October 2019 Update

Today is the one year anniversary of Job's heart transplant.

Seth wrote a letter to our donor family a few weeks ago and I got to hand it to our transplant team this week. It's up to them if they want to receive it or ever respond. We will be so happy to let them direct our interactions.

When we first started talking about heart failure and possible transplant I started wondering how to think about it. What to say about it.

How do you possibly ever articulate such immense gratitude?

We talked about having some sort of family celebration to acknowledge this day, because it feels like the most monumental day in our lives. The big boys were especially troubled by this idea, however, because how do you celebrate a day that is also such a monumental day of pain for another family?

Ultimately, we didn't really get to decide how to acknowledge this day because Job decided for us. He had his annual biopsy a few days ago and needed a great deal of oxygen during the procedure and then spiked fever after fever in the PACU and CICU. When febrile his heart rate and respiratory rate skyrocket, so we're still inpatient on quite a bit of oxygen and don't expect to go home for at least a few more days. Though he's not showing typical cold symptoms, he did swab positive for a virus so that seems to explain at least some of his recovery issues. It was a great relief to get a viral explanation because, of course, everyone's mind immediately flies to possible rejection. His biopsy results came back completely clean, however! No rejection.

He is terribly irritated to be in the hospital and to have extra cares and to feel so sick. This is the first time he's been able to express himself verbally while hospitalized, and so he's letting us know that he "really, really, really hates it". He mostly ignores his providers and me but if he does acknowledge us then he does so with utter scorn and derision.

I've joked to some of the transplant team that they're so nice to him that he keeps wanting to come back for more attention and to celebrate his transplant in the very place it took place. All "joking" aside, SCH takes such wonderful care of us; we're so very grateful for this place and these people.

I think this admit and its drastic change of plans for our week underscores what transplant life means. What CHD means. We are immensely grateful for this beautiful gift of a new heart. But it didn't fix Job. He will always struggle to stay marginally healthy. He will always be in and out of the hospital. He will always need close monitoring. He will always mess up my plans. We're so incredibly thankful to have this extra time with Job but it's still really hard and it will always be really hard. I've struggled for months to write any sort of update because of this tension but I've been realizing that this will always be a tension we will wrestle with and I won't ever be able to articulate it well.

If I'm being completely honest I don't feel very celebratory. I'm so frustrated to be at the hospital again. And that feels like such a betrayal to our donor family's sacrifice. I struggle to feel all my feelings and still hold them accountable for what I know to be true. This is really hard AND I'm really grateful. This really hurts AND God will use it for my good and His glory.

August 2019 Update

Job continues to do really well. We still have lots of concerns about his vocal cords but are working with Oto to schedule that nerve re innervation surgery this fall.

He's adding new words every day, which is just thrilling. It took him so so so long to say anything and now he just won't stop talking! It's very hard to understand him, but he is talking!

July 2019 Update

Job is now 9 months post-transplant and, in so many ways, really thriving.

His heart is so stable and we're just thrilled. We don't have to see cardiology until he has his annual biopsy in September! September!! That's two whole months between cardiac appointments! What is this?

The only other time we tried to go two months between appointments was back in January 2018, but we only made it a month because as soon as he started walking we knew something was wrong and indeed, he was in heart failure.

The main focus this summer is speech therapy and helping Job advance his expressive language capabilities. Weekly speech therapy has been very helpful but we're also working with Pulmonology and Otolaryngology to try to optimize his lung and throat capabilities before we hit cold and flu season.

His vocal cord gel injection back in June helped a great deal - he can now speak with more volume and perhaps a bit more clarity, he can now swallow safely although still on very thick fluids, he can move his body

We haven't hit any of his "transplant" anniversaries yet but we keep marveling that he can do xyz "summer thing" this summer because last summer he was declining so rapidly. We're just starting to write a letter to our donor family because we can send it to them in October, at the year mark. But how do you write such a letter?

We're well aware that this transplant won't last Job's whole life. It may not even last through his teens! We're well aware that he will continue to have many doctors appointments and he will always be on medications and he will need therapies for many more years. As he runs (he runs!!) and plays this summer and everyone around us is (rightly!) excited about his very good health, I struggle to know how to hold these things in tension. My excitement and oh such deep joy that he is still here for me to hold and play with is tempered by my awareness that this gift has come at great cost to our donor's family (and to our family).

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.