Half a Heart: Sunley's Diagnosis Process Part 2

Continued from Part 1.

After what felt like FOREVER, the cardiologist pulled out a drawing of Sunley’s heart and I immediately thought “Thank you Lord! There are 2 ventricles!” The cardiologist showed us her two working ventricles and I was so relieved. She had malposed arteries, and a small mitral valve. Then the doctor pulled out a drawing of an HLHS heart, and started talking about the Norwood, Glenn, and Fontan procedures. We couldn’t understand why she was even bothering to show us these. Derek asked, “Right but she doesn’t need these because she has two ventricles.” The doctor replied, “Well unfortunately, we can’t fix the mitral valve, so we will have to reroute the heart to operate with only her right ventricle.” We were CRUSHED. Derek asked her if the mitral valve could grow, and she said that it was very rare, and not to get our hopes up. But IF the mitral valve grew, then she would only need ONE surgery and would be cured. No lifelong condition. She called her condition Double Outlet Right Ventrical with a mitral atresia, and we left with hope that the valve would grow, just like the left ventricle did.

We prayed SO hard for that valve to grow, and I truly believed it would. We prayed that she had just seen it wrong, or that God would develop it. Two days later, I got a phone call from the cardiologist. She HAD seen it wrong. And it was much, much worse than she thought. She was very apologetic for seeing it wrong, and she explained that actually Sunley had no right ventricle, and that we should expect to outlive her, even with the 3 surgeries. There was no hope for living with two ventricles. Oh, and by the way, the mitral valve is normally-sized. I was taking it all okay until she told me about the mitral valve. I quickly got off the phone, fell on my bathroom floor and cried until I threw up. Second Pleading Mother Cry. Derek and the kids had returned from an errand while I was on the phone, and he was so confused when he saw me, and probably terrified. I told him what had happened, and asked him to call Mom and Dad to come take the kids because I knew I would not be done crying for quite a while. They rushed over of course and played with the kids (I guess?) while I finished sobbing. I was just so confused. WHY in the world did God answer my prayer and give me something WORSE? I think this was the first time in my life I’ve asked God why. This misdiagnosis was the miracle I had asked for. And it ended up being a bad thing?! My favorite part of Job is when he asks God why, and God’s response is not exactly what you’d expect. Instead of saying “Everything happens for a reason,” or “Don’t worry, I will make good things come out of it,” He says things like “And where were YOU when I created the universe?” And He goes on for a LONG time. It completely puts Job in his place, in a strangely comforting way. Job responds with “Therefore I have uttered what I did not understand, Things too wonderful for me, which I did not know.” After I finally calmed down, I just said “Okay, Lord.” I got up, and went to talk to Hadelyn, who had unfortunately witnessed some of my breakdown. I think part of the giant wave of emotion was just being SO jerked around with the diagnoses, the stress and exhaustion that so much “switching gears” had done to us. The healed mitral valve that got us nowhere was just the last straw. As soon as my parents went home, I sat down at my table and frantically wrote out a list of prayers that God HAD answered, including the mitral valve, and taped it to my fridge. I think the only way to survive this is to FORCE yourself to see silver linings, even when it feels like they aren't there. Because they are ALWAYS there.

We were still extremely confused about the diagnosis, how she had mistakenly seen a right ventricle when there was not one, and decided we needed to go to Texas Children’s in Houston for a second opinion. We weren’t angry with the cardiologist at all, and honestly it’s amazing they can see anything at all on a fuzzy monitor with a heart that small. But we had pretty much already decided on Houston anyway, and this was an easy time to make the switch. I’m sure that care in Dallas would have been good too, but Houston is ranked #1 in the nation for pediatric cardiology, and they do 4 times the amounts of Glenns and Fontans that Dallas does, so we just couldn’t ignore that. We had also heard very extreme polar opposite reviews about one of the Dallas surgeons, and that made us uncomfortable. One friend of a friend was a Dallas cardiologist, and even she recommended that we go to Houston instead.

I called my doctor first thing in the morning and very urgently requested an appointment in Houston. They worked very quickly and secured an appointment for me just one week away. We started praying again for a new diagnosis, or a cure. Tuesday night, we flew to Houston (my parents paid for that, and we are so grateful! Tickets from Midland to Houston are so expensive.) and stayed in a hotel across the street from Texas Children’s. I woke up at FIVE in the morning and just couldn’t go back to sleep. We arrived at our appointment at 10, and met our cardiologist, Nancy Ayres. She was accompanied by a nurse and a fellow and the three of them went through another long echocardiogram, during which they spoke in “cardiologist code.” Unfortunately, we knew what most of it meant by now. We heard terms like “hypoplastic RV,” “Double Inlet,” and “Double Outlet.” We knew the diagnosis before it was even over. As of right now, Sunley has Double Outlet Right Ventricle (DORV), Double Inlet Left Ventricle (DILV), with a large Ventricular Septal Defect (VSD – Hole between the ventricles), slightly Malposed Great Arteries, and a severely Hypoplastic (underdeveloped) Right Ventricle. That last one is the biggie. Sunley basically has half of her heart. Unless that right ventricle miraculously grows, she will live her life with only one ventricle, meaning that her heart will always be working twice as hard. In fact, her right ventricle is so small that she is not even a candidate for the somewhat experimental surgeries in Boston that create two ventricles.

The good news is that if you had to pick one ventricle to have, the left is the one you want. We were told that these babies usually handle surgeries and life better than HLHS babies. Also, her aortic valve SHOULD be malformed, and the doctors are very surprised that both valves look strong and well developed, aside from some abnormal positioning. They were very encouraging, and there was no talk of outliving Sunley. In fact, Dr Ayres mentioned that she’s had some patients go on to go to college, get married, have kids, etc. This was very encouraging, even though we are well aware that things could go very differently during and after any of the surgeries.

We were also glad to learn that Sunley does NOT need the Norwood Procedure. This is the riskiest of the HLHS surgeries, and happens at just days old. Instead, she will have a pulmonary banding surgery at 1-2 weeks old if we can wait that long, followed by the Glenn at 3-4 months, and the Fontan at 3-4 years old. While it is impossible to predict what her life will look like, she could possibly have a relatively normal childhood full of sports, hiking, etc. We don’t have to “monitor” her activity or make restrictions for her – she will simply feel tired when her heart and lungs have had enough and will naturally slow down if needed, just like you and I do with normal hearts. Of course, these surgeries are some of the riskiest open heart surgeries that exist, so complications can arise, other surgeries may be needed, etc. But we are praying for speedy, eventless recoveries. Actually, that’s not entirely true; We are praying for miraculous healing before she is born.

The day after her appointment, we found a great apartment near the hospital and signed a 9-month lease. I will need to move there at 33 weeks in case I go into early labor (I did with Ruger), and so our lease is March 8 – December 8. That means 9 months away from my home, my church, my family’s events (my best friend, older sister, and sister-in-law will all have babies during this time) and of course I won’t be shooting weddings at all (expecting two friends’ weddings during this time too). That’s the down side. The up side is that we used to live in Houston so we have church family there too, we LOVE the area where the apartment is, and we have decided to rent out our house in Midland to help cover the cost of the Houston apartment (It won’t cover the whole thing, but it will help tremendously). Our dog will go to OKC to stay with my pet-loving in-laws, so she will be WELL taken care of.  We will put most of our stuff in storage or in my parents’ soon-to-be-empty rental property, and will purchase a few needed items (sofa, sofa bed, table and chairs, etc) from IKEA as cheaply as possible. Derek found out that he can work from Houston and that is a MAJOR blessing.

And that’s as far as we’ve gotten. Because of how day-to-day Sunley’s condition will be until her 2nd surgery, we can’t really make decisions yet about whether or not our kids will get to be with us. That is by far the hardest part (right now). Honestly, I can barely even talk about it. Any parent will understand that. I’ve had some other moms reach out to me, and I’m sure as they share their experiences and this process goes on, I will understand the diagnosis, surgeries, etc much better than I do now. I’m learning how to do this one day at a time. God has been so RIGHT there with me through all of this, even during the brief moments that I questioned why, and I know He will continue to comfort us and guide our decisions.

To be Continued.