Well, in keeping with how the process has gone so far, we might have a slightly different diagnosis for Sunley soon, although it will not change her plan of care or prognosis. Before today, her diagnosis was Hypoplastic Right Heart Syndrome – Double Outlet Right Ventricle, Double Inlet Left Ventricle, a large VSD, severe Hypoplasia of the Right Ventricle, and slightly malposed Greater Arteries. After the Echocardiogram today, we aren’t sure that Sunley has any right ventricle wall at all, which IF that is the case, would change her Double Outlet Right Ventricle to Double Outlet Left Ventricle, which would make her anatomy even more EXTREMELY rare. This isn’t worse or better, just different. We won’t know if she has Outlet Right or Outlet Left until after she is born. Either way, she will still most likely have a pulmonary banding surgery around a week or 2 old, a Glenn procedure around 3-6 months old, and a Fontan around 3-4 years old. This is the same plan we’ve had since January, so no surprises there. Her body (specifically her lungs) will hopefully respond positively to these surgeries and allow her to live a relatively normal life. Each case is very individual, and thus completely and utterly unpredictable, which we are slowly getting used to.
The doctors also couldn’t tell if her arteries are crossing at all (normal arteries cross into the separate ventricles). So, she may have Transposed Greater Arteries or just malposed, but not too severely. They can’t tell if there’s an extremely small right ventricle that barely formed, or not one at all. This could affect the pressures which will determine when her surgeries are. That is all very much in a nutshell—it’s all very complex because, ya know, GOD DESIGNED IT and it didn’t just happen by chance. Somebody tell Bill Nye he needs to look at the complexities of a perfect, working heart. But I digress. If there is a small right ventricle, I THINK that means that her pressure could be slightly better. But honestly, I’m not sure I fully understand that part yet, so I’ll move on.
We don’t have to look far to see major answered prayers. Though there has been no improvement in her condition, I am beyond relieved that nothing is worse. Specifically, her aorta should be malformed and it’s not. Her tricuspid valve shows very minor regurgitation, but nothing that the doctor is concerned about, so I will pray that remains the case. Also, right now, even though all the blood is mixing instead of separating (In a normal heart, the blue blood and red blood separates and goes to different parts of the body), the blood is flowing in the correct directions.
We were able to spend more time with our AMAZING cardiologist (Dr. Nancy Ayres) this time, and were able to understand things much better than last time. The pulmonary banding surgery will require surgeons to open her chest, but they shouldn’t have to stop or cut into her heart, which means that it does NOT require bypass!! She also might not need that surgery for quite a while, if her breathing and saturations are good enough. That was really good news. The doctor is “pretty positive” that she will need that surgery, but it’s just too difficult to predict when that would be. She even mentioned the slight possibility that we could bring her home to the apartment before that surgery. How wonderful would that be?!
We also had a tour today of the NICU and the CVICU. We saw lots of strong little babies, and some very VERY exhausted parents. While it was a lot to take in, more information is always good, and we are just that much more prepared for what we are about to do. Our tour guide told us that our kids would not be allowed to come into the NICU, but we found out later from the Neonatologist appointment that they ARE allowed in, as long as their vitals show no signs of sickness (The nurses will take their temperatures each visit). Relieved to hear that. Hadelyn needs to see her sister, in my opinion. Ruger…might not care too much. I was also happy to hear that I can bring my cameras in to take photos. Every NICU/CVICU has different rules, so I wasn’t sure what to expect before today.
The neonatologist who we met with was very encouraging, and spent a great deal of time discussing the importance of faith and prayer in medicine, which made us feel right at home. Overall, we just feel like we have the BEST team here. Everyone has been blunt when they need to be, but still very encouraging. We have had some difficult discussions about different options, and we feel like we can really rely on the advice we get from everyone here, if and when we are faced with the really tough decisions on her care.
There are absolutely endless possibilities with Sunley’s future, both short and long term. Our surgical plan is really just a rough draft – Everything will be very day-by-day, and that has taken and will take a lot of getting used to for us. Our doctor discussed some possibilities for other surgeries she may need, but they aren’t even worth mentioning, because she is currently just as likely to not need them as she is to need them. She may respond really well to breathing, eating, noise…or she may need to be intubated, tube fed, etc. Her surgeries may “take” easily, or they might not, thus drastically or immediately shortening her life. There are hundreds of risks and different routes that her condition could take, so that’s why it’s so important that we find a way to function as a family in our new day-by-day normal.
Both doctors that we met with today seemed really surprised that we weren’t a crying hot mess. We explained our faith to them a little bit, but they didn’t seem used to seeing people NOT crying. I’m not sure if that made me feel encouraged or not. Like I’ve said, I definitely have my meltdowns (we both do), but it just is what it is. God is finding ways every day to strengthen us, mostly by sending us really loving helpers.
On Tuesday, a very talented photographer friend offered to shoot a maternity session for me. Since I can’t find my nail polish anywhere, I decided to squeeze in a manicure before the shoot, even though I didn’t really have time. I Googled the closest place, and walked in without an appointment. The nail tech started asking me about my pregnancy, so I eventually shared with him some of her health issues. A few minutes later, the woman sitting next to me said, “I’m sorry, I’m not trying to eavesdrop, but I could hear you saying that your baby will have heart surgery, and I just wanted you to know that I’m praying for you.”
She was so kind, and invited me to her church, very genuinely asked if there was anything she could do, and I kind of figured it would end there. But get this:
“I’m actually a neighbor of Dr. Adachi—our kids play together, and he is a really good man and surgeon.”
Dr. Adachi is one of the congenital heart surgeons at TCH, and suddenly I felt like I was talking to a celebrity. He could very well be the one operating on Sunley at some point. She gave me her name and phone number, and said she would mention our name to him. I mean, COME ON. That is not a coincidence. If my friend hadn’t offered me a session, if I had found my nail polish or gone to the nail salon at any other time, I never would have met her. I did text her the next day, and she really is such a kind person. I have no idea what will come of meeting her, but it was very encouraging, and I look forward to seeing what God does with this new connection, even if it’s just receiving some encouragement through the process, or finding a way to give back and help others after we get through this part. By the way, our photoshoot was so fun, and Amy got ALL of the photos back to us within 24 hours which is INSANE impressive. I didn’t realize how much I wanted a maternity shoot until I had one! Unfortunately, Amy knows all too well the grief that comes from watching a child suffer. She has been a well of strength and encouragement for both of us, and she’ll probably never fully understand how deeply grateful we are to her.
One thing I’ve had a hard time with lately is communicating my optimism to people without downplaying Sunley’s prognosis. I really don’t want people treating her like she’s sick for her whole life, but I think sometimes I overdo it on the positivity, to the point where people think we’ve found a real “fix.” There is no cure for what Sunley has. Statistics are pretty irrelevant because treatment is still very new, and because each baby reacts so differently, AND because her condition is very rare. We can’t predict whether or not her heart will bear the extra workload, but we have chosen to put her through all of this medical intervention because there is a chance she can make it to adulthood and have a very full life. I’m mostly good about not dwelling on the negative possibilities, but of course those possibilities are always there. We’ve had lots of people ask us about heart transplants. We are very VERY much hoping to not go down that path because:
1. Hearts for babies rarely become available
2. The wait for a heart can do a lot of damage to the rest of the body
3. Anti-rejection meds are rough on the body
4. You are more likely to get cancer with a heart transplant
5. Heart transplants typically do not last longer than 10 years (often, less).
So, if she got a heart transplant as an infant or young child, it would most likely not be the last, and it would definitely altar her quality of life. That doesn’t mean we wouldn’t consider one at all if her surgeries don’t turn out well, but it just means that heart transplants are not a cure, and are not as cut-and-dry as I thought they were.
Just a few of the hundreds of things I am praying for now:
Sunley will not need prostaglandin.
Sunley will not need a PA banding surgery for a while, if at all.
Her surgeries will WORK and giver her really high sats.
Sunley will not need any extra surgeries.
God will show us how we can fit into and help the heart community long-term.
Sunley will not have scary long-term complications from the surgeries.
Thanks everyone for the encouragement and prayers! (Photo below from Amy of Lovebird Photographie here in the Houston area)