At this point, Brian and I have come to appreciate the unsurprising, and that was pretty much what we were given during our appointment at Boston Children’s Hospital (BCH). The pediatric cardiologist (PC) at BCH was happy with the images they were able to capture during Theo’s echocardiogram, and we were provided with essentially the same results that we had received at Strong Memorial Hospital in Rochester a week prior. We did, however, discuss a couple of treatment options that would either be preferable or only available to do out of BCH. The challenge is that we will not know which treatments will be best for Theo until he is born and continues to grow.

The following is a brief description of the treatment options that we discussed with the PC while at BCH:

1. Patent Ductus Arteriosis (PDA) Stent:  If Theo is in good enough condition following birth, he could be given a PDA stent instead of a BT shunt to keep the ductus arteriosis from closing. The PDA stent would be preferred because it is less invasive and would not require an open heart surgery.  The less time Theo spends in surgery the better, since less recovery time would mean less developmental delay.
2. Biventricular Repair: The typical treatment of single ventricle defect includes a series of surgeries that ultimately results in blood flow to the lungs with only one functioning ventricle. As an alternative, successful biventricular repair would restore ‘two-ventricle’ circulation.  Because Theo technically has two ventricles (albeit two ventricles that aren’t separated by a wall), he may be eligible for this treatment option.  The biventricular repair would put an artificial wall between Theo’s right and left ventricle so his heart could function more like a normal, two-ventricle heart.  However, because the right and left ventricles are inverted, it makes the biventircular repair more complicated than normal.  Again, time will tell whether this will be a viable option.

In addition to hearing about some of the treatment options available at BCH, we also learned more about what our lives would look like if we pursued treatment there.  We would need to be in Boston around Week 37 in case Theo makes an early debut.  Once born, we will be able to hold him for a few minutes and then he will receive medication through an IV to get him ready for his first procedure, which will take place about a week later.  Fortunately, breastfeeding should be an option and Theo would be able to visit with family and friends before undergoing his first procedure.

The first procedure will be either the BT shunt or the less invasive PDA stent.  If Theo isn’t able to receive the PDA stent and thus undergoes a surgical procedure to put in place the BT shunt, he would need to be in the hospital recovering for 4-6 weeks.  If he is able to receive the PDA stent, the recovery time would be cut in half.  We are fortunate enough to have great friends and family in and around Boston for support during this time, as well as housing made available through BCH and some very dear friends nearby. We don’t have all the details down yet, but we are lucky to have options that make receiving treatment in Boston a workable possibility.

We also spoke with the PC about what home care will look like once we are in a position to return to Rochester.  The PC reassured us that our home would not need to be a hospital.  There will, however, be some at-home monitoring that we’ll need to do twice a day.  Additionally, a nurse from Strong will visit briefly twice a week for normal check-ups.  Although Theo’s treatment will be an ongoing process, it is not as daunting as we originally anticipated, and we are feeling as capable and optimistic about it as we ever have.

Over the next few weeks, we will be weighing our options and eventually making a decision about whether to deliver and receive treatment at Strong or BCH.  We’ll keep you posted.  Otherwise, hopefully the next few months are uneventful as Theo continues to develop in the womb.  In the meantime, we’re feeling his kicks and sending him lots of love.

Our appointment with the high risk obstetrician (HROB) was initially scheduled for tomorrow, but after deciding that we would look into a second opinion from Boston Children’s Hospital later this week, we moved the HROB appointment up to today so that it was on the same day as our appointment with the pediatric cardiologist (PC). After all was said and done, we spent around four and a half hours with our doctors today. There was a lot of information to process, needless to say.

Teddy remains a giant pain in the you guessed it during his scans. He’s incredibly “active”, the doctors have said. We say hopefully this isn’t a preview of what he’s like as a baby. But from the images the HROB and PC were able to obtain, we were given some relatively good news.

First, the HROB told us that there wasn’t anything alarming about Teddy’s brain, kidneys or anatomy other than his heart. This was reassuring to hear since there were signs at our last HROB appointment that there may be issues with a part of Teddy’s brain and his kidneys. Although there is still a chance that abnormalities develop over time, at least for now we are in the clear.

Second, the PC gave us some reassuring news about the condition of Teddy’s heart. Since there’s a lot here, it’s easier to lay it out in a list. Full disclosure, I am a lawyer and not a doctor. Some of this may be completely wrong, but it is the best I can recall, Your Honor:

• Ventricular inversion and large ventricular septum defect. We were unsure if Teddy’s heart was missing a second ventricle entirely or if the second ventricle was just too small to see without an echocardiogram. After the echocardiogram today, it turns out that Teddy has both a right and a left ventricle but that they are inverted and not separated by a ventricular septum. We learned that this means his right ventricle is actually on the left side of his heart, and his left ventricle is on the right side of his heart. Since the two ventricles are not separated by a wall (the ventricular septum), his heart is essentially functioning like a heart with a single ventricle. Although Teddy’s heart still technically has a single ventricle defect, the outcome tends to be more positive when a child has two ventricles that are connected than when a child is missing a ventricle entirely.

• Moderately hypoplastic right ventricle. The right ventricle, located on the left side of Teddy’s heart, is moderately hypoplastic. This means it is moderately underdeveloped. That it is underdeveloped is not ideal, but that the underdevelopment is only moderate is a relatively positive finding.

• Pulmonary atresia. The valve that is responsible for allowing blood to flow out of Teddy’s heart and into his lungs has not formed properly. According to the Mayo Clinic, “Instead of opening and closing to allow blood to travel from the heart to the lungs, a solid sheet of tissue forms. So blood can’t travel by its normal route to pick up oxygen from the lungs. Instead, some blood travels to the lungs through other natural passages within the heart and its arteries.” In Teddy’s case, blood is traveling through his ductus arteriosis to the pulmonary artery and then to his lungs. This is OK while in utero, but the first of Teddy’s post-birth surgeries would be to use intravenous medication and a Blalock-Taussig shunt to keep the ductus arteriosis from closing.

• Healthy aortic arch. In some cases of single ventricle defect, the main blood vessel that transports blood from the heart to the rest of the body (the aorta) is underdeveloped where it bends (the aortic arch). In Teddy’s case, the aortic arch has fully developed and appears to be functioning properly. This is important because the first post-birth surgery for babies with single ventricle defect typically includes an aortic arch reconstruction, which would not be necessary for Teddy. That also makes the prognosis a bit more favorable.

• Left outlet transposition. The two blood vessels that carry blood away from the heart — the pulmonary artery and the aorta — are both connected to Teddy’s right ventricle on the left side of his heart. I’m not sure what that means, but apparently it’s weird.

While we were able to learn more about Teddy’s heart today, the structure of his heart does not fall into one of the “standard” types of single ventricle defects. This means that there isn’t a standard treatment protocol, but we are optimistic that with our doctors we will put the proper treatment plan in place. Until we learn more, we plan to get a second opinion in Boston and go from there. Fingers, toes and ventricles are crossed…

I was surprisingly calm when we heard at our 16-week ultrasound that Theo had a single ventricle defect. I felt like I had cried out all of my tears during the four weeks prior. Instead of feeling crushed or blindsided, Brian and I felt like we had finally received a bit of clarity.

We made a decision as we left that appointment – a difficult one but a decision that I think will help us tremendously through the balance of our pregnancy. We chose to look at the information we learned as a blessing, and we chose to express acceptance and gratitude instead of anger and resentment. Although we still had more information to learn from the cardiologists, we were one step closer to a full diagnosis and one step closer to a plan. We were not yet out of the woods but the uncertainty was largely behind us.

The longer I live, the more I realize the impact of attitude on life. Attitude, to me, is more important than facts. It is more important than the past, than education, than money, than circumstances, than failures, than success, than what other people think or say or do. It is more important than appearance, giftedness, or skill.

It will make or break a company, a church, or a home. The remarkable thing is that we have a choice every day regarding the attitude that we will embrace for that day.

We cannot change the past, we cannot change the fact that people will react in a certain way. We cannot change the inevitable. The only thing we can do is plan on the one thing we have, and that is our attitude.

— Charles Swindoll

Brian and I agreed that we would learn as much as we could about single ventricle defect and connect with as many families as we could who have gone through similar experiences. Opening up was refreshing. We learned that essentially everyone knows someone who has experienced a complicated pregnancy in one way or another, and we’ve come to realize that many know parents who have a child with a congenital heart defect (CHD). We came to feel less alone, and having a child with a single ventricle defect became less frightening and insurmountable.

I joined several Facebook groups during my first trimester – “First-Time Pregnancy” and “Mothers Due April 2016”. Once we learned that Theo was dealing with a heart defect, there was of course a shift in our focus. I’m now a member of seemingly every possible CHD group on Facebook, including the Rochester chapter of “Mended Little Hearts”. The conversations went from, “How is your morning sickness?” to “How many open heart surgeries has your child had so far?” It’s not where I thought I would be, but I continue to remind myself that this is our path – this is the pregnancy we have been given and there is no doubt in my mind that we are up to the task. There is a larger plan and all we can do is ask questions, learn, connect and be incredibly grateful that we still have our baby boy.

I’ve had several long conversations with mothers of thriving children with similar heart defects. None of them sugarcoat the difficulty of the first couple of years, or the NICU time or the necessary surgeries. But they are so proud to share pictures of their healthy little ones after it all, and the joy on their children’s faces makes it all worth it.