Helping my child cope with an ICD/Pacemaker

She’s never felt a shock. She’s come close. That’s one of the great things, it’s monitored. She has a monitor in her bedroom, so if there’s some activity that Dr. Alexander doesn’t like, he calls us in. Immediately I’ll get a phone call, “You need to come in. Nothing major, but we need to take a look at something.” And then he’ll either increase her medication, or he just recently changed her medication and for that she had an overnight stay in the hospital.

His calls are less now. In the earlier days it used to be a little bit more because as he explains to us, “we’re dealing with something that’s individual to each patient, it’s individual to each child.” The severity of it. He’d see a little bit too many arrhythmias that we’d need to address, so we’ll bring her in and we’ll increase the number of beats before a shock.

She’ll get a bit nervous about a shock—she’s like, “What does it feel like?” I tell her, “From what I’ve read, it feels like you get a really hard kick in the back, it’s quick, and sometimes you may pass out beforehand.” They said that her defibrillator’s got a year of battery life left so it will need replacing, the battery will need replacing. It’s something that she knows. She doesn’t mind, because she loves staying in hospitals. She likes the environment of a hospital. She likes to see the other kids.

 – Jim, father of Chloe, age 13, CPVT


I haven’t experienced a shock, and neither has Rebecca. Actually, since my defibrillator was put in about ten years ago, it has never recorded anything scary, so I feel really very comfortable with my own health. My heart is kind of enlarged to the point where it probably can’t enlarge anymore and I’m able to function really well with it, and I exercise. But hers, she doesn’t exercise, she walks. She is so young for this disease, it usually doesn’t hit until you’re in your twenties or thirties. I think it’s because she was so athletic, but maybe that’s what brought it out. So she doesn’t exercise, and her defibrillator hasn’t recorded anything scary either, and she’s had it over two years. I’ve had mine replaced once. It’s an eight year battery life. Well it was eight when they put my last one it, maybe it’s longer now.

 – Amy, mother of Rebecca, age 19, ARVD

Helping my child cope with taking medications

Alice: It started out being very hard. I remember when he came home six months after his Glenn, we had to start giving him shots to keep him from forming blood clots, and those are twice daily shots, and the needle is like a diabetic needle, but the medicine burns and it’s painful if you ask an adult who’s had them. I do them myself, I did it a couple of times and just me inflicting that pain on him was—Allan had to take that over, and then I felt guilty that I couldn’t do it.

Allan: I mean, when he came home from the hospital after six months, he had an NG tube, and for a good six months, and we probably replaced 100 of them. So we learned to do it ourselves because every single time, almost 2-3 times a week, we’re not going to drive all the way to have them put it in, because then they’re going to want to send it to radiology, and then it’s just going to be a whole process, so we had to learn how to do that.

Alice: That was terrifying at first too. As he’s getting older, he knows how to say “No” now, and he knows how to say “Scared”, and that’s what he says. Some of it, like the infusions, he’s been doing them for a year now, he knows the drill. He knows on Sunday morning he sits in his little chair, he gets to watch TV for two and a half hours, and we do the infusion, and we do things like, “Okay, we’re going to count to ten, and by ten the needle is going to be in, we’re going to have the Band-Aid on.” And I think the structure of knowing what to expect and how to expect it has helped him and I think us doing it makes him feel safer. First we had a nurse come to teach us how to do it, and it’s scary having a stranger. So as much as it pains Allan and I to be the ones to do all these terrible things to him, I think it makes him more comfortable because ultimately he knows we love him and we’re just trying to protect him. His brother even knows how to help with things.

Allan: He’s a mini-therapist.

Alice: He’ll sing him songs, kiss where the shot was, hold his hand for things.

Allan: Helps feeding therapy, and everything else.

Alice: So I think he is starting to get that we’re all here to support him. It’s hard too because he’s developmentally delayed, so he doesn’t have the language to really express to us how he is feeling. “Scared”—he has that word, but that’s it, he can’t elaborate on it or say what helps him or doesn’t help him. He can’t tell us if something hurts really.

Allan: But he understands every single thing that you tell him.

Alice: Yeah, I would say for other parents having to do those types of things, it sucks. It’s scary, you hate to do it, but you get used to it. There’s no sugar coating it, I guess.

 – Alice and Allan, parents of Zach, age 3, Single Ventricle


Nick: They taught her how to sit up, and lay in the bed certain ways. She actually coped with pain pretty good.

Shana: Because she likes medicine.

Jade: I don’t like medicine!

Nick: Not anymore

Jade: Some medicine tastes like there’s pee in there.

Nick: You always say that. But it’s just here in the later years that it’s gotten like that. I would say until she was about four, they didn’t have any issues administering anything to her. I can remember coming to the hospital early mornings and not finding her. The nurses had taken her and she was just like doing rounds with them, the nurses and the doctors. She was fine.

 – Nick and Shana, parents of Jade, age 6, Dextrocardia


When she was born she was taking Propranolol, she was on it four times a day, that was for two years, and then after that she was on it three times a day until she was five—she’s always had to take some medications. We’ve never had any pushback really in terms of taking medication, it’s just been part of what she needs to do.

She started being somewhat resistant to that last year, before she had her episodes last summer, but now she knows she needs to take her medication. We tried to explain the physiology, the “why it is important to” at that point. She’d never had any problems, any cardiac problems, but so she has had seizures because of hypoglycemia, which is actually caused by the medicine that she’s taking. There was some pushback because of that. Until that summer, the only effect of the medicine that she had experienced was having seizures, so the medicine hadn’t helped her at that point. We just tried to explain how the electrical system works and why she was taking it.

I think our medical backgrounds are helpful in that we understand the biology, the physiology aspect of it, so I understand very well that taking medication consistently is essential and that it’s not okay to miss doses. Talking to other people, sometimes I’m surprised that people don’t really understand how drugs work, so maybe they’re not as consistent as they should be. But the beta blockers inhibiting those receptors, your body is going to naturally grow accustomed to the medication, so if you don’t take your medication, it’s actually worse than never taking it. I think that understanding is helpful.

 – Catharine, mother of Willow, age 8, Long QT Syndrome


Chloe is very, very determined. Prior to hospital, our routine every night was I would read to her, and then she would read to me. She was only six, they were children’s books. So when she was making a little bit of recovery, we’re like, “Let’s do that.” So I’m reading a story to her, I give her the book to continue, she picks the book up, opens it upside-down, puts it down and says, “I don’t feel like reading.” She’s just realized at that point that she can no longer read or recognize which way a book is held. Up to that point, she didn’t know she could no longer read. So there was a lot of things that came up afterwards, things like that she couldn’t read, finding words, she would use a different word, she would put a different word in place, and even to this day, you have to give her time when she’s telling you something. If she gets so frustrated with it, she’ll stop telling you. So I’ll ask her a question and she’ll go, “Oh no, it doesn’t matter” and I’ll say, “Well, actually it does, I need to know what the answer is.”

So I let her give me pieces of information as she can. She can’t always string it together, but she can give me snippets of information that then you sort together. So she still has that, and her short term memory- she’s very aware that it’s lacking, and we make a joke about it, I say “You have no short term memory because you had a cardiac arrest, I have no short term memory because I’m old and it’s gone.” So we make lists on everything we do, and as long as we can remember to take the lists with us, we’re good. We try to remember to hang things on the front door if we need to take something.

She takes medication twice a day, morning and night, nighttime medication isn’t a problem, we never ever forget that. Morning medication, I could be driving her to school and all of a sudden it’s, “Oh, sugar, I forgot to give you your medication, let’s drive back and get it.” So we started this thing now that as soon as one of us remembers, we put it on the kitchen table, so that before we leave, we know that the medication has to be taken. So, I play into my own memory loss to encourage her and to let her know that it’s okay. My memory’s not that bad, but I kind of wanted her to know, “Yeah, it’s okay. So you don’t have great short term memory, that’s okay. There’s things you can do to deal with that.”

 – Jim, father of Chloe, age 13, CPVT


He’s now on a diuretic, he’s on Coumadin for the rest of his life—it’s just tough. Because he just wanted to be a normal teenager and he wasn’t. He didn’t look good, he’s just constantly taking these meds, and obviously when we first came home I was overwhelmed by, “oh my God, how am I ever going to get to know this.” And he was still young enough that I was dispensing the meds. Now he does his own pill box and takes the pills. He’s at college now, I’d say he probably misses maybe three doses a week, just cause he’s not good about it and it’s an ongoing conversation with his doctor and nurse. Again, now he’s not even a teenager, but he just wants to be a normal person. And other people, they have to take meds, they take them in the morning, they take them in the evening, their more conscious of the fact, “I’ve gotta take these meds.” Gabriel, he doesn’t take them and then he says, “well I’m fine,” but then sometimes, he doesn’t take the Lasix and then the next day he feels like all puffy and it’s because he didn’t take it.

When he first went back to school after that surgery, he was taking the diuretic twice a day, morning and afternoon and it was a disaster because he had to go pee every like 15 minutes. So he’d excuse himself out of class, so he was missing class time. I talked to his doctor about him just taking a double dose in the afternoon when he got home, so that’s what he does. But even then, this past week, when he goes out with his friends, he doesn’t want to go out with them and then have to go pee. They want to go out to a movie, they want to go out to dinner, they want to go to the mall, and the last thing he wants it to have to go find the bathroom in the mall every 20 minutes. So sometimes he comes home at 11 o’clock, and then he takes the diuretics or he skips it, so it’s not good.

But the Coumadin is okay, because he’s got his own home monitor, so he tests his own blood every couple of weeks. I usually have to harass him because he’ll forget—if they tell him to do it on a Monday, he’ll usually get around to it by Wednesday. But if they have to tweak the dose, they do, that part has worked okay. It’s really more getting him to take the pills and then just the hassle of the diuretics. His doctor has tried to scale back the dose, and it just hasn’t worked. It just builds up the fluid. It just kind of stinks. It’s one thing when your old to have to take pills, a lot of old people, God bless them, just don’t. How many teenagers, early 20’s kids do you know that have to use a pill box filled with like 9 different meds. It just really stinks.

 – Louisa, mother of Gabriel, age 20, HLHS

Helping my child cope with pain

With her fourth surgery, it was the first time that pain was ever a big issue for her. Being in the hospital, seeing her in pain, that was really hard, because you would do anything to take their pain away. You could tell she wouldn’t want to move, she wouldn’t want to get out of bed to walk, you could see it on her face if she moved remotely she would grimace. We would try the Tylenol and the Motrin first, and I would try distracting her, but you could just tell by her not wanting to move and by her face that she was in pain, so it was the first time we ever had to say “Hey can she please have something stronger?” So then they would give her the narcotic for pain, and within like a half an hour her whole mood would change. She’d be willing to play a game with you again, she’d be willing to go to the activity room. But prior to that she didn’t want to engage she didn’t want to interact when I brought her brother and sister in, she didn’t even want to talk to them, she would just wasn’t herself. But she wouldn’t ever say, “Mom it hurts” but you could tell in her face and she didn’t even want to move an iota, she didn’t want you to sit on the bed, she just didn’t want to be moved at all until the pain felt better. And then when it did, she would be great and want to move around and do things.

So that was the first time we had to come home and give her a narcotic for pain, which even as a nurse was very scary to me because she’s just, my little baby and when she’s on narcotics I know she’s not going to feel great, it’s going to constipate her, but I need her to be able to be happy and not angry and want to play and walk and talk. But even at home she only needed it for a couple of days and it was mostly at bed time to help her sleep or when she first woke up in the morning. Then it was like a switch or something, and it just didn’t hurt her as much anymore, and the Tylenol and the Motrin were enough or she wouldn’t need it at all.

Since that surgery she tripped over her soccer ball and broke her clavicle and she had an umbilical hernia repair. She is the toughest kid in the whole world. I mean her clavicle was broken in half and she’s not crying, she’s just like “Mom, something’s not right.” That was really painful, super painful. She had a harder time sleeping after a broken clavicle than she did coming home from the hospital after her heart surgery. With the hernia repair she complained of pain then too. As you got older you’re more in tune to pain and not wanting to feel pain, and you know she was definitely more vocal then about, “My belly hurts” and being able to tell us that she was having pain. But she’s still tougher than any one of my other children, and falls down and scrapes both of her knees open bleeding and she’ll get back out and be aggravated that she tripped. She doesn’t ‘crybaby’ about anything. She’s very stoic.

 – Erica, mother of Isabelle, age 5, Anomalous Aortic Valve

Helping my child cope with scars

Her scar is something, for me, that’s such a badge of honor, I honestly feel that way. Before her surgery I thought, “Oh my Gosh, look at her perfect beautiful little chest now, there’s going to be a scar there.” I don’t feel that way at all anymore. I sent a picture around to people afterwards and someone just responded “Brave heart.” And my cousin said, “That is so cool. I love scars, they tell such a story.” And I really do think it’s such a badge of her strength. It’s going to be something that she can reflect back on that’s tangible. It may fade, but it won’t ever totally disappear, she can always look down and say “I did that, I can do this” whatever that “this” may be, and how brave she was when she was just two years old.

She, you know, she points to it happily, from her—because she’s always used the word “boo” for a boo-boo, so from day one it was “her beautiful boo,” and it still is. It’s just this very cool thing. I just—I really—to me, I’m so proud of it. I’m so proud of her, it is such a sign of her resilience and strength and courage as just this tiny little person.

 – Jessica, mother of Avery, age 2, ASD


So we bought the book “Zip-line,” it was written by a dad, a Boston Children’s dad. It’s called “Zip-line” and it’s about a little girl. It rhymes, it’s very age appropriate, it’s not scary. It talks about how she was very brave. Well, so we started reading that to Emily before Elizabeth’s surgery, because we didn’t want her to be scared when she saw the incision. So we started reading it originally to Emily and now we read it to Elizabeth, and I donated the book to the preschool because they read books to them, and if three of them are there with open heart scars, the other kids need to know. So now she talks about her zip-line, and it’s kind of funny because she’s like “My zip line can get wet, I’m so brave.” Because she sees me at the pool and she’s like “You don’t have a zip-line, I have a zip-line.” She’s proud of it. And I want her to be proud of it. I think about that. Like, the first time she ever takes her shirt off in front of someone, I want her to be really proud of it. It’s almost like a beauty mark.

 – Anna, mother of Elizabeth, age 2, VSD


After her second surgery, I remember it caught me totally off guard, one girl watching her dance class had seen Amelia in her leotard and had seen the scar and it still had the dermabond, and it looked kind of gross, and she pointed at Amelia and was like “Ew! What’s that on your chest?” And I remember Amelia looking at me with fear in her eyes, and I was at a loss for words because it was just so random. It took me a second to pull myself together and I said, “Oh, that’s Amelia’s bravery scar. A doctor had to fix boo boos on her heart, and that’s what he gave her. He gave her this great, great bravery line that shows how brave she is for what she’s been through.” Then the little girl was kind of like, “Oh, wow!” and that was it. Some of the girls in her classroom weren’t as blunt and just said, “Oh, is that from your surgery?” Because the teachers had shared with our approval that she was going to be out, and that she had some problems with her heart, and they made her cards and sent them up to the hospital and gifts had come and been delivered so she felt supported by all her friends and stuff while we were at the hospital. When we came home she got more surprises and stuff like that. But luckily, there’s not been a lot of attention to her chest, to the line. At this point it kind of blends in, it’s hard to see. She doesn’t wear a lot of shirts that expose the area.

When she gets upset, or sometimes she’ll put on a shirt where her scar will show—because after her second surgery, that was bothersome to her, that you could see the scar. Especially when it was more fresh and more noticeable, body image kind of came into play. That’s when I pushed and encouraged like, “That’s your bravery scar. None of the other girls in your classroom have that.” And I was like, “You were this lucky girl that was picked to have this heart and you had a lot wrong with your heart, but the doctors fixed it, and now you’re healthy, and that’s your scar to let everybody know how brave you are.” And slowly with time as it’s healed and stuff, she’s accepted it, and it’s nothing now to her, which is nice. And I said “If you didn’t have those surgeries, you couldn’t play soccer” so that’s how we kind of turn it around again and make it a positive thing that she’s been through.

 – Roxanne, mother of Amelia, age 7, Dextrocardia


I think medicine’s very important, but just realizing there’s also something bigger, and somebody’s taking care of Alexandra, that’s been really helpful for us. I love this story in the Old Testament, they used to put these things called “Stones of remembrance,” and so at the parting of the Red Sea, when they went through the Red Sea, and how God provided, they put stones of remembrance, and so every time we go in and Alexandra has x-rays, you see all these scars on her body, and I think of those as her stones of remembrance, of how God has preserved her life. It’s interesting because God always says that you need a new heart, and Alexandra has a new heart. So I think there’s all these great tangible things about our faith that come through with the story, and that we’ve been able to tell our children.

 – Kate, mother of Alexandra, age 8, Heart Block & Pacemaker


[At ICD/Pacemaker Camp] They have scar buddies, they call them scar buddies, and they show their scars to each other, and they’re proud of their scars. It’s not something they want to cover up. A big thing was when Chloe met a girl, she was Miss Massachusetts at one time, and she had an ICD. She had a problem with her ICD so they had to replace it, and she has quite a large scar, and she said that when she was in the beauty pageant, she didn’t cover it because she’s proud of it, it’s who she is. And she told this to Chloe, so Chloe is happy with her scar, it doesn’t affect her.

 – Jim, father of Chloe, age 13, CPVT

Helping my child cope with procedures and appointments

Visiting the doctor, it’s only become a problem lately, and I think that’s because now she’s old enough that she understands that she doesn’t want to be poked at or touched. Prior to her surgery, she was young enough that by hospital policy she had to be sedated. That made it really easy. It sucked for us but when you knock out a baby it’s a heck of a lot easier. So now she’s too old and she had an appointment a couple of weeks ago and it was just a disaster. She was screaming and wanting to run away, like “No! No!” She’s two, she doesn’t want to be doing that! So I’m not sure what we’re going to do next time, but we’ll see.

 – Leslie, mother of Margaret, age 2, VSD


She skips into Dr. Geggel and the nurse Maureen, and she’s never hesitant and she just gets there and she knows that she needs a height and weight and she takes her shoes off and they need an EKG and they put the stickers on and you know, “Oh, I have to have an Echo” and she lifts her arm up and turns on her side. She just does everything that she needs to do, and never does it with a single complaint. It’s never been an issue. My son—this summer he needed a cardiac workup, and so he needed an echo and an EKG and they were putting the stickers on him and taking them off and he was like “Ow, ow, ow” and Isabelle said, “Chris, I had that done like 100 times!” It’s like, “Seriously! You’re ten, stop it!”

 – Erica, mother of Isabelle, age 5, Anomalous Aortic Valve


She had a lot more questions about the more recent surgery and the worst thing that she hates is needles and having to have bloodwork done. We’re open with her, I don’t tell her a lot of times ahead of time what’s going to happen. We honestly didn’t tell her about the surgery until a week before we had to go up. We talked to her the day before to explain the pre-op care and the pre-op appointments. She knows the little chest x-rays where they take a picture of her heart, she wears the lead apron to protect her, so she knows that that’s something that’s okay and doesn’t hurt her. She knows the echo is the jelly on her chest and she’s fine with that.

The bloodwork is where we get into a little bit of difficulty. She’s never had to be restrained, typically she sits on my lap and I hold her kind of in a bear hug. She sits there and does it, she’s not happy, she normally cries through it, but she gets it done. I explain to her going into it why she needs to have something done, that it’s very important and I say to her, “I would never make you do something that I didn’t feel was important or that wasn’t important to take care of your heart.” She kind of accepts it, she cries, but it’s nothing ever worse than that. We’re always very open with her as much as we can be without scaring her.

The scariest thing that she’s been through was when she was awake and they extubated her. I could see the fear in her eyes. I remember in the ICU when they extubated her, she was awake and she was fighting the sedation, the doctor at first said, “We need you to step out” and I said, “I can’t step out, she needs to have me there, I’ll be able to calm her.” I never go in playing the nurse card, I like to just be the mom, and I remember saying to him, “She’s definitely getting upset,” and they were at the point where they were going to have to tie her arms down because she was trying to take the tube out, and I said “I’m a nurse, I know what this entails, please let me stay, I can calm her down.” He finally relinquished and I looked at her and I was like, “Amelia, you need to calm down, you’re going to do this quickly, mom is going to be right here.” So they did it and I remember the doctors saying, “Wow, I would have never expected her to react the way she did to your voice, and just to be able to calm her and get her through it.” She was more fearful when they were going to take the chest tubes out, she got very emotional and I again said, “it’s nothing that they can’t not do.” So she remembers those sorts of things, so going up to a visit with Dr. Lang is like “Do I have to get the needle?” or “Are they putting anything in and then they’ll have to take anything out?” because she’s thinking back to the tubes and things like that. So I feel like the more open we are, the more relaxed she is and knows what it’s going to entail.

 – Roxanne, mother of Amelia, age 7, Dextrocardia


After that arrhythmia episode last summer, she had a very hard time going to sleep for the month after that because the feeling of going to sleep was like the feeling of when she lost consciousness. So she would often get in bed fine, but then as she was starting to fall asleep she would have difficulty. We actually had her go to a therapist that Dr. Alexander recommended, and she really didn’t like talking to the doctor about this. So I told her, “This doctor is here to help you deal with these feelings and to help you be able to go to sleep by yourself, on your own without crying or being worried that you’re going to die.” Every night she would cry out. So she said, “Well, if I sleep by myself can I stop going to the psychologist?” And that was it! I mean to some degree I think she has suppressed her anxiety because she doesn’t want to talk about it. I don’t know that that’s really the best outcome and the best way to deal with it, but I think fully coming to terms with your own mortality is a challenge for anyone, let alone someone who is only eight years old.

 – Catharine, mother of Willow, age 8, Long QT Syndrome


For her it’s the stickers. She is anxious until it’s communicated with whoever is doing it that she wants to take the stickers off. I think she really, really wants to do that. I’d say, “there’s a lot of things that I don’t like to do too, but this is just part of what we have to do, and we don’t have to like it, but we have to do it.” And then I usually say, “But we’ll go get a treat or do something afterwards, and do you want to bring a stuffed animal? Is there anything you want to bring with you?” And so she often brings her Red Sox stuffed animal doggie with her and we talk about it. I don’t really talk about it until the day before, so she doesn’t have all this time worrying about it because usually it’s scheduled appointments.

 – Kate, mother of Alexandra, age 8, Heart Block & Pacemaker


He went in for a cath when he was about six. They went to put an IV in, and he absolutely wanted no part of it. His anesthesiologist was wonderful with him. He actually brought everything that you use to do the IV, and he had Austin be the doctor and do his arm first. I’ve got pictures there of the doctor with his arm, Austin’s tying the blue rubber band around it. That helped. Another time, I bought a teddy bear and brought it back up to him, and one of the IV nurses, she showed Austin how she put the whole PICC line, if you will, on the teddy bear’s arm, and gave Austin empty syringes. She showed him how to flush it, how to put the medicine in, and the little bear, he still sleeps with every night. Picc the bear, he goes everywhere with him. He goes, “We have to take Picc!”

 – Grace, mother of Austin, age 10, TOF

One time and staged surgeries

A kid can present how they need to have their next surgical procedure in a variety of different ways, from really drastic, the kid turned blue, to what happened with us which was just she wasn’t hitting her growth curve. She was just declining very, very slowly. They were looking at her height and weight and all of a sudden she just dropped off her average. They said, “You know, this is an indication that we need to get in there a second time and do what we need to do.”

From the time that we brought her home after she was born to that second surgery was very different than what our life is now. That period of time was a big waiting game, we were always waiting for the notification of that next surgery. There were frequent doctor’s appointments, we were there every three months. She was having more sedated procedures and then finally we got that decision for her to have her second surgery. We brought her home from that and our lives sort of reverted to normal. They were like, “Alright, now she’s fixed.” And that has created a very different feeling because we’re not sitting and waiting for this call that she’s going to have another surgical procedure. She’s just a normal kid. We were extremely, extraordinarily fortunate. She did great for both of her procedures, the doctors were able to fix everything, she was just lucky, we were lucky.

 – Leslie, mother of Margaret, age 2, VSD


Allen: The waiting is excruciating when you know the weight of what’s happening with the surgeon in the operating room. I was appreciative of the people who come out and give you updates “Okay, she’s on bypass,” “She’s off bypass,” “She’s all stitched up,” “The doctor’s going to come see you in ten minutes.” Those frequent updates— I could see it going either way for people. For us it was good. We like information, so it made us feel good that we knew what was going on. But sitting there was strange in a lot of ways because there’s all these other families in the same area and you know they’re going through exactly what you’re going through. It could be for a completely different surgery. Amongst all the other families who were having surgeries, there’s a little bit of comradery in that, and there’s also a little bit of weirdness in that everybody’s wondering what they’re in for, it was just a very strange experience. But how it’s handled at the hospital I think is really good and helpful from an information standpoint.

Miranda: You’re exactly right. I think they did a great job of preparing us for how she might look in the ICU. We walked in and both of our reaction was like, “That’s my girl. Fine, she looks a little different, she’s got some stuff sticking out of her,” but she looked great compared to what they prepared us for, so that was actually kind of a good feeling.

In terms of advice for other parents, we sent her away with a blanket and a stuffed animal, so when she left us she was carrying that blanket and that stuffed animal. And to the credit of the hospital, when we saw her, she had that blanket on top of her, and that stuffed animal. And in my mind, I could pretend that she had the comfort of them the whole time, even though I know she didn’t. But that was so helpful to me to just have those visual comforts so that she had things from home with her the entire time. I just wanted her to be comfortable, and I loved seeing her and having that with her and it just made me feel really good.

 – Miranda and Allen, parents of Serena, age 2, ASD


The second procedure was to fix her VSDs, which did not get fixed during the first surgery, and to remove the pulmonary artery band, which she had had put on when she was just born. Bringing her in for the second procedure was heartbreaking. It’s much easier to hand over your two day old infant than it is to hand over your 16 month old. At two days you don’t know the baby—I mean you love her obviously, but you know she doesn’t talk, she doesn’t call you mommy, she doesn’t know to be scared, so handing her over at 16 months was immensely more difficult than the first one.

During her surgery it was pretty nerve-wracking, but the hospital does a really good job of keeping you up to date and calling you for the big benchmarks during the surgery. It was fall so it was nice out. We sat outside—I could not be in that waiting room, that would have driven me crazy, but people do sit there all day. I mean we just walked around, we tried to lay in the sun and just relax and just wait until we could see her.

We were both pretty relieved at how good she looked when she came out. Kids can look pretty bad when they come out, they can be swollen, they turn all different colors and they puff up a lot. She looked pretty good, all things considered. It’s hard having a tube down her throat, and tubes all over and IVs and wires, but again the hospital does a really good job of kind of keeping them all wrapped up so you don’t see too much if you don’t want to.

 – Leslie, mother of Margaret, age 2, VSD


After going through what you go through, coming to Boston was kind of like going home. At that surgery, my youngest sister flew out there with me, and my mom, dad and siblings came, and Brian’s twin, Danny. We got there and they were impressed to see how he’d developed and the way he looked. After all the preliminary stuff, they did a cath, which is standard to go in there and check it out, and then they also put coils in. That involves lateral veins, just to help with the oxygen flow and prepare for what they need to do in surgery. That went fine, he just did not handle it well. I slept in the room with him, I stayed with him those nights in the hospital, he was just totally uncomfortable. And they do stuff, his oxygen kind of drops a little, everything is in preparation for what they’re going to do for the next surgery. He was just not happy.

I was just assuming that everything was going to go okay. Everything was looking great in him, and the way his body was handling whatever they did to him. Surgery took several hours, but I was not at all nervous, it was very strange. I was kind of like, “We’ve already been through everything. There’s no reason to think anything bad.” The first time going through all of this I was just a basket case for these nurses—they come and give you reports on the surgery and I remember initially they walk towards you from the hallway, and their faces are just straight. And you know I got dramatic—I was freaking out on them and so it was funny because they would just dramatically put on the biggest smile as they were walking towards me just to say it was okay.

So this time, one of them remembered me and so she would walk back and forth with the news with a big smile on her face just to joke with me. He stayed in the ICU for a day and a half, and then they brought him to the floor. If the cath wouldn’t have happened, we were admitted and discharged within a week from the surgery. But with the cath and the surgery, we were there for almost two weeks. Of course all the nurses, they know exactly who we are because we were there for two months just a few months before. The looks on their faces of just how amazing he looked and just how he handled everything and the second time around was almost reassuring, that he’s going be alright, we don’t need to worry. So that was better.

I think that was the turning point, the second surgery. I’m waiting any day now to get the call for the third one, which is probably going to be the worst out of all of them because he’ll be two years old, and he will just be pulling everything out and screaming for me and me not able to go anywhere and all that stuff. They just let me know. They’re a year and a half this month, and they said 18 months to two years of age, so any time now!

 – Lindsay, mother of Brian, age 2, Heterotaxy


After his first surgery, he was discharged at nine days old. He went home and was in the single ventricle monitoring program. It’s something that more and more hospitals are doing for high risk kids like Zach. There’s a high mortality rate between birth and getting to that Glenn surgery, so there’s a lot of daily monitoring. We had to keep him on a pulse oximeter, we had to do weigh ins and we got a whole binder of things we had to fill out, and then we would call the cardiologist once a week and give them the information we’d been gathering and they would tell us what to do. So that went on for basically four months.

 – Alice, mother of Zach, age 3, Single Ventricle


Because he’s a single ventricle, we knew that there were two additional open heart surgeries that needed to happen to address that. They wanted him to get physically as big as he could before the first stage happened, which is the Glenn surgery, so we were kind of just home waiting for the surgery to happen, which makes you even more paranoid. We knew that there would be signs from him that it was getting too hard on him to thrive, I guess, for lack of a better word, and then that would be the time. So we were kind of always watching for these signs, “is his O2 okay? Is he eating okay? Is he acting lethargic? Is he being fussy? What is happening to him?” And whenever those things started going awry, we knew that the timing meant “Okay, he needs surgery again.” So that’s definitely a hard thing, just waiting for surgery to happen, and not knowing when it’s going to be. But he was four and a half months before he had that surgery so we were home for three and a half months in between the two hospital visits. On the last two weeks or week and a half, he was on oxygen at home. He had kind of started to decline and he was really struggling to keep his oxygen up and was very blue, and so he went on home oxygen and we were basically like, in between the time that they could get us in for the surgery. It was about ten days and then he had his Glenn surgery at four and a half months.

Then he had his Fontan at 22 months. He’s unofficially the quickest Fontan recovery at Children’s. That’s what we were told, I don’t know if that’s official or not. He was walking around ICU less than 24 hours after the operating room, eating a grilled cheese sandwich and drinking chocolate milk, it was kind of weird. He did great. He had his Fontan, he was up rearing around, we were out of the ICU 24 hours post-op. We left on day four. And on day three they said “Really, he probably could go home but they really don’t believe it, so we’re just going to keep him one more day just in case. We’ll let you go home tomorrow if everything stays the same,” and he did, he did great, and we left four days later, which is crazy. It’s absolutely crazy.

 – Amanda, mother of Carson, age 5, Heterotaxy


Nick: The Fontan was eight hours long. That was rough.

Shana: All of them are rough.

Nick: She took it really well. She was in good spirits right up until they put her out. The night before she and I were up skylarking, and then when her mom got up early that morning she was playing. She was in really great spirits about it, she wasn’t worried, nothing. She actually slept through until the beginning of it.

Jade: Because you were all night snoring! I couldn’t sleep.

Shana: It’s hard when they’re older and as a mother, every time they wheel her out you feel like you’re just empty. I think I gave housekeeping a good little bit of cleaning up to do because every time they wheeled her into the emergency room I threw up—my nerves. You go in, they tell you that she’s in there, and then they put the brain to sleep and then they stop her heart—they tell you all that. I guess it’s a good thing.

Nick: I’d say we were probably informed every half hour. So that was comforting, but still, your nerves are taking over. Especially, you’re in the waiting room, and you see families come in and you see families leave, and you’re still standing there pacing or eating or trying to eat and it’s not working so well, and then Dr. Emani came out and he had a look on his face that was like, “I don’t have much to say to you, I couldn’t have asked for a better. Something as serious as this was so routine” That was a big sigh for us, that was a big.

Shana: Yeah, the Fontan is usually done when you’re two or three, something like that and she didn’t have hers until she was six.

Nick: She was very slow to grow. Fighting with weight.

Shana: We’ve been fighting with weight for a long time, she still doesn’t weigh 40 pounds, but they did the Fontan, she wasn’t quite where they wanted her to be, but they couldn’t hold off any longer.

Nick: I think they were worried about her being so little, some of the medicines they can’t give medicines to take away some of the pain and stuff, and they have a five year old, six year old, trying to keep them still, even just as far as trying to sedate them probably to do it, they were really worried because her body is tiny, about her coping with all the poking and prodding, and at the same time it was like, “Well what’s the pain going to be like?” But she done well and they were extremely happy with her. I mean she was due to be in the ICU for 72 hours, they were wheeling her out of the ICU in less than 24. I think that was the hardest part for me was—

Shana: To actually- I mean we’ve seen her tubed up before, maybe one, two, three times she’s been tubed up, but this one was bigger. I mean when she’s tiny, in a little crib, it’s like “Okay.”

Nick: I think the other times we came here, there was something wrong, we knew they were going in to fix something. This time, yes, we knew something was wrong, but it’s like I carried a perfectly fine looking kid into this hospital, and eight, nine hours later to see her just kind of lifeless, it was extremely, emotionally, very hard.

Shana: We knew a little girl who didn’t make it, so that was sort of freaky because she had  the same procedure, and she was probably more severe than Jade was I guess, but we knew that she went in for the Fontan and came home and then three days later she had passed. That sort of was freaking us out. That’s all in the back of your head as well. It really messes you up even more.

 – Nick and Shana, parents of Jade, age 6, Dextrocardia


She was seven and a half months old. It was a whirlwind of emotions, it was hope that this was going to be a new chapter after we got through that day, things were going to be different. It was probably the scariest day of my life, not knowing what was going to come, we were comforted in knowing that this was the place for her. Leading up to it, my husband’s insurance was only going to cover 80% of the surgery, we had gotten insurance through the state at that point, as a secondary insurance, and there was a battle with insurances and paying out of state. I had done appeal after appeal for the secondary insurance and they finally approved it. That was just a huge relief knowing that financially, the surgery was covered. That out of everything else that was going on, that was a relief and that whirlwind of emotions, we had left our two year old son my parents’ house at that point.

Leaving my older son at home was hard, because he hadn’t ever really been away from us, but a relief knowing that we didn’t have to have him with us and go back and forth, that we could just focus on being with Amelia in the hospital. So that morning came and her surgery got bumped to the afternoon because an emergency case needed to take place prior. It was hard, it was hard waiting. All the emotions, it was hard with her because she couldn’t eat or drink and at seven and a half months it was kind of tricky.

The hardest thing was handing her over to the anesthesiologist and knowing that potentially there was a risk that I wouldn’t see her again if she didn’t survive the surgery. Knowing that that would be the last time that I would hold her for quite a while was hard. I remember breaking down in tears watching him walk her away. And we were led to the surgical waiting room where we waited like eight and a half, nine hours. They gave us updates throughout, it was hard. Surgery ended late in the night, close to nine or nine-thirty when Dr. del Nido met with us, told us that surgery went well, that she did great throughout it. It was another hour and a half before we were able to go into the cardiac intensive care unit and see her. Never in my wildest dreams did I ever imagine she would look the way that she did. I think it scared the living heck out of my husband because he didn’t have any idea of what to expect. I remember walking into her ICU room with my husband to see her, and she was this little tiny thing with so many tubes coming in and out of her. Basically a wall of medication drips for the IVs and all the various things, and monitoring her, she was sedated, that was shocking. That was really, really hard to see. Seeing the little dressing on her chest from where they had done the surgery, that was emotionally tough to see her like that.

Then it was just waiting. Waiting to see how she was doing, waiting to have the meds adjusted, just sitting there. That first night, my husband stayed most of the night and then he did go back and sleep, and I remember just sitting just watching the time, and just watching her and just being in awe of our journey from finding out at twenty weeks to now seven and a half months of age just having gone through open heart surgery. You sit there at times and you wonder “Why? Why is it happening?” and “Why her? And Why us?” and just trying to believe that the worst was behind us, that it was a positive thing, and that there were just good things coming and that this was going to be a change of life for her.

Our big one year post-op appointment was great, and even though Dr. del Nido had repaired most of the issues with her heart, there were still some that we needed to watch, and there was the potential for another open heart surgery, but at that point they were thinking that it was going to be quite a while out. We knew after the first surgery that some of the narrowing in one of her arteries was still there, but they anticipated and were hopeful that it would open as she grew and as her heart was pumping more normal, and as her heart recovered. Then in echoes and things like that, it was showing that it wasn’t. In the summer, I noticed as she was running and playing, she was more sweaty, she was more out of breath. I always try to treat her as a normal kid and when she’s sweaty and out of breath it’s like, “Alright, well she’s just been running around, it’s not because she has an extensive heart history.” But she did seem like she was overworking, so we had scheduled an appointment. In December, she had to have another cardiac catheterization, and I did not anticipate Dr. Lang coming out of that and saying she needed open heart surgery again. He said that she was kind of at the cusp of needing surgery, and that we wanted to be ahead of the game and not behind it, so we scheduled surgery then for the spring to go back in and open the narrowing that was in her heart.

That surgery was a lot different and a lot harder in terms of—she was six years old at the time, she wasn’t an infant, she was able to verbalize. I had to be a lot stronger, I couldn’t break down in front of her because I didn’t want her to be scared. Having to explain this to her, explain in a six year olds terms what was going to happen to her. Knowing that she was going to have to miss school, that she couldn’t dance in her dance recital—and getting her to accept it and be ready for it and then get her through it. That was a larger challenge with a different entity than what we had done at seven and a half months with surgery. She did great through it, she was certainly scared. That was hard too, watching them wheel her away. When she was a little baby they had given her medicine to relax so she wouldn’t remember things, but that was hard, just knowing where we had been before and this is going to be where we were going to end up in terms of seeing her in the ICU after, knowing what she had to go through, what she had to entail. But she did great the surgery was only four hours. She again was the talk of the ICU because she got extubated a couple of hours after surgery.

She had been through open heart surgery and she wasn’t in a lot of pain and she didn’t want a lot of medication, which was amazing to me. She’s a tough little girl, it’s amazing. You think about if you had open heart surgery, you hear about adults having open heart surgery who are down for the count for a while, and in a lot of pain, and she was nothing like that. That time we were in the hospital for a week, and then we came home and she was out of school for two and a half weeks, and then went back half days, and then progressed to full days. She wasn’t able to dance in her dance recital, which was probably the most upsetting thing to her in all of this, not that she had to have surgery, that she couldn’t do her dance recital. But she went and watched and the school was really supportive of her, and it was a good thing for her. It was a lot of positives.

 – Roxanne, mother of Amelia, age 7, Dextrocardia


His oxygen saturation was very low for an extended period of time. Every once in a while, they would go down pretty low, but they tended to pop back up within a certain amount of time. And I remember they were low 60s, 50s, and so we called them at noon and an hour later they called saying to get in the car and come back up for the surgery. So, it was like two times in a row when it was like, “Oops! How fast can we make plans and decisions on a dime.” So I think that whole whirlwind of making sure my daughter was set, getting everything we need packed up, getting him up to the hospital, getting out of work, because at that time, the grandparents would actually watch Johnny while I was at work, so yeah that’s where the emergency part came in. It was time, whether we wanted it to be or not.

It was very hard. It’s hard to think about now. It was hard. We were optimistic, and we kind of throughout it all, tried to stay very positive, and kind of always expected that he would get the best care and we would have the best outcome, but despite that, you always have in the back of your mind—I would just remember holding him right before a surgery and we didn’t know. We’re going to say our prayers and hope that we have the best of luck with this surgery, but you still have these thoughts that this may be the last time you’re holding your child. Very hard. He was in the hospital probably for about two and a half weeks after the surgery, which to me was absolutely amazing, that a child with such an amazingly complicated surgery that we were bringing him home after two and a half weeks. So I think we were very fortunate in that respect.

 – Abigail, mother of Johnny, age 9, HLHS


For the next three years after his initial surgery it was 25 hospitalizations. Luckily, none was longer than that first one. We got to a point where the doctors knew us. Every trip up there was like Grace and I going to boot camp. We’d get in the car every morning, Austin was basically too young to almost understand what was going on. I’d carry him into the operating room every time, they let me carry him right into the OR room every time. That was the most brutal thing I think I’ve ever done, three or four times. Just carry him in, lay him on the operating table and have him holding your hand, and then they just walked me out and I hit the deck. I couldn’t even—it was like, “Whoa.” And then we sit and wait in that damn OR—the waiting room up there, you sit there and sit there and sit there and sit there and all of a sudden you look up and here he comes, walking down like you see on ER or on those shows, they’re walking toward you and you see a blank—there’s no emotional anything on his face. He just comes over, crouches down, looks at you, and God—most of the time some of his phrases were like, “Everything is good. It went well. We’ll see you upstairs in 45 minutes.” It was just like, “this is our life!”

 – Scott, father of Austin, age 10, TOF


Leah’s surgeries were all in Boston. She went back at about four and a half months for her Glenn- we were in and out of the hospital here in our home state three or four times in the interim just with low oxygen saturations. But we went back to Boston for her second surgery, and it went pretty well, we came home and about a day later she was looking pretty puffy, and so we went to the pediatrician and her saturations were low again, and we were immediately hospitalized, and she was very sick at that time. She ended up having an effusion around her heart, she had an effusion in her lungs, they had to put in a chest tube, they had to put in a pericardial drain, and plans were made for us to be medically lifted back to Boston. She was extremely sick, and so they had to intubate her, and we were coming back with her, because she just was in bad shape.  And we ended up staying in Boston in the ICU for about three more weeks with no real answer to what was going on, she just kind of all of a sudden got better. Leah kind of does that, she’s like a light switch, when she’s great she’s great and when she’s not she’s not. There’s really not much of an in-between.

But anyway, she was better and we came back home and she did great for the next couple of years until she had her Fontan, and that was at about two and a half years of age, and she had a hard time with that. We had never gotten a phone call from the ICU in the middle of the night until that surgery, and it was her first night post-op, they called us in the middle of the night and just wanted to let us know that she wasn’t doing well. We were staying at the patient family houses, so we hurried up over there and struggled the whole day, and then the next evening they came and talked to us and told us they needed to put her on ECMO. It was her lungs, her lungs always seemed to be the issue after her first surgery and it took a long time to wean her off of oxygen after her second surgery. She had an effusion and that was part of the issue that had us medically lifted back to Boston after her second surgery- she had pericardial effusion then with the Fontan her lungs just were not liking the changes and she was in acute respiratory distress. And she also had- her abdomen was huge- they put a drain in there and drained like a liter of fluid out of her abdomen. She was sick, she was very sick, very unstable. And she was on ECMO for five days, and then remained on the ventilator for another week, when she extubated herself. Then after that surgery we were in step-down for about another month because again she developed pericardial effusions and needed chest tubes and that was a very difficult time as well because she had been on ventilator for two weeks, she was so weak, she was two and a half years old and had to learn how to sit again, learn how to walk again.

Then we had to give the tough love and take away that wagon out of her room and tell her she had to walk to it, just to help her gain her strength back, and it was hard because she was a different child as well. It was almost like she was depressed. You could look in her face and she just was not the child that we had handed over to the surgeon two weeks prior, and so that was a struggle. But one night in the middle of the night, it was my husband’s night to stay with her, he started juggling this cow that mooed and she cracked her first smile in weeks, and we knew then we had our Leah back and she was going to be okay. But it was the hardest thing I have ever had to go through.

 – Donna, Mother of Leah, age 10, HLHS


He was six months when he had his second surgery. He was cathed at five months, that was a really difficult one. He was supposed to be home the next day and I think it was a week in the hospital, he had some complications. And then the second surgery actually went really smoothly. He was two years old when he had the Fontan, so a year and a half later. It’s really hard because you have this other surgery coming and so you can’t ever really relax. He was very healthy during that inter-stage period for the second. I can’t remember the details too well, it was a long time ago, but I remember there were ear infections and things like that, pretty typical baby stuff. From his third surgery onward, we dealt with chronic infections, but I don’t think he had the asthma in that period. But that year and a half between surgeries, honestly is a big fat blur.

 – Diane, mother of Jake, age 21, HLHS


Courtney: Yeah, the crib was up very high, almost at eye-level so they could work on him, you know, right there. I remember walking in and seeing this raised table with all the people standing around and all the wires and that was really scary. Really scary.

Richard: The first time. When it was the second surgery it was fine.

Courtney: I also remember seeing a teenage boy in one of the rooms next door, and it’s scary to see other patients, because you’re so intent on focusing on getting through your situation, but you can’t help but feel empathy for the others around you. That’s the reality of a hospital. They got David out pretty fast, I mean we were in and we were out, and he healed very quickly and there were no problems at home, the scar was fine. From what I recall of that surgery these many years ago, it was very successful. We thought he would never have another surgery.

 – Courtney and Richard, parents of David, age 23, TOF

Valve surgeries

They kept her in their cardiac ICU for five days and monitored her. She was doing well, tolerating it well, so they sent us home with her and hoped that she would grow just that little bit bigger before they had to intervene. She was just under a month old, when she was drinking her bottle I would notice that she would get really sweaty on her forehead and she would be gulping her bottle and then stopping for a second, like trying to catch her breath, and then gulping her bottle and sweating and I thought “Gosh, she’s working really hard to drink her bottle,” but that was the only symptom that I noticed.

At that point we went back in for an appointment and that’s when they did her first cardiac cath. They said she was symptomatic, it was severe, and they wanted to try to balloon it out a little bit. They did and it was beautiful and she tolerated it without any complications. They sent us home, and then right before she was 12 weeks old, maybe 11 weeks old, she had another balloon cath. The same thing had happened again, she started to become symptomatic again, and they did her second balloon, and that time it went really well again, however the problem with cardiac caths is when they balloon the valve open, then there’s the risk of aortic regurgitation. So after that cath she started to have issues with aortic regurgitation. Coming in post-procedure and seeing her being transfused with blood was like one of the scariest things—I don’t think that I expected that either. Being a nurse and somebody who gives blood, I didn’t think that would be such a frightening thing to see her getting blood. That’s something that I just can’t forget. So she tolerated that okay, but we were frequently at the hospital—monthly we were there for her echoes and appointments.

When she was 10 months old the aortic regurgitation got problematic enough that she needed to have her first open heart surgery. They were able to repair the valve using a piece of her pericardium which was awesome. She recovered really well. We spent those days in the hospital. The first day you’re in the ICU seeing her connected to all the tubes and the vent and all of the medications all at the same time I think still now, every time I physically enter an ICU of my own hospital and hear the vent machines, I see Isabelle and I can’t even get past that at my own job. But the nurses, everybody who cared for her was great. They always talked to us, they would talk to her.

After all of the surgeries, the hardest thing for her was nausea and vomiting from anesthesia. She would wake up and be thirsty and want to drink and would guzzle, and would vomit. She’s had three procedures, and every one that was like one of the biggest things for her to hurdle. But on the floor the first time she ever pulled herself up from sitting to standing in her crib was post-op after her first open heart surgery. It was the fourth day she’s in the crib and she pulled herself up. I don’t know what kids can do that, but she did. She’s just really strong, she’s really tough. That procedure lasted until she was 3 years old. They thought it might last a year, and it had lasted over two, so that was pretty awesome. And again we had really frequent visits every month or so we’d have our echoes and sometimes they would need to do sedated echoes, and sometimes she would just fall asleep on her own. It’s like she just kind of knows when we’re in Children’s that like, these are things that just have to be done, and she’s so great about it even now. And with her second surgery she was 3, we didn’t have any complications, we spent a day in the ICU, they extubated her rather quickly, she gets to the floor which is all great because the faster they can do that and the faster they can get her out of the ICU, the better she can get. But again, I knew the things she needed to do to be able to move and get home and get better, and I remember standing at the end of the hallway and her not wanting to walk and taking five steps away from her and making her walk to me, and she didn’t want to but it was only going to make her better. She needed it for her heart, her lungs, and to get her GI tract back on track because the medicines constipate, and that was really hard.

But again, when we came home from the hospital that time she was hula-hooping outside in two days, she does crazy things. And she always came home on this Tylenol or Motrin, and never needed more pain medicine. It was always enough for her, and she did great. On the second surgery they used what they described as the core matrix of a bovine valve, so they stripped away all of the DNA and just used the core structure in that for her valve. I guess they had shown that they were working really well, and that her own body would grow new cells over it. It wouldn’t grow with her, but it should function well. But it never really did because each echo just was either the same or a little bit worse.

It was 11 months later when she needed another surgery, and that was really hard because it wasn’t even a year later and I felt like for that whole year, her life was on hold. Every appointment it was just “Are they going to tell us today she needs surgery again?” So after 11 months she had her third surgery, and this time they did the Ross procedure. They took her own pulmonary valve and moved it to the aortic valves place because being her own native tissue, this would have a chance to grow with her, and then they used a cadaver valve in her pulmonary valve’s place. So they were able to put a larger size in, so as she grows she won’t outgrow it so quickly. So that was really good, and the positive part of that procedure is you don’t need any blood thinners, so she was on Aspirin therapy temporarily after, but she did not need to be on any other blood thinner, which is really good. Any time she came home on meds they always seemed to be able to taper any med she came home on very quickly.

 – Erica, mother of Isabelle, age 5, Anomalous Aortic Valve


The doctors needed to build a pulmonary system, and he built it using collateral blood vessels, and putting in the valve and the conduit and creating a whole pulmonary system. But it took multiple surgeries to do it. He’s had three open heart surgeries, right in the front of his chest, and then he’s had two through the back, through the left shoulder blade and twice on the opposite side of the back on the right trying to reach all of those collaterals and incorporate them into the pulmonary system. The first two years he had 25 hospitalizations between the surgeries and the catheterizations, it seemed like he’d be coming home from a surgery just long enough for him to recover, get strong, and then we’d have to go back and hand him over again. It was tough. That second surgery was-I mean every time it’s been difficult to hand him over, but that second surgery…it was brutal to hand him over, just knowing what complications had happened after the first one.

 – Grace, mother of Austin, age 10, TOF


Richard: There was a second surgery that happened when David was 16 and we didn’t know he was going to need that until he was like 12 or 13.

Courtney: We never knew he was going to need that.

Richard: David decided he wanted to have the surgery sooner rather than later so he could be more competitive in sports. So we knew that there was going to be a surgery at some point. We had thought it was going to be maybe when he was 18 or 20 or something like that. It kind of hit us like a punch that there was going to be another open heart surgery. Back on the heart/lung machine, and all of the trauma for us as a family, and obviously for David as an individual. But the kick part of the story is that David decided he wanted to have that surgery early because he understood that it would improve his heart’s performance dramatically when the purpose of the second surgery was to put a valve in, in fact a pig valve.

Courtney: A pulmonary valve.

Richard: So David decided he wanted to get this surgery sooner rather than later, so I said “David, that’s really not our decision, that’s up to your cardiologist and a conversation that you should have with him.” So we made an appointment with his doctor to go back in and have the conversation about sports and David’s doctor said, “You’re going to have this surgery when I say you’re going to have it. It’s got nothing to do…” of course he was very blunt with him, in his charming way.

Courtney: He said, “There’s no reason, you’re not symptomatic, you’re doing great, there’s no reason to be having the surgery.”

Richard: But they did some tests and he said, “let me look at this.” He ended up calling that night, and Courtney and I were out to dinner, and all of a sudden David calls and his doctor had called on the house phone, and David had answered, and he had said, “This is your doctor, I’ve got some news that you’re going to need this surgery.”

Courtney: “Your heart is enlarged.”

Richard: “And he’s in a dangerous position, you need to have the surgery immediately.” So that was one of the little bumps in the road. I had conversations with his doctor about this subsequently that we wish that he had waited and had the conversation with me or Courtney first.

Courtney: He did apologize to us afterwards, but he probably shouldn’t have given the information to David. So we started out with a little bump in the communication, but it smooths itself out. We got ready for a surgery a week later. In a way, that’s the best way to figure out you’re going to do it, because you don’t have time to be agonizing. He went in—they removed his scar, his original scar, which grows with you. They removed the scar and they saved it to do stem cell research. We had to sign something that allowed them to take the scar and use it for stem cell. Now at this point he’s in biology in school, he’s 16 going on 17 years old, he understands exactly what’s going on with his body, and is very aware of everything that’s about to be done. He was very brave, incredibly brave.  His friends were very supportive, they all signed a poster, “Get back soon David” and school couldn’t have been more supportive.

Richard: And we fortunately had the same surgeon who did David’s original surgery. He was still at it, in fact he had been doing lots of work to come up with new solutions for pulmonary valves. The surgery went extremely well, I think David was in the intensive care unit for maybe an hour and a half. Then he was in a room, they thought it was going to be for a week in the hospital, and I think he was home on the third day.

Courtney: So at this point his brothers are now an integral part of this because they’re young adults, they’re eight years older than David. So they were there when he woke up, and of course he had a pig valve put in so they’re signing his little pillow that they give you at Children’s, “Oink Oink” and making him laugh, telling him he’s never looked better because he’s all swollen after the surgery, looking very muscular. They made light of everything, but they certainly understood the severity of the situation. And he knew that if something happens to us, they’re there and he can lean on them. That was what I saw during that episode is that they’re there for each other. You couldn’t really ask for more, as far as David’s health goes. This is going to go on for a while, you know he’ll need another surgery probably in five years maybe. They’ll go through his groin this time, so he won’t have to have open heart. That’s to replace the valve. They don’t last that long. When they put it in this time, they set it up for that.

 – Courtney and Richard, parents of David, age 23, TOF

Arrhythmia surgeries

Last summer she had an episode of torsades des pointes so, a cardiac arrhythmia, and lost consciousness because of that. After the episode over the summer, we decided to go ahead with the sympathectomy. The morning of the surgery she was really anxious and actually tried to leave the hospital several times, so they had to give her a little something to calm her down before the surgery. The surgery was like an hour and a half, and then she was in the ICU for a little while. When she woke up she was actually in pretty good shape, she actually walked around the ICU by the evening time.

 – Catharine, mother of Willow, age 8, Long QT Syndrome


She was around 18 months when she got her second pacemaker. We had noticed that she seemed lethargic, and so we went in and they just were like, “She’s outgrown this pacemaker.” But the second surgery was not open heart, they were able to put the pacemaker up in her shoulder and have the leads go intravenous, so actually they took out the old pacemaker that was in her stomach, but the leads are still there from the first one. Because they said “if we don’t have to do open heart, then let’s not.”

I don’t think she has any idea about that first part of her life or that she had a pacemaker before this one. She was still young enough. She knew we had to go to appointments, but now she’s like, “Oh, I’m going to have another surgery, what does that involve?” Now she’s asking questions she wasn’t for that second surgery. But we had a party when she came home had cupcakes and celebrated her making it. But you know, they give you toys, like, they gave her a little stuffed animal and I spent the night with her on that surgery. And the surgeon, I mean you would’ve thought she was getting a cavity filled, he was really nice. I mean, that’s all he does is place pacemakers, you know? He was like “It was routine, she did great!”

 – Kate, mother of Alexandra, age 8, Heart Block & Pacemaker


Shana: Last year’s catheterization one was the first one she actually talked about. Other than that she was just so tiny. But last year was the first one she knew what was happening.

Nick: And they had been giving her medication and stuff to help her cope with it.

Shana: When they’re little they give that memory stuff that makes it so she doesn’t remember it. So she had that for the first couple of times, but last year was the first time she didn’t actually have that medicine.

Nick: The first time when she woke up—most kids are hungry, first thing she says, “I don’t have any clothes on!”

 – Nick and Shana, parents of Jade, age 6, Dextrocardia


We were in the room when they gave Emma anesthesia, and the minute they rolled her away we both just started sobbing, it was too much—that was hard. And she at that point was kind of giddy, but then she started to cry, as she got rolled away. The anesthesia must make you so emotional, she went from singing to crying. And that image of your child getting wheeled away, we both started to cry it was too much. We went downstairs and were on pins and needles, because you know they told us they would call us part-way through and tell us how it went, and also Dr. Alexander did such a good job explaining what he was going to do.

They’d put her heart into SVT, they would find it, and they would either freeze or burn the spot. It sounds like make-believe, like I can’t even believe they can do this, and they go in through the thigh, the vein in the thigh up to the heart- and the idea that someone’s like, touching your child’s heart is—I found it really stressful. And then they called and said it went well, and we went upstairs and it took a little bit for her to wake up. It’s really something that’s hard, and again we’re dealing with a not life-threatening, pretty straight-forward thing, but when it’s your own child and it’s your child’s heart, it all feels really stressful.

 – Meredith, mother of Emma, age 9, SVT


The first time he had a cath it was very nerve wracking. In some respect it scared me a little bit more than the surgery, but he ended up healing fine and everything was fine with his leg, the flow going and all that, everything was fine. After that first time when we had a chance to talk to the doctors and ask the kinds of questions that we had, we were more prepared for the next time he went. But that time he did much better with it and he was there just for the cath, and the surgery was directly after that. We went up as an emergency for his Glenn, a procedure that normally we would have done separately, we actually had to do back to back. It was more the intensity of the emergency of getting him up there, for the surgery, I think that probably impacted the fears, but the second time, I remember it was a good experience. Everything went smoothly.

I remember bringing him for one procedure, and they medicated him beforehand. At that time, the movie Cars was a big thing, and once they did the IV and all that, and he started to get medicated and everything, we stayed with him until he was going down, and we sort of joked with him that he was driving Lightning McQueen and he started to feel woozy and he almost starting giggling and being happy, and it was lucky for us because he was going down the hallway with a big smile on his face, going into the cath, thinking that he’s driving Lightning McQueen. Afterwards they had him up right away, they brought him toys, I remember they brought him play doh and he was playing with the play doh, so he handled it very well afterwards.

 – Abigail, mother of Johnny, age 9, HLHS

Complications after surgery

From February to September we were building up to the surgery and then surgery went great. It was a few hours, she was up and talking hours later, we were just blown away. Then that night she had a series of very scary arrhythmias, and that was unexpected. A whole new round of, “Oh my God, what’s happening?” Things went wild, 20 doctors rushed to her bedside. The unexpected was not something I was prepared for, which is a silly thing to say, that’s the whole point of the unexpected, but I was bulldozed by that. What was so hard on us at that point was that we had sort of let our guard down. We thought we were through. We knew that the next couple of days in the hospital were going to be hard, trying to keep her comfortable—not physically but emotionally, but this was a bump that we were not expecting.

By morning we had a team of electrophysiologists who had a medication plan and they diagnosed her with supraventricular tachycardia (SVT). She was on medication for that three times a day until about a month ago and now we’re trying her off of it and seeing how she does, and she’s done great so far. But each step is hard because we were safe on that medication. It was nerve-wracking taking her off, she wore a Holter monitor for 24 hours a couple of weeks ago, and things looked good from that. It’s still steps of feeling good and confident and safe, but they’re steps in the right direction. We’ll take it. For us going into this, it was hard because we didn’t have a sick child we felt like we were bringing in to make better. She didn’t have symptoms. It’s not like she was unhealthy on the surface. We never would have known. So, we didn’t look forward to the surgery as, “Oh, you’re going to feel so much better after this.”

It was hard because we were saying “My gosh, are we sending what looks like this perfectly healthy wonderful child in for a potentially horrible experience?” You never want to go there in your head, but you can’t not have a sliver of your mind go to the most horrible place. So now to see how she’s benefitting from it, is wonderful.

 – Jessica, mother of Avery, age 2, ASD


The day after they were born, they brought Danny to me and I was taken in a wheelchair with my husband over to Boston Children’s and went to see Brian in the ICU. He was all wired up, and they did a good job when we got there of showing us what to expect in the ICU. He looked phenomenal—they were impressed with his O2 sats, his coloring, his breathing, everything about him was looking great. And beforehand, Dr. Breitbart had explained that it could go one of three ways, “1) He’ll require the first part of the three part open heart surgery within the first couple of days of life, or 2) they can stent him and keep his PDA open (which obviously is a far less invasive thing than open heart), and that way we could take him home and fatten him up and come back in two months for the first surgery. Or 3) best case scenario, he goes home without anything and be closely monitored with whatever they would set in place for us.” But they wouldn’t discharge him until they knew it was safe to take him home.

After the first couple of days it was looking like the best case scenario—that we could take him home, we’d have to care for him without even having to do anything surgically. It was the fifth day of life when we went to check on him because he was discharged to the floor. They had kept his PDA open right at the beginning to help with the blood flow, and then after a few days he was doing so well they decided to see how he would do as it closed. They could see as it was closing that he was declining. The doctor felt that we had to put the stent in, that was just the best thing for him at the time.

That was a blow and a shock—like someone punched you in the stomach because you think you’re going home the next day with him, and now he’s getting a stent and it’s going to be another week and a half that we’ll be here. He was scheduled for that procedure the following day. I remember going in there and he had an oxygen tube and I was just crying, and keep in mind I have another baby and I’m a first time mother and have no idea what the heck I’m doing anyway. I was so drained so seeing him with an oxygen tube in his nose just broke me. And of course he couldn’t eat and that’s all babies want to do at that time, so they’re bringing him down to the cath lab before surgery and he is just crazy crying, crying, crying. I remember them trying to put the IVs in, and the veins were blocked, they couldn’t get it right, of course his veins are very small plus he’s a baby so it’s hard to find them, so it was just one thing after another. I don’t even know what he was feeling, but I was just helpless, I couldn’t do anything for him.

He got into surgery and we went down to the waiting room and prayed a rosary over and over again. Literally 20 minutes later, the doctor come out like, “It went phenomenal! I don’t think it could have gone any better, he’s in recovery, everything’s fine.” And then he’s mid-sentence and his beeper goes off and he walks out of the room because he has to take it, and then he walks back in and he’s got a different look on his face. Basically he says that, “There’s a complication, apparently Brian has just thrown a clot in the stent.” It happened in a matter of less than 10 minutes, later they said it’s literally the world’s fastest clot they’d ever seen thrown. Our whole world just dropped, I mean, what the heck? So, now they were now going to do the most aggressive part of our original plan, the open heart surgery. They were able to pull the clot out from the stent, so he was stable, but he went into a surgery called the Norwood. I try to block that first two weeks out of my mind. He went into open heart surgery that night, that’s how rushed it was. He was a lot worse, he was stable but they didn’t hesitate.

We sat in that waiting room and cried and shook and prayed and it seemed like forever, it was several hours, of course. Then it was maybe two in the morning and they were like, “Hey, y’all should go get some rest, he’s stable, they’re transferring him to the ICU.” We go to the Yawkey, and my mom’s with Danny the whole time, she was basically his mother that whole period. I couldn’t have done it without her, I was still breastfeeding too, so that was just a whole other element of exhaustion. Within two and a half hours—so it’s four thirty or five in the morning—they call and say that Brian has thrown a clot in the shunt that they put in, so it’s not looking good. They want us to come down, they’re having to do CPR. The worst call you could probably get.

We rushed down there and they had stabilized him. The doctor and one of the nurses come in and they basically say that they’re very concerned but, “We have him stable, he should be okay to rest through the next few hours. Y’all go home for a bit.” So, we’re freaking out, we go back to the house. Then they called maybe in an hour of us being there and said that he’s going downhill. My husband and I were just, “What does this mean? What is happening.” My dad had called to check on us, but we just yelled at him and said, “It’s not good” and kind of blamed my sister who had died a few years ago, like “Why can’t she do anything from up there?” Just, you know, crazy things going on in your head.

We went back to the hospital. They wanted him to rest for a couple of days, and then he’d have his next surgery. They had got together some colleagues, the best of the best, and this is a very rare situation and not the procedure that they were going do, just the fact that this happened, they can’t explain why he was throwing blood clots—it happens in 7% of these cases. The doctors figured it out and they didn’t put another shunt in, they actually stitched something into part of his heart, one of the muscles. Anyway, that was the second open heart surgery. That ended up being the temporary fix. We were in the hospital for almost two months, just waiting for him to get better from everything that had happened to his little body within the first week and a half. The worst part was over. Everything that was traumatic happened within that first week and a half of his life. Every time my husband got a call, people would just have to say certain things after he picked up the phone, because they’re calling to check in with you about stuff constantly. I just assumed that every call was going to be devastating, because that’s how it was. As the days went on it got much better, his condition stabilized and he was getting better little by little.

 – Lindsay, mother of Brian, age 2, Heterotaxy


We kept going to our local hospital because by then we had established a relationship with the team there, the doctors, the nurses, and they were the ones that we followed up with for the single ventricle program, that we checked in with every week, so we didn’t really think about exploring other options for hospitals. At four months old he went into another heart catheterization, and they decided “Okay, he’s stable, let’s do his Glenn.” So he had that the day after he turned four months old, and he did well with the surgery, but a few days after the surgery he developed fluid around his heart. Typically they like to keep you there for three to five days, but because they used a syringe and took fluid out from around his heart he had to stay in the hospital a little longer.

Unfortunately, around that time he came into contact with someone at the hospital that had shingles, so he contracted varicella. He got chicken pox three days after his open heart surgery. So it kind of changed everything. He went from being a kid with a heart condition, to a kid on life support, not sure what was wrong with him, they didn’t know why he started crashing. It took almost a month for them to figure out that he had contracted the varicella, and by that time he was on life support in a medically induced coma. Once he started being treated for the varicella, they tried to take him out of the coma and see if he could breathe on his own, and that’s when we discovered he had a major stroke when he was under. So he went from being a child born with a significant heart defect to being a completely medically complex hemiplegic. He’s got hemiplegia on the left side of his body, lots of developmental delays, feeding issues, breathing issues. So it became so much more than just the heart.

 – Alice, mother of Zach, age 3, Single Ventricle


That night after his surgery I was going to sleep bedside, my husband was going to sleep in the family unit. I was laying on the window bed with the little curtain drawn, Austin’s little crib was probably six feet away from me, but on the other side of the curtain. The nurses said to me, “The first night post-op there’s going to be a lot of adjusting things, a lot of activity, but no need to panic when you hear a lot of activity out here.” I said “Okay.” Then probably about 45 minutes later, the nurse called a code. I just sat up, but I was still behind the curtain, and I was in shock. I was saying “What’s going on?” I could hear all the commotion on the other side of the curtain, and I remember saying, “This isn’t happening, this is a bad dream, this isn’t happening, what’s going on?” And I pulled the curtain back and at this point the room looked like the operating room.

When I got into the hallway, I just dropped to my knees. Another couple was hugging me, and all they kept saying was, “God is good. God is good. God is good.” Somebody said, “Can I get you something?” and I just said “Somebody get my husband.” They sent somebody downstairs to wake Scott up. My husband got up there and he sat—we were on the floor in the hallway. I couldn’t take my eyes away from the bed space because I didn’t want them to stop trying to save him. At some point they went by with the ECMO machine. He spent the next three and a half to four days on ECMO, and thank God was able to gradually be weaned off of that. And knock on wood, he has no ill-effects from being on the ECMO. It was a scary time. I mean, I look back now and-he was in the hospital for a total of 13 days, which seemed terrible at the time. We didn’t know if we’d ever be bringing him home. I can look back now and say it’s truly amazing.

 – Grace, mother of Austin, age 10, TOF


Melanie: He had surgery on Thursday, and on Sunday in the morning the nurse practitioner came in and said “Well, we did some labs and an echo, everything looks good, you can go home today,” and I’m like “huh?!?” because we’re three hours away, and Dr. Newburger and I had talked—she said maybe Monday and I said, “Do you think we could push it to Tuesday because I’m really nervous about going home that far from the hospital,” and she said “Yeah, we can.” He had the labs and he went down for an echo, was fine when he went down, insisted on walking to the elevators and stuff, and we were down there way over an hour with him lying mostly still that whole time and we came back and all of a sudden he said, “Mom, I don’t feel good.” I went and got the nurses and his blood pressure was like 240 over 140 and immediately, zoom, swarmed, doing vital signs and stuff. Dr. Newburger happened to be the on call cardiologist that weekend. For us to have her on call just made me so much more comfortable because she’s the expert on this.

Walter: She came flying in and in about ten minutes he was in the ICU.

Melanie: I couldn’t pack fast enough to get back into the ICU. And then they needed to get another line in him because what she came and told us, “We need to do another CT scan because I’m afraid the repair has failed and that we’ll be going back to the OR.” So that was for both of us a total kick in the stomach and I’m thinking, “I’ve been through this once, I don’t know if I can go through this a second time,” knowing what you face and what he faces. He didn’t know the first time, so often when you don’t know, it’s a little easier on a patient his age I think.

Walter: So they needed a CT scan. And of course weekends, nobody’s there—Dr. Newburger’s on the phone, dragging somebody out of Brigham—

Melanie:—and she’s talking to Dr. Emani, who’s at home. It took hours, agonizing hours to get his blood pressure down, and then finally they said, “We’re going to do the CT,” even though his heart rate wasn’t as low, because they get better pictures the lower the heart rate is. They do the CT scan and then Dr. Newburger found us shortly thereafter and said “The repair is fine, we don’t know what happened.” So they continued to work on getting his blood pressure down, and he spent the next day in the ICU being bored out of his mind, because he felt okay by that point, his blood pressure was better.

Melanie: He was out of the ICU in less than 24 hours.

Walter: He still had his chest tubes in him, but he was so determined that he’s going to play soccer, and whatever it took to make it happen, he was doing it.

Melanie: It didn’t matter how much it hurt, he was going to do it. And I hear from other people, a colleague whose daughter has had several cardiac surgeries said afterwards, “I didn’t want to tell you, but that happens a lot that they go back to the ICU” and I’m like, “We were going to go home until we were back in the ICU.” It took a couple of days to get his blood pressure under control with oral medication, so he was discharged on two oral anti-hypertensive medications. That was Sunday that it happened, and we went home on Wednesday.

 – Melanie and Walter, parents of James, age 20, Anomalous Coronary Artery