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.

Muddling

We're muddling through these last weeks until the cath. It's been harder than I expected, both emotionally and practically, but there's only eight days left.

Job is so very excited to that he's walking. If he wasn't so proud of himself, I probably couldn't let him walk so much - but how do I stop him? Walking IS a good thing... It's just hard to watch him turn so blue.

Likewise, he wants to be outside all the time. His big brothers are outside, so that must be what big boys do! He asks so nicely, so often, but when we do go outside he gets so cold so quickly... And turns blue.



How do you find the balance appreciating and even encouraging such a huge developmental skill (that we have worked SO hard to achieve) that is also hurting him? How do you mostly deny requests to be outside in the fresh air, playing with balls and looking at new spring plants?

I wish he'd be interested in vegging out and binge watching some TV instead. 🙄

It's also been a rough month because we've had a good 4+ appointments a week. I'd forgotten how consuming such a schedule is - however did we make it through the first year of Job's life?!

So that's a weird place to be too: so grateful for so many people working so hard to help Job... But also really wanting to get off the merry-go-round and just stay home and let my toddler walk around outside.

I'd really appreciate prayers for patience and trust and contentment because all of those things are really hard to come by this week.

Interventions Ahead

Job is now walking!

But his heart can't currently sustain this increased activity so he needs intervention this month.



Job was fitted for orthodics in mid February and, amazingly, began taking independent steps the next day.

He's gained confidence really quickly and, over the last two weeks, has completely quit bottom scooting. He just walks everywhere!

But over these last two weeks we've watched him turn bluer and bluer with each subsequent step.

So at our regular cardiology appointment last week we did a stress test, hooking Job up to a pulse oximeter while he walked the hallways (with his brothers' encouragement): video

Job doesn't get enough oxygen-saturated blood and never will. Whereas you and I "sat" at ~98% (that is, 98% of our blood is saturated with oxygen) and with saturations of 90% would be admitted into hospital care, Job will at best sat between 75-85%. (Which has and will continue to cause organ injury and function impairment - we just want to limit this as much as possible.)

When he got off of supplemental oxyegn in August he was satting in the low 80s but we've watched his numbers slowly drop over the last 6 months to the mid 70s. This alone told us he would need some intervention sooner than later but we weren't really expecting to need to do something so soon (I guess I had next fall in mind).

Walking (a sharp increase in energy expenditure) has now forced this issue. At rest in the office, Job was satting at 78. He walked about twenty feet down the hallway and dropped to 50. We had him stop and rest and he climbed back up to the mid 70s. Then we had him walk and he dropped to 48.

It is so exciting that Job is walking! And it's scary. He very visibly turns bluish purple, first in his finger tips and lips, then his cheeks. Then he starts panting for breath. But he's so excited that he can move around like a big boy that he just keeps going and going. He does eventually get really tired and finally sits down but right now I'd rather he just never walk.

Cardiology didn't give any restrictions - we don't want to halt such huge developmental progress! It would be great for him to be on supplemental oxygen but it's virtually impossible to put a toddler on oxygen without using hand restraints and we're not going to do that.

But we need to intervene.

So we have a cardiac catheterization scheduled for April 27th. Caths are the best diagnostic tool to assess the state of the heart. How is the function? How is his tricuspid valve regurgitation? How are the pressures in his Glenn? And why is his blood this oxygen depleted?

We expect them to balloon some vessels or place a stent or two and therefore we plan to stay at SCH for at least one night. But we're now also tentatively planning on a surgery this spring. This will absolutely depend on the results of the cath, but Job's little half heart needs assistance and likely more than a cath can provide right now.

We had a long year off from intervention and I think we were lulled into a bit of a false sense of normalacy. No hospitalizations and only one ED visit!? So this news, though not unexpected, was and still is jarring.

It's also been a hard month as many of our heart friends have been in and out of their respective hospitals for various complications and surgeries and we've also had two deaths at our church. It's been impossible to blissfully ignore cardiac complications and death, like I at least pretended to do for the year previous. The timing of Good Friday/Easter and Job's birthday in all of this is, of course, not coincidental and has also been a large part of our emotional reflections.

We know that God is moving in Job's life for our family's good and for His own glory and we are resting in that, even in our sadness. It's important to grieve and it's important to preach truth to ourselves.

Practically speaking, we need to keep Job cold free over the next few weeks or his procedure gets bumped and we need to find that balance of letting him walk around and gain these necessary skills... but also swoop in and pick him up when he's blue in the face but not willing to take a break yet. We're on high alert right now, hoping his desats hold steady until the cath and monitoring his O2 closely.