On Wednesday, September 28, 2011 Erica and I went in to Mass General Hospital for our 18 week ultrasound. We we’re excited to find out if we are having a boy or a girl. It’s a boy, and he has a special heart.
In this case, special = big challenge. A Critical Aortic Stenosis was detected at ultrasound, confirmed at echocardiography the next day by two pediatric cardiologists at MGH, and re-confirmed Monday October 3 at the Advanced Fetal Care Center at Children’s Hospital in Boston by the world’s leading pediatric cardiologist in this particular condition.
Everything you ever wanted to know about Critical Aortic Stenosis can be found here on the Children’s website. If you want to know more, Google around, but it’s not all up to date.
In this area of the Children’s website, you’ll find a bit about the treatment options, including in utero fetal interventional catheterization/balloon valvuloplasty. Either this Wednesday (Oct 5) or next Wednesday (Oct 12) this is the path we’re headed down. Oct 4 we head to the Brigham for prep for possible Oct 5 surgery. (Ah, if we were only headed to Brigham’s instead for some rasberry lime rickey, but not to be. Not to be.)
We’ve been consumed with understanding the diagnosis, options, and possibilities that come along with fetal critical aortic stenosis. What we’ve talked about could fill a book, but no time to write a book this week. For now, we’ll stick to a few of the most frequently asked questions. Not a bad idea, actually.
Frequently Asked Questions:
What do you know about baby Schultzmeister’s heart right now?
Aortic valve is mostly closed. This is bad. So the blood in the heart is flowing in all the wrong directions. Thus the left ventricle, whose purpose eventually is to pump blood throughout Schultz the Younger’s body, is working overtime to try to push blood through the valve. But it’s closed. So the left ventricle pushing harder and harder. Building up pressure. No rest. No release.
With the normal exit door closed, blood is flowing out the in door…in this case, the mitral valve. In other words, the mitral valve is leaking, and is likely also be starting to be damaged, at least a little. In this case this is good as the leakage relieves some of the pressure on the left ventricle. But it’s just temporary. Untreated, all sorts of things you don’t want to happen start to happen.
What happens if we don’t treat with surgery?
99.9% chance of Hypoplatic Left Heart Syndrome (HLHS).
HLHS is what we’re doing everything in our power to avoid. So we’re trying to head it off at the pass with the surgery.
Wayne Tworetzky, the doctor in this video, is our doctor, along with about 10 other world-class specialists. And there are a bunch of other folks on the care team, most of whom we’ve yet to meet. It’s a team that works together specifically on these. As Dr. Tworetzky put it, kind of like the guys in Space Cowboys, but it’s still a new, experimental surgery. I guess half of the Boston medical community is sounding the alarm for this one. Attention all Colonial units: Cylon attack underway. This is no drill.
What’s with the surgery?
We’re good candidates for the surgery because Erica is young, in good health and shape, the baby’s left ventricle is currently still Hans and Frans, and we’re early in the pregnancy: as of today, 19 weeks, 1 day. Average surgery time is 23 or 24 weeks. But the stenosis is severe, so good thing we caught it early.
To our knowledge, there have been about 140 cases of the surgery. Ever. Dr. Tworetzky and his team have done 89 of them. We’ll be number 90. (Son now has a basketball and soccer number, and a must-beat golf score.)
Been now 10 years since they started doing these surgeries. As they describe it, 10 years ago was the stone age. In the last 3 years, techniques and outcomes have improved significantly, though there’s still very little data and every case is different. No matter what, we’re rolling the dice, but we’re doing what we can to cheat at every turn and fix the game so we and the little bugger win the jackpot. Come on 7…baby needs a new pair of valves.
We’ve been given about a 10% chance that the baby won’t survive the surgery.
10% chance that it won’t be “technically” successful, meaning that they won’t be able to inflate the balloon in the proper place in the aortic valve. If it’s not, they’ll try again. And then if it’s still not successful, we’ll re-evaluate all options.
The surgery risk is minimal for Erica. She may not even go under general anesthesia, though we don’t know yet. The surgery is not dissimilar to an amniocentesis, commonly used in prenatal diagnosis.
While surgery is never risk free, Erica should be feeling well soon after, and get a band aid. Seriously. (I think He-Man band aids would suit her well. By the power of Greyskull…)
After the surgery, we’ve been given a 70% to 80% chance the baby will not develop HLHS. Again, this is what we’re fighting (not as hard as the kid, but trying nonetheless) to beat.
Then there’s after the big birthday to deal with.
If it works, then what?
If it works, we get an echocardiograph shortly after the surgery and hope to see changes in the blood flow. Then we monitor. The doctor said after a few weeks we’ll have a sense of whether it’s going/flowing in the right direction or not. If it is, we keep monitoring. If not, we evaluate options again.
At birth, if there’s no HLHS, there’s an 80% chance he’ll have surgery within days to work on valves and make sure everything’s working as well as possible. There may be no surgeries, 1 surgery, or up to 6 or 7. If there is HLHS, it’s much more complicated. But let’s assume no HLHS for now.
We were told to expect 2 weeks to 2 months in the hospital after the birth. Toby is already upset, as he wants to meet the baby and will miss us. When he stops snoring and dreaming about rummaging through your trash, he’ll tell you himself. RayRay couldn’t care less as long the baby doesn’t eat his wet food or compete with him for time with his beloved Eyeore (another story).
Anyhoo, 2 weeks to 2 months, though if it takes a turn for the worse, it could be any amount of time. And we fully expect a lot of 1 step forward, 2 steps back, even if it goes well. Touch and go is the name of the game. (Not like the one you played in college. Stop that…)
If all the stars line up, there’s no HLHS, and we make it through the first few months, there’s a good likelihood that the kid will have a normal life (normal as it can be with me, of course, influencing him). Football, fishing, slamming his body full speed into walls like any two year old…all fair game if it works.
There’s likely to be future valve surgery, but in the scheme of things, future valve surgery would be the jackpot, as the kid could have a great life with just a few more cardiology appointments than most kids, a bit of cow or cadaver valves floating around inside, and periodic stays in the Aorta Suites Hotel for a week or two every once in a great while.
Before we headed down this highly experimental path, we needed to hear that we were good candidates for the surgery and that the kid had a shot at being like any other kid. It looks like that’s a good possibility, so we’re going for it. Flutie to Phelan.
Who knows where it will take us, but we’ll go together with a hell of a lot of support, love for each other, a warrior on the way…
…and danger is already his middle name.