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

Tuesday, July 10th - Gastroenterologist

Starting with the good news - Dr. Christie (Mario's Gastroenterologist) liked the color of Mario's poop (we were able to view a live sample) saying that the color indicates bile IS getting out of the liver. This is good. Other than that, really nothing to report. His weight is the same as it was on Friday when we left the hospital...actually 30 grams less (4.48 kilos Friday, 4.45 today). We obviously want him to GAIN weight but the doc said not to get too worried yet, he is eating more each day (as proven by my tracking sheets) and urine/bowel activity is consistent with what he is eating so I'm supposed to go back next week to check weight again before I worry (easier said than done). Mario's bilirubin levels are also still high, but again, we are not supposed to worry. Dr. Christie said that is normal and might take awhile to come down. I quote "We (Dr. Christie, Dr. Sawin-the surgeon, me and Israel) are all in a waiting game right now" so it is best to be patient. Like I said, easier said than done... I see Gastro doc on Tue and surgeon on Friday next week so time will tell. Keep the positive energy flowing in Mario's direction and we'll keep you posted as we know more...love, phoebe

Monday, July 9th

I got another great photo - see right - of Mario today smiling. This was taken only moments after I injected him in the leg with one of his meds. He is such a trooper! Eva's birthday is Monday, 7/16 and we were planning to have her party with her cousin in B.C. this year...so, I'm quickly changing plans and getting things organized to do a little afternoon party at school for her. She's been a great big kid through all of this...also quite the trooper! More tmrw after we see the doc - love, phoebe

Sunday, 7/8

Well, the weekend flew by. We're getting our routine down with Mario's medications. The nurse approved my spreadsheet to track everything. I did end up having to do an injection...3X/week. I gave that one to him in the hospital on Friday with supervision from the nurse and it wasn't as bad as I expected. Mario is being a trooper, tolerating all the goo we have to put in his mouth. He gets uncomfortable at times, his incision gets sore, etc. But he continues to smile and eat and poop - all good news.

I have blood tests and appt with the Gastro doc on Tuesday so I will probably have more news then. Meanwhile, thanks for your continued good wishes.

Friday Afternoon

430PM: Well, we made it home, Mario has a full belly, bravely took his afternoon meds and is taking a nap. We are going to try to relax this weekend and be "normal" for a couple days. I have tests and doc appts on Tuesday for follow-up. Sounds like we might not know conclusively how well the procedure is working until as late as 7/20. That's when I have a series of appts with the surgeon and more testing. Hopefully his blood work will be heading in the right direction on Tuesday. Let's hope. Meanwhile, enjoy your weekend. I'll update this blog Tuesday after we get home from the marathon of appts. Our love, Phoebe, Israel, Eva and Mario - home together for the weekend!

Friday Morning

10:00AM Well looks like I have to do the injection afterall, they are bringing me an orange to practice on. wish me luck with my training :)

9:00AM I had to prove I can administer his meds... 2 antibiotics, 1 steriod, 1 pain, 1 gas, 1 vitamin = 5 oral doses, 3X/day. They were going to give me an injection too, but substituted a different oral supplement we can give (another vitamin). They are rounding everything up from pharmacy so hopefully released around lunch time. I'll update tonight from home! Sounds like I have multiple docs and tests on Monday so will be here again soon to see how things look longer term...

7:00AM Wow, I'm still in disbelief at the good news. The surgeon said that since Mario has pooped (especially since it is brown) and is eating well that we can probably take him home today. Probably...waiting for the GI doc to agree. He is still on a steady regime of antibiotics and steriods - all can be given orally. He lost a little weight yesterday, but he said that is normal fluid loss. He also said they will continue to check his bilirubin levels, but won't get anything conclusive until next week. So, no reason why we can't go home and come back in to be checked out on Monday. My GI doc did say that he thought it would be Monday before we leave, so I'm not getting too excited yet. But it is all great news! All the good positive energy and/or prayers are working. Thank you and keep it up! Love, Phoebe

Thursday update

AFTERNOONG: Big development, Mario pooped! Unassisted and good qty and color :) He has been eating like a trooper today! Up to 4.0 ounces at a go, I don't have quite that much milk to contribute so we've added a special formula that is easy to digest, as well as some special vitamins that are easier to absorb (both formula and vitamins specifically for his type of condition where the bile wasn't getting where it needed to go). Doc said we still have to wait to get conclusive blood test results so I'm supposed to focus on fattening him up and getting him to poop today. :) Seems so simple. Stefanie was here for lunch and Mario was able to get his IV tubes removed (still has the IV in, but not hooked to the machine at least) so we went for a "walk" and explored all the outside places, kids outside play area has a nice garden, plus there are a couple patios (5th floor and 3rd floor). So Mario got fresh air (mum too).

MORNING: Mario is steadily eating now. I forgot to mention yesterday that they took the stomach tube out. I have a photo to post once internet is back up. Should be soon. I am typing on my wee blackberry keys to post this. They stopped his morphine drip just now. One less tube is nice. Might get IV out today. Nurses are checking to see if meds can be done orally. He is still on antibiotics and steriods due to the surgery. They did some pretty major revisions in there. We still need him to make a normal unassisted BM (he had one last night with the help of a suppository). Details! Oh and I had a lovely view of fireworks from the window. More soon, Phoebe (.5.181)