Heart to Heart with Anna

Learning about Goldenhar Syndrome

May 19, 2020 Bonnie Hunt Season 15 Episode 268
Heart to Heart with Anna
Learning about Goldenhar Syndrome
Show Notes Transcript

Bonnie Hunt is a mother of three children, including a medically complex child, who has spent a lot of time in hospitals and doctors’ offices. Greg has
Goldenhar Syndrome, as well as LPA ring-sling complex. She is from the
Canadian Prairies and is familiar with many hospitals in the country.
She is a devoted mother and advocate for families dealing with
Goldenhar Syndrome. 

Bonnie joins Anna to talk about what Goldenhar Syndrome is and how this rare birth defect has affected her family. She also talks about what it means to have a child with special medical needs in rural Canada and the special challenges her family faces dealing with hospitals that are many miles from her home. 

Recorded during the height of the COVID-19, Bonnie and Anna discuss what it means for a medically-fragile child to deal with germs, how the coronavirus is affecting the congenital heart defect community, and what language is now part of the world's experience and how that vocabulary might help Bonnie in the future.

To learn more about Bonnie, check out her blog: https://dwellinglogs.com

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Bonnie Hunt : 0:00

Your child is fearfully and wonderfully made, and never let the medical version of normal interfere with you seeing your child is perfect.

 

Anna Jaworski : 0:13

Welcome to Heart to Heart with Anna. I am Anna Jaworski amd the host of your program. We are in our 15th season and we're happy you're here with us today. I'm very excited about today's show to feature a special heart mom. Today's show is entitled "Learning about Goldenhar Syndrome." Bonnie Hunt is a mother of three children, including a medically complex child who has spent a lot of time in hospitals and doctors offices. Greg has Goldenhar Syndrome, as well as LPA ring-sling complex. For those of you who do not know what LPA is, that stands for left pulmonary artery. So, that's Left Pulmonary Artery ring-sling complex. She is from the Canadian prairies and is familiar with many hospitals in the country. Bonnie is also a homeschooling mother. Previously she worked with children with autism. Now she is a devoted mother and advocate for families dealing with Goldenhar Syndrome. Welcome to Heart to Heart with Anna, Bonnie.

Bonnie Hunt : 1:12

Thanks so much for having me. I'm happy to be here.

 

Anna Jaworski : 1:14

I'm excited for us to talk about this syndrome. I had never heard of this syndrome before. So I think we're going to be introducing something probably pretty new to a lot of our listeners. Let's start by getting a good definition from you. What Goldenhar Syndrome actually is.

 

Bonnie Hunt : 1:31

If you Google Goldenhar Syndrome, you'd probably see a lot of pictures of people, or children, with asymmetrical faces. Goldenhar is sometimes mixed up with other craniofacial anomalies or other conditions. One that it's commonly mixed up with is Cranio-Facial Microsomia, or CFM. But it's usually that with an epibulb or dermoid in the eye, like piece of skin in the eye, and vertebral anomalies in the spine. And to be properly diagnosed with Goldenhar Syndrome, my understanding from our doctor's appointments and specialists is that you have to have at least five anomalies in certain categories. And those categories are your eyes, your ears, your spine, your heart, your lungs, your kidneys, your central nervous system. And there's a few other categories as well, but to get the Goldenhar diagnosis, you do need to have five in that list. 

 

Anna Jaworski : 2:19

Okay, 

 

Bonnie Hunt : 2:20

So early on, I even read that less than 5% of Goldenhar is genetic. What was explained to us is that basically, it's just a glitch that happens in the first trimester of pregnancy. But something goes wrong in that first trimester, and every single thing that was developing at that very small moment in time now will have an anomaly in it, which is why you get all these different things. It's not a condition that just focuses on one area. It's so many areas because it happens early on. So that's how our specialist explained it to us is that it's just kind of a glitch, a chance thing, not something that we ever really have to worry about happening again. It's...yeah

 

Anna Jaworski : 2:59

Oh, okay. So that's why you didn't have any concern with having two more children, because you knew that it was very unlikely that it would happen again.

 

Bonnie Hunt : 3:10

Yes, that's right. We did already have our healthy oldest son, and then our second son was born with Goldenhar syndrome. And that was actually a really big conversation with our geneticists, because we weren't sure if we wanted to have more kids. I think anybody with a child with a disability kind of goes through that motion of like, hmmm, should we have more kids? Is it fair to have more kids? So it's a big conversation, and part of that was talking about the genetic end of Goldenhar syndrome and why it happens. So Goldenhar syndrome is considered to be a rare disease. And in our case, it was quite unusual even in the goldenhar syndrome realm because our child is quite mildly affected cosmetic wise, but his heart anomaly has severely impacted his airway. So it's definitely kind of a different branch. Every single goldenhar case will probably look very different because It affects so many different areas in millions of different combinations and severities.

 

Anna Jaworski : 4:05

That's amazing to me that there can be that many different combinations. But I think you're right, I think from what I saw, because of course, as soon as I saw your guest survey form, and you had said you were interested in coming on the program to talk about this. I immediately went to Google and started to read about it, and I couldn't understand some of the things that I was reading. There were terms that I was unfamiliar with. So my solution to that is to go to google images. 

 

Bonnie Hunt : 4:34

Yeah. 

 

Anna Jaworski : 4:35

So, I went to Google Images, and then I was like, 'Okay, now I understand some of those terms.' Plus, I also used some of those medical online dictionaries to help me understand, because they were using a lot of medical jargon that's not heart related. And I was a teacher of the deaf and I studied speech pathology, so I was used to certain terms but, these are terms I had never heard of before and then it's just started to make a lot more sense to me. So what you're saying then, is that it seems like your son's heart and trachea issues were the main concerns.

 

Bonnie Hunt : 5:13

Those were his life threatening things, that-that was the biggest concern. Everything else for him is a cosmetic thing. It's not affecting his quality of life at all. 

 

Anna Jaworski : 5:22

Right 

 

Bonnie Hunt : 5:23

And it's not typical. So his heart and trachea issues, they are related to his Goldenhar syndrome. But in a typical Goldemhar case, that's very rare.

 

Anna Jaworski : 5:32

Okay, well talk to us about what LPA sling-ring complex is because that was something I had never heard of before.

 

Bonnie Hunt : 5:41

Right. So like you said, LPA is left pulmonary artery. So a ring-sling complex is two things and first, it is complete tracheal ring. So normally, when you have a trachea, you usually have and are born with with these c shaped cartilage rings that kind of make your trachea pipe and so the 'C' is hard cartilage and that soft spot is what stretches and grows as you grow, then your air pipe will get wider and you'll be able to breathe as your body grows. So complete rings are when you're born with no 'C' shape but a complete ring. So your trachea can't grow wherever there are complete rings. In our case....yeah... in our case

 

Anna Jaworski : 6:20

Wow! That's dangerous! That sou,nds very dangerous. Oh, my gosh! Okay,

 

Bonnie Hunt : 6:25

... so the complete rings can vary. Different people with this condition and in that realm can vary with how much of their trachea is affected. Some people might have a few rings that are complete, some people might have just a portion of their trachea, which is your airway, that is complete. In our case, our little guy had complete tracheal rings right from the top to the bottom of his breathing pipe. 

 

Anna Jaworski : 6:47

Wow! 

 

Bonnie Hunt : 6:47

So his was an extreme case that way. And then the other part of the complex is your LPA sling your left pulmonary artery sling. And basically what happens is your pulmonary artery should come out of your heart in a main trunk, and then they split into two. Right, so you have your right pulmonary artery coming off of your trunk and your left pulmonary artery coming out of your trunk. But when you have a sling, what happens is your LPA actually grows off of your right pulmonary artery instead of the main trunk and that causes, instead of your left pulmonary artery, then growing nicely in front of your breathing pipe, it actually weaves in between your breathing and swallowing pipe, your trachea and esophagus. So creating a sling around the breathing pipe. If you can imagine that. I hope I'm describing it well enough.

 

Anna Jaworski : 7:37

You did! You described it very, very well. It took me searching on several different websites before I got a picture as clear as what you just painted for me. 

 

Bonnie Hunt : 7:45

Oh, good. 

 

Anna Jaworski : 7:46

And that just amazed me because as a student of speech pathology, I did learn about the trachea and the esophagus and to hear that the left pulmonary artery was forming a sling. I thought 'oh, my goodness, this is really scary.' Let's talk about his other heart defects because this isn't the only heart defect he has, right?

 

Bonnie Hunt : 8:08

It's the one that's caused the most trouble. 

 

Anna Jaworski : 8:10

Right, right.

 

Bonnie Hunt : 8:11

In his surgeries... 

 

Anna Jaworski : 8:13

in the next segment. 

 

Bonnie Hunt : 8:14

Sure. Yeah,

 

Anna Jaworski : 8:15

but as if this wasn't enough to deal with the fact that the trachea is not going to have the ability to grow like it usually does, and that the left pulmonary artery is originating from the right pulmonary artery instead of where it's supposed to originate from and ringing around the trachea. He's got other things going on. What else does he have going on?

 

Bonnie Hunt : 8:32

He does. So, his pulmonary arteries, in general, are small. So far, cardiology is happy his lung perfusion, like the blood flow, is very nice and even but they are small, so that's something that they are keeping an eye on. The other thing is he does have a small ASD, which is the Atrial Septal Defect, it's a hole in the wall between the two upper chambers in your heart. And a lot of times if kids are born with a small hole, their doctor aren't concerned it will usually close up. In our case, it hasn't closed. And at this point, the doctors are assuming that it's not going to close. It's not causing any problems right now. So the plan going forward is just to leave it. The only other kind of strange thing about his heart, your heart's kind of tipped to the left side of your chest, his is actually quite upright and more in the center of his chest, which is a little bit unique. But yeah, just how everything grew there. So that's kind of a summary though of what's going on with his heart. So, cardiology is pretty happy with us. We're just down to annual appointments now. Just checking out making sure everything is growing nicely, but yeah, we got small pulmonary arteries and a hole/

 

Heart To Heart with Michael Promo : 9:41

Texas Heart Institute were offering us a mechanical heart and he said, "No, Dad, I've had enough. Give it to someone who's worthy." "My father promised me a golden dress to twirl in. He held my hand and asked me where I wanted to go." "Whatever strife or conflict that we experienced in our long career together was always healed by humor." Heart to Heart with Michael... please join us every Thursday at noon Eastern as we talk with people from around the world who have experienced those most difficult moments.

 

Questions and Comments : 10:10

This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. The opinions expressed in the podcast are not those of Hearts Unite the Globe, but of the Hosts and Guests and are intended to spark discussion about issues pertaining to congenital heart disease or bereavement. You are listening to Heart to Heart with Anna. If you have a question or comment that you would like addressed on our show, please send an email to Anna Jaworski at [email protected] That's [email protected] Now back to Heart to Heart with Anna. 

 

Anna Jaworski : 10:51

Bonnie, before the break we learned about your son's diagnosis and complicated medical history but in this segment, let's talk about your son's surgeries. How old was your son when he had his first surgery and what did he have done?

 

Bonnie Hunt : 11:03

He was five months old. Basically, the doctors just kind of wanted to wait as long as they possibly could before the surgery was extremely necessary. So five months is how far we made it. And actually, our pre op for that first surgery was when our oldest son was given your book called 'My Brother is Going to Have an Operation.' So that was so special to us. 

 

Anna Jaworski : 11:24

How neat! Wow, I didn't know that.

 

Bonnie Hunt : 11:27

Yes, yeah. And we ran through it again, just not long ago, and all of the answers for like, you know, you fill in the blank of different things in the book. And that's when I found you on social media. I saw your name and I was like, I'm just gonna try to see if I can find her on Facebook and send her a message to say, "this book means so much to us."

 

Anna Jaworski : 11:46

Well, I'm so glad you did that. But, five months old to have his first surgery. That must have been pretty scary for you, and how old was your older son?

 

Bonnie Hunt : 11:56

So my oldest son was two and a half when surgery happened. And so my Yeah, my so our little guy was five months. That is a tough age. I think any age of surgery is tough, but going through a couple of them, I know that five months is hard because they can't tell you if they're hurting or if thry're grumpy or if they're teething. So that part of it, the healing part, was kind of hard. And yeah, so the surgery he had it was very unique. The surgery had to involve ENT like otolaryngology and cardiology, so that is not very often that an ENT surgeon and a cardiology surgeon have to team up in the operating room. 

 

Anna Jaworski : 12:34

Right, right!

 

Bonnie Hunt : 12:34

So, it was a little bit unique

 

Anna Jaworski : 12:36

Working with that LPA for the first surgery, I'm guessing?

 

Bonnie Hunt : 12:39

Yes

 

Anna Jaworski : 12:39

Okay,

 

Bonnie Hunt : 12:40

Actually, they did both. So our first surgery was a combination, they did an LPA re-implementation, which is just basically taking that left pulmonary artery, cutting it off of that right pulmonary artery and putting it back on the main trunk somewhere, stitching it up. And then, they also did a slide tracheoplasty at the same time, and this actually has to be done behind the heart. It's an open heart surgery on bypass. So they have to actually flop the heart aside and work on the trachea. And the slide tracheoplasty is actually quite a successful surgery. It hasn't been around for a long time at this time our surgeon said that worldwide, there had not been 100 of these done. 

 

Anna Jaworski : 13:21

Wow! 

 

Bonnie Hunt : 13:22

So, it's very successful, but there was hardly any statistics. They couldn't give us probability or outcomes or anything like that. But what a slide tracheoplasty is though, imagine your breathing pipe, your airway as a pipe, what they do is they cut it at the smallest skinniest portion, and they fillet both sides kind of up one side and down the other side. And then they stretch them together and stitch them up. So what you end up having is an airway that's sort of half as long as it used to be but twice as wide. And the trachea is made up of really stretchy material, so it'll stretch where it needs to be. Like it can reach. But yeah, so that's how they get your airway to be kind of twice as wide. And they stretch and stitch it up.

 

Anna Jaworski : 14:07

And by using his own material, it will grow with him. 

 

Bonnie Hunt : 14:11

That's right. 

 

Anna Jaworski : 14:12

And they don't have to worry about rejection or anything like that, that they might have had to worry about. That is fascinating!

 

Bonnie Hunt : 14:20

Yeah, I believe before the 90s. This didn't exist, and they did use a patch, but the trachea, in particular we were told, is very picky and that was very unsuccessful. There was a very high mortality rate.

 

Anna Jaworski : 14:33

Wow. 

 

Bonnie Hunt : 14:33

So, I don't know when the first slide tracheoplasty was, but I believe it was within the last 20 or 30 years.

 

Anna Jaworski : 14:39

Wow, I had never heard of it before. 

 

Bonnie Hunt : 14:42

Yeah, 

 

Anna Jaworski : 14:43

That makes sense that this happened after I was a student of Speech Pathology because I'm sure speech pathologists nowadays are learning about it, even though it's unbelievably rare, probably... 

 

Bonnie Hunt : 14:53

right. 

 

Anna Jaworski : 14:54

not everybody is going to know about this. Okay. So you must have been so nervous in that waiting room because it's not just a heart surgery. You've got people working on his heart and his breathing tube, which either one of those if there's a problem, it could have dire consequences.

 

Bonnie Hunt : 15:14

That’s right, yeah. And our surgeon, it was a little bit difficult because we would ask, what are our chances and he had no numbers, 

 

Anna Jaworski : 15:21

Right. 

 

Bonnie Hunt : 15:22

...there's no percentages out there. There's not 100 cases. 

 

Anna Jaworski : 15:25

Yeah, 

 

Bonnie Hunt : 15:25

He said, it's a big risk, but basically without the surgery, there's no other choice. So in a way, it was an easy decision to go with the surgery because we didn't have another choice. But yeah, the waiting room is never fun.

 

Anna Jaworski : 15:40

No. Now, when our son had his first surgery, it was an eight hour surgery. I'm thinking this was at least eight hours or longer.

 

Bonnie Hunt : 15:48

Yeah, I think it was around eight, maybe six or eight. I don't remember exactly, but something like that.

 

Anna Jaworski : 15:53

Wow. That's just unbelievable. So why did he need a second open heart surgery? Tell us more about that.

 

Bonnie Hunt : 16:00

So yeah, he needed a second surgery. Originally, our specialists and our surgeons had told us this is a one time deal. We get to do it once we can't do it again, you can't keep cutting and sewing the trachea, it can't handle it. And so they said it's a one time surgery. But what was happening is every time he was getting a cold, his colds were getting more and more severe. We went from being hospitalized in the regular ward for every cold to going into close-ups for every cold, and then pretty soon every cold we were in the ICU on BiPAP machines and CPAP machines. And then it got to the place where every single runny nose, we were in the ICU within 24 hours. 

 

Anna Jaworski : 16:40

Wow! And you live in Canada, where it's cold, runny noses are probably pretty darn common.

 

Bonnie Hunt : 16:46

Yeah, yeah. Our closest children's ICU is five hours away. And so, if there was any kind of runny nose, we packed the kids up and we started driving. We've had lots of life flights as well because sometimes cold develop in the night But basically, every cold was getting so severe. And the children's PICU, they were exhausting all of their resources. and it was actually them that came to us and said, Every cold is getting harder for us to help him and we're in danger, and these are just common colds. It was like Rhino virus. We did have RSV once, which you know, all the heart, people know that word. 

 

Anna Jaworski : 17:20

Right, right. 

 

Bonnie Hunt : 17:21

But yeah, so they actually advocated to our bigger hospital out of our province to say, this kid needs another surgery or again, we're back to that place of if he doesn't have surgery he's not going to live long, 

 

Anna Jaworski : 17:35

Right, right. Okay, because the longer he is on ventilators and needing that kind of support, the more difficult it is on his lungs. So it's not enough that he's got trachea problems, heart problems, but then you're going to add the lungs to that's just too dire. So, what do they decide needed to be done? 

 

Bonnie Hunt : 17:53

Basically, what they decided to do was another slide ytacheoplasty, which was another open-heart surgery because you have to work behind the heart So, so again, our surgery hospital, our same surgeons got together, they made a plan. They phoned us we had a lot of phone meetings with them discussing it. Again two have these surgeries, I haven't found any record of it. They weren't sure if it had been done twice before they got together, they made his trachea twice as wide before, mostly working in the middle and upper trachea because that was the most narrow areas. But they said there was some trachea that they didn't cut and sew at the very bottom and down into where your trachea breaks into your two bronchial branches, and the only thing with that is they said, "We don't even want to go down to the broncial branches." They said, "that is new medical territory. We don't know what's going to happen down there." The Carina is what's called that upside down y intersection where your airway goes into the bronco branches and they just didn't want to play with that at all. So we had a lot of talks before surgery and they decided, 'let's go ahead We'll do this slide tracheoplasty in the bottom, one third of his trachea, and we'll see about the broncial branches.' We actually, at one point, decided 'no, we're not going to touch those broncial branches. It's not been done. It's not going to be something that we play with and...' 

 

Anna Jaworski : 19:13

Okay, but hold on, hold on, from what little I know of he heart, okay, if you have a narrowing, it's called stenosis, and it's those narrowed places that can cause problems. I would imagine that if he's having breathing issues where he's having to go into the hospital, it could be because there was that narrowing that led to his bronchii.

 

Bonnie Hunt : 19:34

Right, right. Yes. And in the first surgery, the upper and middle of his airway was the most stenotic, and so now that he has grown as a boy, it was about three years later, now that he has grown, when they went in there did their CT scans. Now when they were looking at it because he's got those complete ratings and nothing stretching and growing. The surgery site, the scar tissue and stuff was able to stretch and grow in the middle and top third, but that bottom, where the Carina is and breaking into the broncial branches, those were now looking like the smallest areas and causing big problems.

 

Anna Jaworski : 20:08

Sure, and that would be really really scary because those were still the highs of the infant that have been born and they're not going to throw. Wow, okay. 

 

Bonnie Hunt : 20:18

Yeah, 

 

Anna Jaworski : 20:18

So I'm always scared for him now and I'm scared for you. Don't leave me on the edge of my seat. Tell me what they did.

 

Bonnie Hunt : 20:24

Yeah, so they went in and just before surgery they said "you know what, sign the paperwork to allow us to go into the broncial branches if it's really small," and we had kind of decided we weren't going to do that, but we signed the paperwork anyways. So the second surgery, they opened him up and I mean, we were in the waiting room I guess and afterwards they came to us and they said, "You know what, when we got him open and we're in there working those broncial branches, especially on the right side, were very small. They said we can see why he was having such severe colds. So they did end up going and doing this slide tracheoplasty right into the right broncial branch a little bit. They didn't do the left side, but the right side. It's really interesting because our surgeons are wonderful, they were loving and they were caring, and we have such a good relationship with them. But it's really interesting and almost exciting to see them put their skills to use, and for them to be excited about doing something that hasn't been done before, or that hasn't done very much before. They're compassionate, but they were quite excited to be able to have a case like this to help.

 

Anna Jaworski : 21:31

Yeah, I mean, what they were doing was saving your son's life without a doubt. 

 

Bonnie Hunt : 21:35

Yes, absolutely.

 

Anna Jaworski : 21:37

And improving his quality of life because who wants to be in the hospital all the time? 

 

Bonnie Hunt : 21:42

That's right. 

 

Anna Jaworski : 21:43

Wow. So it sounds like he was in and out of the hospital an awful lot before that second surgery, and you're a homeschooling family. So how in the world do those hospitalizations affect your other children? especially regarding their education while little brother or big brother, depending which sibling you're talking about, is in the hospital all the time.

 

Bonnie Hunt : 22:06

That's right. Yeah. So first of all, for our family, Ronald McDonald House has been a godsend. And so when we are in the hospital, we have somewhere to stay that we can all be together. And I think that awareness of attention for all your kids is really important. It can't be perfect. When you're living hospital life, you are caring for the one in the hospital and they're your main concern. They're your main conversation point. So life can't be fair, unfortunately, for all the kids all the time when you're not at home. But I think awareness of that is really important. But what's really cool with our other kids is I think, especially I can see it in our oldest child who's two years older, is learning empathy. 

 

Anna Jaworski : 22:50

Yes, 

 

Bonnie Hunt : 22:51

I remember one time we were in the ER in our local town, we were waiting for an emergency lifeflight to come and get us and my husband was on his way to come help and get the other kids And anyway, but we were in this ER and waiting for the lifeflight and the nurses had to do an IV, and my oldest son was sitting behind my younger son and he's like, "oh, buddy, like you can do this! Gotta be brave." So I feel like it has affected our family, and our kids, in both a negative way and a positive way. Like, I don't know how on earth my other son and my daughter growing up too now would have the same practice in their life for empathy and care for people like they would with their brother who needs a lot of care and attention and is in danger sometimes. So I think that that is the one kind of neat thing that does happen at the hospital and as far as education goes, I was telling you earlier that I was homeschooled from K to 12. So homeschooling is not strange for me. I actually definitely would have considered homeschooling even if we didn't have a child with difficulties, and so I was really excited to homeschool and it is different. than I thought it would be. I thought we would be the family that's out and about, you know, evolved and all the community events and all the sports, which isn't the case we do stay away from the public places and the germs and stuff like that. But it's been really great because when you're homeschooling, you can school anywhere you want. We bring school books to the hospital visits, they don't get a vacation when we're living at the Ronald McDonald house. So it's been really, really cool. I love homeschooling. I'm really passionate about homeschooling, and making it fun and wonderful, and it's just so flexible. I don't know how we would cope not only in our emergency trips, and our inpatient stays at the hospital, but even all of our medical trips driving, we take our schoolwork with us because we need to and otherwise we get behind because we take so many trips all the time. So it's really really neat to have the opportunity to homeschool because we just don't have to get behind and we don't have to sacrifice education because of what's going on with hospital life.

 

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Heart to Heart with Anna is a presentation of Hearts Unite the Globe and is part of the HUG Podcast Network. Hearts Unite the Globe is a nonprofit organization devoted to providing resources to the congenital heart defect community to uplift, empower, and enrich the lives of our community members. If you would like access to free resources pertaining to the CHD community, please visit our website at wwwcongenitalheartdefects.com for information about CHD, the hospitals that treat children with CHD, summer camps for CHD survivors, and much, much more. 

 

Anna Jaworski : 26:50

Before the break, Bonnie, we were talking about Greg's surgeries. Now I want to talk about your role as an advocate in a Goldenhar syndrome community. Why is it important for us to know about Goldenhar syndrome and LPA ring-sling complex?

 

Bonnie Hunt : 27:07

In general, I think it's important to raise awareness for all rare conditions. Some people don't know the inside story when you meet someone, and as a family with someone with a rare condition, you don't want to just be talking about your medical life all the time. But there is this inward desire for people to know and understand all the things that you have to go through. So I think just awareness for those kinds of conditions. One thing that we have in our home, we do the beads of courage program. I think you guys have that in the United States, 

 

Anna Jaworski : 27:39

We do

 

Bonnie Hunt : 27:39

...where you can have all these beads that show all the yucky stuff that your kid has to go through and your child can tell their story. When we come home from a hospital admission. My kid can show their grandparents his new necklace and like this has how many needles I got, and this is how many times I had to do this and that. So I think that raising awareness of some of the struggles is really an important thing. The Goldenhar community was really excited a little while back when the movie 'Wonder' came out, actually it was a best selling book as well, right? Actually, I think for craniofacial anomalies, that was huge, because globally and like it was in the media, and people just seemed to really become aware of what someone's life might be like if they look different. So that was a really exciting thing. And also with kind of awareness of rare conditions. I think it's also good to promote awareness for the health compromised. We're living in COVID world right now. COVID world is our norm. 

 

Anna Jaworski : 27:39

Right! 

 

Bonnie Hunt : 27:39

We mask up the kids when we go to the grocery store on a regular day. 

 

Anna Jaworski : 27:52

Do you do socially? 

 

Bonnie Hunt : 28:33

Yes, we do. Yeah, because we still avoid colds. My son's surgeries didn't fix him, they did help a whole bunch, but if he gets a bad cold, he still has to go to the hospital. And so, it's something that is kind of ongoing. So I think it's really important to advocate for the health compromised, which is really interesting because our government right now is saying, "stay home, stay home for the health compromised," and it's really cool in a way for us because after this whole COVID thing blows over, I am now going to be able to have language to say, when someone's asking us about our lifestyle, I can go Yeah, you remember COVID? 

 

Anna Jaworski : 29:22

Right.

 

Bonnie Hunt : 29:22

That's how we live

 

Anna Jaworski : 29:24

Yes, I've been thinking about that and I've been talking to other people in the heart community. And you're absolutely right, especially for us when we were between surgeries, my son was born with a single ventricle heart, and we were told when he was diagnosed, he would probably need three surgeries. And then that inter-surgery time was critical that if there was a time that he got really sick in between those surgeries, it may prevent him from being eligible for the next surgery. So just like you're saying, we didn't go to church. There were a lot of things that we just stayed away from. How can you go to church with an adorable baby, and people not want to hold your baby kiss on your baby and touch your baby's face and all those things that you do not want them to do. 

 

Bonnie Hunt : 30:08

I know exactly

 

Anna Jaworski : 30:09

So yeah, you just stay away. And so you're right, we lived the COVID lifestyle for months, if not years. And I don't know about you, but a lot of my family and friends had a really hard time understanding why... 

 

Bonnie Hunt : 30:23

Yes, 

 

Anna Jaworski : 30:23

...we were doing that, because when they did see him, he looks so quote unquote, normal unless he had a shirt off... 

 

Bonnie Hunt : 30:30

Yeah. 

 

Anna Jaworski : 30:30

...and then they saw the big scar, and they knew that we wasn't normal. But so for us, I think that this whole COVID-19 experience, it is actually April 2020 when we're recording this program, so for those of you who are listening, we're in the thick of things. We're still in lockdown in most parts of the United States, and worldwide, people are staying in instead of going out and I think for the first time ever, people are are, well, maybe not the first time ever, maybe during the Spanish flu epidemic as some other epidemics, people realize the importance of washing your hands and not sneezing or coughing on people. But I think that we had gotten really, really lax.

 

Bonnie Hunt : 31:15

Yeah, yeah. And with modern medicine, we just rely on that to fix us when we get sick, so we're not too worried about spreading or catching germs usually.

 

Anna Jaworski : 31:23

Right! What has been the biggest challenge that you have faced in living with a child with Goldenhar syndrome?

 

Bonnie Hunt : 31:31

Yeah, you know what? Kind of on the coattails of what you were just saying to is the germs and social distancing. That has been probably the biggest challenge, like you were saying, friends and family understanding. 

 

Anna Jaworski : 31:42

Yeah, 

 

Bonnie Hunt : 31:42

That has been really hard. I like to make people feel warm and comfortable. I like to be welcoming and hospitable. And when someone shows up on my doorstep, coughing and sneezing, wanting to come in for a cup of coffee, it just kills me to send them away, or we've shown up for events with our kids. And there's other kids there that have colds and runny noses and you know, boogers coming down. And I've had to tell my kids, you guys back to the car, we got to go. And it's interesting, like my kids are young amd they're so used to disappointment. They're so used to playdates being planned and canceled because of germs. So I think that has been something that we are constantly dealing with and that has been a big challenge. The other challenge has been having people understand, I guess, what we're going through with the severity of my child's cold. You know, that we're not just in the regular ward, which sucks, but we're also in the PICU. A lot of people, now my kid is four years old, so people are used to us saying, "Oh, yeah, we're in the hospital again," and it kind of becomes this normal. "Oh, yeah, that well, they're in the hospital again, that's normal for them," and people don't actually understand the picture of what's actually going on and how intense the care can get and how stressful so that has been a challenge. Also, bringing out my mama bear. That, that's really been a challenge for me. It comes so naturally to other moms and it does not come naturally to me and I have struggled with that. One time we were in a hospital admission with new doctors. And I was on the phone with my specialist and I was like they are not taking care and attention the way they should, we have signs here, things are going south, we really need help. And I remember our specialist, it was our surgeon at the time, and he said, "Bonnie, you got to bring out your mama bear." He's like, "I will phone and I will kick up a ruckus if I need to," but he said, "you get your mama bear out and you tell them what's what." So, you know, I've had to work on doing that. Another challenge is travel. I actually went through our records before this conversation and in the last four and a half years, we have driven more than 43,000 kilometers by car for emergencies and medical appointments. We've flown over, we've flown over 6000 miles by air for planned appointments and we've had numerous emergency Life Flights all in between there because if he gets sick very quickly, we don't have time to drive the five hours to our nearest PICU. We have to of course, go to the hospital and wait, and we've had experiences that were stressful with the traveling and the distance between help, because our small town hospital doesn't have the children's equipment that he needs to breathe. Being so far away from help... 

 

Anna Jaworski : 34:16

Yeah, 

 

Bonnie Hunt : 34:17

...being very remote and kind of a rural family. So that has been very challenging and has been. We have stories of very stressful times. But all through that. I mean, the medical staff are amazing. Our specialists, we have their phone numbers, and so we have care and we have good communication with doctors is key. So it's worked out so far.

 

Anna Jaworski : 34:38

So I'm curious, when my son's heart defect was diagnosed in the 1990s, and we were three and a half hours away from the hospital that took care of him. I asked the surgeon, "Do we need to move closer to the city?" and he actually said "No, that if something were to happen, it wouldn't matter if Alex was on the table on front of him, he probably wouldn't be able to open him up fast enough to save him," which was not very comforting to hear that but it was okay for us to stay where we were, which was a huge blessing. Have the doctors talk to you about "why don't you consider moving to a big city?"

 

Bonnie Hunt : 35:15

You know, I'm sure that we did have that conversation earlier on, nothing recent. And we have talked about that. The thing is, too is that we do farm so moving to a city would mean moving jobs completely, our family has been farming for generations. 

 

Anna Jaworski : 35:31

Yeah, you'd be giving up a lifestyle 

 

Bonnie Hunt : 35:31

Of course we would for our son, but again, that the time that we talked to our doctors about it for them, things have been okay, we've been able to get help. So there's not a really good straight answer for that. I think in the doctor's world, they probably would love it if we live down the block. But I think they really seem to consider your lifestyle and how much are you going to revolve around your medical emergencies? How much of your life are you going to concentrate on that, or are you going to have better quality of life? There's beautiful things about living where we live, and, you know, being away from germs and away from the city and stuff like that, too. So I think all together in big picture terms, they're happy with us for now to stay where we are, not to say that that could change if something became very severe though. 

 

Anna Jaworski : 36:16

Right, right. Well, you've had quite a few years now to live with this. What do you have for families who are dealing with having a child with a special medical condition like this, or especially for families that have multiple children like you do?

 

Bonnie Hunt : 36:32

Yes. Yeah, you know, my list is long for advice. First of all, I think my biggest thing is just to say that your child is fearfully and wonderfully made, and never let the medical version of normal interfere with you seeing your child as perfect. I think that is something that we all need to get right in our heads. Second, second, make a website. It can be private if you want it but I know firsthand that friends and family in very good intentions will play telephone with all the things that are happening medically, and by the time you get five phone calls in, the story is completely different. So the second piece of advice is make a website that you can blog or post on so that the facts comes straight from you and all your friends and family can read it. Ask for paper copies of medical reports and keep them in a home binder. That has been really key, especially for Goldenhar syndrome, probably other things too. 

 

Anna Jaworski : 37:25

Yes.

 

Bonnie Hunt : 37:25

But when you got lots going on, we have lots of different kinds of specialists because of all the different categories that we fall into. So it's really important to have your paperwork. A big thing for me is, for advice, if I could have known this at the start is to be a team player with your medical team. So you do need to learn to advocate when you need to, because not everyone is going to know what your child is going through and what they need. I wish I would have understood the roles of doctors and nurses and understood that nurses can't just change a medication without doctor's approval and they can't just do some of the things that you think you need when you're in inpatient, or even understanding that when you call a nurse in, she might be in another room with a crying child that has no parent at the bedside. So I think understanding the roles of people in the hospital is really good to get a grasp of, and build relationships with your medical team. Build relationships with your nurses, talk to them. If you're awake all night, why not talk to them, you know, they're usually in the hallway, and it's worth it down the road for me to build a relationship with our doctors and nurses because we're in the hospital so much and honestly, they feel like family and there's no better team to help your child than a team that's getting along when you're a parent. 

 

Anna Jaworski : 38:39

Wow, so much helpful advice and I'm sure you could write a book just on advice for parents. I think you covered the most important things, keeping the records, being part of the medical team, understanding the roles of the different people who are part of the medical team. Those are nuggets. They are such pieces of Important information that every parent really should know, but I think a lot of times you are so struck with, 'oh my gosh, my child could die.' I mean, we're so focused on just about that, that we can't absorb all of the other things that are going on around us and the things that we need to do. We're on automatic pilot. 

 

Bonnie Hunt : 39:18

Yes. 

 

Anna Jaworski : 39:19

And it takes us years. I think it takes us years to really get through to the point where we can maybe look at this a little bit more objectively. So for anybody who's pregnant right now, who's listening to this program, don't get stressed out. Start making friends with the professionals that you're dealing with are ready and do understand they will be like family to you. You can get through this because you're not going through it alone, and I think that's probably one of the most important things to know, don't you, Bonnie?

 

Bonnie Hunt : 39:47

Yeah, yeah, for sure. And I was going to add to that for families with children sticking together and having a good team attitude with your family, gives you emotional stability and helps you to face anything. It's important to be conscious om spreading attention to all your kids and if you can be in a team relationship with your spouse or another caregiver that's going to help you through all of your appointments and medical visits and everything. The biggest thing that we've learned is to stick together as a family. Whenever possible. I think you actually mentioned that at the end of your book that you wrote is that, that was really important that you guys had learned that too. That just to stay together and to be a team together means the world when you're in a very stressful situation.

 

Anna Jaworski : 40:28

right? And for us routines, were really important having our bedtime story singing our night nights, having our prayers together, those kinds of normal activities, even when you're doing it in the hospital. Yeah, there's the Ronald McDonald House, wherever you are that the kids know. Well, mommy and daddy are still gonna read me a bedtime story. 

 

Bonnie Hunt : 40:51

Yeah. 

 

Anna Jaworski : 40:52

We're still going to sing the night-night song. Those kinds of activities for me, that's what kept the really stupid crazy hours that you have in the hospital from being too overwhelming, 

 

Bonnie Hunt : 41:04

Right 

 

Anna Jaworski : 41:04

It's so easy to get that ICU psychosis. You don't know what time of day it is. You don't know yjr date. You're going on not enough sleep and too much adrenaline. So I think that you're right, being a team and helping each other. Another thing that was important to me was letting Joey, letting my son who wasn't afflicted by medical conditions, letting him be part of the care team. He was really an important part of his brother's healing. When Joey walked in the room. Alex was all smiles.

 

Bonnie Hunt : 41:35

Oh, yeah. 

 

Anna Jaworski : 41:36

It's Important to have those siblings as part of the therapy, part of the care team. 

 

Bonnie Hunt : 41:40

Absolutely. Absolutely. 

 

Anna Jaworski : 41:42

Well, thank you so much for coming on the program today, Bonnie. I feel like we've been all friends even though we're just meeting for the first time we're definitely kindred spirits as heart moms. 

 

Bonnie Hunt : 41:53

Yeah. Yeah. I'm so happy to come on and talk about this.

 

Anna Jaworski : 41:56

Well, I think we've all learned a lot about Goldenhar Syndrome, but even more than that, I love how you shared being in the farm community and making all of that work and making this COVID-19 experience analogous to what it is that those people with medically fragile children live with every single day every day. Yeah. Well that's it for this week's episode. I hope you all enjoyed listening to it, and if you did, I hope you'll consider being a patron of our program, just head over to Patreon.com/hearttoheart and make a monthly donation. A small donation on your part means a lot to us. Remember my friends, you are not alone.

 

Closing : 42:40

Thank you again for joining us this week. We hope you have been inspired and empowered to become an advocate for the congenital heart defects community. Heart to Heart with Anna, with your host Anna Jaworski can be heard every Tuesday at 12 noon Eastern Time.