This is a special blog post. I have been trying to process it for days. This one is a doozy and will be long, so sit down and find a comfortable spot.
Those of you who have followed our story from the beginning, or have read our blog from the start, know now that Chris and I don't do things the easy way. This pregnancy has been suspiciously easy. Until now.
As I said in last week'a entry, last Monday I was scheduled for a follow-up ultrasound because baby girl was hiding something they wanted to see. So off I went, thinking everything would be routine. Chris didn't come as he had a lot to do at work, and didn't think it would matter. I had a nice lunch with a friend before the appointment then headed over to Mt Auburn. The tech spent a good 40 minutes looking, but couldn't really get baby girl into a good position. In came the Dr. This time I saw Dr Ralston, who is the director of Maternal and Fetal Medicine at Beth Israel in Boston. He comes to Mt Auburn on Mondays as the two hospitals are both Harvard affiliated. This was the first time I had met him, but liked him instantly. I felt at ease. He spent another 20 minutes trying to find what he needed, but baby girl still wouldn't comply. He then stopped and said to me that he wanted me to get a more advanced test, an echocardiogram. And he wanted me to go to Children's. To see some cardiologists. At Children's. He explained that, even though they can't get baby in a good position, if they don't see what they need to see after two tries it is often because it is missing. He suspected baby was missing a heart valve.
He said he was going to get me in later that week. That he had connections and he wanted to know ASAP what was going on.
Through our medical adventures, Chris and I have come to know most of the major hospitals in Boston. I had a breast reduction at Newton-Wellesley, he met with a doctor at MGH for his Barrett's esophagus, we've spent a ton of time at Beth Israel getting the Barrett's taken care of, I've been to New England Baptist multiple times for various running injuries, and then most of our regular doctors are at Mt Auburn. This does not include the infinite amount of time we've spent at RSC (not a hospital) being treated for infertility. The only major hospitals left are Tufts / Boston Medical Center, Brigham and Women's, Dana Farber, and Children's. While it's not ideal to know every hospital in Boston, of those four, there are two I hoped to never see the inside of - Dana Farber (for obvious reasons) and Children's, because that means your child is very sick. So when Dr Ralston said we needed to go to Children's, it took everything I had not to flip out.
I held it together pretty good and asked a few questions. Most importantly, what he thought the prognosis might be. He said probably very good, but it would be complicated and probably involve surgery. He said there may be an underlying chromosomal disorder, and though we have pretty much ruled out Down's syndrome, there are others. He mentioned possibly doing an amnio, something I haven't been keen on since the beginning as there is an increased risk for miscarriage associated with amniocentesis. He told me it wasn't mandatory, but if I had any desire to terminate the pregnancy and not deal with these issues, an amnio would be the way we'd need to go. I told him termination was not an option and explained the brief story of how we got here. I asked him if I should limit myself in any way until we got the full diagnosis, and he said he didn't think that was necessary and I should continue doing what I'm doing.
I kept it together until I walked out if the hospital. I can not tell you the number of times I have walked out of a doctors appointment crying, so this felt a little more natural than walking out with a smile on my face. This was worse though. The uncertainty for my unborn baby's health, possibly even her life was clear. I called Chris but he didn't answer, so I texted and asked him to call now. I completely lost it and was sobbing so hard I was incomprehensible. He tried to reassure me and told me we'd just have to wait and see. Once again, we were on the wait and see train. We have spent the last 4 years waiting and seeing, so this is not a new feeling, but it still sucks.
Tuesday I had a bunch of things I needed to get done, but found it hard to concentrate. Wednesday I was scheduled to see my midwife for my monthly appointment. In the time in between the two appointments, I tried very hard to limit my internet exposure as I had little information and didn't want to self-diagnose. I was looking forward to this appointment with my midwife for some reassurance and direction.
After leaving my appointment with my midwife, I felt a lot better. She gave me a hug when she came in and asked me to explain what I thought was going on (even though I knew Dr Ralston had been in touch). I explained and she told me that her son was a heart baby. He had a defective valve and had it fixed when he was in his 20s. She raved about Children's. She also told me about a friend's daughter who suffered from a condition called Tetrology of Fallot (4 different heart defects) and how she was 20 something and had just graduated college. Both kids have lived an active healthy life. We discussed what would happen with my care. She said I could continue to see her or I could transfer immediately over to Dr Ralston and either way they'd make it seamless. While these discussions were about seemingly little things, they gave me some answers that I could carry with me to my appointment the next day at Children's. Then she examined me - the good news is I'm healthy (more about that in my 22 week update coming soon).
The next morning, we got up early to get to Children's in time for our 8 appt. I didn't sleep well. Between the heat and humidity and overthinking everything, I had gone to bed with a massive headache. It was still there in the morning, but wasn't as massive. We got there right on time, filled out the paperwork and at 8:00 sharp, a woman called out our name and introduced herself as, Chris, the cardiac fellow. So we skipped right to a doctor, no nurses or techs examining us here, which made it seem a little scarier to me, as in whoa, this is really serious. She led us into the exam room and had me sit on the bed to get ready for the echocardiogram, which is really just a high tech ultrasound that allows them to see the function of the heart in a much more detailed way. She was great. She made little jokes that made this much easier and told us much of what she was seeing. She spent about 40 minutes. Then she went to get Dr Friedman, the doctor assigned to our case. It took them about 10 minutes to come back, in which time, I'm fairly certain they were discussing what she found. When they came back, he introduced himself as "our" cardiologist. This was both scary and reassuring. He spent another 20 minutes or so and they continued to discuss. It was pretty clear at this point that Dr Ralston had been right and something was wrong. They told us it was complicated and gave us a quick break so I could get the gel off my belly and take a bathroom break and that we'd reconvene in the conference room.
In the conference room we learned exactly how complicated it was. Many of these terms were scary to hear, but I'm sure in the coming months they will become part of our normal vocabulary. In many ways they already have. Dr Freidman began by explaining the normal function of the heart. Chris, the cardiac fellow, took notes for us. He then started to explain what they thought was wrong, however he said it was so complicated that they weren't even 100% sure this was the correct diagnosis. To be brief, we learned that baby girl has a hole between her ventricles and only one artery instead of two coming out of the top. They originally thought it was the pulmonary artery coming out of the top and that she had aortic atresia (the aorta was underdeveloped). This is a rare defect and was going to involve a very complicated surgery a week after birth. It is a surgery that us not done very frequently and a condition that foes not have a name. She would be put on prostaglandins immediately after she was born to keep a valve that normally closes up open so she could function. They said she'd likely be in the hospital for 2 months, but if she got through surgery, the prognosis was great.
They also explained that I would need to deliver either at Beth Israel or Brigham and Women's, so that she could be brought immediately over to children's. Brigham is slightly better, only because it is connected by a bridge, however my referring doctor is a BI doctor so they suggested I deliver with him at BI. This makes me sad because I love my midwife and I love Mt Auburn. I was looking forward to a very natural birth at Mt Auburn. BI was not in the cards. It's amazing how things change in the span of a couple hours. They did say that even though I was going to transfer to a high risk doctor, that my pregnancy was in no way high risk. They all agreed that we should still go on vacation and that I should still continue to exercise. Nothing would change until she was born. After the doctors left, the nurse sat with us a bit and chatted with us to make sure there were no questions. She tried to reassure us as best she could.
Two and half hours later, we finally walked out of children's. We left there now knowing that things were very different from how we had planned and that the smooth sailing was about to end. By November, the seas are going to get very rough.
I spent most of the rest of the day trying to digest and process the news. A friend and I had decided to go into Boston for the rescheduled 4th of July concert and fireworks, so this was a good distraction. Around 3:30, my phone rang. It was Dr Friedman. I saw the number and guessed that was who it was. Having a doctor I just saw call me scared me a little bit. As it turned out, it was somewhat good news. They had spent the day reviewing our case and had decided it was not aortic atresia, but instead it was pulmonary atresia. This means that the one big artery coming out of the heart was the aorta instead of the pulmonary artery. This was a much more common defect, a condition known as Tetrology of Fallot. The same condition that my midwife had just told me about where the patient was in her 20s, healthy and just out of college. He told me that because the aorta was there, the surgery would be easier and baby would only be in the hospital about a month. This means she'll probably be home for Christmas!! A definite bright spot when I realized that.
Tetrology of Fallot has 4 characteristic defects - one big artery (the aorta) coming out of the heart, a hole between the ventricles (ventricular septic defect -VSD), a smaller, underdeveloped artery also coming out of the top (pulmonary stenosis or atresia), and a build up of muscle tissue in the right ventricle over time (right ventricular hypertrophy), which is not present in our case because we caught it early and the heart is not serving its usual function yet. So what does the surgery entail? They will have to plug the VSD. They will connect the existing, underdeveloped PA to the aorta, and they will insert a conduit (tube) to serve the function of the pulmonary artery. In the case of the original diagnosis, they would have had to make the pulmonary artery serve as the aorta and replace it with a conduit. Either way it is major open heart surgery and, of course, is risky. She will need lifelong cardiac care, but should be able to live a long, healthy, happy, and active life. She will require more surgeries as she grows older to replace the conduit as she grows, but none will be as extensive as the first.
How do I feel? A little numb. A little relieved that we know what it is and that I'm no longer waiting for bad news. At first, I felt very overwhelmed, but it's becoming a little less overwhelming now. Knowing she will only be in the hospital for a month is much better than two months. But most of all, I feel blessed that we live in Boston and have access to the best pediatric cardiac unit in the country.
The care that we have already received at Children's is second to none. The doctors are caring and not overly clinical, especially given they are the best of the best. Not only did the cardiologist call to tell me the change in diagnosis, but I was also given his and his fellow's email address, as well as the emails for both nurses on our case. My new OB, Dr Ralston, called to check in Thursday evening, the night before the 4th of July holiday. He left me his cell phone number and told me I could reach him anytime over the weekend. I finally called him back yesterday. He was reassuring and told me the same thing my midwife told me - that we would make the transition seamless. He suggested doing it sooner rather than later, just so I could get accustomed to his practice, which is the way I was leaning. I told him I would go to my August appt with Phyllis to wrap things up, and start coming to him in September. He thought this was a great plan. He, along with Phyllis and the cardiologist, once again emphasized that my pregnancy was NOT high risk and that baby girl will develop normally until she is born. Her heart is serving it's proper purpose in-utero and I am doing most if the work. All three emphasized that the only reason I would deliver at BI and not Mt Auburn is the fact that I need to be close to Children's for her. Dr Ralston told me there was no reason why I (or she) could not endure natural labor and that I could have as high or as low tech a delivery as I wanted. He told me that even though he was a high risk ob, he was a midwife at heart and he'd make sure I have the delivery I want to have. Aside from baby's health implications, missing out on that natural experience was one of the things I have been struggling with the most, so that was a relief. So really, the only difference in our baby's birth will be that instead of being born in Cambridge she will be born in Boston and instead of an uneventful, quiet birth, she will be met by a team of waiting cardiologists, anxious for her arrival.
I am happy that Dr Ralston (ranked as one of Boston's best Maternal and Fetal Med doctors) caught this early and that we can be be prepared. So many people that have babies with this condition don't find out until their baby is born or much later. In one short week, we have been able to assemble a team that will be prepared and waiting for her arrival, so she will receive the best care possible.
I have gone from beside myself, to in denial, to now, as I sit here feeling her kick me, at peace. Que sera sera. Whatever will be will be. I still feel confident that she will be fine. We have the best care. I feel relaxed and ready to take on this next big challenge. Maybe this is why we had to endure all he struggles we have endured - to prepare us for this last major challenge. I thought we had earned a smooth pregnancy, but apparently this little heart warrior has chosen us as parents, and as special as she already is, this will just make her more special.
We will be sure to continue to update. I thought the roller coaster ride was over, but clearly it's just beginning! Like I said, nothing is ever easy....
I've been enjoying my big gift from my parents for the last two weeks - a new beach chair with cup holders. All in all, a good birthday, especially knowing that I have finally gotten what I wanted for the last 4 birthdays and Christmases - the beautiful baby growing inside of me. I really didn't need anything more - I feel totally blessed. 
