Mid June 2019 Update

We had our monthly transplant clinic earlier this week.

On echo, his heart looked better than EVER before!! They're not worried about rejection but we're all still waiting for his biopsy for a more thorough confirmation. We've rescheduled his biopsy multiple times since April because of Job's cold or SCH's mold issues, but he's on the priority list so perhaps it will happen sometime in July.

It's possible that, pending biopsy results, we might go to every other month appointments. :O



Job switched to a new immunosuppresant (evrolimus instead of MMF). This has shown to be a better long term med in transplanted adults, so several centers nationwide are switching their kids over to it as well. Three weeks in, they're still happy!

It's actually pretty annoying to cut it and crush it, but MMF messed with Job's bowels so maybe it's a fair trade off. We're also slowly decreasing his diuretics, so maybe we can finally potty train sometime soon. Poor Job has been so interested for so long but med side effects haven't allowed him control over those bodily functions.

I just got the call yesterday to set Job up for vocal cord surgery on Tuesday, the 18th. This too has been rescheduled 4 different times and they said they might call to cancel it this weekend, but we're hopeful it will happen. In addition to the gel injections, pulmonology will do a battery of function tests in an attempt to get a better idea of how to optimize Job's lung function.

And we finally got intensive SLP appointments set up to work on Job's articulation. Yay! His speech is my big focus for this summer.

May 2019 Update

Job is really enjoying his newfound energy and stamina. It's awe-inspiring to watch him walk from our car to one of his brother's baseball games, play while he watches said game, and then walk back to the car. We carry him sometimes, but for safety or convenience or cuddles, not because he *cannot* propel himself forward.

His cares and med list are still long and exhausting. We still have many upcoming appointments and procedures. We've never experienced that fabled time of stability and rest and growth that doctors keep telling us exists. But I think that's always going to be our life with Job. Last summer one of the ways I tried to convince myself that Job needing a new heart wasn't horrible was to imagine how easy life would be without HLHS and the imminent threat of death. Now death seems less imminent because Job is very stable from a cardiac perspective. But everything else is hard in different ways and I'm learning to give up some of my misplaced hopes and expectations.

It's a weird place to be: we're so dearly grateful for the mercies we have experienced but we're still grieving a great deal of loss and trauma.

The greatest frustration right now is that it has been three months since Job's vocal cord gel injections stopped working. His surgery to re-inject the gel and do some other vocal cord work and do a number of lung assessments has been bumped. This is our fourth time rescheduling it, because Job was sick or because they had kids needing surgery more emergently. We hope they'll have an opening soon because Job's abilities to breathe, eat, drink and talk are all so affected by his broken vocal cords. It is beautiful that he is functioning as well as he is right now, that we're not the ones bumping someone else from their surgery because we need this surgery today! But it's also sad and frustrating.

Post-Hospitalization Update (May 2019)

Job is home from the hospital again!

We were pretty frustrated to have two different 11 day admits in a mere two months and pushed hard for further assessment. Of course, being immunosuppressed, he's more susceptible to illness and struggles to recover from said illness, but was that "all" that was wrong? Was there anything that could be done to optimize his respiratory health?

We were all pretty sure that the biggest answer to these questions is just that Job really needs his vocal cord gel injections... but he can't go under anesthesia while recovering from these viral illnesses and we've therefore rescheduled his injections three times.

He's always struggled greatly to get off of respiratory support but we've always just chalked this up to his broken heart.

So Pulmonology came by while we were at the hospital and did a very thorough assessment. Apparently there are six pulmonary "compartments" and Job had problems in five of the six. So we started a whole list of new meds and are scheduled for a battery of new tests and procedures. I'm really thankful to start to optimize his respiratory health but am also daunted by how much we need to do.

In the week he's been home he's been so energetic and fun. I was going to say that he hasn't been this healthy since February but really, he was still recovering from transplant back then so really I'd probably have to say it's been at least a year since he's been this active! I took him to the park a few days ago while the big boys were in class and he was running all over the place and didn't even get tired!

Spring 2019 Hospitalizations + Agnst

Job was born with such a serious heart defect that it was an easy explanation for his various physical ailments. Then his quick decline last summer meant we ignored other potential issues, if we even noticed them.

We had various conversations about his lung health, for example, but how much investigating did we need to do? We KNEW he had a heart defect that was causing his ever worsening issues. His poor oxygenation and very very slow weans off respiratory therapy seemed perfectly consistent with his CHD. We sent all of our home oxygen supplies back to our home care company back in November because a new heart had us all hopeful we wouldn't need it again.

Then he got a new heart. He was severely decommissioned from his decline all summer AND from surgery recovery AND was on high levels of immunosuppressants, so we expected when/if he got a cold that it would severely affect him.

So when his sats plummeted at the end of March we were really disappointed but not terribly surprised that we needed to admit for 10 days of oxygen. I really struggled during that hospitalization not to wallow in bitterness because, frankly, I'm sick of hospitals. I want to be home. I've been waiting and waiting for Job to be healthy enough so I would get to be home with all three of my kids since what, February 2016 when we got the initial diagnosis? We've never reached that level of stability and keep waiting for the next thing we need to do that will finally give Job that clean bill of health.

We were initially told that his first open heart surgery (the Norwood) was risky but that if Job survived it and then made it through the next six months (the "interstage" period) to his second open heart surgery (the Glenn), then life would get easier. He'd be considered medically fragile, of course, but would start to thrive. At 3 or 4 years old he would need another surgery (the Fontan) but then after that things would really get easier.

Of course, none of that happened. I don't want to dismiss the fact that so many babies born with HLHS die in the first six months. We recognize that, through God's mercy, Job is still alive! But he's not following the expected pattern. Kids with HLHS who survive the first six months are supposed to start thriving. They aren't supposed to go into heart failure when they start walking, as Job did last spring. My plans, adjusted though they were to include two open heart surgeries and an eventual third, started to crumble yet again.

I searched and searched to find any other toddlers with HLHS needing a transplant. It seemingly doesn't happen. For some reason Job has complication after complication, needing various surgeries and interventions and new consulting teams and new therapies. He doesn't die. He has survived. But he hasn't thrived. At least not like I expect him to. My entire life has been a struggle of laying aside *my* expectations and plans, but certainly I get to practice this in an especially intense way with Job. Or I think I practice it. I'm not so sure that I ever actually hold my plans in a completely open hand. I loosen my grip on said plan a little bit and then congratulate myself that, 'look! I'm being so trusting!'

I'm weary. I clung to the hope of transplant to "fix" all of Job's issues. I really did, even as I got frustrated with people who also saw his transplant as a "fix". 'Job will always have significant health issues,' I would mutter in my head, 'even with a new heart. And he will almost certainly need another transplant some day. He's not fixed.' In actual fact, though, I got really really excited about things getting easier.

So when we had to admit in March, I was furious. I wasn't worried about Job. His heart was fine. He just needed oxygen. I was just annoyed. We finally discharged but he never really fully recovered throughout the month of April. Various assessment appointments I had been looking forward to were cancelled and as the weeks wore on. And he started really struggling again.

We kept him home and just kept a close eye on him until we decided enough was enough and brought him back up to SCH for another admission. I'd been convicted all month that my attitude about our last hospitalization was wrong, so for the first week we were here at the hospital I was even pretty cheerful. I did a better job of preaching to myself about being grateful for each day of Job's life and for such an attentive medical team and people bringing us meals and caring for the big boys and checking in on us and praying for us. I told myself all the things I know to be true about relinquishing control over my timelines and expectations and trusting God's sovereignty and refinement through trial.

I threw myself into advocating for a Pulmonology consult to try to get some answers about Job's lungs and vocal cords and how we can optimize his respiratory health to then diminish our respiratory support.

And I got a lot of what I wanted. We made so many changes this week and started so many new meds and therapies that seem to already be really helpful. We have 10 some new, additional appointments on the calendar for this summer. We should be able to get to the bottom of all of these Pulmonology issues.

But now that I don't have to advocate for something I'm feeling bereft. We don't expect to discharge until next Monday, the 13th. It's hard to love on the big boys and Seth from up here. Job started steroids and is raging. He's missing his preschool classes and new speech therapy. We were on such a strong finish to the school year but that has now collapsed. It's sunny outside and I want to take the boys to parks and museums. Basically, all of my plans have again crumbled around me.

I'm trying to find joy and contentment and just be still in this. It helps that I'm still very convicted by my April pity party. But I feel weak to be able to persist even though the next four days we plan to be here at the hospital. And what happens if he gets another cold? We have another admit?

So, there's my bare confession. It's been a rough post-transplant seasons, emotionally for all five of us. It's been easier to keep it all tucked inside. But that gives the false impression that this is easy. It's not. I can't do it on my own strength. But I'm also struggling to "cast my cares on the Lord" and actually trust that He will see us through. Even though I know that He has already been faithful day after day to do so in Job's life. In my life before Job. In all of human history before me.

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.