November 17th finally arrived

We were finally to the day we had waited for 2 weeks. I was having so many mixed feelings, on one hand I was so excited in hopes that it had all changed & everything was going to be amazing from here on out & on the other hand with what if its the same...or worse? Then what? Where would we go from there & can I handle it?

So my Mom came over to watch my girls & we headed up to the hospital. We got all registered & checked in, which took a while. Then sat down in the waiting area for our turn. My stomach was turning in knots, I didn't know what to think. I just wanted it all to be over & go home knowing exactly what was going on (hopefully as positive of course).

The Ultrasound tech took us back in the room & got started. I tried so hard to really look at what she was looking at. But at that time not much of it made sense. She said "Well it looks like the heart is on the right side of the chest"......& my heart sank! All these questions started rushing through my head, Does his mean that everything with his heart was the same as before? Why would it be on the right side? What causes that? What does this mean? Is just that it is on the right side but its a normal healthy heart??? I knew these were all things that she couldn't answer so I didn't ask I just watched & waited while I tried to fight back the tears. About 15 minutes later she said that she was going to show these pictures to her doctor & she would be back. So Jason & I sat in the room & waited.... Jason said "when she was zooming in on the heart it looked to me like there were 3 chambers, so that gives me some hope" Him & I talked back and forth about what we thought but obviously not knowing a thing. Both of us in fear that we would be going home still not knowing anything until our appointment in the morning. Then the tech came back in, this time with the doctor. The doctor said "It looks like your baby has a very rare condition called Heterotaxy, which means some of his organs are mirror images of themselves or mirror images of where they are supposed to be. Both his stomach & heart are on the right side of his body when they are supposed to be on the left. Other organs that are usually effected by Heterotaxy are the lungs, kidneys & spleen. As far as we can tell they are all okay but at this point in pregnancy it is hard to really say because everything is so small, some things we just wont know for sure until he is born." We said so other than his heart being on the right side is it okay? & she said "You will have to get further evaluation on his heart over at Primary Children's, they are the only facility that has the equipment to do a fetal echo cardiogram & that is what you will need done to know the whole extent of what is going on with his heart. From there you will meet with surgeons to go over what all will happen when he is born. We will get you an appointment there tomorrow before you meet with Dr. Manuck."

I was screaming inside!!!!! I had so many things rushing through my head. I just wished I could wake up from this horrible nightmare! The words she said kept going through my head over & over..."from there you will meet with surgeons" what does this mean? My baby is going to have to have heart surgery? How serious is it? Is my baby going to survive? I was trying not to make myself crazy but how could I not? I just wanted the tech & the doctor to leave so I could lay on the bed & just cry. Jason came over & helped me up & we walked out together just clenching each others hands as we walked. It was exactly what we had feared & maybe worse. I was shaking & I couldn't say a word knowing that if I opened my mouth, all that would come out is cries. I just wanted to go home & be left alone. The drive home was hard, neither of us could say a word, we both were so shocked & with still not knowing everything that we were up against we were numb & terrified of what more we would find out the next day.

We got home & of course I got right on the internet & started searching Heterotaxy. I was surprised at how much I found. It said that it is more common in boys than girls & that it is not something that comes from genetics, it happens randomly to 4 in every 1 million babies & that it usually comes with some sort (or many sorts) of heart defects. I found so many stories of families that had gone through the same thing, some were so comforting and others I just cried the entire time I read it. After a while I knew I needed to get off the computer or I would drive myself crazy with all the what if's & questions. At that point I started getting lots of phone calls from my close friends & family that I know care so much & wanted to know how it went, but I knew I couldn't do it, I couldn't talk to anyone it would make it way harder than it already was.

The next morning we headed up to Primary Children's Hospital. Just walking through that door was such a surreal feeling! It's the type of place that you have to be SO grateful for but at the same time pray that you never have to go inside. It was amazing seeing all the sweet babies & children going though so much & still having smiles on there faces & positive attitudes, it gave me some hope. We sat in the waiting area & a woman walked in with her arms so full, she was pushing her stroller with one hand, & a baby in the other. The baby had a breathing tube in her nose & an oxygen tank strapped to the stroller. She came & sat down by us & my heart just ached for her! She seemed so exhausted & doing it all on her own, I just couldn't even imagine! It made me so incredibly grateful for my husband, knowing that he was by side no matter what & I would never have to be alone in any of this. We were watching this beautiful baby girl, she was probably 6-9 months old & she was so adorable, the Mom was talking to her & she was talking back. Jason said to me "She reminds me so much of Addi" & she did, she looked so much like our sweet little girl so of course that made it even harder to see how much she was going through. Just before we got called to our appointment the Mom went to adjust the baby girl's shirt & I saw the staples that closed up a huge incision down this sweet baby's chest obviously from a heart surgery. Tears just started rushing down my cheeks uncontrollably, knowing that not only our baby would probably need the same thing but seeing a little girl that was just like our little Addi with that type of an incision on her chest just really gave me a good reality check as to what we may be in for.

The nurse took us back into the Echo room & got started. She was very quiet & just got right to work. She told us that we would be able to meet with the Cardiologist after she was done with us so that we would get all of our questions answered. After about 40 minutes she went & got Dr. Menon (the Cardiologist) to come take a look. He came in and they spoke medical gibberish back & forth for a while, nothing that made any sense to either of us & then after about an hour they said that they would meet us in the conference room.

We walked into the conference room & there is a whole team of people & nurses there waiting for us. We sat down and the cardiologist sat down right in front of us. He took a deep breath & said "Well, to start... your baby has a very rare condition called Heterotaxy that usually comes with some pretty serious heart defects, for starters lets me show you what a normal heart looks like." So he showed us a normal heart & how it is supposed to function then he started to pull out numerous charts, he put all the charts in front of us & started pointing out things on these charts of everything that was wrong our baby's heart. The main things that he pointed out was that our baby's left ventricle was extremely small almost non existent this is called Hypoplastic Left Heart Syndrome a very serious heart defect & that his aortic arch was under developed but that they couldn't tell just yet how under developed it actually was. He said "Your baby's quality of life may not be very good with this heart condition & the only way he would survive at all would be with a series of 3 heart surgeries. The first at 1-7 days old, the second at 4-6 months old & the third at 3-4 years old, after these heart surgeries we still cannot say how well off he would be & weather or not his heart could carry him into adulthood or even teenage years." I asked him what the chances of survival are? he said "With just the heart condition that he is dealing with I would say maybe 70% chance of survival, but then you throw the Heterotaxy in the mix & that would drop it down to maybe 60%" I didn't know what to say! Jason & I just sat on the couch listening to all of this clenching each others hand just praying that the bad news would stop! But it didn't. I wanted to ask so much but I was terrified of the answer he would give! He then said "I'm sorry to put this all on you both but a decision on what is going to be done needs to be made soon." So he told us we have 3 options: 1-to terminate the pregnancy (which he highly recommends) 2-to proceed with the pregnancy with no surgeries (0% of survival) or 3-to continue with the pregnancy, set up the surgeries and prepare ourselves for a long hard road. Automatically both Jason & I said "We want to continue with the pregnancy & prepare for the surgeries!" I was so proud of both of us & the relationship that we have together that we didn't have to stop & think about it or even look at each other before we both made the exact same decision! There was no doubt in either of our minds that terminating the pregnancy was not an option.

From there he said "As much as I like your decision I have to let you know that it is going to be an extremely hard one because as much as I am sure you hope it will change this WILL NOT CHANGE! The human heart is developed in the early embryo stages of pregnancy & whats done is done. I know a lot of people think that prayer or positive thoughts are going to change it all, but from a medical stand point its just not possible! I'm sorry to tell you that but it is just the facts! So if you still chose to proceed with the pregnancy just be aware that it will be the hardest thing you will ever experience & you will need a ton of help from friends & family!" Then he asked "Do you have other children?" & we told him we have to little girls together & Jason's two boys every other week, so exactly 50% of the time. He said "Are you close to your families?" & Thank goodness we could tell him yes we are extremely close to both of our families! He said "Good, because you will need them now more than ever, especially for your older children needs because I can promise you, you both will want to be up here with your baby as much as possible." He also asked about both our jobs & again we were grateful to say that neither of us have high demanding jobs that we have to be there specific hours. He told me that the chances of me being able to work with a baby in these circumstances are unheard of so to not plan on it.

After being in there for quite some time & speaking with both Dr. Menon & the team of nurses we found out so much overwhelming information. There are so many variations of our baby's condition that they were unable to give us an average lifespan for our baby, but they did say that a lot of it depends on weather or not he has a spleen (spleens are too small to be able to tell until he is born if he has one or not). Sometimes with this condition the baby is born with either no spleen or multiple tiny spleens that are in most cases, pretty much useless. Without a spleen he would have no immune system, therefore he would have to take an antibiotic everyday for the rest of his life. Also, a spleen makes a huge difference in how he heals from these surgeries & factors in a lot of other things down the road like weather or not he would be eligible for a heart transplant because he said they cannot do any transplants without a spleen. Another big factor in this condition is the fact that his stomach is on the wrong side of his body, this can make the intestines twist around themselves making it so he would have to have stomach surgery soon after birth to fix it. He said there are many people out there that live completely normal lives with Heterotaxy & some that don't even know that they have it. It just depends so much on how the organs are all placed & weather or not everything is there.

Before they left the room he gave us a list of all the things that our baby was dealing with, the list was so overwhelming & absolutely heart breaking! It had at least 10 major things that they either knew for sure were wrong or that they were still questioning weather or not it is wrong. I sat there holding the list as he asked if I had any other questions....what could I say? Of course I had other questions, I had a million! I just wanted to know everything now & what the end result of it all would be. The head nurse coordinator handed us a box of tissues & said I'm so sorry that we didn't have better news for you both, we will leave you alone, feel free to stay in this room as long as you need.

As soon as she shut the door I laid in Jason's arms & lost it completely! I felt like I was gasping for breath....How could this be happening to us? This is the sort of thing that you hear about but not ever have to experience in your own family, let alone your very own baby...I had so many mixed emotions I couldn't do anything but cry, but I knew we needed to get over to the U for our appointment with them that we were already late for.

So from there we were sent back to the U where I was told we had to find a new OB because we would have to deliver at the U where they specialize in high risk pregnancies. Thankfully we found one named Tracey Manuck that was so helpful, sweet & very honest with the facts & what we have to prepare ourselves for. She went over everything with us that she could at that time. We will be delivering at the U of U hospital where we will be in a "High Risk" delivery room that is linked to the NICU by a window, so as soon as he is born they will send him straight through the window to the NICU to have an IV through his umbilical cord to provide his nutrients & to keep his PDA vessel open, then he will have numerous tests done to find out all the ins and outs of his condition. From there it all depends on how everything looks as far as how long he will stay in the hospital & what surgeries will need to be lined up for him. There were three huge things said in this conversation that really stood out to me- One was that our older children would not be able to see him (besides in pictures or video) until he is sent home from the hospital which could potentially be around 3 months old. All I could think was "How am I going to tell my sweet Braylee that she has to wait for an extra 3 months to see her baby brother that she has anticipated for long before we even got pregnant with him? She is going to be devastated!" 2- That the chances of me being able to hold him would be slim to none, that in most cases the baby comes out, the doctor holds up the baby so the Mom & Dad can see him & then sends him straight through the window to the NICU & that I would not be able to nurse him until he came home (again around 3 months old) & 3- That we need to mentally prepare ourselves for the possibility of having a still born, that the chances are pretty high of him having heart failure before the labor is over or in some cases before the pregnancy is even close to over. She said I hate to tell you these things but they are things that you as the parents have to be aware of so that if it does happen you are as prepared as you can be.

The last thing she had talked about was that there is a test to be able to tell weather or not our baby's heart condition is from genetics or if it was just random, she said it is an amniocentesis test where they put a needle into my stomach to pull out some amniotic fluid so it can be tested. I asked her if there were any risks with having this test done & she said "well yes there are risks such as the needle breaking the sack which would force you into pre-term labor, but the chances of that are very low" Jason said "how low" & she said "about a 1 in 400 chance of having that happen" Jason got a little defensive & said "Well the condition our baby is dealing with right now has a 4 in 1 million odds so I think the odds are against us right now & we are just not willing to take that risk!" I was so happy that he spoke up on that one, because it was so true & he worded it just perfectly, obviously the odds are against us right now so we will try our best to take every precaution possible!

By the time we were home I think both of us were so mentally & emotionally drained & exhausted. My Mom stayed at my house after we got there for some time so I could have some time to myself, she was really sweet to not ask too many questions knowing that it was just to much to handle at that time. Once again my heart ached for my sweet little girls, they just didn't understand why Mommy was crying so much, yet they were both so sweet, loving, & understanding through it all. As much as I wanted to sit them down & tell them what was going on, I wanted to tell all four of our kids at the same time & even then how do we start & how do we explain it all to children??

The next morning I was up really early looking through my phone & just feeling horrible that I had ignored so many calls & texts from my close friends & family. I knew that they were all so worried & wanting to know what was going on & to let us know that they cared. I really wanted to let them know what was going on but how? I knew that if I even picked up the phone & thought about the words I needed to say no words would come out, only tears. It was just to hard to talk about so I decided to start an email. Needless to say it was the hardest email I had ever written, it took so long because I had to walk away from it at least 5 times, but once it was done it was so worth it knowing that everyone that cared about us knew what was going on & they could let us know their thoughts & feelings through email so that there were no words necessary.

So from here the U of U hospital has set us up on every two week appointments so that they can monitor him closely. They said as soon as I hit 30 weeks along I will have multiple appointments each week. Then at Primary Children's they set us up an appointment for December 19th to get another fetal echo cardiogram & consultation with the cardiologist so our baby can grow bigger & they can see everything with his heart a little better. Hopefully we will know more then.