Dr. Files poured over Job's records and cath results this past month. Job, as we've said before, is keeping him on his toes. We got the preliminary results of the cath a month ago, but I've been eagerly anticipating the results of Dr. File's research.
I was desperately hoping for a "yep, cath results look great! See you in a few months!" sort of appointment. We've never had one of those appointments, but I keep hoping that NEXT time... I think I need to stop hoping and dial back my expectations, though I've said that before.
Job has three significant cardiac concerns at present:
1.) The pressures in his lung artery and Glenn are too high. (I'll come back to this.)
2.) His tricuspid valve regurgitation is moderate (and has been for months). He will need a valve replacement but we're hoping this leaking will continue to stay in the moderate range so we can wait to replace the cable when we need to do other work on his heart, rather than an "extra" OHS "just" for the valve.
3.) He continues to need oxygen, unlike most Glenn circulation kids with HLHS.
The interventionist who performed the cath was quick to point to Job's diaphragm as the primary explanation (and CERTAINLY it is a big factor) but it shouldn't affect Job so drastically. Actually, right now Dr. Files has three other kids between 1 and 2 years old, with HLHS and paralyzed left diaphragms, who sat 85 off oxygen. PT is working really hard to develop Job's accessory and intercostal muscles to help him breath deeply without the diaphragm (and I'll cover that in a future post), but Dr. Files isn't content to accept the diaphragm paralysis and subsequent plication as the only reason Job needs oxygen still.
I told him that I, emotionally, need to just believe Job will be on oxygen for the next few years. I can't handle the roller coaster of hoping he'll get off! only to be crushed by my disappointment after that next intervention fails too. Dr. Files is determined to keep trying things, so maybe one of these days something will work!
Circling back to Job's Glenn pressures... Job's June cath measured his Glenn pressure at 16, up from 12 in his Decemeber cath. This is too high to allow blood to flow easily and so now we begin a great deal of effort to lower this number. In fact, just to give an idea of how big of a deal this is, if his pressures stay this high, Job would be disqualified from the third planned OHS the Fontan. (This is because that surgery reconnect the veins and arteries of his lower body back into circulation. Since there is no pump, blood needs to flow passively but would not be able to with the high pressures in Job's Glenn at present.) Collateral veins will also likely start forming to "help" with the increased pressure - but because of Job's current circulation, those would need to be cauterized or coiled in another cath.
Why did his pressures rise so dramatically? The only thing we know is now different is that Job is off of one of the medications he was on back in December: the vasodialator, sildenafil. It's a very expensive prescription (because it's viagra and pharmacies can charge $$$$ for it) and it must be given 3-4 times a day, so we DCed it (back in the fall) because it didn't seem to be helping Job get off oxygen.
Sildenafil may help Job get off oxygen as well as lower his Glenn pressures, but it bumps us back to three med times/day and nine medications total. As well as some extra appointments in the next few weeks to monitor his blood pressure closely.
Also, for extra fun, Job's axillary fistula was not audible in his check up today, sending us quickly off to a (previously unplanned) echo. Echo results seem to show that the fistula is closing up. We will monitor this, but if it does close up Job will likely start forming AVMs again.
Bottom line? Job is complicated. We get to go back to monthly cardiology visits. We need another cath in a few months. We are up on meds and med times, not down.
So I probably don't need to tell you how disappointed I am. How tempting it is to sink into discouragement and frustration.
But then, Job plays with his brothers like this and I'm thrilled at how happy and active he is:
https://www.facebook.com/autumn.faee/videos/10100434599446470/
