Happy Sunday from our hospital room :)

I was so thankful to wake up to sunshine today after a few days of cloudiness here.  I'm always reminded at what a huge difference the simple pleasures make when we're stuck (quite literally) in a situation like this.

Landry finally had an extended break from vomiting (retching, really, since nothing has been in her belly) and she is more alert for us, which is especially encouraging. Things are starting to look brighter and the sunshine is just the icing on the cake. 

I have been trying to think of how to word an update on Landry's brain MRI and consults with neurology to give good information without being overwhelming. I don't know if I'll make that happen, but I'm going to try.

First of all, her MRI went beautifully. Putting her under anesthesia wasn't a decision taken lightly and, up until shortly before was being questioned, so I think I was more anxious leading up to that than for anything the images might actually tell us. I was thankful for a thorough and wonderful anesthesiologist who took the time to really talk to us before taking Landry back. He did everything he could to protect her airway and, in the end, she came out of anesthesia better than she has before, even at her baseline. Such a relief!

As for results, it was an extremely informative MRI that helped teach us a lot about her brain and functioning and also facilitated some great conversations with neurology, but unfortunately, it did not give us any straightforward or fixable causes for vomiting or other symptoms. 

I had a conversation with the on-call neurologist Friday evening and a good experience with them overall all week, but yesterday morning I was SO happy when our own neurologist walked into the room. He no longer does regular hospital med and only takes 4-5 on-call weekends a year so it was a total God thing that it coincided with our stay. No matter how great the hospital physicians are, it is such a welcome relief when you see someone who truly knows your child and has seen them at baseline in a clinic setting.

He really took time with us and had a thorough conversation. The MRI confirmed what we knew, that Landry has cerebral atrophy, but we are glad to know it's stable from past MRIs and not worsening right now. This isn't unsurprising considering the mutation on her CHAMP1 gene caused her brain to stop developing prematurely, but we've never had it explained to us in correlation to her brain having less reserve and, essentially, a lower threshold for being able to withstand illness. 

We've known that Landry has dysautonomia, which is a dysfunctional autonomic nervous system. Part of this system is the sympathetic nervous system, which is better known as our bodies' "fight or flight" response to stress or danger. It controls involuntary bodily functions like heart rate, blood pressure, tear and sweat production, body temperature, and so many other things that most of us don't think much about because our bodies keep them regulated for us. 

Landry's autonomic attacks, or you may have heard me call them neuro storms or autonomic storms, are not new to us. Her body reacts to stress much differently and more easily than the typical person and we see this often for sometimes no reason at all. 

Essentially, the same thing happened this week as her brain responded to the coronavirus (again, not covid), but on a level we've never seen.  Not knowing how to handle the stress from the virus, her brain malfunctioned and went into autonomic crisis causing fluctuating heart rates, high blood pressures (120s/90s), fluctuating fevers, behavior/alertness changes, tremors, skin rashes/blotches, and, ultimately, triggering her cyclic vomiting syndrome.

Cyclic vomiting syndrome is not new to us either; we've always known that her vomiting at the beginning of an illness (even those not known to cause GI problems) is neurological and more of a stress response to a virus than an actual stomach problem. We just have never had an episode that lasted so long and didn't respond to any combination or strength of medication, which is what made it so unusual and puzzling.

Everything lasted six days total when we look back to the significant changes in alertness that started last Sunday. Her body worked so hard with no relief and is so tired, but she does finally seem to be coming back to a more regulated state.  It makes me so happy to see her eyes open and sitting up in bed.

Today's plan is to work on transitioning back to continuous formula feeds at a very slow rate.  Right now 1/6th of her maintenance fluid rate is continuous formula (only 10 ml/hr) into her feeding tube and the other 5/6th being IV fluids.  We'll work on this very slowly, probably over several days, until she's eventually getting her maintenance fluid rate at 100% formula. GI will come back into the picture this week with a longer term plan for home feeds and new medications, but as long as she can tolerate some formula over the next few days, we should be able to avoid PPN (IV nutrition), which is ideal.

This was an intense and scary week that I don't think I'll even fully feel the extent of until we get home and I can process, but in the midst of all that, we have felt so much love and support from home.  Your prayers, texts, gift cards, money for meals and just to relieve some of the pressure of being away from home means the world to us and that even feels like an understatement.

I know that we've had people praying for Landry and our family that we don't even know and I can't tell you the comfort that brings.  Landry is proof that God is good and able to heal even in the scariest of circumstances. Sometimes I wonder how there can possibly be a greater plan in all of this and I wish all the time it wasn't my child that has to suffer, but I know there's purpose here, even if I can't see it, and I pray that someone, somewhere, is encouraged by me sharing our story.  

All my gratitude,
Lindsay