I've been sharing a lot of information about Lucas and what he has been/is going through on Facebook and Twitter in sort of short bursts. It serves well to keep family members and friends informed about what is going on, but it can be a bit disjointed. So I wanted to take a minute and kind of put a number of things in one place, as well as where he is headed, so anyone who is interested can be on the proverbial "same page".
We found out on the day we were supposed to take Lucas home that he had a congenital heart defect called TAPVR (Total anomalous pulmonary venous return). So the first question everyone generally has is "What is that?".
TAPVR is when none of the four veins that take blood from the lungs to the heart is attached to the left atrium (left upper chamber of the heart). This sounds bad, and in all honesty it is, but luckily his body compensated for this and he has an extra vein called a "vertical vein" that helps his blood somewhat get to where it needs to go. Unfortunately this isn't an effective system, and if left uncorrected, would prove to be fatal.
The next question just about everyone asks is "Why didn't they catch this before he was born?"
TAPVR effects how the blood flows to and from the lungs. When a baby is in utero, they don't breathe oxygen, and therefore don't really get or need the blood flow through the lungs. So in essence it just doesn't show up on an ultrasound. Also, if it is diagnosed in utero, they have no way of correcting it until after birth. In Lucas' case, he looked like a healthy baby, so at first there was no concern. Fortunately for us, his pediatrician heard a heart murmur while checking him before sending him home with us and had him checked out.
While I'm answering questions I will follow with "How did they diagnose him?"
To figure out what was going on when he came to Children's Hospital to be checked out, they did what is called an echocardiogram. This is essentially an ultrasound on his heart. This allowed them to look at his blood flows, the structure of his heart, as well as a number of other things.
And lastly everyone wants to know "How do they fix this?"
To keep it simple, they cut an opening on the pulmonary veins that came together behind his heart, and they cut a hole in his heart where they were supposed to go and sewed them together. Technically there is a lot more to it than that, but that's the short and sweet version.
So now with a little background, here is what went on with Lucas a bit more specifically. Like I mentioned previously, he appeared to be a perfectly healthy baby for the first couple of days after delivery. We were getting set to take him home when we were told that he had a heart murmur and would have to go from Akron General to Children's Hospital to have it looked at. After a number of hours of tests, we sat down with the cardiologist who explained to us about the TAPVR and that he would have to have open heart surgery.
Initially they put him on the positive end of the spectrum since he was healthy, decent size for his age (8 lbs, 9 ounces), and just so darned cute. We were told that the thought was that he had about a 90% chance of success with the surgery, and though it was scary we were optimistic. They were planning on doing the surgery the next day, Friday morning.
Lucas wanted to make us worry a little more though, so he developed some issues with his kidney function. The doctors decided since he looked healthy otherwise that they would postpone the surgery until that Monday in hopes they could get a better grasp on what was going on with his kidneys. That Friday night, unfortunately his condition seemed to worsen and his breathing became very labored. After some more xrays, ultrasounds, and tests they found out that one of his extra vein structures had become obstructed. This is a very bad thing, and what determines whether a child with TAPVR falls on the "healthy" side of the spectrum, or the "very sick" side.
So the surgeons were called and he was scheduled for emergent open heart surgery to correct the TAPVR first thing Saturday morning. The operation was about 6-8 hours long because it require a lot of prep as well as a lot of steps after the surgery. So we waited in the surgery waiting room with a lot of family and friends (whom we can't thank enough by the way), while getting periodic updates from one of the doctors that watched over the operation. After sitting down with the doctors that morning it was made clear to us that he was no longer in that 90% range, and could very well be in the 50% range of coming through the surgery. To say that thought made this a long day is an understatement.
Luckily every update we got was good and he made it through his surgery without any complications. By about 7 pm that night we were back in his room hanging out with our baby boy and enjoying pretty much the best Christmas present we'll ever get. When they do open heart surgery, the person's body generally retains a lot of fluid and they swell up so they leave their chest open. This sounds a little gruesome, but it really isn't. He had a patch sewn in place and some dressing over top of the area, so you really couldn't tell. After about 5 days his swelling had gone down and they closed up his chest. He just has a thin line now with a couple of really tiny stitches.
At this point I realize I left one question unanswered that may be fairly important, and that is "Why is his recovery taking so long?"
So since his blood didn't flow through his heart how it would in a normal baby, the left side of his heart isn't as big as it normally would be. So once they did the surgery, it had a lot more blood flowing to it than it had previously had. This means it has to work harder than before. Along with this the condition causes pulmonary hypertension. This basically means the vessels in his lungs are really constricted and need time to open up. So now that the surgery is over his body basically has to grow to adapt to the changes. The good side of this is that he is a baby, so growing is what he does. The down side is of course that it takes time.
After the first time going back on the ventilator, the doctors decided to do a heart catheter on him. This basically means they run a wire through one of his arteries and into his heart and use it to get a better look at everything and how his blood flows. This showed them that his heart still had high pressures and needed more growing time. It also showed them that one of his four pulmonary veins connected a little lower than they had previously thought. They think it will need to be corrected at some time, but not until he is bigger and healthier.
To help his body make this adjustment the doctors have put a number of things in place. Some of these are drugs that help his heart work easier, as well as Viagra that helps his vessels in his lungs relax. The main thing is the breathing tube they have him on, which does his breathing for him so he doesn't have to work as hard as he would if he was doing it on his own.
Obviously one of the goals is to get him off of the breathing tube and breathing on his own. Before they do this, they do what is called a CPAP trial. This is just basically them turning down the ventilator support for a short period of time to see how he handles it. A week or so after his surgery they decided he was doing well enough to take out the breathing tube. The first day he did well, but then he started to get worse again, so after a couple of days with it out they had to put it back in. After doing this he started to get much better again and the doctors were very pleased. So at the beginning of this week with the thought that the breathing tube would be coming back out they tried again. He did alright with his first CPAP trial, but on the second one he had some trouble.
So this brings us up to now. Over the past day or some we've had some discussions with doctors about what they think on his progress so far and his path going forward. Their general consensus is that they gave him a short period of time to let his heart adjust, but it hadn't grown enough to handle the load. So would giving him another short amount of time see much change, probably not. So what can be done?
The answer we have been discussing with the doctors is a tracheotomy. Basically they cut a hole in his throat and insert a more secure breathing tube there. This of course sounds bad, but when you weigh everything it seems like the right answer for a number of reasons. First of all, this will allow him to be more mobile. That means mom and dad will be able to hold him, and he will be able to come off of his sedation and be able to act like a baby as well as get treated like a baby. Secondly it gives us an avenue to eventually take him home. The doctors said most likely once he gets it we can expect to take him home in about 4-6 weeks. Of course nothing is set in stone, but just the idea of finally taking our boy home is a huge thing.
The other plus side to this is it isn't permanent. It is something that can be taken out, and will leave just a small scar. Along with the tracheotomy they are going to change how they run his IVs to reduce the risk of infection. So as parents we never want the best option to be "another procedure", but weighing everything it just seems like the best thing to do for Lucas. We're excited to see him awake and able to move around, even if it means a little bit of extra hardware.
So that is Lucas' journey so far and where he is at. I feel like it is worth saying that the staff at Children's Hospital are not only responsible for saving our child's life, but also for saving our sanity. They are some of the nicest people we have ever had the pleasure to meet, and we owe them more than we could ever give. This has been the hardest time in our lives, and I honestly hope no one ever has to go through anything like it. Even once you make it through the phase where you have no choice but to take things day by day, you enter a phase where you're constantly unsure of what the future holds, and when the future holds it.
We've been in the hospital for a little over three weeks now, and it is hard to stay positive, but it's something we have to do. Again we owe a lot to friends and family who have helped us carry this since it all began.
The best thing to remember, is at the end of all this it will be worth it.