Day 43: Frankie Firebrick is an Insomniac

It's true. This boy does not sleep. He has slept for about 35 minutes of the 10 hours we have been here. Frankie Faun has been in a better mood than yesterday, but a far cry from happy. We took turns consoling him all day. Today's high lights include a clogged feeding tube. The new one is now in his right nostril.

We have seen an improvement in his stats while he has been Crankie Frankie on roids. The steroids are a super strong anti inflammatory and they hope to wean him off the oxygen while he is on them. We were unaware of this until tonight. His heart rate sits in the 110-120 range when relaxed and the 140-150 when he is up. He used to be in the 170s. His oxygen saturation is sitting above 95% while his air is at 3 liters at 35% right now. His breaths per minute are at 70 BPM at an actual count. The weaning of the oxygen is a catch 22 since he gets pissed easily and needs more air but then doesn't require as much due to his steroids.

Last night they did remove his PICC line. That was the more permanent IV. They had stopped using it, but kept it in in case they needed it. It was flushed daily with a blood thinner to keep it from clotting. But it clotted anyway and they just went ahead and removed it. It is so much nicer now that we have Frankie Flax's arm back. We can dress him so much easier now. His Viagra and steroids are given "orally" through his feeding tube.

As for the omphalocele, the surgeons finally made it by at 8:30 this morning. They looked at it and honestly did not know what it was. Their educated guess was it was his liver we see. That things has shifted around. This is quite possible since it did only take one piece of xeroform to cover it. Since it was not compromised or leaking, they did not have much concern about it. They did stop the silvadene (burn cream) for the moment. We did not get to see the whole thing today because there was no need to re-wrap it again after the surgeons did this morning. We did replace the outer gauze because Frankie Fuchsia did pee on it and we didn't notice any problems.

Frankie Flesh Tone did sleep for about 35 minutes earlier when we walked over to the Central West End for a quick dinner. What a double disappointment. We were told there were tunnels that connected our building to a building closest to the CWE. I was stoked.  I thought we were going to an underground maze that included all the maintenance systems. Nope, these "tunnels" were sky walks. Boring. Then Jimmy Johns just doesn't make a good sandwich. But Frankie Fern has been asleep the whole time I wrote this, which is roughly 40 minutes, close to a new record.  I lied, I just handed the computer to Alex to have her proofread and she said, quote, "He just woke up and took a fat shit, it has been only 28 minutes." Such a classy lady I married.

Day 42: Frankie Ferrigno Roid Rage

Frankie Fairchild has roid rage. Plain and simple. But before that, lets mention tonight's visitors. When we arrived, my father, stepmother and stepsister were already here. It was Jaimi's first time meeting the boy. She cried. Being a mother herself, she just couldn't understand how we are doing this. Guess what, neither do we. But we chose to carryout this pregnancy, we are responsible for this little guy, and we love him more than life itself and wouldn't trade it for the world. Unfortunately we had a few more guest tonight and kinda, unintentionally ignored the family while they were here. The Capriano family came by also and we got to see Miss Sophia for the first time since she was born. She is 4 weeks older then Frankie Fairbanks and they have an arranged marriage. The lovely Miss (which should be Mrs.) Hollie also came by for the evening.

Unfortunately for our visitors, Frankie Faulk has not been feeling well. The steroids he is on are clearly making him crawl in his own skin. It is terrible to watch, nothing consoles him. He is a strong boy and I have a tough time holding him while I change his diaper, wrapping, or bath. He has been pissed before and cried for a couple hours, but normally exhausts himself and it's over. Not so much on the roids. He is inconsolable and this lasted for 4 plus hours until we finally had to give him a sedative. He is quite asleep now, but it is terribly hard to see him like this. But the rewards will outweigh the pain, so we have to continue these. It is until Thursday when he has the echo. My heart really goes out to the boy, I hate seeing him like this. This is going to be a rough week ahead, so you might wanna not read the blog until Thursday. If day 2 was this bad, day 7 is gonna suck. I just hope that this improves the PPHN and Frankie Fallon hasn't done this in vain.

As far as the omphalocele goes, we have a little concern with it. We noticed a, for the lack of better terms, a blood blister on it. It was about 2 inches by 1 inch but shallow. The surgeons had a trauma case and therefore have not been by to see it since we found the defect on the defect earlier. I believe that it is a popped blood vessel or something like that from crying, screaming, and clawing/kicking so hard today with discomfort. It is not leaking nor has it been compromised in any other fashion, but still a reason to raise concern.

Has everyone paid any attention to Frankie Farley's nicknames lately? They have been themed. Can you figure out the what the theme is each day?

Day 41: Frankie Fawn's First Worlds Series Win!

Alex and I came in at 1:00 for our meeting with the specialist. He was a very intelligent, kind and informative person, but we did not come away from the meeting with much new information. Joanie the night before had given us roughly the same info. He explained there is not much information on infants with PPHN, children with omphaloceles, and definitely not any info on them combined. The best thing we got from the meeting is that Frankie Ferrett is a baby, he grows. Meaning he can grow new lung tissue with better vesicles in it. Some of the questions we asked are below...

What are his chances of surviving?
All three Doctors had high expectations of him surviving, but with lack of info, especially for kids with both PPHN and Omphaloceles, there were no numbers they could give us.

What are the long term effects of this?
Unknown. I expected him to be "asthmatic" at the least, but they said if he outgrows it, he outgrows it.  

What is causing this?
They believe it is in his lungs only. This is a form of lung disease. Whether it is undersized lungs, underdeveloped lungs, or defective lungs are unknown at this point.

How long will we be here at the NICU?
Obviously there is no glass ball, he could wake up tomorrow healed or quite the opposite, good days and bad. But they could not give us anything for right now. There is another heart echo planned next Thursday. We have started steroids, and we will see how those work. Although this is in his lungs, the heart echo will tell the pressures coming and going from the lungs.

Treatment?
Obviously the oxygen and Viagra we have already spoke of. The newest thing he started are steroids. This is kind of a trial and error thing since there isn't much literature out there. We don't think he has responded well to these. He has barely slept and has been very upset in constant waves since we have been here. When he did finally started to relax it was time for another round. They will reassess this at the latest at the next Thursday echo. If he doesn't improve, then a lung biopsy, cardiac catheterization, and CT will be the next course of action.

So from here, once again, time will only tell, but I can tell you this, if Frankie Foxhound is as stubborn as his parents, he will be terrorizing the neighborhood in no time.

We did sneak away for a few hours tonight to eat dinner and watch the game at Cody and Anna's. We want everyone to know how much we appreciate the support, love and encouragement we get from our friends and family. Everyone reading this feel free to give yourself a big hug, but anything else you do is not from us. Depending on who you are of course.

Day 40:Frankie Fredbird Wins It In Extra Innings

Up and down day for Alex and I. We accidentally slept in today because my alarm didn't go off, so we took the day off. We ran a few errands and went and hung out at Forest Park for a little while on this super nice day. We followed that with a late lunch at Pi in the Central West End. Another good thing happened, I don't know if you heard, but the Cardinals won game 6. So there will be a game 7. I will tell you about the boy before I tell you the potentially bad news.

Frankie Firebird was awake when we got here and was cheerful. We cleaned him up, gave him a bath, changed his wrapping and diaper. I remeasured the omphalocele. The circumference has grown but the bust has shrunk. The circumference is 11.25 and has grown a .5 inch. but the "bust" has shrunk from 16" to 15.5". Keep in mind that Frankie Fairlaine in growing, so a losing a half inch is a bigger number than you think. We took turns holding him and he slept in my lap until the 8th inning when I had to pee. He got a little fussy after that and it took some gas drops to quiet him down. We had some problems with his oxygen saturation not keeping above 95% during this time. It was dropping into the lower 90s. But a little kick of the oxygen mix and a re-alignment of the sensor on his foot cured it. We were able to drop the air mix down lower than it originally was before this episode once we fixed the sensor. Besides all that, he was an angel as always. His feedings are now bolus. He gets 75ccs over 2.5hrs and then a .5hr off. This will be worked down to eventually full feed at 3hr increments. Also his Viagra has been increased again.

Now the bad news. When we got here, the doc on duty came and talked to us. Keep in mind these doctors rotate through, and this one has been the best, most informative, and most proactive doc we have had since we started. None of the other doctors did anything about the pulmonary hypertension, told us anything about it, and brushed it off. Joanie had a frank talk with us. We are working on his breathing with oxygen and Viagra and a specialist that works with kids with PPHN will talk to us tomorrow. There is talk about steroids and other treatments. Half way through the conversion she said something about PPHN could be terminal. We were dumbfounded. Obviously we had read about that on the internet, but thought nothing of it. I mean, if this was serious, then the doctors would have mentioned it, treated it, informed us, right? Not take 40 fucking days for a great doctor to tell us? Right? Joanie thinks Frankie Fleetwood can and will survive this, that it is mild enough to out grow it. But there is a possibility there. Wow. Seriously, if this was this serious why are we waiting this long to do something about it and waiting this long to even inform us. This drastically took the wind out of our sails. Everyone has been brushing this off for 30 something days. I could maybe see not letting us know if we weren't involved parents, but we spend 10 hours plus a day here and do everything we can while we are here. Frankie Ferrari will be fine. He will be burying us someday, not the other way around.