I remember vividly being at a Cardiac Kids conference many years ago and hearing the Chief of Cardiology talk about the new generation of survivors and the shift that it has caused in the world of cardiology. One thing he said really struck me--he said that we needed to get away from using the word 'fixed' when referring to surgically repairing cardiac lesions in kids. They are indeed 'repaired', but not fixed. He wasn't trying to be a doomsdayer, but wanted to hit home the point that even though many kids get life-saving surgeries, their anatomy is forever different than a child born with a normal heart.
We were given lots of hope 15 years ago after Cooper was repaired; however, he was part of a new generation of kids repaired using techniques that had only been around for about 10-15 years. There was no data on kids living with repaired TGA as adults. Then, when he got his pacemaker at age 3, I saw his doctors scratching their heads trying to figure out how to implant a device made for adults into my tiny toddler and make it last. It was explained that pacemaker manufacturers don't spend a ton of time on research to figure out how to make a pacemaker work for 60+ years in the same person. Most people get a pacer after age 60. In any event, we have been a part of cutting edge medicine and experimental medicine all at the same time. Cooper continues to teach his care team about his heart.
Cooper has been followed every six months to a year since he was born. He doesn't know any different and for the most part, the appointments have been very routine. We've had some bumps in the road--learning he needed a pacemaker, discovering that his aortic valve (which is really the pulmonary valve when he was born) is leaking a little and watching some hypertension that developed after he got his second pacemaker 5 years ago. Oh, and Coop will never let the doctors forget that he had to go to the OR twice when he got that pacemaker--once to put the new one in and a second time to take the old one out--something that should have been done at the same time.
I need to set the scene a bit before I talk about the news we got on Kinley's birthday about Cooper's heart. Cooper's cardiologist, Michael Schaffer, is an odd duck. Nice guy and very positive, but a little odd. His catch phrase is, "Your kid is fine." He's said that to me a hundred times.
The day before Kinley's birthday, Cooper had a check up. He gets his pacemaker interrogated and about once a year, he has an echocardiogram to assess the valves and suture lines and function. I was reminiscing about how difficult the echos were when he was a baby and how nice it was to watch him take a nap this time. :) Dr. Schaffer came in to review the echo results with us and was acting a bit weird. He ended up spending a ton of time talking to Cooper about his weight lifting practices and explaining to him that straining and holding his breath, which comes with heavy lifting, has to stop. OK, that was weird. He also decided to put Cooper on blood pressure medicine (lisinopril) to take some of the workload off his aortic valve with the hope that the valve can hang on for many more years before it has to be replaced. OK, we expected that--his BP has been high for almost 5 years.
The next day, I was in Kinley's classroom helping with their Valentine's Day party and bringing in birthday treats. My phone rang and I was about to silence it when I noticed that it was Dr. Schaffer. He told me that he didn't want to scare Cooper the day before, but his echo didn't look good. His LVEF was down from 55% to 40%--a number considered to be congestive heart failure by most. The ejection fraction of the left ventricle is a measure of how much blood is pumped from the LV to the body with each beat. A normal heart ejects 55-70% of the blood volume in the LV with each beat, so Coop was on the low end of normal two years ago (a number I never knew because it's never been an issue!) After I almost threw up, I started asking some questions, but it was noisy in the classroom and not the right time to talk. He said we weren't talking transplant yet, but we needed to take the news seriously and make a plan. I hung up and gave Kinley a hug and left to go have a breakdown and call Doug.
Over the last 2 weeks, we have all been on an emotional roller coaster. Cooper took the news pretty well and yet the news is too close to what he witnessed with Spencer, so he had a look of panic in his eyes, too. I saw my husband cry for just the second time in 20 years. We wrote down all of our questions and I called Dr. Schaffer a few days later only to be told by his secretary that he was in South Africa for two weeks. What? Really? Sure wish I had known that he was leaving.
So, I emailed him our list hoping he would have internet connection. Instantly, I got an auto reply stating he would be gone and wouldn't have access to e-mail. Ugh.
My next step was to ask someone else some of the more pressing questions that we had as a family--what about baseball? What about moderate to intense exercise that didn't involve weight lifting? What caused this sudden drop in function? My stepdad's relative is a fantastic cardiologist in Greeley and he was familiar with Cooper's story, so on a desperate day, I called him. Not only did he answer all my questions, but he gave me a few more to ask Dr. Schaffer. In a nutshell, this is what Dr. Marsh said.
1) The cause could be congenital (a percentage of kids born with TGA also have LV dysfunction). Really hoping it's not that. 2) The hypertension and resulting aortic valve regurgitation (leaking) has put enough extra workload on the LV that it has hypertrophied and lost some function. Fix his BP and fix his exercise regimine and it should improve. 3) He has developed LV dyssynergy as a result of the location of his pacer wire in the RV. Long story short, some people do not not tolerate a heartbeat that is not in sync with respect to the ventricles. The fix for that is threading another wire into the LV and attaching it to his existing pacemaker and creating a more synchronous beat. There is no way to know the cause of the decrease in EF with Cooper until we try some different approaches to correct it.
The tender mercies of God were with me that day because as soon as I hung up with Dr. Marsh, I got an e-mail from Dr. Schaffer from S. Africa. He apologized profusely for dumping that news on me and then leaving the country and addressed each of my questions one at a time (and then jokingly complained that he doesn't type well and his fingers were cramped, which made me relieved because I needed to hear something light from him). So, we now have some of his answers and more new questions and we plan to meet with him when he gets back. His answers were mostly that he believes the cause to be either his pacemaker or the hypertension and he is confident we can turn it around and/or prevent it from worsening. Still so much to figure out and it was a huge reality check for us, but we are going to do all we can to help Coop with diet, exercise, meds, reducing stress and getting more sleep. The rest is in God's hands and we are coming to terms with that, too.
Cooper has been amazing through it all. He's had some tears, but his resolve to do everything in his power to turn this around has been truly amazing. I'm so scared, but so proud. We have very much appreciated the calls, prayers, and hugs. They have kept us afloat for the last two weeks!
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| For now, I choose to focus on the fact that having a teenage driver is taxing my heart! :) |
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