Bentley's Heart-March 6, 2018

Since moving to Tracy, I have realized that a lot of people aren't aware of Bentleys heart. Not that I don't want people to know, I just have a hard time bringing it up. Before I update his condition, I'll just give a little background on what it is.

Bentley was born with a rare heart condition called Congenitally corrected transposition of the great arteries (cctga).The easy way to explain it is the arteries on his heart are switched, the larger artery which is supposed pump blood to his body, is pumping blood to his lungs, and the smaller artery which is supposed to pump blood to his lungs, is pumping the blood to his body.  Cctga comes in 3 forms and Bentley has the most rare. His Cctga also causes him to have a heart block, which just means his the top and bottom of his heart aren't beating in sync, causing his heart to beat at a slower rate.

 Most of the times when diagnosed with Cctga kids have other major issues with their hearts and need open heart surgery in the first months and year of their lives. Now with Bentley, this wasn't the case, he only had the heart block. and at birth was monitored very closely and even though his heart rate is low, his body is handling it very well. As he has grown, his pulse has slowed down,which we knew would happen. The way to solve this is to put a pacemaker in. Our first cardiologist said that with how well Bentley is tolerating his heart block, he estimated that he would need a pace maker when he was around 4-5 years old. 

As for his Switched arteries, the smaller artery pumping blood to his body will start to wear out. While the larger artery, since it is not doing the work it was made to do, is losing muscle because its not working as hard.  The solution to this is to have a series of surgery that leads to them actually switching the arteries to their correct position. The first surgery they place a band on the larger artery and restrict it in a way so that it is forced to pump harder and build up muscle. The second surgery would be 6 months later and they will tighten the band a second time. then six months after that the artery should be strong enough and they do the actual surgery where they physically switch the arteries to where they are supposed to be.

Now looking at Bentley, you wouldn't ever know that anything is wrong with him, unless you took a stethoscope and listened to his heart, or looked at an EKG reading. His body is handling the CCTGA and heart block so well. The cardiologist said there have been cases where people have what Bentley has and have lived their lives without ever knowing it, or finding it when they are in their 60-70s. There are other cases where they have heart failure early in their life, and at that point the only solution is a heart transplant.

So here is where it gets tricky for us. He could live his life with just a pacemaker until a ripe old age, or his heart could fail in the next 10-15 years. There is just not enough research or studies done because his condition is so rare to know which case he will be. 

When Bentley was born, in the first year of his life, after being monitored closely the Cardiologist and we, decided to take a wait and see approach to see how his heart managed the Cctga and go back every year to asses the next step. That was our plan, and we were content with it. Then we moved here and everything changed. When I took him in to get our new cardiologist up to date with his condition, he was concerned that we never even considered the surgery when he was younger, and frankly we didn't know that was an option. He did the routine ekgs and echos and gave Bentley a 72 hour monitor and we set up an appointment a few weeks later so we could discuss the results.
At his next appointment we decided that is was definitely time for a pacemaker, but he still didn't have any answers about the surgery to correct his Cctga. I got the impression that he seemed to think it was a really good option, but again looking at Bentley he appears to be a normal and very active kid, he didn't want to just tell us to have him go through surgery. So he proposed that they take his case to Stanford during their weekly meeting where they discuss difficult cases, just to see what the specialists had to say. Another couple weeks go by and we hear back from them. And it turns out there is no straight answer. There are pros and cons for surgery and there are pros and cons for waiting it out.  Because once again they just don't know if his heart will fail, or if it will thrive with the Cctga, and there is no way to find out. So we decided to go talk to his surgeon, and see what he thinks. Once again he said he has nothing against waiting, and nothing against the surgery. He did say that if we chose to do the surgery its best to do it now, because the older he gets, the less chance he has of the surgery being a success. And basically leaves it up to us.  He then suggested that if we wanted to start the surgery, and put the band on his artery it would make his heart stronger, and healthier even if we opt out of the complete surgery. And the band would stay on his heart for his whole life. It wouldn't ever need to be taken off. Or if we decide we can opt to do the full surgery a year from now once he has had the bands and his heart is stronger. Of course there are risks going through with the surgery. There is even a small chance that the bands won't work, and in that case we wouldn't be able to do the complete switch.

Anyway I know that was a lot of information, and probably more than you wanted to read. But after much fasting and prayer Alex and I have decided that we will go ahead and put the first band on his artery and go from there.  The surgeon said putting on the band is a simple surgery and that it can be done along with his pacemaker. That way we can see if it will hold, and if it does it gives us 6 months to decide if we want to put our almost four year old through open heart surgery. But we have decided that if we can prevent heart failure in the future we should prevent it. A surgery now will be less risky than waiting around to see if his heart fails then wait for a transplant.

My heart has been aching for the last few months over this. I wish so badly that I was the one with the heart problem and I was the one going through with the surgery. But I know that for some reason it has to be Bentley. Any surgery is scary, and I am anxious and nervous for him. I am learning to put my trust in my Heavenly Father more than I ever have before. Also I have to say that Alex has been such a great support. He is my rock and always seems to know what to say.

Any and all prayers are welcome,  his Surgery is Scheduled for March 6 (for his pacemaker and his artery band). Recovery will be at least a week in the hospital, then after that they said he should be feeling like himself after a couple of weeks.

Anyway, thanks for reading, I'm always happy to answer any questions about what's going on, you might just have to bear through some tears on my part!

Love always
Lezlie and Alex