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