April 2019 Update

April was a pretty dismal month.

Job never fully recovered from his cold + swallowing issues... but never really got sick enough to need to go to the hospital... Until we took him in Monday, the 29th, for another admit for respiratory distress. We'll see how long we stay this time around!

This means that we miss even more appointments, like our annual cardiac neurological assessment at UW at the CHDD clinic, our regular weekly Speech Language Therapy and Physical Therapy, his preschool classes at the Early Learning Center, his 6 month post-transplant biopsy and, most devastatingly, his gel injections.

He started preschool, though he missed his entire first week and only made 2 out of 4 days of his second week and missed his third week (and counting). It was such a big decision to send him in the first place but he's hardly actually been there, so the jury is still out on whether or not it's a good fit for him and our family.

It was a month of decreased verbal communication, comparative to what he was doing just before his gel injections "expired". While he spoke much less, he is much more verbally responsive to questions and conversations than ever before. He tries to tell stories, but since he can't say much we fill in a lot of the words for him. For example, we went to the zoo and saw the lion sleeping on a rock. So when he decides he wants to talk about it, Job will make a "roar" noise and we'll say "oh! are you telling us about the lion?" Job will nod and we'll ask "what was the lion doing?" and he'll say ""'leeping" and we'll say "yes, the lion was sleeping. Was the lion small or big?" and he'll say "big!" It's obvious that he now wants to talk but he's just very limited by his vocal cords. He sometimes gets a phrase out, like he'll say "juice yuck!" when we offer him juice or he'll say "no, no, water" as he's also signing "please" because all he wants in life is the taste of cool, refreshing, non-thickened water.

It has also been really really hard to get enough food and drink into him. He wouldn't have gained weight this month were it not for his obsession with chocolate milk (to the exclusion of almost every other beverage). Our goal is 30oz/day and it often feels like we do little else but try to get him to drink all day. That's certainly an exaggeration, but it is a constant concern and I always have a running ounce total in my head.

Despite these difficulties, we did have pockets of fun! We went on a lot of walks and to the zoo and to a Mariner game and started preschool and played with brothers and watched Paw Patrol and played with friends and celebrated his third birthday and looked for Easter eggs and discovered a love of chocolate milk and chocolate ice cream.

March 2019 Update

The various standardized testing we did at the end of February (for the school district) and at the beginning of March (for the NICU follow up clinic) showed Job's receptive language to be in the 98th percentile, yay! He understands most of what he hears!

But his expressive language was in the 5th percentile. This wasn't a surprise to us, but it was still disheartening to see such a discrepancy and to see "on paper" how very delayed his expressive communication is.

But then he just started talking! For the first time in his life he began to consistently imitate the sounds we made for him. It's as if it finally clicked that expressive language is magical. Say something and get a response! We really didn't do anything differently - we've always talked to him and read to him and modeled communication for him in dramatic ways, but with little to no appreciable response from him.

He's been in weekly SLP since recovering from surgery and I think it just took some time for him to feel better from surgery and realize he *could* make more sounds.

As he is about to turn 3 years old, we will no longer be eligible to receive our speech therapy through our local Birth to Three program (A Step Ahead Pierce County) and so I scheduled an eval with a SLP at Mary Bridge on March 12th. I sat there and told the therapist all about Job's long medical history and my concerns and frustrations but then also told her how excited I was to suddenly see such incredible interest and effort on his part. "He's talked more these last two weeks than he ever did in all the months previous, combined!" I told her, just bursting with pride and hope.

And then we came home and he took a nap. And woke up barely whispering in a throaty, raspy growling tone. I chalked it up to overexertion but when he kept whisper-growling all the rest of the week, I started calling Otolaryngology.

This dramatic change in his voice and the amount he spoke was concerning enough, but paired with a huge difference in his food/drink intake beginning the weekend of March 9-10th, this gave me enough "ammo" to convince Oto we needed an appointment sooner than our scheduled April 10th appointment.

Job's left vocal cord was damaged in his first open heart surgery when he was five days old, but then he learned to compensate with his right vocal cord to at least swallow safely. We couldn't conclusively attribute his delayed expressive communication to his damaged left vocal cord and when I started trying to advocate for more testing as to why he wouldn't/couldn't talk, his heart started failing and cardiac testing to get on the transplant list took precedent over Oto appointments.

But then his right vocal cord was damaged during intubation in his October 2018 transplant, and he was again aphonic and completely unable to swallow. I said I wouldn't leave the hospital without an Oto consult, because I had long be concerned with his vocal cord function and I wasn't content to just wait and see if his function would improve.

So on November 9th we gave a short acting botox gel injection in his left vocal cord. The procedure itself only took about 20 minutes, which was a surprise to me - I remember not even having enough time to go get tea before they were calling back to see him. Recovery seemed so smooth, too. Even immediately post-op his voice quality was incredibly improved! He ate and drank well (or at least well for an opinionated toddler) and began to make HUGE strides in his expressive speech.

Until (as stated) March 12th, when he woke up from his nap with no voice and a diminished ability to swallow.

We were just starting to feel pretty confident about making our way through cold/flu season unscathed when Job decided he missed all the attention and wanted a little hospital stay to spice things up.

I took him to the Mary Bridge ED in the middle of the night (on March 25th) when his work of breathing increased and his sats plummeted. I thought about driving up to SCH but felt he was in too much distress to drive so far. And sure enough we transferred up to SCH by ambulance so that the transplant team could assess him. He just needed to be on oxygen for a while and then headed on home.

It was a hard hospitalization because it was such a comparatively "minor" issue that I really struggled with bitterness and discontentment that we even had to be there, at the hospital, even while also being grateful we have such an attentive team so dedicated to Job's care.

Here's to hoping Job recovers quickly (even though immunosuppressed) so we can get that gel injection soon!

February 2019 Update

Job has a echo and clinic visit every month and we still have near weekly blood draws. His heart looks great!

We also had another set of ultrasounds on his left arm (to assess his clots from his central line placed during transplant) and his clots have resolved enough to get off of his lovenox injections! Hooray! He's back on aspirin, as he will be for life, but that's a simple half a tablet I grind and sneak into a beverage.

Overall, he's doing pretty well! We seem to be reaching that point where he's recovered from the trauma of surgery and now able to regain strength to get back to pre-transplant baselines, even taking in the drastic decompensation of July/August/September.

We are in the process of potentially enrolling him in our school district's developmental preschool, but are still considering the "pros/cons" list of such a big decision.

Physically, Job is doing really well. We're so grateful! But it seems that the more stable he gets, the more the rest of us fall apart. We're trying to work through the trauma of the last three years as we have been so focused on Job just surviving. One of the big boys is particularly struggling with a great deal of emotional pain and anxiety and so we're doing our best to love him through his big emotions, which is probably a helpful way to force us to talk about all of these hard things. I don't think I've cried this much since when we were interstage (in between the Norwood and Glenn)!

The healthier Job gets, the more clearly we see how very very sick he was and how very very close we were to losing him.

Our next big issue to confront is Job's (lack of) verbal communication! It's exciting to now have the opportunity to be proactive about aspects of his health and development instead of "just" reactive.  

January 2019 Update

Job had the tiniest cold this month and developed an ear infection. We expected that his body fighting a cold/ear infection would bump up his numbers (like BNP which is a little snapshot of how stressed out the heart is in that exact moment you test it), and sure enough, his numbers all skyrocketed enough that we got to go up to SCH for an extra visit. He was just fine, but we're grateful SCH is so very attentive.

He's actually so stable that Seth and I left him and the big boys for 48 hours in the snow at a youth retreat with high schoolers from our church. How amazing is that?

We also attended an award ceremony at A Step Ahead Pierce County to help present a grant to their organization and got to see Miss Janet, Job's former specialized instruction therapist who worked with him from July 2016-January 2018. He really didn't remember her, which just breaks my heart because she was such a lifeline for us, for me emotionally and for Job developmentally. I've long realized that Job wouldn't really remember his various providers but to actually see it happen already? I guess that's a large part of why we try to take photos and keep up this blog. 

December 2018 FAQ

I wanted to update on a couple of issues that we've been getting a lot of questions about:

NG TUBE:
Job is now tube free! It perhaps got caught on something because on 12/8 Job came running to me, crying and holding the tube half in his nose. Ezra held him down while I tried to get it back in, but it's really a 2-3 adult job, so I just pulled it all the way out and called to get permission to try all his meds by mouth.

He's doing well still, though it takes us alllll day to get his meds in, because there are so many and so many that taste terrible, but he's doing a great job with them! And as long as that continues, he doesn't have to have the tube.

FEEDING:
Job can eat solid foods by mouth! We have to be careful about hidden liquids and we have to thicken all of his drinks (to about a milkshake consistency). He loves to eat but now that the novelty of being able to eat again has worn off, he's being a picky two year old. What he loves one day he suddenly doesn't love the next day, but he is gaining weight.

IJ INFILTRATION/NECK WOUND:
Perhaps a week after his transplant, so mid October, Job's right side IJ infiltrated (leaked meds) and had to be replaced with a left side IJ. Because these central lines are so very important to give ("life support") meds, our first concern was to get a new line in. But many of these meds are toxic to the skin cells and the concern was that his cells would die at the infiltrate site and calcify and eventually necessitate surgery. The team acted so quickly to inject other meds to counteract the infiltration and it worked! He hasn't had any issues so far, though they will continue to assess.

CLOTS:
Because they need multiple access points while patients are on life support, after they placed the IJ on the left side of his neck they also placed an additional central line in his left arm. And when they pulled it out right before discharge, they found 8-9 clots in his left arm. So we've been giving twice daily injections of an anticoagulation drug (lovenox) to treat these clots. Job really really hates these shots and it's hard for pin him down firmly enough to administer it without injecting myself or somewhere else on his body.

Because taking a blood pressure or drawing blood could dislodge one of these clots, we can't do either of those things in his left arm. Which means his poor right arm veins are scarring up and becoming more difficult to draw from. We've basically alternated arms his whole life because he's had so many clots or because of that fistula he used to have in his right arm, so this isn't a new frustration, but because he already has small and squiggly cardiac veins, it doesn't make for fun blood draws.

The weird clot they found in his left leg (that must have traveled down to his leg, since he didn't have any lines in his left leg this time) has resolved, but the clots in his left arm have not fully resolved. The ultrasound he had earlier this week found that they're smaller but still potentially dangerous. So we have to now do another 6 weeks of anticoagulation therapy.

DONOR FAMILY CONTACT:
We are working on a letter to the donor family and will send it to the organization that facilitates contact in the next month or two. But they won't give it to the donor family until a year after transplant.

If the family would like to make contact with us, they can then do so, and we can exchange a round of letters without specific information, and then eventually can share such information and even meet someday if we'd like. The donor family gets to direct our interactions, as of course they should.

THERAPY:
Job is receiving weekly PT and weekly SLP therapies and we're so grateful for both of them! He's still rather delayed but is making great progress, and we anticipate being in weekly therapy for most of this next year and then perhaps spacing out our visits to twice a month or monthly for the next year after that.

I love that people ask about these things because they have all been such areas of concern, so that people are asking means they have been praying for us specifically on these issues. I can't ever express how encouraging that is to us.

Here's a bit of video I took earlier this week of Job invading the children's choir at our church Christmas pageant. He loved being on stage so much, he took every opportunity to run up there even when the sheep weren't really supposed to be on stage. It was a really sweet visual, however, of how this new heart has given Job such energy and an enthusiasm for life.

https://youtu.be/hlZ2NNbx5Ik

December 2018 Update

Job is loving life. He has so much enthusiasm and energy and practically bounces off the walls - oh, and while talking constantly!

I'm absolutely exhausted keeping up with him but am so very very grateful to have the opportunity to be worn out from attending to his needs instead of, quite frankly, grieving his death.

Sometimes it's really hard to be content with all of the things we're being asked to do for him. I really want a break from all things medicine, to be quite honest. At the beginning of this week I was feeling myself slide into resentment because I had three days of appointments at SCH and therefore 9+ hours of driving Seattle traffic and childcare to figure out and I'm really annoyed at his tacro levels. I asked a few dear friends to pray for my attitude; for stamina and gratitude and patience, because I know quite well I can't spontaneously cultivate those things myself. I lose sight of the big picture - the big picure of Job's whole life, let alone the bigger picture of the whole of human life - and get bogged down in my daily troubles... Then I (sometimes) catch myself in my ingratitude and start preaching to myself... But sometimes I wallow for days first. At least right now it seems I ricochet from one extreme to the other, with little to no balance.

One particular daily trial is that his tacro level has been all over the place these last two weeks, from a low of 4 to a high of 26.4, instead of staying anywhere in the 10-12 range like it's supposed to. We're frustrated and concerned (and very sick of near daily blood draws), as are his doctors, though they are more confident we'll find the right dose than we are at present. This is his most important medication to manage his rejection and is essential to get in range asap. Too low and he's at risk of rejection (even with juat a few days of too low a dose!). Too high and the side effects start piling up.

His cardiology appointment a few days ago was fantastic though. The team is THRILLED at how well his new heart is doing! He looks and sounds great.

The doctor actually came into the exam room just as Job was racing across it, running away and squealing with joy as he played hide and seek with the big boys. She just started laughing and joked that was all she needed to see - he must be feeling better.

He has some mild tricuspid valve reguege still so we can't yet wean off his diuretics or the supps to replenish his electrolytes that the diuretics are stripping. So we're still on lots of meds, but as his heart grows to better match his body's needs then we should be able to wean down.

He really hates his sodium chloride though and I think it is becoming quite a problem as he tries so hard to refuse it and sometimes spits it up. It tastes horrid (can you imagine drinking an ounce of salt walter 3x a day?), so I don't blame him at all. Buuuttt he needs to take his meds no matter the taste. I don't want him to get used to gagging and refusing a med and then that turning into refusing all meds. We're going to try putting some of them in food this week but that gets tricky because then he has to eat all the food instead of just swallow a syringe full. Other meds make him queasy so now that he's taking everything orally we're essentially giving meds all day long, to space them out. It won't be sustainable long term but it's helping now while we have so many meds.

All in all, he's doing really well and we're so thankful.

Official: Not Treating Rejection!

It is now official: we are not going to treat for rejection.

Diuretics have made a world of difference and now his echo looks great and his BNP has reached baseline. His tricuspid and mitral valve are no longer "leaking".

We now have to figure out if we want Job euvolemic (to have a normal fluid balance) or if he needs to be dry or wet. His electrolytes are all out of whack now, so they are also determining which supplements to give.

And all of that will probably take a few days.