Tomorrow's Decision Day

I was trying to explain to my friend how very odd this hospitalization is.

Job feels fine. He's irritated by the pokes and confinement, but he's getting close to his pre-transplant attitude plus with more energy. He actually probably feels better than fine. His *smile for the camera* still looks like a grimace though.

He had a grand ole time today, with his brothers and toys and room service food delivery. He even started insisting on sitting on the toilet rather than wearing his diaper, but we'll see if that lasts.

Tomorrow is the big day: have the diuretics made enough of a difference to definitively push aside the suspicion of rejection?

His BNP has gone down and he's been peeing well and now his Tacro is in range - all good things. He'll get labs at 8:30 and an echo around 9am or so and then the team will convene and hopefully be able to make a decision.

If it's clearly that his too-small, stiff-from-being-on-ice heart couldn't handle being fluid overloaded and the diuretics did indeed make a big difference then I'm not sure what we'll get to do. Go home, but when?

If they can't clearly see the diuretics made a difference then we treat for rejection, even if there aren't any other clear signs of rejection. And that will mean at least 4 days of further treatment here.

Thanksgiving Day

We spent today playing and eating, just the five of us.

Despite intensive diuretics, nothing changed on Job's echo... which is very disappointing.

He gets two more days to keep trying diuretics and if nothing else changes then we have to start treating rejection. Even though the biopsy came up clean, if fluid retention isn't the answer for his issues then rejection must be. Although it's not ideal to give Job such high doses of steroids with confirmed rejection, giving unnecessary steroids is a small price to pay compared to not treating rejection at all. Something is wrong with his heart and they have to treat the worst thing.

His Tacro dose is climbing up, closer to range, but his dose also went up since it's not in range yet.

He's pretty happy and active and enjoyed spending the day off monitors, wandering around his room playing with his big brothers.

The big boys and I ran over to the Ronald MacDonald house and picked up a turkey dinner and brought it back to our room. I'm so thankful for the legions of volunteers at RMAC who daily donate their time and resources to feed and house families at SCH. I wish, mostly for the big boy's sake, that we could get a room there and they could spend the night but I understand and fully support giving rooms to families from much further away. It's just a big blessing that they now give out day passes to families like us.

I've been thinking all week about the timing of this hospital stay with the Thanksgiving holiday and really, I've been thinking about this since we listed for transplant. I'm thankful for Job's life... but I don't know if I'll ever be able to understand how grateful I am, let alone ever express it. I've almost felt numb to the emotions of all of this (this week and this past month) and I think that's largely because I can't even comprehend the magnitude of it all.

Biopsy Day Updates

9:00pm update:

The biopsy results came back.

Showing no sign of rejection.

We're all surprised - the symptoms Job is experiencing seemed to match rejection so well. His Tacro dose was so low that the bias was immediately towards rejection and necessarily so, because treatment had to begin.

They stopped his intensive course of steroids (methylpred) but can of course restart it if it is determined later that this is rejection that just didn't show up on biopsy.

It's possible this is a false negative and so further monitoring is needed in order to determine what is going on in Job's body.

He'll get an echo in the morning and more labs and the focus will probably be on finding the right course of diuretics for him. We'll need to stay here for several days, probably not to treat rejection  but to figure out what we need to treat and how. I guess Job just likes to keep his team busy researching.

Job is back to himself after this morning's activities, engaged and interactive. 

He's not quite back to his pre-transplant self activity-wise, but this little hospital stint doesn't seem to be negatively affecting his attitude much (at least not yet). He spent most of his time out of his crib and had our full attention and a steady stream of new toys from Child Life, so he was pretty happy.

5:00pm update:

Job drank 38oz of thickened juice from 12-5pm today. And he's eating a lot of food as well.

He's drawn and played with blocks and animals and done puzzles and watched his favorite video multiple times. He's been mostly happy - some pain at the incision site for his biopsy at his right jugular vein.

12:00pm update:
Job is back to his room and recovering well from the procedure!

All signs point to rejection (increased pressures and decreased function and fluid overload) but pathology is on a rush order to get his samples processed as soon as possible to make the official declaration.

We're starting treatment as though he's in rejection though because he probably is. He'll be on high doses of IV meds and we'll get lots of labs. A discharge this weekend could be possible, I suppose, but we'll see how it all goes.

Theoretically he'll be happier this admit because he can eat and isn't on viral precautions but he is starting mega doses of steroids so maybe we'll just be back to raging.

8:30am update:
Job is already off in the cath lab!

Their first case was cancelled and Job fell asleep and stopped eating/drinking early so he was eligible to go! (So glad I didn't wake up to my alarm in the middle of the night to force feed him in the middle of the night as I had planned to do!)

Going early means less time NPO which means less time grumpy! We basically woke him up to give him his pre-op "bath" and then whisked him away.

He should be back in his room by 10 or 11am and, because this is probably just a biopsy (measuring right side pressures and taking tissue from the right ventricle) and not a cath (assessing his vasculature and measuring venous and arterial pressures) they will only go in through the neck which means he doesn't have to lay flat for 4 hours like he would if they needed groin access. That's so great! It has been so hard to lay still as needed the last few caths.

Possible Rejection

Well, we brought Job to the Emergency Department at SCH this afternoon to assess for possible rejection.

He is scheduled for a biopsy tomorrow as second case (so around 11am).

In the biopsy they'll go in with a catheter through his neck vein and snip off pieces of tissue from his right ventricle, which should go to pathology by afternoon. We find out whether or not he is in rejection (and what kind of rejection) tomorrow evening or Thursday morning.

Rejection is treatable but certainly this is hard news. Frankly, we don't even know enough about transplant yet to have perspective on all of this.

He's not terribly upset when he's eating/drinking and not being poked.

More details:

Tacro is Job's primary immunosuppressant medication and his "level" of it is supposed to be between 10-12. It was a 6 last week so we upped his dose and drew labs again yesterday morning. Those came back today at a 4. So we upped his med but his level still dropped.

Also yesterday we noticed that his face was pretty swollen and puffy and his stomach was huge and firm. The team had us give extra meds last night and he did look less puffy this morning, but we were already on high alert when his Tacro level came back so low today.

So we packed our bags and drove north. He's had his echo and EKG and x-ray and a whole team of people assessing him.

He has some fluid in his left lung but the main issue of concern (other than his inadequate Tacro levels) is that his mitral valve and tricuspid valve reguritation is moderate after two echos with no regurge (one each week).

He could just have excess fluid in which case we'll up his diruetics and go home. But the head of the transplant team told us he'd give that about a 20% chance.

Or we'll stay 3-7+ days and treat for rejection. And reset the clock on his intensive course of immunosuppressants and steroids (when we were already on week 5 of 12).

Good bye, Home Care Oxygen Supply!

I'm thrilled to be sending our tanks back and yet also packed them up with tears in my eyes.

I hate our old CHD. Hate it! But I miss it too.

Hypoplastic Left Heart Syndrome was familiar. After studying it for almost three years, it finally felt comfortable. Normal.

I could do single ventricle care. I knew the procedures and medications.

The projected outcomes were still terrible but I understood them. I had sorta kinda made peace with them.

I was really invested in the current research focus on the Fontan.

I knew the SV doctors and nurses and medical assistants.

I hardly needed our pulse oximeter to accurately assess Job's oxygen saturation at that particular moment because his color and work of breathing and energy levels were so telling to me.

I wanted to keep his native heart, as broken as it was. As scary as the projected outcomes were for Fontan physiology.

So even though I now look at Job and marvel at how pink he is and weep with gratitude that some other devastated family chose to donate life, I'm also grieving the loss of HLHS deeply.

And giving up Job's oxygen supply is a very tangible reminder that we have a totally new disease now.

Grief is weird. Emotions are weird.

These last five weeks have been so very full I haven't been able to do much beyond just the next step. Processing anything emotional or spiritual has seemed a superfluous luxury, but very much needs to happen.

The big boys are probably most helpful to this process with their constant questions. And can you even imagine explaining all of this to Job someday?

VFSS Results

JOB PASSED! HE CAN EAT AGAIN!!!!

Oh, I couldn't be more excited but I think that pales in comparison to Job's excitement.

Job got to sit in this special little chair and I gave him food/drink with barium while they took x-rays.

He swallowed food and purees just fine but aspirated on thin liquids (regular water/juice). So then we tried nectar thick liquids (juice with a thickening agent in it) and he aspirated, so then we tried honey thick liquids (juice with extra thick thickener in it, to the consistency of a thick smoothie) and he could handle that. YAYAYAYAYAYAYAY!

Our first stop after the appointment was the cafeteria and I bought him whatever he pointed at.

He even asked me to stop midway home for more food! And then once home we scrambled eggs and he devoured four eggs before I made him take a break.

This means that the intervention (gel injection) Oto did last week worked! We assume it's his right vocal fold working to overcompensate and reach to the plumped up left vocal cord to give him voice but also to protect his airway as he swallows.

It's just not all the way back to pre-transplant baseline to close in time before the fast moving thin liquids approach his airway.

We'll reassess in about 90 days with another VFSS but we'll see Oto in 33 days to talk about whether or not we need to do a more permanent intervention.

First Week Home

Short version: Job has been home four days now and transplant-wise is doing great! Attitude-wise it's been pretty rough: he's grumpy because of steroids.

Long version: We had our first outpatient transplant (TX) clinic today and his cardiologist was very very pleased. His Tacro (main immunosuppressant) level was really low, so we're going up on his dose, and Job was vascularly dry so we're taking away one of his diuretics and adding more fluids.

We're also working to increase his calories and get some real food blends into him. Thursday is his swallow study so it's possible he'll get to start eating some foods by mouth after that assessment, but in the meantime we'll start giving fruit and vegetable purees by tube 6x a day. It's a lot of work to get his feeds ready and to make sure we're on time with them. Much more work than I remembered from two years ago when we were on tube feeds, actually.

It's hard to avoid eating or drinking in front of him - we have to be very calculated about who will go eat where and when and who will entertain Job while everyone else is eating. But it's hardest to avoid talking about food and drink in front of him! Wow - food is a common topic!! And we're all a bit dehydrated because we can't carry out water bottles around and drink from them all day.

Every day Job gets stronger and more active and can stay awake longer. He's actually sleeping pretty well at night - better than we were expecting!! We're starting up PT and SLP therapies next week.

His meds aren't very difficult. It's actually an easier schedule than we were on pre-transplant. Two of his meds are very time sensitive and have very precise blood draw times, so that is a bit of extra work, but it's not so bad. I almost have all his meds memorized by name and dose and bottle size/color and med color, which happened more quickly than I was hoping and makes life much easier. I could have drawn up his old meds with my eyes closed and was apprehensive about how much extra time it would take to learn his new meds, but for naught!

'Roid rage is the hardest part of post-TX life right now. Job gets so very angry so many times throughout the day. He's most grumpy with me but will play for moments of time with his brothers or dad or grandma and grandpa. He's really sad to not eat by mouth and keeps asking for food/drink politely... and then angrily. And he's deconditioned from surgery and all that bed rest, so he's irritated about that. But he just bursts into rages all of the sudden. Sometimes as (over)reactions to some provocation but sometimes completely randomly. We'll be off steroids on November 3pth and that day couldn't come soon enough.

He likes walks in the stroller outside and baths always but is rather fickle about other activities and people that he did like pre-TX.

It is completely understandable that he's this emotional. I can't even imagine how scary and sad and frustrating and confusing and uncomfortable his life is right now (and I am usually the one forcing him to do xyz)! But it's definitely hard to figure out how to encourage him and entertain him and take care of him right now.