Sibley Heart & CHOA

#heterotaxy #avcanal #sibleyheart #childrenshealthcareofatlanta #raisetheruth

The last post that I made, I shared that we had finally received a diagnosis, and we learned something new about Ruth's heart condition. As we now know, this is pretty much going to be the theme of my doctor's visits; we learn something new each time, good or bad! This particular post will focus only on our visits with Sibley Heart Center - one in Albany, and another in Atlanta. I did have other appointments in between these two with Sibley, but for the sake of efficiency, I think it will be best to focus just on Ruth's heart for now.

On June 15, about a week after my doctors confirmed that Ruth has Heterotaxy Syndrome, we headed to Albany for yet another fetal echocardiogram. This time, however, we were going to a satellite office of The Sibley Heart Center, a pediatric cardiology group, to meet with a new doctor and a new time that came highly recommended from a sweet mom friend who also has a heart warrior kiddo.

Just to give you a quick rundown of Sibley Heart in case you ever need to meet with them, they have offices all over the state. Their mission is that parents should not have to drive halfway across Georgia for quality heart care. TECHNICALLY, their closest office to us is in Valdosta, but we cannot go to that office until Ruth is born because they do not offer fetal scans. Because they are, essentially, heart sonograms of a person within a person, fetal echocardiograms are performed with very different imaging equipment than newborn/child echocardiograms. Only Sibley's Albany, Macon, and Atlanta offices offer services to pregnant women. And luckily for us, they ONLY perform those fetal scans at 8:30 in the morning! Meaning, we had to leave the house by 6AM. Ruth is already preparing us for no sleep, so that's nice.

We got to the Albany location (found within a medical office building that shares walls with the hospital there) and were already more impressed with Sibley Heart than our previous cardiology visits to the "other" office in my previous posts. I'm not saying that they rolled out the red carpet or anything, but the fact that staff made immediate eye contact, acknowledged our presence, and weren't on their phones made a big impression. Our sonographer took us back to the imaging room, and for the life of me, I cannot remember her name. She was very kind though, and even shared a bit about her own life, her own heart differences, and her child's journey with a rare lung disorder. We found out that she is also a believer, so it was very nice to share that kinship in the Lord when you're under a great amount of stress. In addition to the obvious stress that comes with having a child with a heart difference and coming to a facility like Sibley, I was also stressed out because Heterotaxy impacts so many different areas of the body, but we were only there to look at Ruth's heart. You want to ask about lungs, liver, spleen, brain, etc., but you just can't, and it drives you crazy.

I've mentioned before that Ruth is very active all the time, but especially during scans. I was lying down, positioned so that I could not see anything on the screen, but Kane said that Ruth was leg pressing against the probe, and it was giving the sonographer a fit. The scan took about an hour of wrestling with Ruth, and then we were shown to a separate room where the doctor would come in and meet with us. This is also a cause for stress in these situations: the sonographer can't really tell you anything about your scan. The doctor has to go behind his curtain, read your scan, decide how to explain things to you, and then come meet you for the first time and then potentially flood you with new information. So we waited.

It's worth pointing out that Albany & Valdosta patients are usually seen by Dr. Benjamin Toole, but even doctors need to take vacations. As a result, we ended up meeting with Dr. Brian Cardis. He was very personable right out of the gate, and even shook our hands as he came in the room. This was the first time that a doctor shook hands with us post-Covid outbreak. Again, it's the little things that go a long way with us. Dr. Cardis wanted to know a little more about our discontent with Savannah, so I explained to him that I truly had no intention to ever meet with that cardiologist again. Dr. Cardis then began to talk about delivery in Atlanta, and I could feel my blood pressure begin to rise. Because Sibley's main offices and surgical centers are within Children's Healthcare of Atlanta, I could deliver at Northside Hospital, which is ten minutes down the road from CHOA, so that Ruth could immediately be seen by Sibley cardiologists. However, the catch here is that I would have to get a new OB and move my Maternal & Fetal Medicine visits to Atlanta.

There is absolutely no way that this would work for our lives, outside of it being an emergent medical necessity.

I'm sure my eyes were about to bug out of my head, because Dr. Cardis looked at me and said, "But..." I like that conjunction. Buts are good.

"But, this option is only if you want to deliver in Atlanta and only if you want Ruth to immediately see *us.*" Dr. Cardis continued and I'm pretty sure I stopped breathing. "Based on what I'm seeing today, I do not think Ruth will need emergency surgery within the first few moments of life, or even the first weeks of life."

PRAISE GOD FROM WHOM ALL BLESSINGS FLOW. This was such a big blessing - and I don't even remember everything that was running through my mind at the time. I think I was too focused on getting a plan and expectations into place to show my true gratitude to the Lord. Isn't that kind of a lesson in itself, though? We're too busy thinking about how we will collect our winnings to realize we just won the lottery.

Dr. Cardis then pulled out this drawing to explain to us what he was seeing on Ruth's echo from that morning. Going from the left side of the picture to the right, I'll go over what he said to us.

Ruth does have an interrupted inferior vena cava, but it has actually branched off to her superior vena cava through something called "azygous continuation." This means she may be asymptomatic (!!!!!) or she may have some vein issues in her lower body, but we won't know until she is born and even potentially for years. Either way, it's not something that he is concerned about or that needs fixing.

He explained to us that she actually has a Transitional AV Canal, meaning that there is a (very) large hole in her atrium, and a smaller hole between her ventricles. The problem with the top of her heart is that her septum that would normally divide that left and right atria is nonexistent; therefore, she has one common atrium. Typically, unoxygenated blue blood would pump into the right atrium, and oxygenated red blood would pump out of the left atrium. Essentially, Ruth's common atrium is mixing and sharing all of that blood into one chamber which will cause her oxygen saturation to be lower until it is fixed. Where normal kids have OSats in the 90s, we should be happy to get hers into the 80s. BUT, the fact that her ventricular septal defect is quite small in comparison is a very good thing - this is apparently what determined that she did not need emergency surgery.

The last thing that we learned from Dr. Cardis was that Ruth has a common AV valve. That thick black line separating the top and bottom portions of Ruth's heart is supposed to be two separated valves, but hers is just one. As best as I can explain it, the two valves should look like one valve with just a single line dividing it, and another that looks like a peace sign. Ruth's common valve looks almost like a camera shutter. This is something that will have to be surgically repaired as well, BUT it can be done in conjunction with her transitional AV canal surgery.

Dr. Cardis went on to explain that, although this was a scary situation for us and a lot of unknowns due to Ruth's Heterotaxy, he believed that surgery could take place when Ruth is about 3-6 months old. This is MUCH better than the original projection of weeks/months. Again, this is such an answered prayer.

Because of this news, I asked Dr. Cardis if delivery in Savannah was still an option. I really wanted to know if he and his wife received the same news, would he feel comfortable delivering in Savannah? He came back with a resounding YES. He did remind me that Ruth would have to see the "other" cardiologist for an echo after birth and probably a few more times during our stay in the NICU, to which I agreed. I did, however, make it very clear that I wanted Ruth to be a patient of Sibley, not anywhere else, and that we would be doing follow up appointments there upon hospital release.

Dr. Cardis did want us to have a follow-up appointment in Atlanta at Children's Healthcare to meet with other cardiologists and the main cardiothoracic surgeon over the program. The team has a 100% success rate with the type of surgery Ruth would need, which was comforting; as a parent, though, Dr. Cardis understood the importance of meeting with and getting the feel for people who would be performing the actual surgery on your child. We agreed with that 100%, and were thankful for such insightful care on behalf of a new patient.

After several days and balancing doctors' schedules and existing appointments, we finally got booked to visit Children's Healthcare and started putting everything into place to travel to Atlanta over July 1-2! I'm not sure if this is the case every single time you visit CHOA or if it was just our particular circumstance, but we received a phone call from a case manager pretty immediately. She asked us some questions to get our paperwork started, and because we live so far away from CHOA, we qualified to stay at the Ronald McDonald House for the length of our stay. She quickly got us in touch with them and we began the process to stay there. In order to stay at a RMH, you (and your spouse, or whomever will be staying with you) have to pass a background check first, so we needed to get the ball rolling on this process ASAP. Additionally, because some COVID protocols are still in place and are changing daily, there was a chance that we would not be able to stay in the house proper. RMH only has so many rooms available at any given time, and during COVID, that number was limited even further in order to spread people out. They did, however, have partnerships with several hotels around the Decatur area, so in the event that we couldn't get into the House, they would try to put us up in a local hotel. We wouldn't know where we were staying until the day before we left because RMH is typically a "first come, first serve" deal; this means that you don't call in advance and reserve a room for X number of days. The day before you need a room, they'll call you and tell you where you're going. For those of you who like to fly by the seat of your pants, this may be fine for you. I do NOT like to live like this, so I was slightly anxious, LOL. Additionally, mentally preparing just to go to Atlanta is a big deal for me. Some of you may roll your eyes at that statement, and that's fine. But to me, pretty much everything above Georgia's Fall Line might as well be Atlanta, with the exception of Athens. Also, it was a huge mental preparation for me to leave Alora to go that far away. These are the hard things that no one really thinks about when you're in this situation. The background difficulties, if you will.

We finally received a call that we would be staying in a hotel, courtesy of Ronald McDonald House, in Decatur, about 8 minutes away from CHOA. We got there, and y'all...it was nnnoooooooiiiiiiiiiccceeeeee. We were on the top level, corner room with windows everywhere. We could see the top of the Emory Nursing building! That may have come off more "country come to town" than I had hoped, but let me give you some perspective. I think the last time that we were in a hotel on any sort of "vacation" was in September of 2019 for my brother's wedding. So yes, the fact that I got to stay in an air conditioned room and not worry about bath time and bedtime and that I didn't have to clean up the next day?? Again, I say nnnoooooooiiiiiiiiiccceeeeee!

We woke up early and arrived at CHOA for our 7:45AM echo & meeting. First of all, the parking deck is below ground, and when you get into the building, they take your temperature on a tablet. A TABLET TAKES YOUR TEMPERATURE. Children's Healthcare is living in the year 3000, everyone.

We met our lovely nurse, Laura Lei Castillo (like the Gilmore Girls), who really guided us around and gave us the most practical information during our visit. She is also a certified snack supplier, so I really appreciated that when I got lightheaded during our scan. Ruth is positioned on my vena cava, so much so that when I lie flat for too long, I get really lightheaded and can't breathe. After our echo, we moved to a consultation office with a roundtable to meet with a cardiologist. We met with Dr. Rita Sachdeva who was very straightforward but explained things very well. I had my handy-dandy binder that I take with me to all of my appointments, a list of questions for her, and Dr. Cardis' drawing of Ruth's heart condition. She used this drawing to answer all of our questions and better explain Ruth's heart anatomy specific to Heterotaxy. Fantastic meeting.

Dr. Sachdeva left, and Dr. Paul Chai, the chief of Pediatric Cardiothoracic Surgery came in to speak with us. We talked about surgery and I got to ask all of my questions, like if the 3-6 month timeline was still relevant (it is), and what type of surgery we should be expecting. Because of the size of the hole in Ruth's heart, it will be an open heart surgery. It can't be done via heart catheter, so her sternum will have to be opened up. His plan is to take some existing cortex from another area of her body and patch her ventricular septal defect, and then take some of her pericardium (the tissue around her heart) to create a septum for her common atrium. He will then have to repair that AV valve that I mentioned earlier. The whole thing should take about three hours, and at least one, maybe two weeks in the hospital for recovery. After all of this, Dr. Chai still felt very confident that this should be a one and done surgery. Of course there is always the potential for something else to happen, but this one surgery should catch all of Ruth's main heart problems. Again, fantastic meeting.

Dr. Chai left and Laura Lei came back to speak with us. Wouldn't you know it, she gave us a BINDER with all of the information I could ever want. It's got pictures of providers, business cards, tabs, info on what to do after delivery, and information on what to expect from their ICU care.

So we left, and finally made it back home in time for Alora's bath & bed. We were all glad to be back together and our own home. It was a long drive home, but it wasn't until we got back to our house that I felt that I could begin to process everything we learned in the last 12 hours. It takes me a while to process big information - which is partially why all of these blog posts come out about two weeks after I experience them. I felt very much like..."Well, what now?" We are very much in a waiting game now. We know a lot, and probably as much now as we're going to learn, but there is still so much that we can't know until after Ruth is born.

So we wait, but I don't like the wait. I like to hear from doctors daily to update me on tests, situations, specialists I need to see, and things I need to do. But we wait anyway. And as we wait, I'm reminded of one of my favorite chapters in the Bible, Isaiah 43. In verse 1, God says, "Do not fear; for I have redeemed you; I have called you by your name; you are mine." While I'm fearful or worried about all of this temporary stuff and the day-to-day, God knows my name and has taken care of my biggest need: paying the debt of my sin through the blood of Jesus. He has redeemed me, so I can trust Him when He says, "I will be with you when you pass through the waters, and when you pass through the rivers, they will not overwhelm you. You will not be scorched when you walk through the fire, and the flame will not burn you" (vs. 2). God specifically says when these things happen to you, not if, that He is there with you and has not abandoned you. In verse 11, He says very plainly to us, "I - I am the Lord. Besides me, there is no Savior." And even now as I type, I feel the Holy Spirit reminding me that God is my Savior, not these doctors. These doctors are not with me day in and day out, they do not know the future, and they do not promise to hold my hand as I go through various trials; God does. So if you're perfectly healthy, how does this apply to you? God is your Savior, not your best friends. God is your Savior, not social media. God is your Savior, not your job or social status in which you put all of your time, energy, and mental energy. I'll go even further and say that God is your Savior, not your spouse.

So we wait. Thankful, SO thankful that our appointments went very well and that I don't have to turn over my newborn for surgery while I'm still recovering from delivery! We know that the Lord is providing and is answering prayers in this process, every step of the way. He is good, He is our Savior, and He is trustworthy!