Vaccinations and Listing Info

Job's favorite thing right now is keys, followed only by his love of trucks. So he was pretty pleased that he got to sit in the truck and pretend to drive.

Job will be presented to the selection committe on Wednesday, August 1st. They will review all of his evals and determine whether or not to list him for transplant.

Later today I'll take Job in for his second set of MMR and varicella vaccinations. Typically these would be given around age 5, but because they are live vaccinations we're accelerating that timeline as Job cannot ever have a live vaccination post transplant.

And, of course, if we list then we must be ready to recieve an offer that day, so Job cannot be listed until he is sufficiently vaccinated as per the recommendations made by the Infecious Disease team.

He cannot be listed until three weeks after receiving live vaccinations (which MMR and varicella are), which would put us at a possible listing date of August 17th.

There's a chance SCH would need additional testing and there's a chance that they will determine he is not a candidate for transplant, but we don't currently have reason to anticipate these outcomes. This week will be an exercise in patience and trust because nothing is official yet and we want to hold our expectations in check.

Final Transplant Eval

Today was Job's last transplant eval appointment!! (Unless they ask us to come back for some other testing.

Job really enjoyed his extra, exclusive time with Daddy and didn't fuss too much about his ultrasounds (which he slept through!) of his lab work.

One really exciting thing to me is that we officially got approval to do right arm blood draws! We haven't had right arm access for at least two years because of blood clots the first year and because of his axillary fistula this last year. Now that the fistula has clotted off we're not worried about blowing the fistula with the needle. His left arm is so scarred that he's often a hard poke, but now he has an arm fresh with untouched veins!! Lab draws have always been such a simple but sad thing.

Nutrition clarified some of our questions about foods prohibited post transplant.

We met a new-to-us cardiac anesthesiologist and the other transplant coordinator we'll be wotking with, should Job be listed.

The most beneficial part of this week was the 1.5hr meeting we had with Dr. Nuri (the surgeon who has done all of Job's surgeries). He didn't give us much new information but he carefully sumarized the entire transplant process with just the perfect amount of careful detail. That he's a doctor we know well (and so greatly trust and respect) made his synopsis even more valuable. And we finally got a picture with him, something I meant to do after Job's first surgery and his second and third and fourth.



I have so much more information to process (through writing most likely) about what we're tentatively expecting in the next weeks and months but that will come later!

Penultimate Transplant Eval

Today went well!



We tried so hard to get an O2% read while Job raced through the hospital halls to "prove" how low he desats with exertion but had no such luck. The pulse ox doesn't read well with such activity.

His dental exam and blood draw labs were tear-inducing but went better than we expected. Yay!



One more set of appointments on Wednesday and then we wait for the selection committee to make a decision!

July 2018 Recap

We've had lots of fun in the sun all over Washington state this month. Job considers himself one of the big kids and doesn't slow down much. We've learned lots of tricks to make him take breaks but he's pretty stubborn these days.

The hardest thing right now is finding an appropriate balance of when to hold him accountable to what we ask of him (stop running towards the street, don't throw blueberries on the ground, no car keys, etc) because so often his response to a "no" turns into pretty scary breath-holding, purple-faced screaming fit. We can't actually let him cry even for a few minutes without medical repercussions... But we can't let him have or do whatever he wants either.

Our older boys were such early communicators that we didn't experience the "terrible twos" (age three was much harder than two!), so this stage of parenting such a strong willed toddler who struggles to communicate is definitely challenging. How do you parent special needs appropriately? So far lots of tears and uncertainty and prayer.

We got our schedule for Job's remaining evaluations. This week we see GI and then next week we see Heart Failure and Transplant twice, then pharmacy, infectious disease, dentistry, social work, psychiatry on Monday... and then two days later see nutrition, cardiothoracic surgery, anesthesia and then radiology for chest x-rays and two sets of 3 hour ultrasounds (one upper body and one lower), plus several labs. These will be very long days but it will be so nice to see so many specialists in a close period of time so as to be able to ask follow up questions to each discipline.

All of the data they've been collecting on his eligibility will then be presented to the selection committee at one of their Wednesday afternoon conferences (probably on August 1st).

Better Days

Job's hair had been driving me crazy so this morning I buzzed it. His brothers begged for a mohawk, so he has them to thank for his new stylish 'do.



Incredibly, the last four or five days have been really good days for Job. The downward trend we were seeing seems to have stabilized! It's not nearly as scary to wait longer for intervention as it was a few days ago.

He is much less active than he was two weeks ago, though. Perhaps he finally admitted to himself that it's exhausting to be on the go constantly.

He's using some of the energy he's conserving to be even more ridiculously silly because his brothers laugh and laugh at his slightest provocation.

Cath Postponed

Welp.

The cath was cancelled.

The cardiac anesthesiologist on Job's case had a family emergency and isn't able to to work tomorrow.

So it's now set for May 9th except I told them how worried I am about Job's rate of decline and so he's on a shortlist for cancelled appointments and we might admit if we need to.

We had an opportunity to take a slot this Monday but with a different interventionalist that I'm not familiar with and so I decided to wait for Dr. Rubio's next available slot. He knows Job's body and has been able to get femoral artery access when other doctors have not. It was a hard decision to make, though!

This will be subject to Job's cardiologist, Dr. Files, who might urge us to take the appointment on the 30th. But as long as Job takes breaks from activity, he's ok right now.

They're not going to bump someone else for him yet - though we've been on both sides of cancellations before... cancelling on us because some other kid needed an emergent cath and another kid getting bumped because Job needed an emergent cath.

We're watching him closely and have lots of numbers to call if we're worried. We'll see how he does the next few days though!

Upcoming Cath

And we're officially on for Job's cardiac catheterization for this Friday! I just spoke the the nurse and firmed up his NPO (no food or drink) and med times.

I think he's actually second case, but regardless will be in the OR for a few hours. We expect to speak with his doctor around 2pm or so and then I plan to spend Friday night there at SCH with Job.

A "cath" is foremost the best diagnostic tool we have to assess the state of Job's heart. A small spaghetti noodle sized catheter will be inserted in his left groin area or his neck and then be threaded through his vessels to 1.) assess the pressures and oxygen levels throughout his heart and 2.) locate any narrowing and 3.) check his heart function and 4.) monitor his tricuspid valve regurgitation.

But beyond diagnostics, a cath can also be interventional with options to balloon up a narrowed vessel and then employ a stent (essentially chicken wire) in a vessel to keep it from narrowing and thereby restricting blood flow. Job has one stent in his pulmonary artery that made a world of difference for his lungs last year and we expect some sort of intervention this time. Actually, we are desperately hoping for an intervention because that would bring some immediate relief.

This will be Job's fifth cath but they haven't seemed to get easier. I still find them really scary. In a twisted way, perhaps even more so than open heart surgery. Job has had 9 clots following his caths. His body is bigger and stronger now, and, most importantly, his vessels ought to be big enough now that there will be more room for the cath to move through them without causing clots and scarring (which have caused pretty scary and/or long term complications).

I'm also quite curious how a 2 year old with definite opinions and increasing awarenss of his independence will handle the pre and post op work. Especially the 4 hours of laying completely flat on his back following the cath.

This has been a hard week. Job has had some digestive issues and has been generally lethargic and sad. He gets really tired really quickly, like today playing at his water table. He didn't last long standing by it and walking around it before he was breathing so heavily that I made him sit down to play.

It seems that this has been the case almost every time we've needed an intervention: A few months beforehand I can't imagine giving my baby off to the cardiac anesthesiologist and watching him disappear down the hallway to the OR. But then we watch Job decline and quickly become desperate for intervention - we even wish it could be scheduled sooner. Not that I'm actually excited about it, of course. But we really love and trust SCH and being there has always been the most pleasant experience a hospitalization ever could be.

I don't know how I wrote so many words for a relatively simple procedure, but suppose you don't expect much less by now.

We really appreciate the prayers and child care and meals offered up to us this week. As I've said, you have been the hands and feet of Christ to us and we're so grateful.