Tuesday, November 24, 2015

1st Heartiversary and the Houston Saga

Sometimes, real superheroes live in the hearts of small children fighting big battles.

Dear Abby,

Today is a pretty emotional day for our little family: it is the first anniversary of your open heart surgery, in which Dr. Bryant closed your ASD and PDA. If I'm being totally honest, all of last November was quite trying, and remembering it a year later has been emotional on the whole. I will never forget the moment when Dr. Bryant came into your CICU room just days before your operation to tell us that not only was he not sure that this operation would improve your overall quality of life, he wasn't even sure that the operation would get you out of the hospital.  We had to try though.  Your respiratory episodes were getting more severe and more frequent, and your work of breathing had noticeably deteriorated. It was thought that it could be because of the holes in your heart, which he could fix, or it could be a byproduct of your hypertrophy and suspected airway issues.  Honestly nobody was sure.  But God watched over you and Dr. Bryant, and though the period immediately post-op was quite taxing, not only did the operation get you out of the hospital, it left you able to grow and develop in ways your body couldn't previously allow because of how hard it was working to keep everything simply functioning.

Only a few days ago we feared that this week may hold another heart surgery for you.  Thankfully, your team of doctors (which includes quite a few more nowadays) decided it was not yet time to have to face that fight again. We get to keep you at home, snuggling, playing, and hopefully celebrating an uneventful holiday season!

We are so in awe of how far you've come and how much you've conquered this past year. You fought through a life-threatening event mere days after your heart surgery, an additional procedure to repair the sternal wires that were damaged during that event, countless tests and procedures, a bajillion appointments and therapy sessions, as well as a cross-country move! You are absolutely amazing. As always, we don't know where the future will lead us, but today we are so thrilled to have you here with us and able to celebrate your miraculous journey. 

We love you so much, baby bird - our heart warrior!!!!

Your Mommy


The Houston Saga:

I made a harried post documenting our whirlwind trip to Houston last week, which I wanted to delve into in further detail.

After completing multiple echocardiograms in the last month that seemed to suggest that Aberdeen's hypertrophy was getting exponentially worse, we scheduled a surgical consult with the cardiothoracic surgeons at Texas Children's Hospital to discuss what options there were for Aberdeen at this time.

We ended up having to precede that visit with a trip to the San Antonio Children's ER last Monday night after Aberdeen spent a whole day presenting symptoms of what could have been heart failure (increased work of breathing, lethargy, sweating, etc). Naturally, by the time we reached the ER that evening, Abby had decided she was over whatever had ailed her and was acting 100% fine.  Such a stinker.  They sent us home and told us to follow-up with her local cardiologist the following day.  We did, but he said that as long as she was acting fine, we could keep her home until her scheduled appointment in Houston the following day.  She continued to behave herself, so we hit the road Wednesday morning and arrived to her appointment a full hour early!

Upon arrival at TCH, we were told that her appointment had been cancelled, because they had received word from San Antonio Children's that Abby was being transferred from their facility directly to the TCH CVICU.  We were obviously confused, considering that we'd been sent home from San Antonio Children's and were never even admitted.  No one seems to know where the breakdown in communication happened, but we were quickly assured that her appointment slot had not been filled, and we would be seen by the surgeon as previously scheduled.  We sat in the exam room for three hours after having vitals taken (we'd actually been sitting for four hours, thanks to our super-punctuality), without anyone ever coming to check on us. After some prodding on our parts, we were encouraged to go get some food, as it was still going to be quite a bit.  When we returned from dinner, we talked to one of the nurses (who was wonderful) about what could possibly be going on.  She explained that this surgeon was extremely meticulous in his consults and gave each family as much time as they needed to discuss and explore every option out there.  Cool.  We like anal-retentive people, especially if they're going to be potentially performing surgery on our child.  But good grief, it had been a long time to keep a 15 month old confined to either the car seat, exam table, or our increasingly tired arms.  Abby handled it like a champ though, thanks to some toys supplied by another awesome nurse.  Five hours after her appointment time, we finally met with the surgical team, who started things off by apologizing that our appointment slot had been filled after the mix-up with San Antonio.  Cue the confusion again, as we were told this HAD NOT happened.  Anyway....

The team was very thorough, as promised, but ultimately left us pretty crestfallen. Aberdeen's condition simply isn't one that is easily fixed, by any means.  The first option we discussed was a septal myectomy (removal of excess heart muscle).  I've explained previously about the thickened muscle associated with hypertrophic cardiomyopathy - here's the thing though, it isn't thickened normal muscle, the cells of the muscle itself are different, and even if they remove the muscle that is blocking the outflow from their heart, they cannot change the cell structure associated with hypertrophy.  That means it can grow back, and no matter how much muscle is removed, it wont reduce her risk for life-threatening arrhythmias and sudden cardiac death.  The surgeon explained that the risks of the surgery itself may outweigh the benefits.  He also explained that her size really impedes his ability to remove any muscle effectively at this point.  Ideally, they like to wait on this sort of operation until late childhood, once the aortic valve is large enough to use an entrance point to the left ventricle.  Meh.

The next option we discussed was heart transplant.  We'd been warned in the past that many facilities refuse to perform transplants on patients with Noonan's Syndrome because of the likelihood that things will not turn out favorably.  However, we know of a family whose son with Noonan's received a heart transplant at TCH in the last couple of years and is doing well, so we thought this was as good a chance as we were going to get if transplant became necessary.  However, because of Aberdeen's ongoing cranial issues and airway problems, the surgeon was not convinced that she would be a good transplant candidate.  While it felt as though he was saying that she wasn't worth trying it on, the more I thought about it, the more I think he was trying to say that she simply wouldn't make it through the process. Meh again.  However, Dr. Bryant wasn't sure her first surgery would make a difference for her and it absolutely did.

They essentially said that there were no good options, and with her not having consistent symptoms at this time, they wouldn't recommend any surgical interventions right now.  They did recommend sticking around Houston until Thursday to meet with the cardiomyopathy team to get their take on things and have another echo and EKG performed.  

We left (at 8:15 after a supposed 2:00 appointment) and crashed at a nearby hotel until her appointment the next day. On our way out we asked one of our new nurse friends about the whole filled-appointment thing, and she said that our appointment had NOT been filled. They'd scheduled four consults for that day; when ours was cancelled, they were down to three, and when ours was reinstated they were only back to four again. Ugh, whatever.

Day Two: Aberdeen received her echo and EKG right on schedule and a member from the cardiomyopathy team was in shortly to discuss the results with us.  The gradient associated with her left ventricular outflow obstruction was still quite high, however, it was nowhere near as high as the readings she'd received at her two echoes here in San Antonio.  In fact, it is almost exactly where it was when we she was last seen in Cincinnati in August.  CUE SO MUCH CONFUSION. The cardiomyopathy doctor drew us a lovely picture of Aberdeen's heart and explained that in the case of the two local echoes, the "gnarly" (his word, not mine) regurgitation across the mitral valve (also not good, but not as concerning as other things), was contaminating the measurement used to obtain her gradient. So, it didn't magically get better, the echo was simply being read incorrectly.  While this sounded like good news to us, ultimately it didn't make one bit of difference from the cardiomyopathy team's perspective.  They have literally NEVER cared about that gradient number - which we've always found hard to believe, I mean, they have to consider the quantitative data relevant at some point, right?  Nope.  Not at all.  The effects of HCM are truly a case by case thing.   One person may be horribly symptomatic with a gradient of only 50, while another completely asymptomatic with a gradient well over 200.  They really, truly only care about Aberdeen's symptoms, especially considering that there wasn't anything on the echo suggesting that she was experiencing pulmonary hypertension.  They do care about that potential for pulmonary edema and hypertension a lot though, and because of that actually felt like any muscle removed during a myectomy could have a very positive impact if she were indeed symptomatic.  We found this encouraging: if she does become consistently symptomatic, maybe there is an option we can try, outside of transplant.

While the cardiomyopathy docs made it very clear that the change in perception regarding her gradient number didn't matter to them, it does feel different for us.  We'd begun to feel like we were on a runaway train that was hurtling toward a brick wall.  Now, it feels like we're on a train that's going a little too fast for comfort with an unreliable conductor that may or may not run the train into a wall at some point.  Which feels...a little better, I guess? I don't feel as panicky, and am crying less than I was a week ago, so that seems good.

We left Houston with a Holter Monitor (a 24 EKG, essentially, to make sure she isn't having arrhythmia issues, as we may need to adjust her meds if that's the case) and plans to follow up in a month (with a local cardiologist visit - sans echo - in between).  I'm still concerned about some out-of-the-norm behaviors she's been exhibiting off and on, in addition to the fact that she hasn't really gained any weight in the last two months, when she had been steadily gaining since her heart surgery a year ago.  Ultimately, we'll just have to wait and see how she does though.  Going into Houston, I thought that sounded like the worse possible option - waiting.  Now, however, I'm okay with it.  I feel like everyone is on the same page now and she's being very closely monitored.  We're just going to enjoy our Thanksgiving and all of the wonderful moments we get with our precious girl!

Thursday, November 19, 2015

Consults in Houston

We had Aberdeen's surgical consult at TCH yesterday. It was a very long, disappointing day. We're sticking around Houston today for some more appointments and I will update here as we know more. Abby's still acting like her spunky, smiley self, for which we are so grateful.

From Facebook last night:

Well, very little today went as hoped. Due to some confusing circumstances, we ended up waiting 5 hours past Aberdeen's scheduled appointment to actually be seen by the surgeon. He essentially said that there are no good options. They can try the myectomy, but because of her size, they really won't be able to remove much of the thickened muscle. And while removing what they can MAY reduce the risk of things like pulmonary hypertension, it won't reduce the risk of sudden cardiac death, arrhythmias, etc. Because of everything else she has going on (ongoing cranial issues, sleep apnea), he's not convinced she'd make a good transplant candidate. We've scheduled a last minute echo for tomorrow and will hopefully meet with the cardiomyopathy docs to get their take on things and determine if they think she needs to be monitored inpatient. We're pretty heartbroken right now and hoping for even a smidgen of better news tomorrow.

Update after today's appointments:

We just finished up with our appointments in Houston. You want to hear something completely baffling? It turns out, Aberdeen's left ventricular outflow gradient is no different than it was when we left Cincinnati...it never was. Her first echo here read a little higher than 100 because she was being a fuss-bucket, but the two really high, panic-inducing readings from the local cardiologist were inaccurate. Aberdeen's heart is a confusing place, and the regurgitation across her mitral valve (whose numbers can be quite high) was being mistaken for the gradient across the aortic valve (the outflow from the left ventricle). Apparently it was an understandable mistake given Aberdeen's anatomy, and we should only be doing echoes with a cardiomyopathy specialist from now on.
Just as the cardiomyopathy docs have always told us, the gradient number doesn't really matter, her clinical symptoms do, but it DOES mean that her condition isn't worsening at an exponential rate. It doesn't actually change anything the surgeon said yesterday - if she becomes symptomatic tomorrow, we'll still be in the same place we thought we were yesterday, but because she is currently mostly asymptomatic (she's had a weird day here and there that have given us pause, but her symptoms haven't been consistent so far) it isn't a discussion we have to have TODAY. Which we'll take.
They've upped her beta-blockers and are doing a Holter to make sure there aren't any underlying arrhythmias, and we'll follow up with TCH again in a month. We have to keep an eye out for all of the same symptoms of heart failure we always have, but I think I'll at least sleep a teensy bit better tonight.
Thank you so so much for all of the prayers and positive thoughts coming Aberdeen's way. 

Tuesday, November 10, 2015

15 Months Old!

Aberdeen is 15 months old today!!! That's right, today - look at me being all on time and stuff. She has gained all sorts of skills in the last month: giving kisses, making kissy noises, brushing her hair, and standing while hanging onto things for brief periods (without my help!). She loves waving at everyone and showing them how big she is. She is now just under 20lbs and is cutting a few teeth - oof. 

Things have been going really well in all of her therapies! We've taken a new approach with feeding therapy: everything is on her terms. She isn't actually eating anything by mouth yet, but she is playing with the food and occasionally even bringing it to her mouth, which is a huge step forward. Us trying to feed her was always a disaster, so if we can get her to a place where she is actually interested in the food, maybe we'll have a chance at making some progress. We aren't sure of how effectively she is swallowing these days, but we'll get to that in time. OT and PT are happy with all of the strides she's making as well!

The following pictures somewhat capture how active she is nowadays: much more interested in grabbing the camera than cooperating, haha.

We got to celebrate Halloween at home this year! Yay! She was teething and had a cold, but we still had fun getting her dressed up and walking to a couple of neighbors' houses (since it was still quite warm out. Oh, Texas). We had a chance to go to a pumpkin patch earlier in the month, and have been generally trying to do all of the things we couldn't last year while she was in the hospital. It was a blast.

On a less stellar note: things with Abby's heart are not going well. We had hoped that her obstruction would at least level out after the jump we saw in her gradient at her echo last month. Instead, however, it increased at an even faster rate, and is now in the 170s (it was 140 last month). We've been told that if her gradient were to reach 200, she would almost definitely be showing signs of heart failure. We are getting scarily close to that number now, and nobody is happy about it. We are so thankful that she's been asymptomatic up to this point, but we're now worried that with how significant her obstruction has become, if she were to start showing symptoms of heart failure, things could deteriorate quickly. Our local cardiologist called Texas Children's to update them on her echo findings, and they are working on getting us scheduled for a surgical consult. We really aren't sure what they're going to say. We're hoping that they'll be amenable to the idea of the myectomy (removing some of the thickened muscle), though last month they felt that she was still too small, and she really hasn't grown much since then. If this is still not an option, we may talk transplant, or they may tell us that we just have to wait and hope that by the time she becomes symptomatic she'll have grown to a more favorable size. We do not like the idea of rolling the dice and keeping our fingers crossed (option 3). When Abby does things she doesn't mess around. With the increasing obstruction, there also becomes an increased risk for an arrhythmia, and our local cardiologist doesn't know if we'd get a second chance if she went into ventricular tachycardia (such fun conversations we get to have).

wish I could say that we are holding up great under the circumstances, but I have to admit, we're really stressed out and nervous about what comes next. We're really, really hoping TCH will have a proactive option.