I have spent the last few weeks trying to figure out how to write this and put my thoughts to actual words. Luckily Jacob is able to write and because of that he will be the one maintaining the blog most of the time. He's usually the more eloquent writer anyway so it works out.
It's been quite the swing of emotions since our appointment with the maternal fetal medicine (MFM) team. In case anyone is wondering, it is totally possible to feel pretty much every single emotion in one day... I think I've done it a couple of times now.
We've been getting asked a lot of questions that are more or less the same so I figured I would answer them and try to explain things a bit more.
Q: Do we have a name picked out yet?
A: Yes. We've pretty much known what we were going to name our kids since shortly after we were married. That's part of the reason having a surprise gender was never a big deal to us.
Q: What are you going to name him?
A: Y'all aren't privileged to that information!!! If we can't keep the gender a surprise then the name is going to be a surprise!
Q: I thought you weren't going to find out the gender. What made you change your mind? Did you find out on purpose?
A: After the appointment that changed everything, and everything felt like it was crashing down on us, Jacob and I decided together that we would find out. We wanted to find out on our own terms, not someone slipping up down the road. Since we knew we'd be having a lot more doctor's appointments and various ultrasounds we felt the probability of it slipping was quite high so we turned in to detectives ourselves.
At our MFM appointment they made us a DVD of the entire ultrasound. Since that ultrasound was very detailed and covered everything we knew we would have a decent chance of figuring things out on our own. At each ultrasound the tech has always said that although our baby never wants to show off the face or heart or anything else they were really looking for, it was always happy to cooperate with showing them what gender it was. (Definitely takes after one of his parents already.... I’ll let you figure out which one is the stubborn, slightly uncooperative one) So after some slight indecisiveness on my part we popped in the DVD and did our best to figure out what on earth we were looking at. Well when it came time, it was not too difficult to figure it out. He is one very proud boy! The techs were definitely right, that as long as we're not looking for anything we really want to look at, he doesn't have a problem showing off.
The hard part after this was keeping it a secret! We wanted to tell our family first but had to wait until we could actually see most everyone. This is part of the reason it took so long for is to tell everyone else.
Q: Aren't you mad and/or upset at your OB for not catching it earlier?
A: Not in the very slightest!!! I absolutely love my OB doc! Plus, finding out earlier would not change anything. If anything, it is because of my OB that we even know that there are issues at all.
We went in for our routine 20 week ultrasound, and like it's been said already, our little peanut is quite camera shy. He generally hides face down and back up toward the ultrasound probe. After changing positions multiple times trying to get him to move around the white flag was waved. We were able to get a decent look at most everything except for the face and heart. The tech was able to see that there was in fact a four-chamber heart but it was very shadowy due to an arm being right there too. (A four-chamber heart is a good thing btw). So at our appointment with our OB he said he'd like to try again before our next appointment the next month. That he would give the little peanut time to grow and flip around. So 4 weeks later we were doing the same thing.... with pretty much the same results. Right before we were about to call it quits again he rolled over, ever so slightly. Just enough that the tech could catch glimpses of his heart.... again with an arm shadow. The pictures were sent to our OB and off we went. When we saw our OB he said he would like to send us over to the maternal fatal medicine doc to do a better ultrasound. He was able to see more of what he wanted with this one, but wasn't super comfortable with it, especially the heart because of the shadow and he said he couldn't be sure if there were problems or not because of the angle. So he's the one that hooked us up with our amazing MFM doc! That fancy ultrasound was scheduled for the next week. A week is a long time to wait when you don't know exactly what's going on!
Fast forward to the next week and we get official ultrasound number 5. (#1 at 8 weeks to confirm pregnancy #2 at 12 weeks because he's stubborn and the doc couldn't find his heart beat with the Doppler, so we did a quick scan to make sure he was still in there #3 at 20 weeks #4 at 24 weeks and #5 MFM ultrasound. Plus many other unofficial ultrasounds in between…it’s one of the benefits of working in the medical field) We spent the first part of the ultrasound in pretty much the same boat as the others –back up and camera shy. The tech was able to get measurements and pictures of pretty much everything except for the heart and face. Every time she was able to get the heart on the screen to start measurements and looking at blood flow, the little guy would move. It’s like he knows about his heart defects and is scared about getting in trouble so he hides them. So after about 30 minutes and a few position changes we took a break and tried moving around and drinking cold water to see if he would turn around for us. Sure enough, that did the trick and we were able to start getting some decent pictures of the heart, and even some of his cute little face (although there is still an arm and hand in every face shot we have). After the tech did everything she was able to the doctor came in and sat next to us and, like Jacob said, was very straightforward and blunt telling us that there were some pretty significant heart defects. He explained to us that he didn’t want to beat around the bush and there was no nice way of saying any of it. We truly do appreciate the bluntness, and feel that he explained everything to us in the best and most tactful way you could tell anyone bad news. He told us that he believed that the left ventricle of the heart was substantially smaller than the right side, that there was a hole and possibly two holes between the ventricles (VSDs), and that the pictures they had looked like it was a Double Outlet Right Ventricle (DORV –meaning that the aorta and the pulmonary artery are both coming off the right ventricle instead of the aorta coming off the left ventricle). He continued on to tell us that there are four different subtypes of DORV and he was not sure exactly which one because it is such a rare defect so he wanted to send us up to Primary Children’s Hospital to get an echocardiogram to take a closer look just at the heart. He also did an amniocentesis to run some further tests, mostly looking at chromosomal anomalies since there is no true definitive cause of CHD but can often times have other abnormalities that occur too. Needless to say that day was not what we had prepared for in the slightest and was incredibly overwhelming! Our one-hour ultrasound appointment ended up being a multiple hour life changer. Even though we were given news that scared us (and continues to do so) we were, and are, so grateful to have such amazing doctors and members of our healthcare team that have been so helpful, thoughtful, and supportive.
Q: Why did it take so long to find out?
A: As discussed in the previous question, a big part of this is that our little peanut is very camera shy and makes looking at what needs to be looked at a bit complicated. Finding out earlier would not make any difference anyway. But the most frustrating and hardest part for me has been the waiting. In between the 2 ultrasounds at 20 and 24 weeks was okay –we didn’t know anything was wrong or different. The week in between the ultrasound at 24 weeks and the one at the MFM clinic was a bit harder just because it was stressful not knowing if there was something to worry about or not. But the three weeks between our MFM appointment and our echo and consult at Primary’s was absolutely horrible for us! There were multiple breakdowns and emotional rollercoasters; one minute I would feel at peace with things, the next I would be in a full panic attack or would breakdown in tears over anything. I turned into the patient that I tell all of my patients not to be and did exactly what I tell them all not to do: I turned to Mr. Google for any grasp at answers. Since we had an idea of what was going on Jacob and I both tried to find out anything we could about CHD and more specifically DORV. But since a DORV is more of a rare defect there isn’t much out there other than explanations of what it is anatomically. Even looking up scholarly articles didn’t turn up many results of outcomes, prognosis, and what to expect surgically. I had one moment (okay, maybe more than one, but one major moment) where I was writing down all of my questions that I wanted to discuss with the peds cardiologist and it turned in to a bad idea, very bad idea. I ended up thinking about all of the worst-case scenarios and what ifs, and it all terrified me. It turned into a very rough night for me. It was eye opening to see everything that could happen, but I would not recommend it –I guess there is a reason I tell patients not to look everything up on Google ;)
I have learned that I hate being on the patient side of the healthcare world! It has given me a whole new perspective as to why some of the things I see in the ER are the way they are (but there are still a whole lot of other things will never ever be able to be explained). I have always told people that I would not make a good patient, but this has added a new dimension to it. Having to wait to hear back from people, waiting to hear from insurance companies, waiting to schedule appointments, and waiting for appointments to come up is the worst! It gives me way too much time to think, which has always been a dangerous thing. Trying to schedule the echo and consult at Primary's while emotional and stressed is another bad idea. Being told that the next earliest appointment was over a month away did not bode well at all. I remember calling Jacob while he was at work and I couldn't even talk to him because I was so upset; since he's the best hubby in the world and most supportive guy I know, he came straight home so we could talk and he could calm me down again. He ended up putting me to sleep and working from home the rest of the day (told ya he is the best). Luckily, I have some awesome people on my side and an amazing healthcare team that understood how rough the wait was for me and worked their magic and got me an appointment set up for 3 weeks later instead...still not what I wanted at the time but it turned out being perfect. We just wanted answers and knew that we wouldn't really have many until that appointment. It was still a long wait though. The waiting and unknowns has continued to be the most difficult part for me.
Q: What caused the defects?
A: Your guess is as good as everyone else's. CHD in general does not have any clear cause. The fancy people that research all of this have not found anything exact. Some believe there is a genetic and/or environmental component but nothing very convincing. They also say odds are higher with chromosomal abnormalities (such as Down Syndrome or DiGeorge Syndrome) which is part of the reason why they did the amniocentesis, but since that came back normal we can rule that out. As far as we know there's no one in our families with CHD either. CHD occurs in about 1 out of every 100 births (1% of babies born). Tricuspid Atresia occurs in about 5 out of every 100,000 births (0.005% of babies born). The heart forms in the first 8 weeks of fetal development and for whatever reason the Tricuspid valve on our little peanut didn't form correctly. Thus, leading to the right ventricle not forming and growing correctly. But the human body and hearts in particular are amazing at creating their own work arounds...the VSD (hole between the ventricles or bottom half of the heart) formed which still allows for some blood flow to get oxygen and be pumped to the rest of the body. Pretty much every single doctor and person we have talked to has made the point to tell is that there is absolutely nothing we did to cause it. I've known that deep down, but I still feel somewhat guilty and responsible, so it is nice to hear it from everyone. So essentially this happened all by chance. (Unless you want our churchy answer...then it's not by chance at all, but that's a different discussion that you can find me for later.)
Q: Where will you deliver? Why can't you deliver in St George?
A: The hope and plan is to deliver at The University of Utah. They are connected via a skybridge with Primary Children's Hospital. Since Primary's is where our little guy will need to be, that would be the easiest and best for all of us. Primary Children's is strictly a pediatric hospital which is why they don't have a labor and delivery unit. No one wants me to deliver in St George because then we would have to Life Flight the little guy to Primary's pretty much immediately after birth. Then I would still be recovering in St George while Jacob and peanut were in Salt Lake....not a good idea for this mommy! So we are just waiting (my favorite thing) on the insurance company to give their approval to deliver out of network at The U then we can finish plans and figuring things out and have less unknowns...we're slowly getting there. I'm either going to learn to be a very patient person by the end of this, or all of my patience will be so far used up I will never have any patience ever again.
Q: Will you have to have a C-section?
A: As far as what everyone has been telling us we don't have to unless something else with the pregnancy changes. So far everything else with the pregnancy is going smoothly so there's no reason for a c-section. There is a possibility of an induction but that is still in the figuring out part of things. We are hoping to have some more concrete ideas and answers after our next set of appointments with everyone the beginning of the year.
Q: Will our baby have to have surgery right after birth?
A: This all depends on how cooperative and effective his heart and lungs are after birth. From what has been explained to us so far, there are three possibilities for the first few days of his life: Option 1: There is not enough oxygenated blood being circulated through his heart and out to the rest of his body so they would have to divert the blood flow through the heart through a procedure called a BT Shunt. Option 2: The pulmonary artery is too big and is allowing too much blood to back flow into his lungs essentially causing congestive heart failure. This would require a procedure to place a band around part of the pulmonary artery to constrict it so the blood flow was more restricted. Option 3: The pulmonary artery stays just the right size and the VSD allows for enough mixing of oxygenated blood flow that we can monitor him for a few days and then we would be allowed to go home. It's like Goldilocks and the Three Bears 
and we're of course hoping for the "just right" option.
We do know that he will for sure have 2 open heart surgeries later though. One at about 3 months old, and the other between 2-4 years old. You can refer to Jacob's earlier post for a little more information regarding those.
Q: How are we really doing?
A: Since our echo and appointment at Primary's we're doing better; having answers and a little direction makes a huge difference! We definitely still have bad days and bad moments. Like Jacob said in his first post though, luckily we balance each out and haven't had our worse moments at the same time so we've been able to lean on each other. We have also been very blessed to have great family members, friends, coworkers, and bosses that have been very understanding and supportive to lift whatever they can off of us so we can focus on whatever we need to whenever something comes up.
From my perspective, Jacob is actually handling things as well as can be expected. He has enough medical knowledge from being around me and my friends that he has a pretty descent grasp on what the doctors and books they've given us are saying. Plus he is just the type of person that if he doesn't know, he's going to do what he can to find out! Primary's gave us some pamphlets and books that we've read together that helps. Plus he pulled nearly every journal article you can find and studied them. He could honestly probably write a research paper himself on pediatric hearts and CHD that would look like something from med school. He seriously is the one to talk to right now if you need to know anything about pediatric hearts, Tricuspid Atresia, or CHD in general! It's just the way he comes with things. Something that we've both really worked on the last few years is communicating more effectively with each other, and Jacob has really stepped up him game since we found everything out. He will come right out and say that he's stressed out, struggling, or having a bad time. He will also say when he's feeling pretty good about things and his thoughts on things. It has really helped me to know he is going through the same emotions that I am.
Personally, for the most part, most days are pretty good. Before Primary's I was a wreck most of the time. Now I feel much more at peace about things. I still get scared out of my mind, but it's easier to think about than before. I mostly just stress out about all of the other things - all the traveling to Primary's the next few years, the finances, the time off work for both of us, how to keep our little peanut healthy in between surgeries and in general since his immune system will not be as strong and he can't afford to get sick, being away from family and friends, staying in Salt Lake for unknown amounts of time, all the unknowns of surgery, fears of labor, not knowing if I'll be able to hold my baby after he's born, finding people willing to babysit later, etc... Honestly at times I overwhelm myself which is why I try not to think about a lot of this right now. It's not because I don't care, it's that I know I'm stressing myself out over stuff that I shouldn't and that I don't have much control over. On the plus side, it seems like every time I'm having a rough time the little peanut starts to kick around and move more; he's always letting me know that he's there and doing just fine and that he's a fighter. He already knows how to comfort his momma
Q: What has helped us the most?
A: I don't think I can name just one thing that has comforted us more than another. Caffeine free Dr. Pepper is amazing, especially on really bad days! Haha. Like already discussed, having answers and a direction was a big deal. I think for both of us music has been extremely helpful. Jacob has played a lot of the piano, which has always helped him through stress....and the little guy and I don't mind one bit I have listened to countless hours of Pandora and YouTube playlists (The Piano Guys, One Voice Children's Choir, and Josh Groban to be specific). We've joined/been added to a few support groups online which have all been amazing so far. It sucks that others have to go through this too, but it is so nice to know that we're not in it alone. A few people from those groups have reached out and we've been able to see their journey, learn from them, and help each other. There are a lot of great people out there that I've never met before, but feel like we've been friends for so long already. Jacob and I both have read and studied more than I think either of us ever did in school. This has brought the two of us closer together more than just about anything. It has forced us to talk about hard things together, we've spent a lot more time together as we go to various appointments, and we read our fancy heart book from Primary's together every night that I'm not at work. Being able to rely and lean on each other is awesome! Of course one of the biggest helps to us has been all of the love and support from everyone! Seriously, it would be so much more difficult without all of you! We have had so many different people contact us and share information, share stories, give hugs, offer thoughts and prayers, or just words of encouragement. Every single time it makes a difference. So thank you!!! More often now I find myself crying out of sheer amazement of the love that I'm surrounded by and the comfort that I feel, rather than the situation. I get a little overwhelmed at times with it all, so if you send me a message and I don't reply for awhile just know that it means the world to me, and I just need some time to process everything because this is all a new thing to me. Thank you!!!!!
Q: Will I be able to visit after you have the baby?
A: Yes, of course. We would love to have visitors! But please, please, please know that we absolutely cannot have anyone that is sick, or has been around anyone sick, come visit. No matter what kind of sick! The simplest cold can have life threatening consequences to our little guy. This is not just the rule while we will be in the hospital, but at our home as well. The hospital already has visitor restrictions in place (refer to Primary Children's Visitor Guidelines), and since we will be in the CICU for at least part of it, visiting will be even more restricted. We will update about this later, but just to be on the safe side, please contact either Jacob or myself before coming to visit :)
I was already a little paranoid about having a baby during the peak of respiratory season, but now knowing that little peanut will have a weakened immune system makes the mom and nurse side of my paranoia heightened. Especially in the first few months before he can receive his immunizations and while we're preparing for surgery we simply cannot risk him getting sick. I really don't want to be the mean or overprotective person, so if you could all help us out and respect our rules of absolutely no visiting if you or ones you've been in close contact with have been sick that would be hugely appreciated (even if you are "on the mend").
We really do appreciate everyone's concerns, love, support, prayers, and well-wishes. Please do not be afraid of talking to us about any of this; we are more than happy to answer any questions that you may have. We would much rather people come to us with questions or concerns than for things to go around the rumor mill -that is a big purpose for this blog. We will try to keep the blog updated as often as we get new information. Our next round of appointments is this upcoming week in Salt Lake, so stay tuned.
