Sunley had a big series of check ups in Houston this week with Texas Children's Fontan Go Clinic (this is the clinic that our nonprofit funds). The purpose of these visits are to get a big-picture look at how her whole body is doing overall, and to make necessary adjustments/interventions when needed.

We arrived Sunday night, and stayed with the Blankenships. If you have followed our story over the past several years, you remember our heart buddy, Oliver. They live in Houston now, so we sometimes stay with them when we come for checkups. We decided to bring all the kids this time since Sunley had 2 days of appointments, and we made a little reunion vacation out of it! My dad also had checkups across the street from TCH at MD Anderson, so they came a day early to hang with our crew after Sunley's Monday appointments.

During our stay, we also finally got to meet Heather and baby Hannah! Hannah has a similar diagnosis to Sunley, and our family has been cheering her on since before she was born! She was even cuter in person, and Sunley asked afterwards, “Does this mean I have a new heart buddy?” You betcha.

We also got to visit a bit with Kimberly, another heart mom who reached out to me when Sunley got her prenatal diagnosis in 2018. Kimberly’s daughter had just been through her first year of treatment when Sunley was born, so she was extremely encouraging to me. Her daughter is now inpatient waiting for a transplant, so it was wonderful to touch base and send some love her way.

Monday's appointments were tougher than Tuesday. We had all the imaging/testing done. Sunley had a liver ultrasound, bloodwork, stress test, and a 24 hour holter placed. She absolutely hated everything except the stress test. I love her fight, and it serves her well, but it's so tough making her do things like this when I know there's no real cure. She understands why she has to do it all, and that helps. And she knows that her doctors are on her side.

Tuesday, things were a lot more chill. We had consultations to discuss results of all the testing. The beauty of the Fontan Go Clinic is that I make one phone call to the coordinator, and she sets up all the appointments for me! We met with hepatology, cardiology, and pulmonology, and at one point we had 2 specialists in the room at the same time. This facilitates so much wonderful discussion and patient/parent education. It really was fantastic. I have been so incredibly impressed with the team there, and the communication between the team and us is so helpful as I continue to learn about Sunley's heart and body.

Cardiology:

Sunley's cardiologist actually decided not to perform an echo (heart scan) this time. She had a fantastic scan in May, and at that time her heart was showing no signs of enlargement or valve leakage. This was phenomenal news, and so cardiology decided to just look at the overall picture this time and not specifically her heart. Honestly, this was kinda tough for me. I love getting a peek at her heart each time, and it gives me a lot of reassurance to know that everything is holding well. Her heart physiology is a very delicate thing, so skipping this scan was tough for me to get on board with. But her team is (and we are) always weighing what is really medically necessary, and the truth is that we just didn't need this extra look, so it was in Sunley's best interest to skip it.

We discussed her May scans, and this week's stress test, bloodwork, and liver ultrasound. Her Fontan Go cardiologist is really pleased with where Sunley is at. She is definitely on the “higher end” of outcomes, and so that's a great thing to hear him say. The phrase “For a Fontan patient” is always used lots of times in these discussions, and I hate it, but it's something I just have to get on board with.

Her heart is doing great, for a Fontan patient.

Her stress test looked excellent, for a Fontan patient.

Her bloodwork is stable, for a Fontan patient.

I'm always wrestling in the middle of insane gratefulness for those words, while also hating the fact that she has to live with an asterisk.

Hepatology:

This was our first time seeing a hepatologist, and I absolutely loved our discussion. I was really wanting to know where we were with liver progression. When a kid has a Fontan physiology, the liver almost immediately begins receiving damage, and Sunley had never had a liver scan before. Unfortunately (or fortunately, depending on how you look at it), she explained that we can't really get a good “measurement” of where Sunley is on her liver timeline without a liver biopsy – and none of us are advocating for an invasive test like that just to satisfy our curiosity. Sunley's liver numbers are not good, BUT for a Fontan patient (there's that phrase again), you can not apply “normal” liver number standards. Her liver does have elevated numbers, and it is showing increased stiffness, but this is expected with her diagnosis. Also…there's nothing we can really do to stop it. So a liver biopsy makes no sense. Someday, if we are seeing symptoms or have any added issues, a liver biopsy could be helpful. But not right now. Right now, we just watch her enjoy being 8 and we pray that the numbers don't get worse for a very, very long time. Hold the dam – that has been our prayer for years. And He is doing it.

Pulmonology:

We had a great and very educational conversation with her pulmonologist about Fontan physiology and lungs. We don't have any blaring concerns, but Sunley does need a sleep study to rule out sleep apnea. She has some symptoms that are consistent with this, and it is something we can manage if needed. A sleep study has been added to her next cardiology check up in April, and we are praying everything there comes back normal! We can deal with it if we need to, but I would love to not collect any more diagnoses.

All of these discussions were helpful and encouraging. Everyone on team Sunley is very pleased with how her body is doing, and it feels like everyone is expecting many more years of Sunley.

At the end of all the encouragement, her coordinator came in with a prescription for Make a Wish, and told us we could pursue that whenever we're ready. I definitely was not ready for that conversation, and actually I wish she had discussed that with us in private instead of in front of Sunley. While Make A Wish is an incredible gift, and so much fun for Sunley to think about, the timing of it was not my favorite. It was just an unexpected reminder of her diagnosis, and I wasn't ready to talk about it. I'm so glad that Sunley will get to do that someday, but we've tabled that discussion for now. Being offered a wish is still, regardless of my emotions, a very wonderful thing to celebrate and when we are ready it will be a beautiful thing to participate in! Getting a wish does NOT mean that Sunley is nearing the end; it means that she has been through a very hard medical journey, and is being offered a spark of hope and joy. And I'm so grateful for that.

After those appointments, we were finally done! We celebrated by having a short visit with one of Sunley's school friends who recently moved to Houston, then we made the short trek back to the Blankenships to rest up for one more night before heading home to Oklahoma.

I realized after all of this that I really wanted a measurement and a timeline for where Sunley's body is at. And I don't get to have that. We didn't even look at her heart, and her liver scans and bloodwork only give us a small glimpse into her liver’s overall picture. We got a baseline for her, and we will be watching trends more than numbers when it comes to her testing and imaging. That's hard for me. I want a number, a solid prognosis, and instead I'm being pushed into this abyss where I have to hang on to hope. Hope – a completely abstract thing that no one can measure. I think there is quite a bit of hope available for me to take hold of, and I can feel myself resisting it if I'm being honest. I don't want to get burned.

But I'm not in a hurry, and neither is He. Time is Sunley's enemy anyway. He will teach me slowly how to accept more and more hope. And I know He's good for it. My arms already feel covered in rope burns from holding on to Him so tightly, but I wouldn't have it any other way.

What a weird, good place to be. One foot here, one in heaven already. If this is what it takes to keep me there, then so be it.

Onward we go. Hope today. Sight tomorrow. Faith somewhere in between those two.