Challenges of coping with complications and a medically complex child

Our experience in Boston was a learning experience and it taught me to ask questions, even if they are stupid questions—sometimes stupid questions can lead to smarter questions. So when Elizabeth had to have ear tubes done over the summer, I told the hospital in our home state, “in Boston they let you stay with your baby until you fall asleep. And in Boston there’s a pediatric anesthesiologist.” That was important to me. But they didn’t have a pediatric anesthesiologist here, so I booked an appointment at different hospital to do the ear tubes. I met with the ENT at the new hospital. They had a pediatric anesthesiologist, pediatric nurse anesthetist, and a pediatric anesthesiology nurse in the surgery, in a five minute surgery. And the ENT was like, “It’s not that anything’s wrong with Elizabeth’s heart, but we’re ready.” I guess what I’ve learned is just ask questions and be an advocate.

 – Anna, mother of Elizabeth, age 2, VSD


The first couple of years, the challenges that he’s had are not really cardiac related, they’re more heterotaxy related. He has kind of a weak lung-sinus-ear system. He gets really stagnant fluid in his lungs and ears, and that has definitely played into recoveries from surgeries. And he was super prone to ear infections, sinus infections, upper respiratory infections. Up until last winter, winters were challenging because he would get sick and then he couldn’t get over it. He would have a lot of upper respiratory infections. Aside from that, he’s done really well actually. With school, you would assume he would get so sick being around all these kids, but he really hasn’t. I think balancing good hand washing and sheltering him a bit, but still allowing him to be around other kids and get sick and touch germs, it’s actually been good. I think it’s helped build his immune system and he’s done well.

 – Amanda, Mother of Carson, age 5, Heterotaxy


Her recent pneumonia is when the needle fear started. She had lost a lot of weight, and wasn’t eating and drinking so she had to have IVs. She was so sick and lethargic that she couldn’t really fight much, but they used this stuff that numbs the area so it doesn’t hurt, and they talked to her and explained what was going on. And they gave her options, “Would you like to have this done? Would you like to be the one to take this off?” They let her have options and it helped her feel like she was making the decision.

With a child who has a heart thing, as a parent, it seems more intense when they’re sick. But one day when she was in there she goes, “I prayed that I wasn’t going to have to have any more needles and God heard my prayers, and what he gave me instead was the treasure chest.” That’s where they got to pick toys. So I think children have a great way of making situations that aren’t the most desirable into something as good as possible.

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


When Austin was four he was getting low grade fevers that just would come and go. I took him to the ER, they said it was just a virus. We went back to the ER and they thought I was crazy because it’s just a fever. And I said, “But every night he’s been getting it.” They did a blood culture, said it’s probably just a virus, and sent us home. The next night, the ER doctors called and said, “There’s something growing on the culture. We need you to bring him back in.” I said, “Okay, when?” And he said, “Now.” So we threw a bag together, got him up, and went off. We found out that he had endocarditis—an infection that settles and attaches itself in the heart. He could have died from it. He had to have six weeks of IV antibiotics which was really hard for him.

 – Grace, mother of Austin, age 10, TOF


When she was about three and a half, she had pneumonia. A couple months after the pneumonia, she coughed up something that we looked at and thought, “This is really weird, what is this?” I had asked one of the EMTs that I worked with and he said, “It’s probably a mucus plug.” But when we talked about it to her cardiologist, he said “If this ever happens again, save it and let me know.” That’s when he told us about this condition called plastic bronchitis. A year or so later she did it again and then it started happening more frequently. We’re in the medical field, so science is very interesting to us. We started using a toothpick to measure it so that we could keep track of the sizes and frequency. They were becoming more and more prevalent and some of them were really hard for her to get up. That sent us back to Boston another time or two for heart caths, just trying to see if they could figure it out. It’s such a rare thing, that there wasn’t a whole lot of information on it.

After she had had a year of the plastic bronchitis and a year of coughing up blood, they wanted to do a transplant evaluation on her. We went in for an evaluation and they also started her on some medications. She hasn’t had any issues since starting the medication, so whether it’s coincidence or the extra diuretic has helped or he changed her Aspirin too—if it helped with it or it’s just coincidence we won’t know until if and when it happens again. But right now her plastic bronchitis seems to have taken a backseat and she’s doing okay. And she has not had a transplant. My husband was not ready to list her, so we waited. Now two and a half years later she’s been doing fantastic. It’s been two years since her last episode of plastic bronchitis, and we’ve backed off on some of the respiratory medicines in the hopes that maybe it’s gone.

 – Donna, mother of Leah, age 10, HLHS


He had the third surgery just before his 4th birthday. And that was supposed to be the last surgery. And then it wasn’t. Then he developed PLE (protein-losing enteropathy). I’m not sure you’re always told all the potential complications because you’d be worrying, “oh my God, he’s going to develop this, maybe he’s going to need another surgery,” but the down side is not knowing and thinking everything was swell for 10 years, and then all of a sudden it isn’t.

It really wasn’t until after his surgery for PLE that he came home with just a slew of meds. And he was angry, because that surgery, he was almost 13, it was totally unexpected. He’d been having migraines and he was looking bloated, but he was putting on weight, he was growing, he was 12, pre-pubescent. He went in for his routine 6 month cardiology check-up and met with the nurse practitioner first. She left the room and then she came back with his cardiologist and that’s when it was like, “This child definitely has PLE.” They did a test and confirmed it. That was on a Monday. Our cardiologist called us that night and basically said that he had to have surgery, and he had surgery that Thursday. That’s how quick it was.

Gabriel was angry. Because here he was a teenager and it was spring break, and he was just angry. We were blindsided. I think he was scared, I remember we met with the surgeon and we had never met him because another doctor had done all three surgeries, and I remember Gabriel asking him, “do you have good results?” It was the funniest question. He was in for five weeks. He was back and forth—his sodium was off, his potassium was off, he was restricted on fluids, it was really hard.

 – Louisa, mother of Gabriel, age 20, HLHS


For him, and for our family, the bigger issue from the age of two forward was not heart, it was the asthma. The asthma has been an incredibly controlling aspect of our life. Literally, the joke at work for me is that I don’t take vacation. I use vacation time every year, but the reason I don’t take vacations is because anything we ever planned had to be cancelled. We’ve missed family events and vacations. Jake would get sick, and we couldn’t travel when he got sick. I felt badly about that for both Matt and Jake. They don’t know any different, so I’m not sure they would identify that as a loss, but I do. We’ve never been to Disney World for example, the “be-all-end-all”. Things that most families do, we never did. I think you’ve just got to reach out. The isolation people feel, and the not wanting anybody to think that you can’t handle this, drives some people, and it did for me, to be very private and not share what you’re really going through. And I think that that’s dangerous. Maybe things didn’t need to be as hard on us but hindsight is 20-20. Reaching out to others when times are tough would be my recommendation.

 – Diane, mother of Jake, age 21, HLHS


Courtney: In addition to the Tetralogy of Fallot, which is often the case, his midline development was impacted. What that means sometimes is a Cleft Palate for some kids, he had hypospadias. At the end of his freshman year, he told us that he’d been having a pain in his side. We took him to his pediatrician, set him up for an x-ray and it was a kidney issue, so he had robotic surgery at Children’s. They had to un-twist the connection leading the kidney to the bladder and he hasn’t had any pain since. But it manifests itself at puberty—he could have had it all his life, they think he probably did. Because of his life as a college student, one doctor said, “Do you drink beer, David? Because if you drink beer, your kidney is having a hard time processing it. It’s causing pressure.”

Richard: One kidney was working 100%, the other was at 20%. And when he was trying to keep up, that was when he was having the discomfort. So I mean they could have just removed the kidney but his doctor said if you’ve got two good kidneys, you should take care of them. So we went ahead with that surgery. So once again back to Children’s and back to full anesthesia.

Courtney: We had surgery, he was fine, in fact he was better than fine, he thought he was 100%. Of course there’s no stitches or anything because it’s all robotic, and just little teeny-tiny holes that they go in, and he came home that day. At 11 o’clock that night came into our room in excruciating pain. We rushed back to the emergency room at Children’s, he’s in all night on morphine and he had an ultrasound, the girl said it looked like he was at the bottom of a rugby pile, that he was just loaded with blood clots, he was just stuffed with them. But that was the most excruciating pain I’ve ever seen him in, and it didn’t have anything to do with his heart. So that was really hard.

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

Challenges of having a child with with CHD

Hearing that we were part of this one percent of children born with a heart condition was so shocking to us. You never think you’re going to be that one in 100. I think that was sort of the biggest challenge for me, was moving from this pool of, “Oh no, does she have an ear infection, is she teething,” and having that be a big deal and going to the pediatrician for that, to going to sitting in a cardiologists office. Now you’ve leaped into a different pool of kids who have a heart condition and for me that was really difficult. What did I do wrong? Did I do something during my pregnancy? I thought I did everything right. I breast fed for a full year, I did everything by the book. How did this happen? It’s hard not to question yourself in that way. But then you’re in this pool of kids and you’re dealing with these specialists who for them, it’s their normal.

 – Jessica, mother of Avery, age 2, ASD


The first thing in my mind would be just getting answers. The first challenge was getting a diagnosis and getting answers. That felt like a huge challenge. And then, once we had that, the second challenge was just the waiting game and the uncertainty. Once we had the answers and we had the diagnosis when she was three months old, it was the uncertainty of knowing that if she hadn’t gained half an ounce today, she could be in heart failure again. Going in and out of heart failure* saying, “Are we going to have surgery? Are we not going to have surgery?” Part of it is just, I’m a planner, I always have a plan, and there could be no plan. There was nothing that I could do to change it. And that was huge. That’s hard to feel so out of control.

 – Anna, mother of Elizabeth, age 2, VSD


I think it would just be the feeling of being so alone in it and not having resources—not having a book I could consult, like “Okay, Zach has all of these issues, where’s my textbook on it? Where’s my road map for what to do?” Just feeling alone in terms of how to best help him and also finding people who could even begin to relate or understand what we go through as parents too. Even just between us, we handle things so differently that we’ve been in the same situation but you can still feel very lonely because you just don’t know what to do.

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


The greatest challenge I think is just accepting it all, initially. Accepting that she had such a complex heart. I questioned for a long time why it happened   to her. I had a lot of guilt that it was something that I had done in my pregnancy, even though I was told repeatedly it was just the way that her heart developed. I think it’s been a lot of challenges instead of just one particular thing. Things like accepting what was going on, then the challenges of just trying to be ahead of the game, anticipating what’s going on and when she’ll need to go back. Also being her advocate because she can’t be her own advocate just yet.

Day-to-day, there’s still worry even though she’s been through two surgeries and her doctor is telling me that she’s at a really good place, she’s healthy. I think it’s fear too—not knowing what the future holds, hoping that she’ll never need a heart transplant or that she won’t need another surgery, that she won’t need another surgery after that surgery. It’s hard to put into words exactly what the challenges are, it changes. When we got closer to the surgery, it was the challenge of getting through it, emotionally, mentally, being strong for her and then the challenge of day-to-day after that through her recovery.

A lot of times, luckily, it’s not at the forefront of my mind that she’s got all these heart issues, because she acts like a normal kid, but the challenge again is just accepting what the future could hold and not knowing for sure what avenue we could go down again with her.

 – Roxanne, mother of Amelia, age 7, Dextrocardia


The most challenging issue is the uncertainty about any time there could be an emergency. I think it has helped to try to be as prepared as possible, to be CPR* trained and to try to be as prepared as we can so friends she goes over to their house, their parents are aware and are CPR trained.

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


There’s one conversation we’re going to have to have, but I’m not sure how we’re going to do it. It’s about the fact that I have the Lupus antibody* that she has, and the complete heart block. I think one day we might have to have that conversation. I’m wondering if that’s going to be a hard conversation, but for the most part, it’s really just the fear of having another operation. I will say, she ended up having an IV in her head at one point, and that was a hard thing. I look at the pictures and tear up. I think I’m on this side where I’m so much more optimistic, but it’s a hard thing to go through, and I don’t want to be too light on that.

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


The greatest challenges were the surgeries for sure, the more invasive procedures and getting through and trying to stay as positive as possible. We’ve been so fortunate that he’s been a healthy kid that sometimes we almost forget at this point. Every once in a while I get knocked back into reality and we’ll get a little panicked thinking about if the next shoe is going to drop kind of thing, but I think we’ve been fortunate that life has been kind of normal. It breaks our hearts when we see that he has a hard time keeping up with other kids, and sometimes that affects his relationships. But it’s not something that we dwell on every day, but as a parent, it’s very hard.

 – Abigail, mother of Johnny, age 9, HLHS


The biggest challenge is the unknown and knowing that things could change on a dime. God willing, they’ll just continue to go well. I remember the first time we were up at Children’s, talking to doctor and my husband finally asked the question, he said “What does this mean? The longevity of his life, how old is he going to live to?” And the doctor looked at him and said, “Married with children.” Which was great to hear.

I remember many, many times when Austin was a baby being told, “He’s a very sick boy. He has a very life threatening heart defect.” It’s been repaired, but my husband and I both know that he needs lifelong cardiac care. His heart is still very different from what most babies are born with. So that’s probably the biggest issue. In life in general, there are no promises. Anything can happen and you don’t take things for granted. But there’s that extra sense of, you just hope that he continues to thrive the way he has been. He’s an all-star on his little league team, he played last night and won. He was slamming the ball, he was pitching it, mouths were dropped because he’s this peanut on the mound and he’s throwing the ball faster than a kid twice his size. We just went to the beach for vacation, and he’s out on the golf course with my husband playing 18 holes of golf. Sometimes playing tennis with my husband. He’s just a remarkable child that loves life and he’s just truly inspiring.

 – Grace, mother of Austin, age 10, TOF


It’s hard to explain to people that my 11 year old is in heart failure. Not congestive, but essentially she’s in heart failure. She doesn’t look like it, but she is, and it’s never going to go away. You can’t think that the myectomy* completely fixed it. I had so many people that would come up to me after her procedure thinking that she was fixed and healed, but that’s not the way it works. And as she’s this age, she’s getting tougher about things. She wants to push herself more and she wants to do more and she gets very frustrated with me because it’s typically me that’s doing the reminding or saying no. I’m sure there are days that she doesn’t really like me. I think overall I’m still good in her book, but she has her moments where I am not the top person on her list.

 – Dana, mother of Raegan, age 11, Cardiomyopathy with a Pacemaker


The biggest challenge was that it was not something that we ever expected, because no person would. Then you have to face what happened to you and people come up to you and say, “You’re very brave, I don’t know how you could have done that,” and I want to say to them, “Nobody knocked on my door and said ‘Do you want to do this?’ I was never given a choice.” None of us were given a choice, it just happened to us. And as a matter of being brave, nobody’s brave,  it’s not “brave”—you have to deal with what’s happening as it’s happening, because it’s not about you, it’s about your daughter, who it is actually happening to. And that’s a big challenge to keep in perspective—how it’s affecting you, but you have to almost step away from that a little bit because my job isn’t to worry about me, it’s to worry about her.

When we were in the Pediatric ICU, one nurse said to me, “You have to sleep at some point, because if you don’t sleep, you can’t look after Chloe,” which is terribly cliché, but you’re like, “Well if I sleep, something might go wrong.” But I’m not treating her, I’m just sitting there. So if something does go wrong, the people who can help her are awake and they’re there. So you have to let go a little bit. As a parent you’re protective, but you have to let other people who are more qualified than you to actually deal with the situation.

People’s reactions to us are a challenge sometimes. We live in the small town, so sometimes people know us when we don’t know who they are. After this happened to Chloe, there was a lot of media attention about her, so more people knew who we were. In the early days, we’d be walking around the supermarket and people would stop you and start asking questions. I had one lady who came up to me and said, “Oh you know I think you’re wrong, she didn’t have a cardiac arrest. Look at her, she looks great!” So people will say stupid things, and that was a bit of a challenge. People would approach my wife about it, and she’d get mad. And I’d say, “People only mean well. They just don’t know what to say a lot of the time, so they’ll say something. You may think it’s stupid what they say, but people just mean well.” People are not being malicious—even the lady who said “I think you’re wrong” was just trying to make us feel better.

 – Jim, father of Chloe, age 13, CPVT


The greatest challenge is knowing where the limits are. Knowing what she can and she can’t do, how seriously we take things, how not seriously we take things, that’s definitely the hardest thing. Do we let her go away to college? Nobody knows the answers to these things. We have guidelines, but it’s that not knowing, not knowing what’s going to happen, not knowing if we’re doing the right things. That’s super scary too. If there’re decisions to be made, I don’t know if we’ve made the right decisions.

 – Amy, mother of Rebecca, age 19, ARVD


You feel like you’re alone in this, and even though James doesn’t have to come back to see his cardiologist again, I will be emailing her and telling her if she wants to continue sending families our way we are more than happy to continue talking with them, because it’s very positive for me to talk with the families. You feel like you’re all alone and that you’re the only one going through this. Especially, I think with the older kids. If you go on the ward, there’re babies and young children everywhere that are having cardiac repairs done, but not so much in the older kids. Also especially when you go from a kid that you thought was completely healthy to having a potentially lethal heart condition, your world goes upside down. We had this vibrant, athletic kid who now could drop dead, and you feel alone. Even on the ward we didn’t have much in common with anybody else, because it was mostly little kids, and I think that is hard for families too.

 – Melanie, mother of James, age 20, Anomalous Coronary Artery


He’ll complain about having to take the meds. Every now and then he’ll say, “I’m going to die young anyhow.” I try to let that stuff go in one ear and out the other because I think as it is, I’ve been a bit of an enabler. I’d probably just say—it’s hard. I mean, it will be challenging some times more than others, but support is available and you should take advantage of it and whether it’s talk therapy for the child or therapy for the family in dealing with it.

 – Louisa, mother of Gabriel, age 20, HLHS


One of the hardest challenges has not been the medical for me, but the emotional, social, academic stuff that Jake has faced. And trying to strike a balance between acknowledging his struggles, and that there are very legitimate, concrete reasons that he is struggling—his teachers at school called him lazy. He’s not lazy, that’s not what it was about. Sure he has lazy days, we all do, but that’s not why he was struggling in school, it never has been. So acknowledging his struggles without letting him sit there and use it as an excuse. Trying to strike that balance has been really hard. I think most of the time we do it pretty well, but there are other times when we don’t.

His transition to college is probably his most recent one that I’m still focused on. That first semester in college, I made a conscious decision to let him sink or swim academically, and he sunk like an absolute rock. I will never forget the image of him sitting at the dining room table as he got his grades and found that out, and being so devastated for him because it was happening and also because I know that it’s because of his half a heart and the things that go with that. It wasn’t lack of effort, it was that he’s different. He is. But at the same time, knowing I couldn’t let him use that as an excuse, and I had to not let him wallow. It was a very sad, difficult period for us and I think we worked really hard at pushing him to take responsibility and fix it and make it right, while also acknowledging that we understand why it happened. That’s a pretty tricky balance to strike.

 – Diane, mother of Jake, age 21, HLHS


We’re fairly social people now that we’re 50, we have lots of friends and we do lots of fun things. We joke we had no friends then. There was a bagel shop a town over and we would go over there at eight o’clock at night, right before they closed with the babies, because they would clean, and there was no one else in there. We sat and had a cup of coffee and a bagel. For four to five years that was all of our social life. But it worked, because Jake really didn’t get sick, if he got sick it was really infrequently. That was kind of the sacrifice that we made to keep him well. If someone would come over to look at the baby, we’d just run out of there. So that was a challenge.

There’s the physical challenge of just getting them through it and making the sacrifices that you have to make, and of course we’re happy to do it. And watching him, emotionally watching this kid go through what he goes through, like all parents of sick kids do. So there’s that. But there’s also things like—Jake’s illness hurt us financially, it set us back quite a bit. So there’s the emotional part of watching your sons, one watch it and one go through it, it’s terrible, you lose a lot of sleep. And then there’s the kind of nuts and bolts part of it, which is, you don’t have any vacation and you don’t have many friends and you don’t have any money, but you have a child that’s getting better. That supersedes everything. So as far as the biggest challenge, above and beyond I think for us would be physically and then emotionally it’s a haul.

 – Bill, father of Jake, age 21, HLHS


For David and for us as parents, the challenge is always getting the reality check that he’s not like everybody else. There have been conversations about drinking or marijuana or his diet or exercise is one. He has to live a heart-healthy lifestyle among other things, so for us it’s been the ongoing challenge is just not losing sight of that. It’s so easy to become overconfident, because I mean when you see him he looks so healthy and you’d never know.

 – Richard, father of David, age 23, TOF