Finished Transplant Evaluation

Well, yesterday was a VERY LONG day. Mario was a trooper through it all. Israel and I were pretty much mentally spent by 430pm when we left the hospital. We continue to be impressed with how organized everyone is. Mario is in good hands. He seems to have passed on the cardio, EKG, etc. tests to ensure that the rest of his body can handle this major surgery. The nutritionist confirmed we're doing all the right things with food/vitamin supplements as Mario is still gaining weight. The challenge is to ensure it is "real" weight and not weight from his liver and spleen which are starting to enlarge. So, her job will be to monitor that closely now. He will start to be at higher and higher risk of portal hyper tension, varices and other issues in the weeks/months pre-transplant. We met the hemotologist and one of the surgeons. All very informative, helpful. We'll get confirmation on Mon/Tue but it sounds very likely that they will decide on Monday to list him now. His PELD score is a 1 (range -10 to 40)...the higher the number the more critical the need for a new liver. The benefit of transplant outweighs the benefits of continued growth/development at around 15. Mario is O+ so the most common blood type, but they also consider size appropriateness of a liver...since he is small this is a major consideration. It sounds like they do about a 50/50 split of whole liver versus split liver transplants, so it could really be either one. We can also do work to identify potential living donor(s) in advance. Israel and I are starting to work out how that works (the donor is processed through UW Medical). Those are the highlights...I know this is a not very well laid out update. As you can imagine, my brain is full of various facts and details - these are the ones that seem noteworthy at the moment. I'm answering email regularly so feel free to drop me a note if you have specific questions. Please keep Mario in your thoughts, our love, Phoebe and fam

Not much to report...day 1 of tranplant evaluation

It was a long day, but I think Tuesday will be longer. Mario did great, smiled at everyone and didn't even flinch when they drew blood. His vit levels are okay with the extra doses of supplements we're giving him and he was up to 6.96 Kilos - still gaining weight (all this translates to a lower PELD score, meaning he would be lower on the list for a liver so we probably have a bit of a wait). We met our xplant coordinator and got a good review of the process. Met lots of people. More tests on Tuesday, and we get to meet the xplant surgeons. Then they will meet on Monday after that to decide if they will list him right away. Assuming they do, we give out all our contact details, get assigned a pager and wait...giving blood every couple weeks in the meantime so they can keep updating his score. I'll update again on Tuesday as we should have more to tell after we talk to the head xplant surgeon. Hope everyone is well.

Mario's Evaluation Scheduled

We have our transplant coordinator and the scheduler confirmed yesterday that we will complete Mario's transplant evaluation testing and appointments on the 19th and 25th. A couple of you recently asked if we ever got the bridge fixed so I added a link to the Flood and Bridge Repair photos on the right. Seems like a long time ago now, but I guess it wasn't really :) it is repaired (517ton of rock!) see pictures it is pretty amazing. More on Mario's appts next week, etc. Take care, Phoebe

The road to a Liver Transplant

Well, as you can imagine, I've been doing even more reading, research, etc. since we found out we're headed toward a liver transplant. If anyone is interested, I found some great details on how the PELD scoring is done as well as general information on the types of transplant options. For a pediatric liver, you can do whole, split (adult liver to 2 kids), or even living (take one lobe of an adult relative). Email me if you want to read more - some of the best details are actual presentations that were archived from a liver conference last year. Many of the topics were specific to pediatric liver transplantation.

Latest on Mario: he responded well to the Vitamin K shot, so we will check his blood work bi-weekly for now. He has an ultrasound on 9/24 and more tests. The head GI nurse (my new best friend :) ) called today to tell me the transplant coordinator will get ahold of me in the next day or two. So, we'll be getting a lot more information soon. They seem to have a very organized approach, prepared to walk us through everything from listing criteria to insurance requirements, etc. Lots of hospital resources available to help us through this.

Thanks to those of you who have called and written. I might not always respond, but we sure do appreciate hearing from you. Know that.

Friday, September 7th

We met with Dr. Christie today. In short, Mario will need a liver transplant eventually. We are now just trying to determine when - his best guess is 12-18 months. The HIDA scan indicated that there isn't secretion - meaning the Kasai isn't functioning the way we would like. His Bilirubin levels are not more elevated yet and he has solid weight gain, so we do not go immediately on the transplant list. Instead, we have a series of more tests. Today, they checked vitamin levels and determined he is low on K. Poor enough levels that they are making Israel drive back to the hospital for a shot. Later this month we will have an ultrasound to determine the level of scarring in the liver. Then, within the month, we will have a consult with the transplant team at Children's so they can get us in their database and start working on Mario's scoring. Over the next months-year, Mario will be on the list of patients discussed as the team determines when to list him.

It is unfortunate to get this news, but on the other hand, at least we know what is coming. I've done a ton of research thus far and feel very confident in the team at Seattle Children's and the process we will go through. Please keep Mario in your thoughts.

HIDA Scan day, Monday 9/4

Israel and Mario spend the day at Children's today. IV draw at 10am, blood draw, injection at 1030am and HIDA Scan from 1030 to 1200. Then another 30 minute scan at 2pm. We might have to do another scan at 10am Wed morning as well. Poor Mario then gets his 4 month shots on Wed afternoon and we meet with the GI on Thu to get all the results of HIDA Scan and blood work. This will hopefully tell us if bile is getting OUT of Mario's liver as it should. We are hoping for good results to show this from HIDA scan as well as lower Bilirubin levels from blood work. These results will give us hope that we do not need to start getting assed by the tranplant team anytime soon. So think positive and send him good vibes please! He is SUCH a trooper.

Oh yeah, I start work today...first day back after 6 months. Not quite like the day Mario will have, but something. Eva also starts her EAGLE class today at school. Officially PRE-K.

HIDA scan scheduled for 4th September

Radiology can't fit us in until the 4th! I guess the good news is that means we are not considered urgent in any way :) Meanwhile, we play the waiting game some more. I emailed the GI and asked him to do another set of blood work orders too so we can check the bilirubin, etc. at the same time. The HIDA scan is a 90min procedure on the 4th (start an IV, inject some radioactive dye, then strapped to a table for 90 min laying still while the machine takes photos), then we do a follow-up session of 30 min (just pictures) on the 5th and meet with the GI on the 7th for the update. So, we will hopefully have a good update in a couple weeks.

Meanwhile, we are going to continue to try having a "normal" summer with the kids before I start work (coincidentally on the 4th). Doc said all our plans/travels were okay. We are going to keep our plans to go camping over Labor Day weekend at Kalaloch, see the ocean, have some fun and I'm taking the kids to AZ to see Michelle, Heather and Emma tmrw for a few days. Wish me luck on the plane with the two of them :) Israel will be working - his last day Friday before he is off for 2 months taking his "paternity leave" when I go back to work.

More in September! Love, Phoebe

August 20th - GI follow-up, bloodwork, etc.

I'll start with the good - Mario weighs just over 6 kilos/13lbs now and is slowly moving from 10th percentile up toward 20's on the growth chart. He continues to smile and laugh, etc. Unfortunately, his bilirubin levels went up again. We had hoped that would continue to get lower (toward zero is our goal) from the 1.9 it was last month...today it was 4.8. This is by no means off the charts, but headed in the wrong direction. Our GI has ordered the HIDA scan and I'm waiting for the radiology folks at the hospital to call me back with the appt schedule. This will help determine how good his bile flow is. I have all of Mario's lab results from the last 3 visits and I'm studying up on all the liver function stuff in the reports, trying to find some numbers that might be pointing in the right direction... wish me luck :) He also increased Mario's dosage of Ursodiol...hopefully this bile acid medication will help things along. The HIDA scan results will determine our next move, but today was the first day that Dr. Christie mentioned meeting with the transplant team at Children's. I KNEW this was a possibility from all the reading I've done (most Kasai kids end up needing a transplant eventually - only a handful have made it to their 20's without) but the reality of having the doctor say it outloud was still hard to hear today. Please continue to send your positive energy and prayers to Mario. Our love...Phoebe and fam

August 16th

We went in to have Mario's blood drawn in preparation for our appointment on Monday with Dr. Christie. I've been reading a lot online and found a great site called liverfamiles.net where families of kids with BA and other liver diseases meet, discuss, share information. I have found this site to be quite helpful, where you can get information from others who have gone through this and understand our situation.

On a lighter note, you can see from the latest photo that Mario is diong just amazing. He is happy and smiles, laughs, etc. He has a period of extreme fusiness every evening around 7-8pm where he seems to be in pain...we think gas, hoping that is all it is. I, of course, will bring this up with the doc on Monday.

So, we'll report back next week. Meanwhile, hope you are all enjoying your summer!

August 9th

Here is a cute photo taken today of Eva reading to her brother. She has been showing LOTS of interest in anatomy and doctor stuff lately, more than before. So, she has a bunch of new kid books about anatomy and the body. The book she is reading in the photo is one of her "BOB" books, all 3 letter words that she is learning to sound out.

Mario is still eating well, doing well. We have an appt to do blood tests again next week and meet with the GI on the 20th. He said we will probably do another HIDA scan to see how well the Kasai is working and give us some idea how long the procedure should last. I've been doing lots of research and have found a number of adults in their late 20's, even 30 years old that have only a Kasai and haven't yet needed a liver transplant. So, we're hopeful that Mario will be in that successful group. Only time will tell!

Love to all, Phoebe

7/29/07

Just adding a new photo today...Mario with his "tough man" belly exposed... gaining weight and sporting a nice scar.

July 25th - an attempt to answer your questions about long term diagnosis

Now that life has started to regain some of it's normalcy at home, I've started to do more research. Reading all the articles about biliary atresia that I read so franticly in the hospital and just trying to get my head around long term care for Mario. I found this story today about a little boy who went through what Mario is going through - hit very close to home...and thought it was worth sharing: http://www.chop.edu/consumer/jsp/division/generic.jsp?id=85239

Many of you have asked about long term impacts, next steps, etc... I'll do my best to explain what I understand so far:

Like the boy in the story, we have to be very careful about fevers now. As the doctors explained it, Mario is at risk of choliangitis. Caused by bacteria being able to get from the small intestine (backwards/up) to the liver now since he has had a Kasai. So, unlike normal kids who get a fever and you wait a few days to see if it passes, Mario has to be taken in immediately if fever occurs and the GI doc said they usually just give antibiotics so they can be sure he doesn't have/get choliangitis.

That is really the main concern for now. There is no way to know if Mario might at some point need a liver transplant, we will have to take things one day at a time, month at a time, year at a time for now on and just keep our hopes up that the goods news we've had thus far will continue. We have some great doctors and have every reason to believe Mario's liver will function normally going forward at this point. So, continue to think positive and we'll certainly keep everyone posted as we get more details in the weeks to come.

"Functioning Kasai"

Well, mostly good news today! Mario is up to 5 Kilos and into the 25th percentile for weight (higher than he has been since birth). Bilirubin levels are down almost 50% from where they were when he was admitted to the hospital. Bile seems to be flowing as it should and the doctors are happy to let us keep "pushing the calories" for now and check his blood in 3 weeks to make sure the liver is still functioning as it should. So, we aren't out of the woods yet, but WOW what a relief to hear the surgeon say "he has a functioning Kasai" :) Oh, and I don't have to give him shots anymore. He will stay on the remaining medication and supplements indefinetely. I'm running to dinner so I'll update more or answer your emails if you have questions over the weekend but wanted to get this bit out as soon as I could. All our love, Phoebe and the fam

Tuesday, July 17th

Mario weighed in at 4.72 kilos today...about 300 grams more in one week (since last visit 4.45). This is great news. The Gastro doc is still happy with the color of his poop as well. As Monika put it "Pooray!" - I had to use it :)

There is not much else to report. Dr. Christie wants to see us again on Friday after we get blood work and see the dietician and surgery folks at Children's. So, we have this marathon day Friday of blood test, appts in Seattle, then swinging by his Bellevue office on the way home. It will be good to do the check in with him in Bellevue though because he said he will take all the notes from the other appts, blood work results, etc. and give us some more detail about next steps.

On next steps, here's what he did say: some of his meds are "indifinite" - specifically ones that help bile production and absorbtion as well as vitamin supplements (iron and a water soluable form of the fat soluable vitamins). Although, I can possibly stop doing the injection if blood work looks good on Friday. Friday's bilirubin results will also give us a better idea how well the bile is flowing out of the liver - we know it is since his poop is a good brown/green color, but he is still slightly jaundice. Dr. Christie said it can take up to 3 months to get jaundice levels back to normal...but the sooner that happens, the better the chance of longer term success with the procedure. If the bilirubin levels are still high/haven't lowered enough, we will go back to children's one day for another HIDA scan.

So, send good vibes for lower bilirubin levels this week and I'll update again over the weekend. Thank you all and all our love, Phoebe and fam

Friday, July 13th

Nothing much new to report, but wanted everyone to know Mario is doing just fine. Today is the last of the high dose steroids then just 5 more days of a very low dose until he can stop taking that one. Also just one more day of one of the antibiotics, so the meds are tapering off - which is nice! I still have to give him the injections, and the vitamin supplements he's taking are for the long haul (they taste the worst too and one is a scary bright orange color). But, he is a trooper and doesn't fuss too much with all the things being pushed in his mouth.

Hope you all have a nice weekend! Our princess Eva Q turns 4 on Monday so we're doing special birthday things with her, making cupcakes to take to school Monday, etc. I'll have an update on Tuesday after we see the doc again. Love, Phoebe