Echo of Hope

Action stations. Actions stations. Set condition one throughout the ship. This is not a drill.

Why blog?

Why start telling a story when we so obviously don’t know the ending?

Why invite everyone in to our ‘house’ with the laundry all over the floor, the dishes piled up in the sink, and the leftover cashew chicken having spent more than a day too long in the fridge?

At first, it was practical. Each mini-chapter of the story seemed to take some telling to cover all the bases. By the time we’ve told it 5…10…15 times we get pretty tired out. The 16^th person deserves to hear the update as much as the 15^th, but by that time the telling tank gets pretty empty.

Then it was therapeutic. Getting the words down helped us wrap our heads around, and come to grips with, what happened at each turn.

Why do we keep the blog going now?

It gives Ari his voice.

And it allows us to bear witness to his fight.

He can’t say how much he misses his room at home. How he’d so rather be doing something else besides whiling away the time until the next echo. How much it hurts when they have to stick him again and again to draw labs.

That he’s afraid.

That he’s here.

Damn right he is.

But being here hasn’t been easy. It’s not about to get easier.

Neither is the story telling. Figuring out just the right tone for these posts has been an exercise in self-doubt. When we first heard the news of the congenital heart defect, the experimental fetal intervention options, and the uncertain future, we knew people who love us would worry.

Nothing we could do about that, but, with a bit of levity, we could give everyone the sense that we were hanging in there, and perhaps decrease the worry level by a degree or two. We didn’t, however, want to sound cavalier, or give the sense that the gravity of the situation was somehow lost on us.

It wasn’t. It isn’t.

We also didn’t want to leave out what was going on medically, but a series of lectures on heart anatomy and physiology was neither within my reach to deliver, nor would they serve much of a purpose. We’ve tried to find a balance, though it continues to feel elusive.

It’s funny. I’ve written a couple of books, but most days I don’t feel like a ‘real’ writer. Real writers are forcefully expressive. They treat the trickiest of subjects with elegance and grace.

Real writers make it seem so natural.

In On Writing Well, Bill Zinsser makes the case that writing is more a craft than art. That toil and care are stronger factors in success with the written word than talent and inspiration.

As he put it, “Hard writing makes easy reading. Easy writing makes hard reading.”

As hard as I might go at writing this update, I fear it’s beyond my abilities to make the reading any easier.

I could work at this for years. It would never feel natural.

Here’s what’s up.

Heart catheters – Ari’s now had 4 – are balloon procedures. The idea is to blow a balloon up to widen the valve or unstick the leaflets that might not be functioning properly at the top. Ari came through last week’s cath without complications, but it did not have the intended positive effect.

We were told (not like we had to be) that, in this journey, steps back would accompany steps forward. Simon says…step back.

You’ve met Ari’s aortic valve and his left ventricle. Today we introduce two new main characters: his right ventricle and his lungs.

Right ventricles send blood to the lungs to pick up oxygen.

The pressure in his right ventricle is “systemic” at the moment, meaning it’s riding up and down with his blood pressure. The right ventricle pressure should be half of this or less.

If my understanding is correct, the high pressure is a function of his lungs, like a super tight muscle, not relaxing like they should.

The label for all of this is pulmonary hypertension, the threat of which cannot be understated. No joke warning: do not look up pulmonary hypertension if you are not prepared to read something unsettling.

The question now is this: Is the pulmonary hypertension a permanent condition, or is it caused by the persisting severe aortic stenosis and the scar tissue in his left ventricle?

Our cardiologists and surgeons believe that it is not a permanent condition, but that we need to help his heart out more, and we have reached the limit of what we can do in the cath lab to effect this.

Since we cannot stand pat with pulmonary hypertension, the next order of business is open heart surgery with the intent to repair the aortic valve.

Note that by “repair” we’re talking more duct tape to keep it holding together for a while versus remodeling and restoration to the blueprint nature intended.

With pulmonary hypertension now in the picture, the “trump card” that our cardiologist has had in his pocket since day one, the Ross Procedure, may have temporarily lost its trumping abilities.

With the Ross, the doctors would take out Ari’s aortic valve and throw it in the circular file, remove the pulmonary valve and put it where the aortic valve is now, and put a homograph where the pulmonary valve used to be.

But the homograph can’t sustain the pressure of pulmonary hypertension, so we are now left with a likely less-than-ideal attempt at temporary aortic valve repair.

It’ll still need to go someday, and someday will come soon more likely than not.

Given the pulmonary hypertension, the urgency to move forward is now approaching emergency (action stations, action stations…) We got to the hospital last Thursday with lower right ventricle pressure and the expectation of discharge days later.

0 for 2.

The most senior cardiologists at the hospital are now setting the wheels in motion to get Ari into the operating room for open heart surgery (OHS as we call it in the hood) as soon as logistics allow. We need an MRI first to map out the scar tissue. This will require another anesthesia which takes time for ramp-up and ramp-down. We’ll OR time, surgeon availability, and so on.

We are to make no mistake, however. The intent is to get him in there this week.

The medical team does believe at this point that the pulmonary hypertension is an effect of the severe aortic stenosis and perhaps the scar tissue in the left ventricle. After the OHS, if the pressure in the right ventricle drops even some, Ewoks will rejoice, and we will begin a process to work that pressure to a lower, less dangerous level.

The risk of open heart surgery is, in and of itself, not insignificant. Ari’s chest will be open for the better part of a day, and he will be on heart-lung bypass for up to a few days. This is assuming all goes well.

At the same time, while OHSs are big surgeries, our doctors have told us that our surgical risks are not high, and that he should pull through it fine. (Whether or not we will pull through it fine is another question.)

In any case, the train has left the station. Next stop, open heart surgery.

Then the story will continue.

But for now…

Condition one is set. All decks report ready for action.