Tuesday, June 21, 2016

The Plan...

Yesterday's surgical conference resulted in a recommendation of a septal myectomy, as soon as possible. So, Aberdeen is scheduled for her second open heart surgery on the 14th of July, with a pre-op appointment on the 13th to meet with the surgeon (Dr. Heinle), the cardiac anesthesiologist, child-life, and to complete whatever labs the team deems necessary. She will most likely be first case on the 14th (meaning, she'll be the first surgery of the day), and then we'll start the long road to recovery. 

Ultimately, this was our best-case scenario. The fact that they think the myectomy may be successful and that she hopefully won't need a heart transplant yet is a really good thing. On a purely logical level, I know this is what needs to be done to give Abby a chance to continue thriving. On an emotional level, I just want to grab my baby and run far away. I feel sick to my stomach to have to put her through all of this again and wish there was another way. However, we can't really deny that she's symptomatic at this point.  Mornings are still good. Afternoons are not. I can't for the life of me figure out how to get a video on here, but if I could, I would show you just how awful her breathing sounds in the evenings. It really is starting to be reminiscent of her breathing before her first heart surgery, which is very worrisome. Hopefully things stay the way they are until the 14th and don't continue to get worse. We're thankful that we don't have too long to wait. 

Thank you for your continued thoughts and prayers as we deal with a lot of conflicting emotions heading into this surgery. 

Friday, June 17, 2016

Brief update

I just wanted to provide a brief update as things have progressed since last week. Our cardiomyopathy docs at TCH met with the cardiothoracic (CT) surgeon, who agreed that Aberdeen's echo looked worse and it was time to do something. She will be presented to the surgical conference on Monday (this is not something we are present for, just a conference between the cardiologists and all of the surgeons) to determine if we move forward with the myectomy, the transplant evaluation, or both. If there is concern about whether or not the myectomy will be successful, they will probably recommend both. The eval itself would last multiple days and most likely require a blood transfusion following all of the necessary labs. This may be something they want to do immediately, or it may be something they wait to do while she's still inpatient post-myectomy. We just don't know yet. 

Days at home have kind of been all over the place. During the mornings, Aberdeen seems like herself and isn't really showing any obvious symptoms. After her nap, however, something seems to flip - she becomes noticeably sweatier, despite always being in the highly air conditioned house and not being particularly active, her breathing sounds like trash, and she's puking every day again. Next morning, she seems like she's back to normal. It doesn't seem to fit with a classic heart failure presentation, but it isn't good either. We're glad TCH is moving things along, as it seems things may have been caught just in time. 

Thank you for your continued thoughts and prayers. We're pretty stressed out, but trying to trust that everyone is working in Aberdeen's best interest.

Wednesday, June 8, 2016

22 Months and A LOT of other info.

So, you know how the last few months' posts have been a fairly uneventful summary of all the seemingly mundane, though pleasant things that have been happening in Aberdeen's life lately? This is not going to be one of those posts.

However, let's start with a little pleasant and mundane:

Aberdeen will be 22 months old on Friday - I'd like to note that my monthly post is early for probably the only time ever.  We are all really enjoying signing with her and working so hard on that transition from laying down to sitting.  Given the fact that she is sitting so well now, pulling to stand, on the verge of cruising furniture, and a mad woman in her gait-trainer, we know that this is the last step to really getting her mobile and it has been so hard to make any progress on.  Abby has never been particularly keen on putting weight on her arms, and pretty much all of the versions of getting from laying down to sitting require such weight bearing.  She would much rather try to do a massive crunch to get to sitting, but just doesn't have the muscle tone in order to accomplish that.  We'll keep working and keep working, but man, this has been a tough spot.

Remember how last month, I was all, "Sure, let's just add a bunch of volume to each feed, no biggie?" Well, it didn't work out in quite the way we'd hoped.  Abby started in with some increased nasal congestion and we were back in puke city.  We transferred the volume to the overnight feed with the hope that we will eventually be able to (very slowly) add it back on to her daytime feeds.  

We took a really nice trip to Dallas over Memorial Day weekend to visit friends and family in the area.  Some we'd seen recently, others we hadn't seen in many years, but it was nice to catch up on all fronts and see a little more of the gigantic state in which we now live.  My parents also came to visit last month, so Abby has had the chance to be exceptionally social as of late.

And now, our trip to Houston...

As mentioned in my previous post, this trip to Houston was to include a plethora of medical appointments, a Noonan Syndrome mini-conference, and a surgery to place ear tubes.  In typical TCH fashion, very little went as planned.  

Quick summary of the first few days:
Thursday evening, we packed up basically the entire house, including the dogs, and made our trek out the Rice Village area of Houston where we would be staying for the week.  Friday included a meeting with the pediatric anesthesia team to make sure they were well aware of Aberdeen's medical history as well as an echocardiogram later that afternoon.  Saturday was the mini-conference, which Abby was not very interested in, so we let Jameson stay and soak up the info while we headed back to our temporary abode.  Sunday was our day off, and we spent part of it at the Johnson Space Center, which was lots of fun, and I wish we could have spent more time there.  

Monday was when her tubes procedure was scheduled.  After getting checked in and settled into the pre-op holding area, we were visited by the head of anesthesia and the ENT who was supposed to be performing the surgery.  Based on the results of Friday's echocardiogram, they felt that the risk of anesthetizing Abby even for the five-minute procedure was too high a risk, and recommended cancelling.  The ENT said that she'd never heard the anesthesiologist give such a recommendation for an ear-tubes procedure, and that she took his advice very seriously.  Ultimately, we decided that risking Abby's life for a potential gain in hearing just wasn't worth it and cancelled.  We'll have to do some studying up on our sign language, but obviously that is totally doable.  There was a part of us that was vaguely disappointed by the cancellation, but the predominant feeling was one of relief. Neither of us had been able to sleep the night before due to anxiety, and the doctors' decision just felt right. More concerning to us was this apparent change in her echo...

Tuesday was our big appointment day (cardiomyopathy, ophthalmology, and craniofacial). Our cardiology appt started with a general rundown of how Aberdeen has been doing (increased sweating, grunting, but otherwise acting like herself), we all agreed that her symptoms were vague at best. However, it seems as though her echo may actually be worse. There had been a question about whether or not the echo had been read correctly due to the confusion last November, but this time even the cardiomyopathy docs at TCH couldn't agree. Which, in combination with anesthesia's refusal to do any sedated procedures, has prompted cardiomyopathy to suggest that we need to move toward surgical intervention. 

You may recall that in November, CT surgery essentially told us they would not do a septal myectomy, and that the only other option is a heart transplant, which they may not consider her a candidate for. Basically, what cardiomyopathy wants to do is start the evaluation process for a transplant, with the hope that CT surg will come around and recommend trying the myectomy first. There are so many complicated aspects of starting the transplant process, not the least of which is the necessity that Aberdeen and I move to Houston. We would have to be within one hour of TCH 24 hours a day, 7 days a week for as long as she was listed - which could be anywhere from days to years. Considering that she just doesn't seem sick enough at this point to be listed as one of the more urgent statuses, this time would most likely be longer than shorter. We're formulating our questions and talking to cardiomyopathy constantly, but we really really hope we can go the myectomy route and potentially buy a few years before needing to revisit the transplant discussion, but I just don't know if it's going to work out that way. We're discussing second opinions and third opinions, but agree that if a very highly respected anesthesiologist won't sedate her with her heart the way it is, then we have to do something. Thankfully, ophthalmology and craniofacial agree that we don't need to do anything urgent regarding her skull, so we can focus on her heart, but they did order a 3D CT scan just to make sure (which we did this morning before packing up and heading home).

We're feeling pretty overwhelmed right now. Lots of conflicting emotions. There are a lot of things to consider and options to weigh before we make any kind of decision. We don't have any idea of a timeline right now, but I'll try to update as we continue to discuss and gather information. If you wouldn't mind praying for some wisdom, or guidance, or something, we would greatly appreciate it.