Monday

5PM Well, the doc is watching ultrasound for bile duct inflamation and there isn't any (yet). If we start to see any, they can insert a stint into the duct to help ensure proper bile flow. These stints can last weeks, months, years. So, a good option. We do not have hepatic artery flow (yet, again I keep hoping there will be) and the team seems skeptical that there will be...so the liver is functioning on the blood flow from the portal vein and we continue to wait and see when/what effect it will have.
3PM still waiting for the doc to come back, he's busy, hopefully soon. Here's a picture.
930AM Back from the ultrasound...got called away so didn't finish the 730 update. Still unable to find the hepatic artery. I'm waiting for the docs to come around at rounds to see what they're thinking. White blood count was up a bit yesterday but seems to be coming down today so that is good. Still not drinking anything again yet...lots of bile in the tummy (which is fine) just not passing it yet - totally normal, it can take some time to get things "moving" again.

730AM It's Monday morning at the hospital so things are buzzing again. We're headed for an ultrasound shortly. They were able to normalize his main immunosuppressant med yesterday (Tacrolimus for all you google happy individuals).

Got my smile back

700AM Well, Mario had to get his NG tube back in, his little tummy wasn't ready to work on it's own. Other than that, he did finally get some sleep. His last dose of pain meds was 8 hours ago and he seems fine. The gas and bile in his tummy seems to have been the cause of his discomfort. Today is another day!

Saturday

7PM All is well. Mario has had a bit of a rough day. He seems to churn through pain meds and has been uncomfortable. Beyond that, things are going well. He got his ng tube and his cathitor (sp?) out. So, less tubes to look after...a very good thing. They said we could give him a tiny bit of formula tonight 15 ml every 3 hours...not much, but something! He actually turned it down just now. His little tummy got a little too full from the clear liquids he was having earlier (and threw a bit of it up). Slowing down the pace a bit now so hopefully he can have a bit of formula later.

1130AM The doctor came and talked to Israel late last night. There is really nothing to report except that we have to "wait and see" what happens with that artery and the liver. We're watching his liver function labs and hoping for the best. I keep telling myself that just because they couldn't see the blood flow in the ultrasound doesn't mean it isn't there. I mother can hope.

Friday

4PM Mario was moved out of ICU and onto the main surgery floor. This is where he knows all the nurses, has spent many days already. Sort of like "normal" in a weird way. We haven't had the official update from Mario's doc about the artery from the ultrasound today yet. He has been in surgery (like I said, he lives there). We do know that the radiologist had trouble finding flow in the hepatic artery. This is not good news...but hard to know exactly what it means yet. One day at a time now until we know...10AM Liver numbers still coming down (good), fever didn't return, cultures all negative (good). Mario's eye's are white again! He's doing really well, had a poop last night and might get some pedialyte later today. We're going to do a follow-up ultrasound today so I'll update after that. Otherwise, Mario continues to look better and better, so they are talking about getting us out of ICU.

another photo...

130AM Friday Mario's temps are much better. He's been back down in the 36.5-37 since this evening so a great sign that nothing WILL grow in those blood cultures.

There is this stuff called Nistatin (some medicine to keep from getting sores, yeast infections in the mouth). It smells icky...but Mario was so happy to have anything at all to suck on... The only nutrition he gets right now is through his IV still for another day or so. The nurse is getting a good chuckle out of giving Mario the Nistaton (sp?) on a sponge stick...he sucks on it. cute
p.s. Our nurse, Michelle, is from Australia and one of Mario's docs is English and the 3 of us have been having good fun with languages today. Makes me miss all you Aussies :)

Thursday

Mario looks SO MUCH better today when I came in, he looked like my little guy again. Here's a photo. He's doing fine, has a fever still but they are diligently watching petrie dishes to see if anything is "bug"ging him. Nothing has grown so far. Tomorrow morning we'll do another ultrasound to check that artery. Still listed and we keep our status 1A as long as we're in the ICU. I get to hold him today after they remove a couple of his lines.

Wednesday, March 26th

530PM Mario had his breathing tube removed. Israel called to report that he is doing just fine breathing on his own. This is a nice step, and means Mario is much more comfortable and can use his vocal cords again. They also started his TPN (this is IV nutrition). More tmrw...

NOON: Other than the limbo around his liver blood flow, Mario is doing pretty well. He's doing really well on urine output, his liver numbers are great, his blood pressures and heart rate are solid, he'll get started on some IV nutrition this afternoon and he's doing well on breathing. He was running a bit of a temperature but his white blood count has dropped so not too concerned about infection. His hematacrit (red blood lab) is a bit low for my liking, but the docs/nurse all said this is fine. We've heard varied opinions on the rest. It is impossible to predict the long range impacts. We just have to wait and see. I'm going to go eat and rest and Israel will stay tonight. More updates as we know more...

1000AM Dante wrote about a place between heaven and hell, purgatory. That's the feeling at the moment. The blood from in the hepatic artery is not ideal, it is there, but not ideal. So, we have to wait and see. He has been relisted. We knew this yesterday, but were still hopeful that it was just a precaution, so didn't share that detail. Today, it appears more like a necessary back-up. This is all very difficult to explain, but I'll try. Mario's liver is functioning well, very well. The issue is blood flow to the organ. Two possible problems...1st the temporary loss of blood flow could have caused scarring to his bile ducts, 2nd since the blood flow is not ideal, over time there could be issues with the bile ducts. If either occurs, Mario will be faced with infection, frequent hospital visits. It does not appear that his liver will "fail" but he could have continued issues caused by the potential compromised blood flow.

So, next steps are to continue to get him off the ventilator, add some nutrition, monitor him and keep him stable. It is possible to walk out of here and keep an eye on him over time, waiting.

The other scenario would be sometime in the next few days he gets a "great" liver offer. In this event, we will look at all factors and make a call whether or not it makes sense to replace the liver. Since he is already in the hospital, stable, etc. this might make sense to do now...only if we got an offer obviously.

Finally, Dr Reyes indicated he is happy with Israel's liver as another option. This seems more like something we would look at to schedule in the future if we don't get a great offer in the next few days and end up being able to go home.

So far so good

11PM Dr. Healey came by just now (I swear these doctors live here) to check on Mario. He said he's really happy with his liver numbers, he's been really stable. They are giving him heparin to increase his PTT (basically making it so his blood clots slower than "normal"). This is done to ensure that the hepatic vein doesn't clot off again. They have to balance between that and making sure he doesn't bleed (or "spring a leak") elsewhere. His other blood numbers look stable, so he's happy they have the balance and will continue to monitor this closely.

As I said this afternoon, we'll know more when we do that ultrasound in the morning to check the flow. So, that's our main goal at this point. Once we do that, we'll start talking about getting his breathing tube out, etc.

5PM Well, the post op ultrasound shows the vein flowing again. Hopefully, they caught the clot in time. Tomorrow morning they'll do another ultrasound to see how it looks. For now, we wait. The liver was proving to function fine, as long as the blood flow wasn't compromised too long we should be okay. Think positive! I'll write more tmrw.

Tuesday

1130AM They found a clot in the hepatic vein, have removed it and will bring him back soon. Hopefully just a minor set-back. They'll continue to monitor him the next couple days before we know much more. Everyone has tried to prepare us for these potential ups and downs...but still rough. We'll update more this afternoon.

10AM well we have a minor set back...they were unable to pick up the hepatic vein flow when they did the ultrasound this morning. so, Dr. Healey took him back to OR to take a look. Could be a kink or clot, thrombosis. But an "adjustment" needed. This type of thing is very much to be expected in the first couple days post op. But, obviously, still hard to take. He is doing so well otherwise. He's in the OR now so hopefully he'll be back within a couple hours - up to 4.

730AM I'm on my way back to the hospital shortly. Israel stayed with Mario, he is fine. They've been trying to keep him sedated and he was fighting it a bit at first. Dr. Horslen came by and said after they do an ultrasound this morning they will consider taking the breathing tube out. There was a bit of stress in the evening trying to get the right drug mix in place to make him drowsy again, he just kept fighting it and the nurse said the liver was metabolizing things well. So, I'll get more information at rounds this morning and provide an update later today.

Thank you all for your positive energy, thoughts, wishes, prayers. Whatever form they take for you :) It has all helped!

Mario is fine

He woke up and looked at us, interacted, good sign. He'll be in ICU for some time now but he's doing very well considering he has just been through a 12 hour surgery. No visitors necessary at this point, let's give him a couple days to get his breathing tube and some of the lines out. He's a bit of a shocker to see now anyway :) Thanks to all for your thoughts and prayers. He got his gift and is doing fine. Now we prepare for the next phase of this adventure. We'll update more tmrw, for now, know that he is in great hands and doing well.

New Liver for Mario

1030AM Mario is fine, he has been moved from the OR to ICU. The doctor said the surgery went well. We are about to be allowed back to be with him. He is highly immuno suppressed and protected these first couple days. We'll be off-line while we're with him. Wanted everyone to know he finished surgery and is doing fine.

730AM The nurse called to say that the doctor is happy with the ultrasound so they are proceeding with the last bit, connecting the intestine. This will take 2-3 hours.

600AM He is still in surgery, the liver is in place, he is stable, they do an ultrasound to check everything then reconnect his small intestine so it will be 3 more hours or so. The OR nurse said that everything has gone smoothly so far.

330AM They just called to tell me they are removing the old liver now and starting to put in the new one. They said he's stable, doing well and the new liver looks great. More news in a few hours.

Mario gets it

1130PM OR nurse just called to tell me they have started the surgery to remove the liver. New liver should be here within 30 minutes. It will take ~4 hours to get the old one out.

1000PM He's in the OR, we'll get updates every couple hours. It's happening!

830PM We just received word that it's a go. They're coming for us in about an hour and the surgery could take up to 12 hours so try to get some sleep folks and we'll update you in the morning. If there's anything worth reporting tonight and I have time I'll do that too.

730PM We'll know more in an hour or so. The team is checking the organs, will call and report when they're on the way. Dr was just here, said it is possible that intestine is compromised which puts Mario next for the liver. So, at this point - possible. Will update when we know.

"back-up" offer

545PM - we're admitted, did labs, got an x-ray, got an IV...Mario is finally sleeping. Now we wait.

130PM - Well, we're going in at 3pm. Apparently the recipient ahead of Mario is a liver-bowel recipient and since the organs haven't been procured yet, there is a possibility that the bowel won't be in good condition? So, that means Mario has a reasonable chance of getting the liver? This is all so new to me. I'm thinking about the kid who needs the liver-bowel and is actually ahead of Mario. I'm thinking about the donor, sitting on life support somewhere while the paperwork gets figured out. This is all so surreal. Updates tonight when we know more...

9AM - Nicki called to tell us there is "enough chance" that we should admit Mario this afternoon so we can wait on standby at the hospital. So, we're planning to wait overnight at the hospital.

7AM - Israel and I received a call around midnight last night from Mario's transplant coordinator. She was calling to check Mario's condition, health, make sure he doesn't have flu or fever because we have a "back-up" offer. Meaning, there is a liver, another kid ahead of Mario is going for surgery, but if for some reason they turn out not able to have the surgery, Mario would be next. They'll call this morning to let us know. Most likely outcome is the first kid gets a transplant (and probably needs it more than Mario right now). But Mario does need his and this tells me he is toward the top of the list... If we're "back-up" today, we could be "it" someday soon. I'm pretty calm about it now, but when the phone rang and Israel said "oh hi Nicki" I started throwing things in a bag, grabbing socks/shoes...Nicki doesn't call at midnight unless something is up...(Nicki is our transplant coordinator).

Other news from this week I forgot to add:
Mario's 1 year check-up is coming up in a month. We've been told NOT to move forward with any "live" vaccines...this means no MMR or Varicella (chicken pox) vaccine for Mario. We sort of thought this would be an attainable milestone, but getting the live virus in his system means he would need to go 'inactive' on the transplant list for 6 weeks so the docs have said not to. They want to keep him active...so do I, don't want to miss a liver if one comes!

Happy Easter all! There is bunny poop in my yard leading somewhere...Eva will have to help me find the trail when she gets up.

Living Donor Approved by UW, waiting re Children's

Well, Israel has a few more tests to do, more time at UW, but it appears that he has been approved to be Mario's donor by his UW medical team. Meaning, he passes with regard to health/condition and ability to have the surgery. We still need to get the okay from Mario's doctors that they would "accept" his liver. I assume there are criteria Mario's Children's team might look into that would be different than Israel's UW team. Things like size, connections, appropriateness, etc. If they would "accept" his liver, we would still wait and try to get a deceased donor before Mario's health becomes worse, Israel is more of a back-up (later) option in case Mario's condition worsens before we find a donor. Sorry if this isn't straight forward...it just isn't :) I'm answering the best way I can with what we know now.

Home again

Went home Saturday morning, all is well. Crawling up a storm now without the PICC line in his arm. Taking all the DVD's out of the TV stand, emptying the entire box of legos, tinker toys and whatever else he can get his hands on and spreading them across the house. Pulling Eva's hair... Oh BOY, did I mention we have a little BOY and have not properly BOY proofed the house (not baby proof, because Eva never got into this much stuff). BOY PROOFING the house...

We have to go back in 3 weeks (and probably every 3 weeks until transplant) for the same procedure. Better to stay on top of the bleeding electively than end up in the ER. So, planning another one 4/4.

We should know tomorrow if Israel can be Mario's donor. Think positive

Endoscopy today

330PM - pretty routine, varices were flaring up again. Dr. H injected them with heparin and we're here for the night for observation. Sounds like we'll be doing this drill every 3-4 weeks until he gets his transplant since they were flaring up again. He's in good spirits.

We'll be heading to the hospital today for the rescheduled endoscopy. I'll update in the afternoon with news. We'll be there overnight. Happy Friday!

Development Testing...

Everyone is pretty pleased with Mario's development. He's doing well on verbal, fine-motor, etc. He is just slightly behind on gross motor...basically because he isn't pulling himself up yet. He is hindered at the moment with the PICC line in his left arm. He's been trying to work around the line in his arm by scooting (it hurts his hand to flex his wrist because the line is in the way). Long story short, we will go back in 6 wks after he's got the line out and make sure he's on the road (pardon the pun) toward walking. Otherwise, they are very happy with his development. Good marks all around. The idea was to check now, get a good baseline, then keep him on their radar for post transplant so we can monitor his progress and make sure any/all resources are available to us if Mario should fall behind. Again, Childrens' showing how organized and thorough they are...ahead of the game!

Quick Update

All is well. Mario has some developmental testing tmrw, so I'll be at the hospital with him in the AM. Friday we have the endoscopy. I'll update if anything comes of the testing tomorrow, otherwise a check-in Friday night from the hospital after the procedure.

Endoscopy Rescheduled for 3/14, Eva Update

We try this drill again on Friday next week. Same plan, no food overnight, hospital around 11am, procedure at 1pm and overnight for observation. One of the surgeons said they might want a new ultrasound too so hopefully we can do all that the same day. We also hope to hear back about Israel's liver evaluation on Wednesday (12th). So, we'll update when we hear.

EVA news :) Eva is officially registered for Kindy. They had registration at her elementary school (Clark, Issaquah http://www.clark.issaquah.wednet.edu/) this week. My hand hurt from all the paperwork! She's all set there, we hear back about the lottery for Spanish Immersion (Bellevue) on the 12th as well. Long shot for that since we are not in district. I've been reading a lot of great things about the program at Clark, their parent participation, and it is small - only 2 classes in K, so they do one full day and one 1/2 day (we will lottery for full day). Eva also starts her Kinder Kickers (Soccer) in April. 8 week soccer program for 4-5 year olds. She's excited about that. She's also been practicing her Spanish with our nanny - when I got home from the hospital on (some night) this week, she counted to 36 in Spanish without error. YAY Eva!

Wed

Mario came home last night. He is doing fine :)

Tuesday

6pm I just finished up my training on the PICC line...got my box of meds, waiting for them to remove the IV from his foot. I should be out of here in the next couple hours. Our new neighbors (mom) is one of those people that seems to attract customer service issues. In the last 30 min or so she had a dramatic episode about the guy at the front desk that was rude to her, called her credit card customer service to complain about a $27 charge and made a call to her work to complain about a coworker. It is all I can do to keep myself from going over there (keep reminding myself her kid is in the hospital).

Seriously, NO ONE in this place is rude...

Back to "normal" tmrw :)

1130am the Nurse called to tell me the PICC will be put in at 2pm and training at 4pm so we're hoping to get home sometime later tonight.

620am Added a couple photos from this morning...

5am and I'm back at the hospital so Israel can go to work. Nothing new to report really...we expect to get the PICC line later today hopefully. Mario will have to be sedated, so he has to be NPO (no food/drink) for 6 hours prior. Once the PICC line is in we get trained how to do his meds and can start talking about getting released. Hopefully sometime tmrw? We'll see. I'll update when I know more.

Monday

Cultures still negative, good news! Getting a PICC line maybe tmrw so we can administer meds through it. INR was up to 2.0 this morning so he's getting a vit K shot - well into the IV. Still doing antibiotics, will be on those for 14 days. They will train me (and nanny tmrw) on the PICC line, so good timing that she will be coming in then. Mario smiles, flirts, is generally in good spirits. Happy Birthday to ME! Thanks for all your notes, messages, etc. :)

Sunday

Not much new to report. Still in the hospital, they will take blood and do cultures daily now. I thought we just needed one culture to be negative for 48 hours, but one of the nurses just said it might be 3 cultures need to be negative for 48 hours (so 4 more days here?). I'll confirm that at rounds tmrw morning. Either way, we'll go home with a PICC line and have to give him IV antibiotics on a schedule at home. On top of everything else, Eva needs to be registered for Kindergarden this week! Thankfully, I remembered BEFORE :) I'll write more tmrw, I'm hoping they'll raise his PELD tmrw, from my calculations on the UNOS web-site (they have a calculator online) I think he would be a 16 based on the latest labs. Monday will be the day to figure all this out. More then...

+BC bloodculture gram - rods

My medical training continues... in short, the little nasty organisms are growing in the petrie dish. The gram - rods (this is the type and shape, rod shape little organisms) are treated with the antibiotics we're already giving, so we continue the course. Mario will be in the hospital now for awhile as they draw daily cultures, until one is conslusively negative for the organisms (takes 48 hours to confirm negative). So at this point, the next culture is this afternoon so we're here until Tuesday at the earliest. :( The silver lining (yes, I can still find one) is that Mario is a well timed little boy - exposing this the day we were scheduled to be here! If we had not been here already on Friday, we would have likely ended up at the pediatrician Friday, maybe Saturday and maybe being admitted here on Monday. So, we caught it early.

Saturday morning

11AM: Dr. Horslen came and said since the bilirubin was up, that was more evidence pointing toward Cholangitis. So, we wait, watch the cultures and do labs again and watch the bili. We need 2 things to occur now to be in good shape - bili must decline and cultures must remain negative. Labs tonight will tell us bili, cultures reach 48 hours tmrw late afternoon. Worst case, they do a line that we can give antibiotics through at home to complete the longer round if necessary.

8AM: Mario is doing fine. Temperature spiked to 38.9 again last night but has dropped and stayed down now. His bilirubin went up to 7.8 last night too...not a good sign. The resident just came to tell me that 14 hours in there's no growth in the cultures (good). We just need to keep waiting. I'm imagining some lab person going by a petrie dish with Mario's name on it looking through a microscope every couple hours for nasty little organisms.

Cholangitis, not colangitis? I was tired last night :) Here's what I found about it:
Background: Acute cholangitis is a bacterial infection superimposed on an obstruction of the biliary tree most commonly from a gallstone, but it may be associated with neoplasm or stricture.
Pathophysiology: The main factors in the pathogenesis of acute cholangitis are biliary tract obstruction, elevated intraluminal pressure, and infection of bile...Although the exact mechanism is unclear, it is believed that bacteria gain access to the biliary tree by retrograde ascent from the duodenum or from portal venous blood. IN MARIO's CASE this happens more easily because he has had a Kasai. As a result, infection ascends into the hepatic ducts, causing serious infection. Increased biliary pressure pushes the infection into the biliary canaliculi, hepatic veins, and perihepatic lymphatics, leading to bacteremia (25-40%). The infection can be suppurative in the biliary tract. The most common organisms cultured in cholangitis are Escherichia coli (39%), Klebsiella (54%) and Enterobacter (34%) species, enterococci (34%), and group D streptococci. This would be what they're watching for in the petrie dishes.

I've also been meaning to give some temp conversion notes for you all, 39.1C is like 102.5, 38C is 100.5 and "normal" 98.6 would be 37C.