to the conference and the Make a Wish trip for her. And so, one day we were like, "Well, let's see if we can do it." We started by talking to the cardiologist, and when we started talking about it, Zoe wasn't quite stable enough and they weren't ready for it. But about a year and a half ago, they were like, "Yeah, I think she's up for it now."

We started planning it, and it took a lot of work going into it, a lot of conversations with the cardiologist. Again, they're amazing and did all the paperwork for us. You need a letter to fly, and oxygen, and med orders, and all of that stuff, and they helped a lot of with that. Took a lot of planning on our part because France wasn't going to be as ADA accessible as the states are. Zoe's in a wheelchair for long distances. She can walk and do stuff, but she, like many PH kids, gets winded when she walks a long ways.

We knew we were going to have to haul a lot of stuff, and that took a lot planning, but it was worth it. I talked to other parents who had traveled internationally. Another PH mom who's a dear friend who we met down in Houston, her husband was from France, and so they had lived there for a while and she gave me the name of the cardiologist there, and the PH specialist, and so I even had all this hospital information when I went over there, which was really amazing, which luckily we never needed.

We just try to do all the things with Zoe. We don't say no to her. If she wants to do something, we let her do it. She takes hip-hop class, she takes ballet class, and she's learning to ride a bike and do all the stuff, and I just think those are the memories we want her to have, is to do whatever she can. She's a good self-advocator. She knows when to stop if she needs to. And other than that, she'll jump in and do something with gusto, and I think it's amazing.

My name is Shannin Strom-Henry and I'm aware that I'm rare.

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