Deciding on surgery

Allen: We really relied a lot on our conversations with Dr. Newburger. There’s the trade off of intervening sooner rather than later—we knew though, that we were going to have to intervene. They never say that with 100% finality, but it was I guess a hole that they were confident would never close. So then it’s a matter of when do you do it? When we initially talked with Dr. Newburger, the target was around three years old. Serena was at the time pretty petite, and also not eating very well. She was playing active, but her growth chart both in length and weight had sort of flat lined. She was falling down in the percentiles, which could or could not be part of it. But talking with Dr. Newburger at length, we said, “well, if we have to do it anyway, why would we wait? Why would we wait to see if this is the reason why her growth has plateaued? Is it a reason? We don’t know, but why don’t we take that variable out.”

Miranda: And one of the hardest things was that we saw three cardiologists in Connecticut, and they all disagreed. As a parent, it’s really hard to know what to do then because you’re like “Well, I like this person, so I want to agree with them, but I’m not sure I actually do, and I didn’t really like the attitude of this guy, but maybe he knows what he’s talking about. When you’re not medical and you’re relying on medical opinions, I think that’s really tricky. So when we’re faced with something like that we just go get more information because we don’t have a tiebreaker. So that’s what drove us up to Boston. It was really helpful that up in Boston they were super clear with us. And I mean, she basically sat down and said “There are these new methods, but we wouldn’t recommend one of them.” Having that clarity—I didn’t like that open heart surgery was what we were going to do, but I felt good about knowing with certainty what we should do.

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


Deciding on an ICD/Pacemaker

We had a lot of communications with the doctors when she was five because there started to be a bigger change. The doctor was seeing some thickening that was speeding up a little bit. We did a couple more tests that year and we went to six-month appointments. After she had just turned six they brought her in and did a catheterization and an MRI. During those the doctor said, “Based on those results it’s really time for her to have this surgery, the myectomy.” It made sense for us at that point to talk with an electrophysiologist and get her ready for something like that, because they knew at some point she was going to need a defibrillator. With kids, because of the size of their arteries and veins, it has to be an open-heart surgery. So if we’re already opening her up, it doesn’t make sense to close her back up and maybe two years from now say, “It looks like she needs one, open her up again.” So we knew that the wires themselves aren’t generally the problem in little kids when they’re not hooked up to something, it’s once they’re hooked up to something that the stretching can be a problem as the kids grow. So it seemed to make sense for us to at least put the wires in at that point and then see how it went from there. Now when she gets the device in it doesn’t need to be open-heart surgery because the wires were the part that needed the open heart. Now it’s just the placing of the actual device. It’ll be a much less invasive surgery this time. Of course now there’s a new device that goes under the arm and it only has one lead, but she can’t get that because she has the wires in for the other device—it’s kind of a glass-half-empty side of it, that we can’t do a smaller, easier device now because of what we did a few years ago, but we knew she was going to need a device at some point so it made sense at the time.

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


We had to work with the surgeons and Dr. Lang to figure out what we were going to do. The decision laid in our hands, which was a big surprise to me. You always think doctors know exactly what to do, but there were several courses of action, all of them had their pros and cons. We had to ultimately make the decision of what we thought would be best. That is when you really feel alone and stranded. The surgeons and doctors obviously guide you, but there is a certain point where you need to say, “I’d like a mechanical valve for these reasons, or I want a bio prosthetic valve for these reasons, or I want to do a repair in place.” Those are kind of the three options. We chose repair in place which is Dr. Emani’s specialty and he was sort of leaning towards that direction. There were pros and cons to both but, the biggest pro was they were fixing needed tissue and they had gotten better at that over recent years. That was the least invasive, least foreign material, so that is what we went with. That was attempt number one. We made that decision and it worked for six months and then it failed catastrophically a lot quicker than the first time around.

As a parent, I felt “Wow! I made the wrong decision there!” And your child is the one who has to deal with that. That’s one of the things that parents need to know, is that in some heart conditions, yes, there are certain prescribed methods for fixing it. In her case, there isn’t. Especially being female and pre-adolescent, it is the worst of all factors combined together because she is still growing. Mechanical valves, you need medications that will affect pregnancies in their future and cause birth deficiencies. All these things you have to weigh in your head. If she was a 60 year old male it would be a mechanical valve, you don’t even think about it. So we had a lot of decisions to make and had to learn about a lot. I mean you have to become a cardiologist yourself pretty quickly to communicate with the surgeon and the doctor.

 –  Paul, father of Sierra, age 13, Valve disease



We didn’t want the defibrillator at first, but when she had that second episode in New Hampshire there was no question for me about it, I just wanted the thing put in fast. Having a defibrillator has not been a bad experience for me. For her, it was body image stuff because she’s thin and it shows a little bit. But it’s not bad, with her prom dress you couldn’t see it, it was okay. She’s okay with it.

 – Amy, mother of Rebecca, age 19, ARVD