7/11 Update & The Pain of PAH

Today started with such promise. Before I left my room, I called my doctor and asked if he would up my meds and call in the prescription to the pharmacy at the hospital. Before I arrived at the hospital, I had a message on my cell that he had approved it and it had been sent. On my way in, I decided to have breakfast from Biggby. While I was in line ordering, I felt a soft hand on my shoulder. I turned to find PD's surgeon. He had spoken with the nurse clinician and wanted to let me know that he had time to talk with me today about any questions I had. We made plans to meet later in the day and I headed upstairs.

When I arrived, Patrick wasn't in his room--he was hanging out with the nurses, flirting. They held him while I ate my breakfast and then we went for a stroll around the unit. Plans for the day were simple--begin bolus feeds in anticipation of a pH probe on Monday; reduce milrinone and increase enalapril; and begin sildenafil (a pulmonary hypertension med). Easy-peasy.

When I placed my happy, smiley boy back into bed after our walk, I plugged him back into the monitor. His heart rate registered in the 170s. Huh. Weird. He had been in the 150s when they took him off, which was higher than yesterday but not horrible. And, they hadn't implemented any of the changes for the day yet, so none of those could have been the cause. Then the diaphoresis began again. They took a blood gas--it looked good; great even. They gave him some morphine to see if it was withdrawal; no change. He began to get fussy. They took an x-ray. Nothing. And yet, his heart rate continued to increase into the 180s.

About this time, the nurse clinician came to take me to meet with the surgeon. We had a nice long talk, and he answered a lot of questions. However, I learned I may not be coping as well as I thought when he said, "We're worried about you. You're here by yourself, and we know this is really hard." After we talked, he let me know that he wanted to be available for me to talk if I needed it. Very kind; pretty unheard of. In any event, I was actually feeling pretty positive coming out of the meeting. Hopeful, even.

One of the other things the surgeon said was that he pretty much wants Patrick to force him to re-intervene. He didn't want a single-inflated number or any one thing to make them rush back to the cath lab or into surgery. He admitted that he waited a really long time before putting in the stents because he wanted the surgery to scar over; had they gone in too soon, it could have blown it open and he was pretty sure that would have been non-recoverable.

Anyway, I guess Patrick's soul was listening because when I got back to his room, his heart rate is pushing 190. A gentleman came in to give him an echo. Patrick lost his stuff, but they finally got all of the pictures they needed. Maybe 10 minutes later, the doctors arrived to round. I was holding Patrick; he was quiet, but unhappy. The nurse clinician shared the bad news--his echo showed that his pressures were systemic--that is, his arterial pressures were the same as his blood pressure. They put in immediate orders for him to be put on nitric oxide. It works the same way as the sildenafil, but provides immediate assistance while they slowly increase his sildenafil dose. In addition, if his pressures responded well to the nitric, it would be a good clue that the meds would work. She advised that she would inform the surgeon about the echo results.

The respiratory therapy folks arrived. Patrick was quite unhappy to have to wear a cannula again, but the effects were immediate. Within the first 5 minutes, he had dropped from 185 to the low 170s. Over the next two hours, it dropped significantly and was 146 when I left. So, the good news is, it looks like the meds will help once we get them up to dose. How well still remains to be seen, but it's something. Still, I am totally freaked out that his pressures rose again that quickly.

During my talk with the surgeon, he had indicated that his next move would probably be to open the hole in his patch further. It would sacrifice Patrick's sats, but would provide a pressure reduction on the right ventricle. The cardiologist on rounds also indicated that was likely his next move. So, although I haven't been informed of anything, I think chances are good that come early next week Patrick is headed back to the cath lab for them to further fenestrate his patch.

Obviously, getting this news going into the weekend was difficult, particularly because I know the wheels of healthcare are pretty much booted until Monday. Still, the fact that they got him on the nitric and it significantly reduced his heart rate gives me comfort. Hopefully, it's making enough of a difference that he can hold out until Monday. For now, I just have to keep pushing ahead one day at a time.

I will tell you that my gut says Patrick is going to need a transplant. The surgeon didn't think we were anywhere near that point, and he may be right; he has certainly done this a lot more than I have. And I would love to be wrong. But I'm putting this on the record. I think Patrick's telling us that he needs something more. Only time will tell.

In the meantime, here is another "delightful ditty from Detroit":

The Pain of PAH (to the tune of The Sound of Silence)

Hello nitric, our old friend

You've come into our lives again

Because my baby has had sweat dripping

And PA pressures keep on increasing

And his heart, it can't withstand the strain

that remains

so we enter, the realm of PAH

I sit beside his bed alone

The beeps go on like metronomes

'Neath the glow of your monitor

I remember this from your sister

And my heart feels stabbed by a flash of memory

it's so scary

the struggle and pain of PAH

And in that moment it was clear

Although my eyes were wet with tears

That I would do ever-y thing I could

So I could bring you back home to the 'wood

Once again to be a family of four

nothing more

and cope with him having PAH

So now he's on sildenafil

And sippy fruit he'll learn to swill

And he might also need bosentan

Which means we'll fight our insurance again

But we will get whatever else he needs

Guaranteed

for treatment of Patrick's PAH

And someday it may come to pass

That his heart gives out at last

But even in a future that looks grim

A heart might be transplanted into him

And even if that fails, there's something he should know,

before I go:

I love you, my son, with PAH.