Let's Catch Up

This is definitely a message I wrote to my sister a couple of days ago, catching her up on the things that Kilian has accomplished so far. To save time and sanity, I'm going with good old copy and paste here - not even going to proofread. Forgive me as tommorow may be a big day in way of progress, and I'm hoping and wishing to be able to spend my day writing about that.

So the biggest issue we face right now is his Persistent Pulmonary Hypertension (in a nutshell, pressure that wreaks havoc on your organs, ending in heart/kindey/lung etc failure). Thankfully they think it's because he's premature (common) and not because of his anatomy. Early on it was causing the right side of his heart to be enlarged, but that has since gone down. They have him on a medication called Remodulin for it, yesterday I spoke with cardiology and they are lowering his dose as it looks like the PPH is getting better. So that's fantastic.

He has one lung on his left side, and the concern there would be function. At one point he was on 100% oxygen, then 70, and now he's between 20-30%, which is really good. So they have said it seems to be working just fine.

Originally they had him on a machine called an Ocillator, which was holding his lung open and giving little pulses of pressure. Its a gentle respirator but you cannot move when on it, so he was on a paralytic..paralyzed. The goal was to get him back on the ventilator (he was at Theda and when he got to MKE, but needed to switch to the oscillator) and off the paralytic, and he's accomplished that. The vent settings are also not very high, and they catch him breathing above it at times so that's great, the sooner he can come off that the better.

When he was born they immediately intubated him but had trouble getting the tube down far enough. The transport team from Milwaukee wasn't confident it wasn't going to come out on the bumpy ride down, so they had to remove it and attempt it again - his heart stopped and it was terrifying but they were amazing and got it right where it needed to be. The trouble came from the fact that his airway isn't completely developed (plus it leans to the right). It goes down and branches to his left lung, but there's only a little bit branching to the right- as there's no right lung to go to. So they thought the tube was either getting stuck in that right side or that there's possibly an obstruction/blockage. ENT is actually coming in today to run a scope down his vent tube and take a look. They want to extubate (take the tube out/off the vent) him as soon as they can and we're hoping whatever is going on in his airway won't cause an issue with that. Also, he can't have his head turned to the right and needs to be on his back or left side, they think this is just because of where the vent tube is sitting but want to make sure so its a non issue going forward.

His one kidney is working just fine and hasn't shown any signs of damage from the PPH. He pees like a boy, all over the place and a lot, he was cathed on the paralytic but the catheter is gone now so we've got lots of pee diapers! They were able to start giving him milk thru a drip into his stomach about 2 days ago, so we're waiting on a poop next. May take a bit with the narcotics and from being paralyzed, but his bowel sounds are good so hopefully that'll happen in the next day or so. 

The anatomy of his heart is pretty normal considering (and of course besides being in the 'right' side of his chest, Dextrocardia). He is missing the arteries and veins going to his non existence right lung, which isn't a big deal since the lung is not there. But he is also missing one of his pulmonary veins going from his left lung back to his heart (you typically have 2 on each side). He also has a VSD which is a small hole, but a lot of people have one and they aren't concerned about it. She actually heard a murmur the other day which in his case suggests that its closing..so that's good.

He has bi-lateral ventriculomegaly, which is enlargement of both your lateral ventricles. You have 2 lateral vents, a 3rd vent and then a 4th. Cerebral (brain) fluid goes thru the 2 lateral vents, then drain into the 3rd and the 4th. If for whatever reason the fluid is not draining, the vents become dialted and there is then pressure on the brain. Anything measuring over 10mm is considered VM, and he was anywhere between 10 and 18 (at the highest) thru his ultrasounds while in utero. 10 is mild, 15+ is considered severe. The procedure for this is surgical insertion of a shunt that drains the access fluid into a cavity around the stomach (its super fascinating, they run that line all the way down and provide excess so it grows as you grow). However, his vents are measuring at about 10.3mm on each side and have remained stable. That suggests that he, suprise suprise, just has a big head!! Nuerology has found no evidence of pressure, brain damage or brain bleeds. Ultrasounds are subject to so many variables that they think those higher measurements were errors, and they're frankly quite bored with him. He'll continue to be monitored but we're thinking we're in the clear there.

He was on Fentanyl for pain (and also acts as a sedative) but has been off of that for a few days, and they are just giving him morphine as needed to keep him comfortable. Slowly but surely he is waking up, moving around and opening his eyes. It's scary, because I'm afraid he's going to be overwhelmed and it'll be too much for his little body - but they continually tell me moving is a very good thing and they'll keep him comfortable. They also have him on an anxiety medication more or less, so this helps too.

After my second echo they had given me a soft diagnosis of Heterotaxy - which is a genetic condition in which organs placement and quantity are effected. Heterotaxy comes with a whole list of other issues, and normally a staggering number of congenital heart defects (CHD). Thankfully, not a single specialty believes this to be the case anymore. His Dextrocardia seems to be isolated, and they believe his heart developed on the right (along with his airway leaning that way) because of the open cavity there from his right lung being absent. We did have genetic testing done and will get those results in a few weeks.

I may have missed something but that's Kilian in a very large nutshell.