End of 2016 Updates

2016, whew, you were a doozy.  From the lowest of lows (facing the daunting possibility of heart transplant, and ultimately the reality of major heart surgery again), to the highest of highs (watching our sweet bird recover from surgery smoothly and growing stronger than ever), you have certainly been one for the books.  While this year was at times exhausting, I am thankful for the progress we've seen in Aberdeen and for the lessons all three of us have learned as we navigated the ups and downs of each month.

I haven't done an update in a couple of months because it's been relatively smooth sailing over here lately. No news is good news, right? Abby is about 25lbs and 32in long these days. She's wearing 24 month clothes for the most part and just went up a diaper size. She still has the teeniest tiniest feet ever (size 3s are roomy), and has more personality than just about anyone I've ever met.  She's working hard at therapy, learning to use a picture system to communicate with in addition to her signs, honing her fine-motor skills, and making progress little-by-little with gross-motor skills. She is so close to mastering the transition from laying down to sitting up - I know she is capable of doing it, but something still hasn't quite clicked so that she's doing it without prompting yet, but she's so so close.  She's just starting to cruise along furniture, and can walk a good ways in her gait-trainer - though she gets tired-out easily and requests a lot of breaks.  She is still 100% tube-fed, but we're continuing to work on desensitizing her mouth in hopes that someday she will accept food orally.  One big change at therapy recently is that I have finally been sequestered to the waiting room.  Abby started throwing epic tantrums during PT, and it seemed like me being there was only making things worse.  While it was really hard for me to accept that it was time that I give her some space, she has done so much better without me back there with her.  My little girl's growing up!

Abby hasn't had too many additional appointments lately.  We've tweaked her feeds here and there to account for her weight gain post-op (it's amazing how much more efficiently your body works when your heart isn't obstructed), as well as her continued vomiting.  The puking isn't as consistent as it used to be, but it certainly hasn't gone away either.  This seems to be par for the course with a lot of kids with Noonan's and we're just hoping that over time it will dissipate.  A recent cardiology follow-up showed no change regarding her hypertrophy.  This is great news.  While her heart is still very very thick and has all sorts of risks associated with it, the fact that the obstruction that was remedied during her July surgery hasn't returned is really positive.  There was a chance that she could have gone through everything she did, just to have the muscle grow right back.  But so far, her obstruction is still pretty much non-existent and no other hypertrophy-related issues (arrhythmias, etc) have surfaced.  She's on a 24-hour Holter monitor right now, but just to have some post-op baseline data to compare to down the road.  Come January, Aberdeen will be two years seizure-free (knock on wood), and we're discussing weaning her seizure meds after a follow-up EEG.  It would be nice to be down one more med, especially if she really doesn't need it anymore.  We'll have to follow-up with the neurosurgery team again in the next couple of months for more discussion on when will be the right time for Aberdeen's next cranial repair, but they may recommend that we wait because...

WE'RE MOVING TO WASHINGTON D.C. IN AUGUST!

We've known for a while now that this would be in the plans, but now that it is officially official, you can all know too.  Just as moving here was challenging, getting set up with a whole new team in the D.C. area will be a difficult process as well.  However, we hope that in the D.C. area we'll stand a better chance of having Aberdeen's care spread across fewer facilities - and that none of them will be 3+ hours away.  While the idea of moving again is daunting, I really do hope that the area will be good for us as a family.

We have enjoyed a wonderful holiday season here in San Antonio, with lots of family visiting, fun with local friends, and way too much food. I will be making my first trip away from Aberdeen this coming month - wish me luck and not too many tears! I know Jameson will be fine with her, but I've never left her for more than 24 hours, and I already know that it's going to be tough.  I need a break though and am looking forward to seeing my family out in their new homes in Seattle.

Since I didn't do a post-Thankgiving update, I'll leave you all with a boat-load of pictures from the last couple of months and wishes for a very Happy New Year!

October Updates

I can't believe October is already over! Despite a string of annoying minor issues, last month really did fly by.  We are currently dealing with outer ear infections in both ears following a cold and stomach virus earlier in the month.  This has resulted in a lot of poor sleep and a lot of puke to clean up.  While it kind of feels like poor Abby can't catch a break, I am incredibly thankful that all that has been required so far is a few extra trips to the pediatrician and that none of these issues has landed her in the hospital.

On a very positive note, Abby has made some exciting jumps on the therapy side of things: After facing a lot of resistance in her gait-trainer since finishing up sternal precautions, Abby is now walking in it better than ever before and with even less assistance - apparently she did NOT want to be strapped in anymore.  Stubborn girl.  She also recently discovered her "oh", and "ooo" vowels and with them, her first attempts at purposeful speech!  She is saying "uh oh", a version of "Elmo", and even the dreaded "no" - which we're completely thrilled about.  She seems to be trying to use some of her other sounds to make worlds suddenly as well; balls and bubbles are sometimes referred to through speech as well as her regular signs, and she is "copying" more and more words as we say them.  So exciting! In addition to those amazing accomplishments, we were also able to get Abby out to the pumpkin patch and had a really great Halloween!

And now, since I've buttered you all up with lots of adorable pictures, I shall step onto my soapbox:

Early last month we trekked out to see the craniofacial team at Texas Children's to discuss the next steps for Aberdeen's fused cranial sutures.  They suggested that now that her heart is in a less precarious position that we could do something about her skull if we wanted.  If we WANTED?  No, we do not WANT, thanks though.  It was suggested that there was enough room in her skull currently that her fused sutures shouldn't be causing any undue pressure on her brain and that surgery right now would be for more cosmetic or "reconstructive" reasons. Due to this, we are going to wait.  The craniofacial team gave us a little bit of pushback concerning our opinions on not doing surgery for cosmetic reasons, stating that "those are important reasons too." Look, we can acknowledge that a person's physical appearance may impact other areas of their life, but with everything Abby has been through we just cannot in good conscience put her through another major surgery for something that isn't currently a necessity. We are prepared to eventually NEED to do another cranial surgery to create better protection for Aberdeen's eyes as well as open up those sutures again, but thankfully that time has not yet arrived.  

Also, in an effort to be candid, I'd like to express how dismayed I am that there is such an emphasis placed on physical conformity within our country that it would be deemed necessary to put a CHILD through a major surgery solely to alter their appearance.  I wish that instead we did a better job of teaching acceptance of those who may look different.  

We had an unpleasant experience in the last week, where an older child made a very rude comment about Aberdeen's appearance.  I can't say that I handled it all that well. Chalk it up to social anxiety, but while I am brilliant at coming up with zingers and teachable moments after the fact, in the moment I am much more prone to either silence or crying when approached with unpleasantness.  I wish I had told that child that Abby is just a sweet little girl who is friendly and strong and deserving of all the respect in the world, but in that moment, all I could do was hold my baby close and choke back some tears, thankful that she didn't understand what he'd just said.  I wish I could advise that child's parents on how to approach people who are different than them. I wish I could ask that they teach them that children like Aberdeen are PEOPLE with thoughts and feelings and worth. However, since I don't know this family, I'll say it to all of you:

PARENTS: Please, I implore you, expose your children to people who are different than them. Explain to them that really they are more alike than they are different.  Teach them that there is nothing to be scared of, that these PEOPLE have feelings that can be hurt, and that they deserve the kindness and respect that would be awarded to anyone else.  Encourage them to ask questions, and genuinely seek out answers, so that maybe in the future, they will greet these PEOPLE with understanding instead of negativity.

We would never shirk a well-intended question.  I'm happy to tell you about Aberdeen, let her say hi to you, and answer any queries you may have, but I can't do that if you don't ask. If you shush your children when they start to ask a question or shy away from making eye contact with us, all you do is increase our otherness. Engage us, we don't bite.

To sum up what has been an unexpectedly cathartic post, I'd like to pose a question to the internets: Since we really haven't had to deal with many negative comments over the past two years, I haven't come up with a good plan for responding to them when they do pop up. I was thinking about printing up some information on Aberdeen's conditions (think business card sized info) to give to people in the event that something like this happens again.  Does this seems like a reasonable and helpful idea?  I don't like relying on my ability to handle situations perfectly, and thought this might at least help start the conversation.  Thoughts?

September Updates

The last couple of weeks have held lots of appointments for our little bird. First, we headed out to Houston for a cardiology follow-up as well as another chest X-Ray to try to get a better idea of what was going on with her lungs at her pulm appointment last month. Cardiology was happy with Abby's echo and highly underwhelmed by her X-Ray findings. We retrieved a few more X-Rays from their department, so that pulm would have more data points to use in gauging the condition of Abby's lungs and headed home. 

The following Tuesday, we saw the local pulmonologist, who agreed that her chest X-Ray looked much improved and we didn't have to start any aggressive treatments. Thank goodness! He still considers her at risk for issues and had her get an extra vaccine for pneumonia prevention, but we were otherwise in the clear, and I was happy I didn't put her through the nebs a couple weeks ago!

That night, Abby had a terrible night. She was puking everywhere and we noticed that her urine smelled funky. We got her into the regular old pediatrician on Thursday, and after waiting hours for her to pee, finally had to cath her to get a sample. Although our pediatrician was hopeful that it would just be concentrated urine due to it being so hot out, I had a sneaky suspicion it was more than that. Turns out, it was a good thing we waited, as I ended up being right and she was diagnosed with a UTI. Uggghhhh. So now she's on an antibiotic course and will hopefully be feeling right as rain soon. Once this clears up, I'm really hoping we can go for a least a couple weeks without anything new!

Abby is otherwise doing well. She's off of sternal precautions now, and is making lots of progress at therapy. We just got her back in the gait-trainer, and while it will be a bit before she builds her strength back up to walking, she's at least scooting around the house at turbo-speed again! She is also gaining weight like gangbusters! So much so, I think nutrition will probably want to back off on her calories when we see them later this month. It's amazing how much more efficiently she can use her nutrition when her heart isn't working so hard. Abby's been in a great mood for the most part and isn't letting any of these little hiccups get her down.

1 Month Post-Op

Aberdeen is one month post-op today, and since I couldn't get it together enough to do an in-depth birthday post, I thought I would provide a few more updates as we celebrate this milestone and deal with some difficult developments. 

Abby weighed in at 22lbs and was 31.5in long at her cardiology follow-up on Thursday. Her local cardiologist was thrilled with how well she was doing and so happy to have this myectomy done and successfully out of the way - he made sure that we understood that overall, her heart is still very thick, but that her obstruction is so much improved that her gradient could be considered normal. NORMAL! 

Abby's mood has been steadily improving the further we put the hospital behind us. We are able to get her down for a nap and bedtime again without a gigantic meltdown, and she's warming up to her therapy sessions more quickly each time. She still wants NOTHING to do with medical appointments and her intense reactions to minor things (like vitals) has me run pretty ragged. I hate that she's been through so much and that it's continuing to impact her in a way it didn't when she was an infant. I just want to give her a break.

But breaks we do not get, because if it's not one thing, then it's another - this time her lungs.

After Abby's cardiologist appointment, we had a routine chest X-Ray done, as requested by pulmonology. Results were "highly abnormal" - terms we're used to hearing, but not in reference to her lungs. Apparently there is indication of chronic airway disease, possibly due to aspiration. We don't really know if Abby aspirates, because she doesn't swallow enough to do a modified barium swallow study, which would diagnose such an issue, but there was enough gunk apparent on her X-Ray to make her pulmonologist (who is new to us) very concerned. He immediately ordered up all sorts of breathing treatments, including nebs and CPT (a technique used to break up mucous and fight atelectasis by banging on her back with a cup thing), at which point I had a big old breakdown in the doctor's office. I don't think I've cried like that at an appointment ever, and only very few times even inpatient. It's just too much right now. I feel like we just got over a huge hurdle only to run into a brick wall. I don't know why, with the 10,000,000 chest X-Rays Abby has had in the last month, NOBODY else has mentioned anything like this, and I don't know how her lungs could be in THAT bad of shape with zero clinical indicators. So frustrating. I agreed to the at-home CPT, since Abby doesn't mind it, but I could not bring myself to make her do a bunch of nebulizer treatments right now. We'll do another chest X-Ray in a couple of weeks and see how things look. Maybe by then, she'll be more receptive to the nebs and I'll be in a better place emotionally. 

I'd like to take a second to give major props to Jameson, who has been awesome and a huge help while Aberdeen and I have been struggling. He's always been an amazing husband and father, but I've been especially thankful for him the past few weeks. So thankful.

Praying for better news for our bird at the next appointment and a little peace for all of us. 

TWO YEARS OLD!!!

This past Wednesday, we celebrated Aberdeen's second birthday!!! At only three weeks post-op, we spent a quiet day at home summed up with cake, presents, and a visit from Auntie Jubie. I've had some trouble composing this post, as I can't quite formulate the thoughts and emotions I've had in relation to this day into coherent sentences, but suffice it to say, I'm extraordinarily proud of our little bird. I'm so incredibly thankful for these past two years and all of the smiles, laughs, tears, and triumphs we almost never had. This girl absolutely illuminates our lives, and while keeping us on our toes, has made us feel more blessed than we could have ever imagined. She is a true delight, and I am so thankful for every single day with her. Even the tantrumy days. Even the hospital days. Every. Single. Day. 

HOME!

We arrived home late last night, a mere two weeks after Aberdeen's open heart surgery!!!

Abby's post-op appointment went great! Her echo last week showed that, even awake and not super happy, her gradient is still less than 15. While this technically means that she still has obstructive hypertrophic cardiomyopathy, her heart is in a much more manageable place for now. Yay! Her X-rays have looked great, she isn't sweating or puking anymore, and we're hoping that this myectomy will buy us at least a few years before we have to talk about surgical intervention again. 

Abby was SO excited when we got home. She was squealing and laughing playing with all of her toys and rolling around on the floor again. She was decidedly less happy about having to go to bed and had kind of a rough night overall, but hopefully now that we're home, her temperament will start to even back out again - holy tantrums, batman. 

She is on sternal precautions for the next month, meaning we can't pick her up under her arms, pull on her arms, or allow her to put much weight on her arms or chest. This is a little tricky now that she's bigger and not yet walking, but we're managing. We also have to keep her away from busy places until she's off of sternal precautions, as we still need her to not get sick. So we'll be having low-key birthday celebrations at home this year, but we're seriously thrilled that we will actually be at home and not in the hospital as we expected! Woo hoo! I can't believe Aberdeen is turning two next week!!!