TUESDAY, JANUARY 27, 2009 09:59 AM, CST

The sadness goes away quickly because his smile is too big to allow anyone to wallow. Such a happy bunny.

I know how lucky we are. He's here and his quality of life is really good. I want a guarantee that he'll have a good life - that he'll outlive me - but no parent gets that. Even with healthy kids, you can't get that guarantee. You just have to enjoy the time you have and try to make every day special and memorable.

I had been worried for a few days because last Thursday he started running a little hot. I took his temp about a million times and it always ranged from 99.1 - 99.6. That's considered in the normal range for an infant so they didn't want me to bring him in but I know it's a little hot for him. We all run cool normally. But the last time I took it it was 98.7. So that's pretty good. We'll see what happens Thursday...

SATURDAY, JANUARY 24, 2009 10:42 AM, CST

sigh. It's funny. I seem to not get sad at the obvious sad times. I just suddenly get sad in the in-betweens. I'm not horribly sad today - just a little twinge.

There is a woman celebrating her 25th transplant anniversary on my liver families support site. That's not a sad thing. But I am sad that all us parents with transplant babies are writing things like "How wonderful! That gives us so much hope!" I don't want to hope for 25 good years for my son. I want that to be a given.

I am sad that when I feel sad, I can't call my mom. I want to tell her about the condo and I want her to come here and hold Jack.

I am sad that the medicine I forcefeed my son 4 times a day is simultaneously saving his life and poisoning him.

I am sad that one of the little girls in the CMH study to see if kids can eventually be removed from all meds has now rejected for the first time ever. She is out of the study and back on the Prograf that poisons the kidneys. I wanted Jack to be in that study. I want that study to succeed so Jack can one day get off the meds.

I am sad that my baby has bruises on his arms from the blood draw fiasco AND red streaks on both upper arms where the elastic bands were tied and retied and retied and retied.

And I am sad that every time something good happens, I am terrified that something will go wrong with Jack to balance things out.

Two different families on Liver Families had their kid's belongings STOLEN from the ICU. The Nintendo DS of a child who is only one week post liver transplant. The special gifts sent by Pat Riley to an especially ill little boy who has had a multi-visceral transplant. People whose hearts are THAT black are running around healthy doing unthinkable things and innocent babies are suffering. Where is the balance in THAT?

Sorry.

On a happy note - Emerson's Colorado Medicaid was reinstated. That is a victory for all transplant kids, really.

THURSDAY, JANUARY 22, 2009 12:18 PM, CST

Well, we are back from liver clinic. Horrible lab experience. Horrible. Terrible. Very bad. Also....really not good.

Jack's blood is supposed to be drawn during his cyclosporine trough - basically between 7:50 and 8:20am. After that, the cyclosporine level will be inaccurate. 3 weeks ago they forgot to put in his bloodwork order and we missed the trough. Last week they forgot we were waiting and I missed the trough. This week I double-checked to make sure they had ordered the CMV blood test and they hadn't. So getting an order for that took 20 minutes. Then they only seemed to have phlebotomists I had never seen before. The first one tried his left arm and dug around until I began to sweat. I finally said "Stop. You have to stop this. Find someone who can hit the vein." She was very nice about it but I was panicked that we were once again missing the trough. I went and stood in the middle of the waiting room and stared at the supervisor. She got a second phlebotomist who tried his other arm and also couldn't get the vein. Then she tried his hand. They kept blaming other things. "The veins are good but the bloods not flowing." Yes. that's right. It's not YOU - it's the fact that his heart has stopped. What the.... Anyhoo- then they went to call the AI team. I think that's what they said - arterial intervention, I think. Not sure. 20 minutes later they had not shown up. We are now and hour and 5 minutes past his cyclosporine dose. I went again and stood in the middle of the waiting area. Alma (the supervisor) said "Did they get it?" I said "No." She said "Jesus! I'll do it myself!" so she came around from the desk and hit the vein instantly - at 9:30am. Those other two women will never touch my son again. Mark my words.

The numbers are slightly improved. His doctor was not impressed. They were each about 10 points lower. If they had been 50 or 60 points lower, she would've considered that the mystery problem was resolving. 10 points lower is basically unchanged. BUT they didn't order the biopsy because they didn't go up and Jack appears very healthy. They also discovered a pocket of fluid above his liver (they saw it on last week's ultrasound) This is really no big deal but if his numbers don't resolve or if he suddenly show signs of illness, they will tap and culture the fluid as well as do the biopsy.

In other news - because dealing with an ill child is not stressful enough for us, Sean and I suddenly find ourselves in escrow on a condo in Evanston. It happened so fast. Well, actually, we've been looking at that condo for 6 months and we got it for a song because no one is buying and the market is so crazy bad. Thank you for the condo, W! It turns out that our mortgage payment will be about the same as our rent here AND we get a big tax break from now on. Also, we can get rid of a car and Sean can walk to work so that saves money AND the laundry is across the hall (not down an icy, outside flight of stairs) and it is only 25 cents to wash and dry. Here we pay $1.50. It doesn't seem like a big deal but I do at least 7 loads a week so it will make a big difference.

It will be sad for me to move away from the city. i love the Andersonville neighborhood. LOVE it. And I love all our neighbors and all the Neo-Futurists live around here and the theater....sigh....BUT I think this is what's best for the kids. A more secure future, an investment, a GREAT school distrist (Orrington elementary, Haven middle, Evaston Township) And did I mention it's a LARGE 3 bedroom? It's big. 2 and 1/2 baths. We won't be crawling on top of each other anymore. of course, the place isn't ours yet. We don't close until March 24th so anything could happen. The bank could decide not to loan us the money, the building could explode (that's the kind of luck I tend to have)...who knows. But probably by the end of the business day we should be in escrow and although I don't know what that word actually means or whether or not I am using it correctly in a sentence, it seems like it's a big deal kind of a thing. My threshold for big deal things has changed considerably so it doesn't feel all that big - but my understanding is that it's a pretty big step....just not bigger than a transplant.

MONDAY, JANUARY 19, 2009 01:30 PM, CST

Well, Jack seems fine. He's eating pretty well and poops are normal. One of the other post-transplant moms in the support group said her son's numbers jumped around for awhile and also that he intemittently had those nasty, mucousy poops for months after transplant. I guess it makes sense. The poor kid's intestines have been severed a few times - there's got to be some adjustment in there.

I really don't want you to think we are sad or suffering over here. We're really happy. Jack is really happy. He's a great, active, smiley guy. I write things in here that I think are pretty lighthearted and then the reaction seems to indicate that people think we're living a tragedy. We're not. We consider ourselves to be extremely lucky. We are adjusting really well to our "new normal." It's true that life doesn't look or feel quite like we thought it would but you would be truly amazed how quickly it all becomes normal and you can't imagine it any other way. It makes you less afraid of any other life changes that may come down the pike. Time will make it all normal - a big move, a new job, even the death of a loved one. These are the things that shape your life. Then you adapt. We are so incredibly adaptable. It's a great comfort, really, to watch it happen. Medicine time is routine now. It isn't fun but we get it done as quickly as possible and then we move on. If you see Jack and try to touch him, there's a good chance I'll intervene and make you disinfect, but other than that we are every other family you might see. Only we got a first-hand look at what we could lose and how lucky we are so we are a little calmer and a have a few more wrinkles and gray hairs. That's all.

We hope you have a great Martin Luther King day. I told Lily it was MLK day and she said "I know him." I said "Really?" rather skeptically. She said "Yes. When he was little he wanted to go to school with everybody else. When he was big, he tried to make sure everyone of every color could go to school together." Wow. Amazing what a little schooling can do, huh?

SATURDAY, JANUARY 17, 2009 09:30 AM, CST

Hi- I didn't mean to sound actually angry at Dr. W. I love him. He's as pompous as one would expect an expert in Hepatology to be and has that social awkwardness that happens when you're supasmart that I find endearing and he did also say that even though Jack is not technically his patient, we should let him know when we're there so he can come see Jack cause he's a favorite. Awwwwwww!

As for Jack - I dunno. He seems to have diarreah now and I don't know how alarmed to be. Is it normal childhood stuff or a warning sign? It's a holiday weekend (isn't it always?) so I would only be able to speak to the GI Fellow on call. I've already established how much I trust the fellows so I am waiting a bit to see what happens.

His ultrasound results were that the bile duct is still dilated but not more than it was last time. They don't feel like it could be the cause of the number jumps because his bilirubin keeps going down. It is a concern, though, and they have to keep an eye on it. If some sort of stricture is causing it, they'll need to go to Interventional Radiology and balloon it open. They feel like they need to give him this next week to "declare himself" - meaning the time to develop some symptoms they can identify. Maybe that's what he's doing now with his GI troubles.

The more I sit here, the more I think I'll probably call the GI fellow and see what he says. sigh. I guess that's better than missing something. I feel like his appetite is decreasing as well. I'll let you know what happens.

In the meantime - I will tell you a quick thing. We had our meeting with COTA (Children's Organ Transplant Assoc.) to begin Jack's fundraising account. The woman told that it was started by a family whose child needed a transplant but they had to raise $300,000 first before they could list him because he wasn't covered. Their community came together and raised the money in 2 months and he was listed but he died while waiting for the organ. Horrifying. We are so lucky we have this great insurance but there's a bnagging fear - what if Sean loses his job? What if our insurance changes? So scary! Then, last night, I was reading about Emerson (I wrote about her before) her full transplant needs to be redone (that's pretty common - it could happen to Jack) and Colorado medicare dropped her coverage so she can't be relisted. If they don't repeal that decision soon, she will die. Her mom is organizing a big letter writing/media campaign - not only to save Emerson but to keep this from happening to others. If you want to help, go to www.cotaforemerson.com and read the last few journal entries. There are people you can write. If circumstances change, that could be Jack someday. We are lucky right now in so many ways. Gonna see if he'll eat some breakfast before I call the hospital. I'll let you know what they say.

THURSDAY, JANUARY 15, 2009 03:56 PM, CST

Well darn it anyhow.

Jack's liver function numbers went up again instead of down. That's the wrong direction. There are 4 possible scenarios:

1) some sort of blockage of his bile duct - though with his bilirubin continuing to trend down, that seems unlikely. Still, he had a very lengthy ultrasound today to try to get a look at it. Results pending.

2) CMV, EBV - some sort of hepatitis. They have put in orders for those tests for his next visit in a week. I am terrified of CMV but they said that since he is on meds to prevent CMV right now, contracting it would be unlikely. Of course, in October they said his liver wouldn't suddenly fail so, really, what do they know.

3) Rejection. If the numbers are not trending back down by next Thursday's labs, they will schedule a biopsy for Friday morning. If the results show rejection, they will admit him and we'll be inpatient again for 3 days. Our neighbor who usually cares for Lily will be at Disney next Friday (oh...to be at Disney next Friday instead of facing a possible biopsy....oh......Lisa, you lucky lucky lucky lucky %$*&^(^^&%$%$#!) Anyhoo - if we have to have that done, we'll need help with Lily that day - whatever day a week from tomorrow is - if you're interested - there is pay. Just don't make me call the service - they always send this really weird woman who creeps us out.

4) My personal favorite - his numbers start trending down next week and everyone gives a collective shrug and says "uh mmmmmmm huh."

As I was waiting for the ultrasound appt, Jack and I sat in the cafeteria and along came Dr. Whitington. He joined us and ate lunch with us. He's famous, you know - in the Hepatology world. This means about as much to regular folks as my fame in experimental theater. There are 3 people in the world who might see me and gasp "Oh! There's City Girl!" Everyone else just thinks I ought to wear make-up and get a haircut.

Anyhoo- I told him about Jack's head growth - something I had been dying to do ever since it started growing again - and his reaction was completely unsatisfactory. He was supposed to say "You were right! That's amazing! I'll remember that the next time a mother comes to me in tears." You know what he said? He said "Good. Now you and your pediatrician can stop obsessing." I said "It was really alarming." He said "I told you there was nothing to worry about." I said "You said it wasn't related." He shrugged and said "Well, liver disease causes all kinds of problems - we know it inhibits bone growth. We know it can cause brain cells to atrophy. Maybe it was that. It all fixes itself anyway so...you know...(another shrug.)" ARRRRGHHHH!

But anyway - he's supasmart and famous and he did save Jack's life that one time and he ate with me so it was still kind-of exciting.

ps - We were put in Exam 1 today and I said "Good cause we always get bad news in Exam 4" and the nurse said "Then you shouldn't go in that room anymore." Hurray for other people as superstitious as me.

'

THURSDAY, JANUARY 08, 2009 03:45 PM, CST

Jack had liver clinic today. His bilirubin went down a bit - that's good. But a couple of his liver function numbers went up. That's not so good. His doctor said she wasn't super concerned right now. They are not elevated to a dangerous level and it could have been caused by the virus he is just beginning to shake or a cyclosporine level that might be too high.

They called later and ruled out the cyclosporine level as being the cause. The level was fine. So they will just wait until next week's clinic and see where the numbers are then.

I knew things wouldn't be perfect when we were put in room 4. There are 4 exam rooms in Hepatology. Exam 4 is where Jack was diagnosed with BA and then we were never in room 4 again until the day he was diagnosed as being in liver failure. Then we were in there again today. I get palpatations in that room. But it seems like a crazy thing to say "any room but 4, please." I am considering it, though. That room has it in for us.

WEDNESDAY, JANUARY 07, 2009 09:31 AM, CST

A few words about Jack's head. If you recall, when last we left this topic, we had seen numerous doctors and specialists and had had a dizzying array of tests - to no avail. We spoke to two different Hepatologists who insisted - time and again - that his head growth could not possibly be affected by his liver disease. We spoke to his chief nutristionist who had no idea what to make of him. We spoke to a Neurosurgeon who said that it did make sense to think that the malnutrition caused by his liver disease - mainly his inablility to absorb fat and fat-soluable vitamins - could affect his head growth but he would defer to the Hepatologists who would know more about that than him. We saw a Geneticist who said that he didn't see any common genetic disorders by looking at him but that didn't rule out a genetic disorder and he had no idea about the effects of malnutrition (not his specialty.) My friend Heather spoke to her professor who is a nutritionist who said that his inability to metabolize fat would definitely affect his head growth. We spoke to two neurologists in the hospital - one said that his nutrition issues could have caused his head to stop growing. The other more senior neurologist said that when brain growth is stopped or slowed due to metabolic problems, the brain grows abnormally. Since Jack's brain was normal on both the CT and the MRI, they would conclude that it could not be metabolic.

During all this, I went from hysterical to resigned to defiant. My pediatrician and I finally decided that the problem was nutritional unless proven otherwise and that once all was said and done with his liver, we would hope for some normal growth.

Well, my friends, Jack went to the pediatrician and had his head measured yesterday. Since his last visit on November 24th, his head has grown 1/2 an inch. From August 28th - November 24th (3 months), his head grew 1/4 of an inch. In the past 6 weeks, it has grown 1/2 an inch. That is normal growth (actually, I think it may prove to be slightly above normal as most of that growth happened in the 4 weeks post-surgery.)

Now, I'm not saying that this growth couldn't stop as suddenly as it started. Maybe it will. Maybe there is no correlation. I don't consider the problem solved. I am staying in the microcephaly support group until Jack is no longer clinically considered microcephalic. But it does seem awfully coincidental......and it makes me very happy in spite of myself.

Another note about Jack. Many babies with BA become very weak and lethargic due to the malnutrition. We always felt lucky that Jack was so happy and seemingly healthy during all this. He has always been so good-natured. He would sometimes be content to sit on my lap for hours while I chatted with people - very different from his sister the whirling dervish. Now we know, however, that he actually was subdued. The real Jack Benjamin loves to bounce. He jumps and bounces and lunges for things and can't sit still. He's a real handful. Right now, I'm very happy about that. Talk to me again once he starts crawling and locating and swallowing all the Barbie shoes we have lost over the past year.

Finally - my latest dilemma - to move or not to move. That is the question. We don't fit in this apartment. We discovered this when we began moving all the baby stuff back in before Jack was born. We are two adults, two kids, and two cats in a long, skinny two-bedroom. It is miserable. In the small spanse of time between my father's death and Jack's diagnosis, we started looking at condos in Evanston. We would like to buy so that we can be a more stable family financially and so that someday we will have something to leave our children besides show posters from experimental theater productions. In the current market, we have just enough to put down a deposit on an affordable condo as long as we don't allow that money to be whittled away by medical bills. We saw a couple places that would be good - great schools, Sean could walk to work, more room. Then we gave up on all that to deal with Jack. Now our lease here is up in the spring. Not sure what to do. I like our neighborhood and have a great support network of friends - this is invaluable for us right now. Also, Lily goes to a great public pre-school here. There are none in Evanston. We would have to find a vacancy in a private pre-school and pay out the whazoo to send her there. I don't know enough about the real estate market to know if we are safe to wait until Spring of 2010 to buy. If prices go up too much, they will go right out of our range. Sigh... It's not really a liver issue but it is what I am worried about today. I have to do what is right by my kids. I don't think it is good for our future as a family to keep throwing money away on rent. Anybody know anything about this stuff?

SUNDAY, JANUARY 04, 2009 05:56 PM, CST

Today marks one month post-transplant. That is a very big deal. There is a 10% mortality rate in the first month. There is a 2% mortality rate from month 2 - 12. Much better odds ...if you are playing the odds. Of course, Jack Benjamin is unique.

We have a whole lot of people to thank. I am slowly working on thank-you cards and stuff but I want to mention it here as well:

Thank you to my sister Robin and brother-in-law John for dropping everything for an entire month to move into our tiny apartment and help us. You are amazing and wonderful!

Thank you to Steve, Jay, Diana, and Chloe for agreeing to be the officers of Jack's COTA campaign.

Thank you to Steph and Bob, Sharon, Liz and Doug, Rich, Angela, and the Grondy's, the great people at the Northwestern student health services, the wonderful members of Erma Benjamin's church, Carol and Tom, Anita and Makoto, for help with lost finances.

Thank you to Lisa D. for taking care of Lily so often.

Thank you to Jill for housing Grandma Erma and bringing food over.

Thank you to Joe, Heather, Rachel, Chloe, Sharon, Jay, Diana, Lisa D, Lisa B, Steve, Siri for hospital visits complete with food, gifts, reading materials, an especially lovely board game, support, and laughs when I needed them most.

Thanks to Steve, Phil, Kristie and Jess, Shoshana and everyone else who brought food over to our apt.

Thanks to Bambi and Siri for taking the 3am hysteri-call.

Thank you to everyone who reads this journal and for all the wonderful posts. I wish I could explain how important they are to a mom sitting next to a hospital bed. I can't. Just know that each and every post means the world.

Thank you to Steve for filling in for me and staying with Sean throughout his entire hospital stay. Thank you to Phil for driving them over and thanks to both for the updates from NMH on transplant day - like "Sean was taken down at 7:15" and "Phil just ate a bagel."

Thanks to Phil for filling in for Sean for the Drinking and Writing shows.

Thank you to the amazing nurses and doctors of 6 West and the PICU at Children's Memorial for taking such good care of our boy.

I'm sure I have forgotten people and I'm sorry if I did. I am so grateful to everyone. Thank you thank you thank you thank you!!!!

FRIDAY, JANUARY 02, 2009 10:41 AM, CST

I have been up for 3 hours and am finally getting a chance to sit and write. First - to backtrack. Wednesday Jack had liver clinic and his numbers were great. His direct bili was 0.7, his total was 1.2. All numbers were trending in the right direction and a few were actually normal. He is a wonder of modern science.

Of course, since life is a delicate balance, as his liver numbers get better and better his cold gets worse and worse. It would probably be worse than the rest of us anyway because he's so little but he's also on 3 different immunosuppressents so he doesn't stand a chance. New Year's Eve was very exciting. Sean and I both fell asleep before midnight even though we were trying to stay up. I was with Jack in the bedroom, Sean was watching the Simpsons in the other room, and the next thing we knew, it was 2009. I woke up because Jack can only sleep an hour and a half at a time. Then he wakes up unable to breathe. He has figured out how to clear out his nose while he nurses so I nurse him back to sleep for about 20 minutes every hour and a half. It's like having a newborn again. At about 1:30 on New Year's Eve, Jack woke up and I started to feed him and then suddenly he was making a horrible barking noise and was having a hard time breathing. If it had been Lily, I would have taken her into the bathroom and turned on the hot water and steamed her for an hour. Since it was Jack, we went to the ER. He was diagnosed with croup and given a breathing treament and an extra dose of a different kind of steroid. Then he had to be observed for 2 - 3 hours. We finally left at 6:30am. Yesterday was mostly about catching up on sleep. So, if the idea that whatever you are doing on New Year's Eve indicates what your year will be like, we might be in trouble.

This morning was my first without help. My sister went home last Tuesday and Sean went to work this morning. It was wild and chaotic with some screaming and flying baby food and lots of snot and a leaky diaper and unreasonable demands made before I could get my coffee and as I sat on the toilet nursing my son (yes, that's what I did -it's not pretty but it's real,) I started to laugh. This was the chaos I signed up for. It was great.

About the passing of 2008 - someone on Facebook wrote "&*$% YOU, 2008!" and lots of people agreed and I considered it for a moment. But even though there was some seriously bad stuff in 2008 - my dad's death and Jack's diagnosis and failed kasai and acute liver failure, there were some great things - like Jack's birth and his life-saving transplant. I can't possibly curse the year this beautiful boy came into our lives. I will be forever altered by 2008 - in many ways - good and bad. I look forward to a less eventful, less life-altering 2009. I wish that for all of us - happiness and HEALTH in 2009!

MONDAY, DECEMBER 29, 2008 10:40 AM, CST

Well, Jack caught our cold and had a bad night of coughing and difficulty breathing - but he still woke up at around 7am with a huge grin. Such a trooper! It isn't a terrible virus. Lily had it first and then I got it. It hasn't affected us too terribly but it is his first on the heavy immunosuppresents and so I worry.

Anyhoo- there is a little girl in Omaha who is very sick. I read her mother's journal - even though I don't know her - the same way many people read Jack's. Her mom is part of my liver support group. She is calling for prayers today and I thought that since you all were so good at sending prayer and healing thoughts to Jack, you might be able to add Emerson in as well. Of course, Jack still needs you - please don't forget him - but Emerson is really touch and go right now and she could use your help. Eme has an unknown metabolic disorder and in June she had her colon, spleen, and 2/3 of her stomach removed and her liver, small bowel, and pancreas were transplanted. It's called a multi-visceral transplant. She came through the surgery and even walked for the first time and then the infections started and she has been touch and go for almost 3 months. She is a beautiful 2-year-old with dark hair and dark eyes and chubby cheeks. She loves princesses and dress-up - just like my fancy girl. She needs our good thoughts right now. You can check on her at www.cotaforemersonw.com

I just got pooped upon. Gotta go.

SATURDAY, DECEMBER 27, 2008 02:54 PM, CST

Once upon a time, I was a late-night performance artist. I did a show that started at 11:30pm on Friday and Saturday nights and often the line to get in curled around the block. My boyfriend and I would do the show until 1am, go out for drinks after, head home around 3am and then sleep until 2pm when we would possibly jump in the car and head to a movie and eat popcorn for our breakfast/lunch. That was so much fun. If you had told me at that time that I would someday soon be up each morning at 6:30, I would have fainted dead away.

But fastforward 5 years and here we are - boyfriend and I are now married with two children. One of them wakes up regularly at 6:30am with the biggest toothless grin. And I love it. It is our time. Just ours. We snuggle in the half-light. Sometimes I sing or maybe we watch the news. We know that in two hours the day will begin - with its 22 syringes of medicine - all given on a rigid time schedule - that have to get down his throat somehow - even if he is screaming in protest. There will be feedings in between that and competitions for attention with his sister. But from 6:30 - 7:30am, there is only me and Jack and we are safe from the cold and all the dangerous things that lurk outside (there's a whole new fear level when you think "Oh my god - can you imagine getting him through this transplant only to have him hit by a car?!") And it's so quiet. There's so much peace - away from beeping, blinking monitors and alarms marking the emptying of another syringe or IV bag. When you wake up, for weeks, to doctors at your son's bedside or a stranger draping a lead sheet over you to protect you from the morning chest x-ray, it is a beautiful thing to have that peace - and the ability to appreciate it.

I have heard many mothers of sick children claim that they wouldn't have it any other way. That sentiment always horrified me. And certainly, if I could choose health for my son over this horror show, I would. But I used to be so irritated to be up at 6:30. I would feel so bleary and awful. Now I know how beautiful it is - and that I wouldn't trade for anything.

FRIDAY, DECEMBER 26, 2008 06:05 PM, CST

Well, OK. I'm no doctor. Jack's labs are really good. Not totally normal yet but everything is trending in the right direction right now. Platelets are on their way back down. So is GGT. Direct bili is 1.0, Total is 2.1. His cyclosporine trough was between 200 and 300 - just where they want it. He had an enormous poop while we were waiting to be seen (we had to open the door of that little room. whew!) and the color was fine. They don't think there's much to do about the gas pain except give him some mylicon and hope it helps him. Some babies just get that pain worse than others.

Then we came home and Jack ate 2 helpings of cereal and applesauce.

So - there you go. Today is a good one.

THURSDAY, DECEMBER 25, 2008 11:38 PM, CST

It's a funny limbo - this place between undying gratitude and self-pity. I know how lucky we are. Jack was home for Christmas. He had a pretty good day today. For the past two days, he was really irritable - few smiles, lots of crying. It was a constant effort to keep him just satisfied enough to silently stare at what was going on around him. I would love to know the exact source(s) of the problem. I mean, he has plenty of good excuses to be grumpy - but is it withdrawal or gas or post-surgery pain or hunger? Are the drugs upsetting his stomach? Is the gas pain a more major issue or just normal baby stuff? No clue. BUT today was better. Today the crying was within the realm of what a normal baby would do. And there were lots of nice smiles and belly-laughs. Of course, there were also some light colored stools that look like the ones he was having pre-surgery. We have labs and clinic tomorrow. When they call with his results around 2pm, I am prepared for them to say "biopsy." I think something is wrong. I hate this place - the not knowing. The place where you live for days and days or weeks and weeks or for the forseeable future with nothing more secure to cling to than "There is nothing horrible happening right this second....that we can see...right this second." Are there people who are better capable of living that way? I don't like limbo. I don't like surprises. I had a CVS test in my first trimester to make sure he was healthy. I found out the sex. I don't want a surprise.

And yes - I know that I am lucky. I have him here. He'll snuggle me while I sleep tonight. There are lots of other babies in hospital beds right now. There are mothers who are being told to prepare for the worst. I'm not being told that. Two children died in a house fire in Chicago a few days ago. Healthy children are suddenly ripped from their parents every day. I know that we are lucky and he laughed at us today and my sister and her husband are here - and my cousin and her husband - and my crowded little apartment is warm and filled with happy chatter.

But there is also the melancholy that lingers just outside of all that light and chaos. When you dream of your perfect, healthy family, it's things like this - holidays with family, birthdays, the first day of school, trick-or-treating - that you daydream about. The information that I really think his bile is not flowing properly hung heavy on my shoulders all day - while unwrapping gifts, while tickling Jack, while talking to loved ones on the phone - it was always there. And that is what life will always be. I can't imagine a time when we will be able to forget and just have fun.

It is selfish to wish for more than the miracle that I've already been given. I apologize. Disregard what I wrote.

TUESDAY, DECEMBER 23, 2008 06:18 PM, CST

Hi-

Just to clarify the last post (because I think I often leave out pertinent details) I am no longer pumping. I pumped in the hospital because Jack went two weeks without eating. I came home with a huge bag of frozen breast milk in the aformentioned crumbling bottles. They take up half the freezer. I think I'm just going to throw away any that look broken. There will still be quite a bit left.

Anyhoo- Jack's ultrasound today was not too bad. The Radiologist thinks his spleen looks good. This is good because at our later appt with the transplant nurse practitioner, we were told that if they were to find a splenic infarct, there is nothing they could to to help it. The spleen would eventually just die and he would live without one but he would have to be on prophylactic antibiotics because the spleen helps fight infection. But right now, they think his spleen looks good. So good.

The Radiologist did see that his bile duct is dilated. They are going to show the ultrasound to his surgeon. It could be that it is simply how it looks from transplanting the liver of an adult into an infant OR it could be a problem.

His labs were good but not perfect. His platelets and white cell count were both elevated last time. This time, one is elevated and one is OK but I can't remember which. oops. His GGT is up again. It was up last time too so that seems to be a trend so that could indicate a problem, too. His total bilirubin is a little bit down but his direct was 0 last time and this time it is 1.1. She said she's inclined to think that the 0 was a mistake more than that his bilirubin is actually rising. I really hope so. His cyclosporine level was too low so they are increasing his dose a bit.

The plan right now is that we wait until Friday and draw labs again and also wait for the surgeons to see his ultrasound and then they'll decide if they think he'll need a biopsy. His NP, Ilana, told me to prepare for him to need a biopsy next week (inpatient again, whee!) but hopefully his numbers will straighten themselves out.

That's it. Barring fever, blood in his stool, or other reasons to rush him to the hospital, we'll be home for Christmas. It still doesn't seem like Christmas to me - I missed all my favorite building-up-to-the-big-day activities. I need to go turn on 93.9fm for some 24 hours of Christmas music. Ho ho ho, everybody!

SUNDAY, DECEMBER 21, 2008 11:28 PM, CST

OK - So, at the hospital, they will give you all the supplies necessary to pump and save your breas tmilk when your child is too sick to eat or is NPO (more hospital lingo - it means he can't take anything by mouth) Anyhoo- they give you these sterile 2 ounce bottles that have an attached lid that snaps on tight and you can also screw a nipple onto it and you can use it like a regular bottle. They put them in bags and store them for you in the deep freeze. But here's the thing - when they get extremely cold, they break. They sort-of crumble. I mean, the hospital buys them specifically for this purpose and they won't let you bring your own - and the ones they give you fall apart. So tonight I made Jack cereal with some thawed out breastmilk and toward the end of his meal, he started to gag. Then he sort-of chewed as if something was in his mouth, then he gagged again. I rushed him to the bathroom and started swiping out the inside of his mouth with my finger. Guess what came out - a piece of plastic from the bottle. Can you believe it? I said "When is this kid ever going to stop scaring the pants off of me???" but I know the answer to that one. Anyway - I thought that was pretty crazy. That's my story.

SUNDAY, DECEMBER 21, 2008 06:45 PM, CST

Today my brother-in-law said "Price of a new liver, $450,000. Hearing Jack laugh again, priceless." It's true. He's laughing up a storm and he just starting chattering again which was the first thing to go away when his liver started to fail and stayed away until last night. He has added "y" to his vocabulary - his "aaaaaaaaaaaaaaaaahhhhhhhh" is now peppered with "ya ya ya ya ya"

Lily caught a cold. It hit her very suddenly this morning. She sneezed twice, coughed, and now she is in full-on cold mode. I am trying not to be terrified by it. We are all cooped up in this tiny apartment - 4 adults, 2 kids, 2 cats - it's 30 below with the wind chill so there's no going out. We are all breathing recycled air. He is on 3 different immunosuppresents. The chance of him catching this is great. We are washing our hands like maniacs and taking vitamin C but it is probably just a matter of time before we all have it. They did tell me that his body can fight it some - just not as efficiently. He can recover - just not as quickly. sigh.

But for now, except for the threat of a cold and the nagging fear about splenic infarction which we have to wait until Tuesday to find out about, he is doing well, eating cereal, and took his last dose of methodone this afternoon (last dose of Ativan is tomorrow.) The nutitionist called and said that as long as he is nursing 6 times a day and eating 3 servings of cereal made with breastmilk, he has no need for formula. No one wants to fatten this kid up more than I do but we're gonna do it the right way. So far so good.

THURSDAY, DECEMBER 18, 2008 04:36 PM, CST

Well, Jack had his first post-transplant liver clinic this morning. It turns out that his doctor whom I really like doesn't follow the kids once they are post-transplant. Now he sees other doctors I'm less familiar with including one that I simply don't trust. The day after we were admitted, minutes before Jack's doctor came in and said he was in acute liver failure and needed a transplant right away, she was about to send us home. He main concern was that she was "shocked" that Jack hadn't been started on solid foods yet and that he needed to start on that right away. Jack crashed about 36 hours later. What if we had taken him home? Not to mention the fact that it is really common practice for a baby who had just turned 5 months old the day before to not have had cereal. Cereal does not have more calories or fat than breastmilk. Our pediatrician doesn't start anyone on solids until 6 months. That is pretty standard.

Anyhoo- she saw him again today and harped on the cereal and on giving him formula again. And she's wrong. So I have to ignore what she says. So that's two strikes against her now so I don't trust her. So what do I do about her advice in the future? How can I not take everything she says with a grain (or 6) of salt? I called Jack's transplant nurse and told her and she agreed with me and promised to talk to the nutritionist and the head of tranplant.

In other news - ummmmm........bump.

Jack's labs were very very good (Direct bili - 0, total bili 2.2) ALT and AST normal, GGT slightly elevated but not alarmingly so. BUT - his platelets and white blood cell counts are quite elevated - yet he has no fever. They had a meeting about him and they believe it is indicative of splenic infarction (which, now that I have googled it, is death of spleen cells caused by lack of blood flow.) Sigh. They said not to panic. They said nothing is urgent unless he develops a fever or other problems. SO - on Tuesday he is going in for an ultrasound and then more bloodwork and then an exam. I am just going to go ahead and bring my suitcase this time.

bumpity bump. Isn't life exciting.

WEDNESDAY, DECEMBER 17, 2008 10:12 AM, CST

It took me a full hour and a half this morning to draw up and administer all his meds. I am not exagerrating. An hour and a half. They cover, when all laid out, HALF of our dining table. Sean has gone to Target to get me a tackle box to organize them in. I am scared I missed one. I counted them over and over but there are so many.....whew!

Jack is all smiles this morning - except for a short bout of post-surgery gas pain. He is very happy. It is amazing to me that on Saturday I stood by his bed and cried. I said "I just want my baby back." It seemed like it would be forever until he was sitting on my lap again while I type his Caring Bridge journal and he pulls all the junk mail onto the floor. But 4 days later - that's exactly what we're doing!

His cheeks are beginning to swell from the steroids. We had just gotten them almost back to normal from the last time. The difference is, though, that last time he got the steroids from a voluntary drug study and I felt bad that they were affecting him so much. This time, they are saving his life. So he'll have giant cheeks (it's called "moon face") So what? There's also a really good chance that he'll have excessive hair growth (possibly his entire forhead and maybe his back) and gum growth - large swollen gums that may require dental surgery. So, he may be a little funny looking. But without these drugs, we don't have him at all. It's funny - the shift. With a healthy child, you worry about the tiniest things. On the day Jack was released from the hospital the first time - after his kasai - I went straight to the pre-school open house. I listened to parents fret over the pre-school curriculum and I started to cry (which totally freaked out the pre-school teacher) but all I could think was "I don't give a rats ass what the curriculum is. I want her to have fun. Life is short. Get messy and have fun. And I want Jack to make it to pre-school. That's all. I just want him to be 3 and a half some day."

Oh - this is kind-of funny. Sean and I were given a brochure for the Make-A-Wish Foundation and told that Jack qualifies for a wish. I think Sean and I must have both turned white as a sheet. So she quickly added "Oh! No! It's not just for terminal kids anymore." In my head, it sounds like "Eggs! They're not just for breakfast anymore." That casual. Surreal. It is funny in an also-turns-my-stomach kind of way. But who knows - maybe some day he'll want to go to Disney or meet a race car driver and we'll give them a call. Wild.

So, we ordered lots of Christmas cards. If you figure there's a chance I don't have your address, PLEASE send it to me at noeykrimm@hotmail.com

TUESDAY, DECEMBER 16, 2008 02:50 PM, CST

I almost can't believe I am writing this - 11 days after my 5 month old (who was in end-stage liver failure and hepato-renal failure only 12 days ago) had one of the biggest surgeries a child can get - they are sending Jack home tonight. I'll write that again. They have removed every tube, every line, every monitor and they are sending him home - tonight.

I had thought that if we went bump-free for a bit, they might send him home on Friday. I am flabergasted and panicked. I considered asking to please stay one more night but the head of the transplant program said "We hadn't planned to send him home but we started thinking - "What exactly are we doing for him here now?" and we would hate for him to catch something here because that is always a possibility in the hospital." So she totally jinxed him by saying that so I have to take him home or he'll catch something tonight.

I just had a session with the transplant nurse - we carefully went over every take-home med (12 different meds total - between 22 and 24 doses per day depending upon which day it is) and I was quizzed on things like:

1) Reasons to call the doctor

2) Reasons to rush him to the emergency room

3) Which pets can he never own

4) What foods and drink can he not consume with his meds.

etc. etc. etc. etc.

Crazy!

I also feel guilty. Little Josephine next door got her transplant 4 days before Jack. She is still here - being biopsied for rejection today. Her mom and I bonded. I don't know how to tell her Jack is being released. It's funny how you wrap your own child's progress in the progress of others. I look at her and gauge Jack's progress - I'm sure she does the same.

The Dr. said that there's a good chance we'll be back. About half the transplant patients that go home come back fairly soon after. But, she said, that means half of them don't! She doesn't mean ever, of course, we'll be inpatient again. He has to come back for every fever - even if Lily also has a fever. Even if we believe it's a cold. It is too dangerous to take a chance. We'll be back when lab numbers are off - and they will be sometimes. We have to be prepared for that.

But today he can go home. He can see his sister and play with his toys. He can wear his own clothes and sleep in his own bed. That's gotta make a guy feel better, dontcha think? After 3 weeks - 21 days - in the hospital? Yeah. I think so.

I'm a little too freaked out to be thrilled. Everyone is asking if I'm thrilled or excited. Mostly I am a little terrified. I guess we'll see what happens. Stay tuned. The adventure is just beginning, I think.

MONDAY, DECEMBER 15, 2008 09:33 PM, CST

It is with the quiet reserve of a British gentleman that I tell you - without pomp or circumstance - that Jack was moved out of the PICU today at lunchtime and is confortably settled in his private room on the 6th floor. Jack's labs this morning were rock star (not the labs OF a rock star - those, I imagine, would be quite bad - they are notoriusly hard on their livers) but very very good. His bilirubin was HALF of what it had been the day before - total - 3.8, direct - 1.9 (which is almost considered the high end of normal) He is smiling and laughing, nursing and pooping - all the baby stuff other moms take for granted.

This is NOT, Universe, any indication that I would expect the same tomorrow. I know you and I am fully prepared for the numbers to rise again. I am bracing myself for the words "rejection" and "infection." But my friends and Jack's fans have been worried and they have been tirelessly supportive, and they deserve to know that TODAY Jack looks terrific and mom and dad are pleased about TODAY. whew. The Universe is a tough lady to please.

By the way, I was squirting the Nystatin into a little cup and it squirted up into my eye. Jack's nurse says my eyeball will definitely not fall out. Yes, I just asked that.

SUNDAY, DECEMBER 14, 2008 12:48 PM, CST

Just a short update today. Jack looks really good. He is awake and alert right now, kicking his legs, chewing on his blanket and smiling a bit. He is almost an hour late for his methadone and he is not displaying any discomfort.

His liver numbers are slightly improved today. his CT scan from yesterday did show two pockets of fluid. One that we already knew about that is in front of his liver and another they just discovered behind the liver in the area next to the connection between liver and small intestine. They believe it is a bile leak but that it is not active. They think he had an early initial leak that has stopped and they believe that it is the reason for his unstable liver numbers They think that it may be putting pressure on the duct and causing some stasis. But his direct bili is down a bit today again. They say his liver numbers are looking better. There is a chance that they will go in with a needle and draw that pocket out. They have taken him off the antibiotics they put him on when his fever started and they are going to see if he spikes a fever again. If he does, they will draw it out to see if it's infected. Otherwise they are just going to watch it.

I need to lie down now.

SATURDAY, DECEMBER 13, 2008 10:24 PM, CST

Santa will be bringing Jack and Lily a very large play kitchen this year. Don't ask me how I know - I just know. Don't tell Lily. I can imagine her running into the room and seeing it there for the first time - I can imagine the look on her face. That will be pretty great. But you know what's even better? The joy she gets from the tiniest things. Like when I took her to the Starbucks over here at the beginning of our stay at Children's. They had just put up their holiday wreaths and when we walked in she said "(GASP!) Oh, Momma! It's Christmas in here! Isn't it BEAUTIFUL?!" and everyone turned around to look at her and smile. Or today, when we went to McDonalds - she did her classic take-the-burger-out-of-the-bun-and-eat-it-plain-and-then-eat-part-of-the-bun thing and she enjoyed it so much - she actually made it look delicious. Or when she clutches the happy meal toy as if it's a treasure or when we took her to see the choir of 10 little Jr. High girls singing Christmas carols in the Family Center and she watched them intently and clapped loudly. She knows how to celebrate the little things.

I was so sad today for most of the day. Sad that Jack looked terrible and continued to be barely arousable. Sad that he had to get an MRI AND an abdominal CT AND respiratory therapy and he was just limply moved from table to bed to table to bed like a rag doll. Sad that when he did open his eyes, Jack wasn't in there - he stared blankly and didn't focus. Sad that when they gave him a shot in his leg he barely flinched and didn't open his eyes. Sad that it had been so long since I'd seen Lily. Sad that when it was time to go, she asked me to please go with her and I had to say no - over and over again. Sad that I wouldn't get to take her to the annual Lucia Festival in Andersonville tonight. Sad that I would be spending another night on my chair in the PICU. Sad that I would be watching them put more and more drugs in my boy that I know are poisoning him. Sad that Christmas is getting closer and closer and our stay here is dragging out longer and longer. Sad that this is a roller coaster ride we will be on for the rest of our lives. Sad.

And then the overnight PICU attending came to see Jack and she was alarmed at his state of non-arousal. She said "I think we need to wean him faster." "So do we!" said Sean and I. So she put in an order to cut his doses in half - and that was enough to make things brighter. It's a pretty little thing in the grand scheme of things, I guess, but it's enough. And then - amazingly - as if he had heard we were working to get him out of that stupor- Jack woke up and his eyes opened wide and he took a couple sips of milk and he pulled out his ng tube and he stuck his pulseox monitor in his mouth and he chewed on his blanket and he looked me in the eye and he gave me about 3 half-smiles. And that's not little at all. That's a play kitchen and a pink tent and a new fancy dress and birthday party with cake all at once.

--------------------------------------------------------

ps - Bambi, I think the question is - what is he NOT on.

Off the top of my head, the quick rundown of his meds is:

Immunosuppressants: cyclosporine, imuran, and prednisone

Prophylactic drugs: Bactrim and another one that starts with a V are antibiotics to prevent infection, gancyclovir to prevent CMV, nystatin to prevent thrush, zantac for his stomach, norvase for his blood pressure, aspirin for blood thinning

Pain and sedation: methadone and adavan

Supplements: magnesium and K

whew! I think that's it. He may someday be on Prograf but they tend to start babies on Cyclosporine.

SATURDAY, DECEMBER 13, 2008 12:06 PM, CST

Hey there-

OK. So. His overnight EEG has a few indications that he had a seizure that they believe was caused by the bad drug interaction between the methadone and the cyclosporine. Unfortunately, it is going to take seven days to get him off the methadone and he can't be removed from the cyclosporine because it is his anti-rejection medication (well, one of them - he's on 3) They have reduced his methadone a bit and he is a bit more responsive and he seems to have equal mobility on both sides. He had his big MRI this morning and it looked good. I still haven't gotten to corner them and make them talk to me about microcephaly.

He still is having a problem with his right lung. There's a word for it and I can say it but I can't write it. Sounds like atalektesis but that couldn't be how you spell it. It looks too weird. He had a tap of his plural effusion which is outside the lung but pushes on the lung so it can't expand. The idea is if you get rid of that he would be able to cough up the junk in his lungs but so far - no coughing. Still, he seems to be breathing a little easier. He got his nasal canula removed (the oxygen tube that runs under the nose) because it seemed to be causing him fits. He was fussing and rubbing his face every 30 - 45 seconds or so for hours last night. Removing the nasal cannula didn't help that but it did prove he could go without it so it was left off. They finally gave him benadryl in case he was itchy. I think it is a withdrawal sign. I don't know if he feels itchy or what but it seems to happen when it is coming up on time for his methadone or adavan. Poor addicted baby. I get sick to my stomach every time they give him methadone because I know it is creating neurotoxicity with his cyclosporine but they can't cut him off cold turkey or there will be lots of other problems.

He doesn't have a fever anymore but they continue to watch his cultures. They also sent away the fluid from his chest for cultures. All are negative so far but it's early.

They believe that there's a possibility that the granulomas in Sean's liver were caused by hystoplasmosis which is an airborne virus that is spread by bird and bat poop which then ends up in the dirt and then when the dirt is stirred up - like by kids playing - and respirated - it gets in your body. Some people never get symptoms. Some get cold and fever symptoms. It isn't really a big deal for people. They sent off Sean's urine for a hysto culture. They said that they take lots of livers from cadavers - about 5,000 a year - and put them in people and probably half of them have granulomas for one of the many reasons they occur (hysto being just one of them) and they have never seen a case where hysto was lying dormant in the liver and was then reactivated when it was put into a different body with a lowered immune system BUT they feel they need to look for it anyway. Just in case. Of course, the granulomas could also be from inhaling the asbestos his dad removed from their house when he was a kid or chemicals from the oil refinery right next to his college etc. There is no telling. But since hysto is one of the reasons for granulomas that could possibly effect Jack, ( the other biggie being tuberculosis but Sean is tested for that a lot because he works in health care.) they feel they need to look for it and if they should find that Jack has live hysto in his body, they might possibly decide to treat it - or not.

Also - while most of his liver numbers are normal or heading toward normal, his bilirubin is rising again. His surgeon wants a CT to look for possible bile leaks or blockages. If there are none, they will give him a bolus of the steroid in case he is rejecting. Sean says that will only increase his power hitting, but you still have to have the talent and ability.

I think that's it. Still bumpy enough that you can't let go but not so much that your teeth rattle inside your head.

FRIDAY, DECEMBER 12, 2008 03:54 PM, CST

Brain bleed and stroke have been ruled out by his quick ventrical MRI. The EEG will tell us if he has brain activity that would indicate he had a stroke. That was hooked up an hour ago.

They still have not tapped his chest even though it is causing him a lot of difficulty.(!!)

Here's what we are told:

By Neuro: if they can rule out stroke, they will begin to assume this is a possible reaction of cyclosporine and methadone doing battle in his liver. Both are metabolized by the liver and they can sometimes cause a reaction that can include fever and drowsiness. They have cut down his methadone a bit now.

By ICU docs - his plural effusion is pretty bad. That can also cause fever.

Neurology is currently standing in front of me whispering quietly about his EEG that they are looking at. I am having rolling anxiety attacks. They are shaking their heads a lot.

OK. They don't see any indication of damage from a stroke or anything. They are going to leave the EEG on for a couple hours after he goes down for the tap but they think there's no evidence of stroke.

The team from Infectious Diseases was here - not once but twice. In fact, at the height of the activity, he was getting electrodes attached to his head while ultrasound waited to look at his lungs and Infectious Diseases spoke to Sean about the amount of time he has spent in the Ohio River Valley. All in our little room. Excitement.

Going to IR. More later

FRIDAY, DECEMBER 12, 2008 10:33 AM, CST

bump.

bump.

bump.

#1) I woke up this morning to a flurry of activity. Jack had spiked a fever of 101.4. They took blood for cultures from both his lines and took urine. Occasionally these tests can come back within the day but generally they take 24 hours. In the meantime, they have started him on some heavy anitbiotics in case it is infection.

#2) They never did a biopsy of Sean's liver prior to transplant. They did lots of other tests but not that. During transplant, there was a small extra piece of liver getting in their way so they cut it off and sent it to the lab. They also sent a lymph node to the lab because they saw one that had a calcification. It came back as nothing so they continued. They got the results of the liver workup on the extra piece and found that it is full of granulomas. Granulomas are formed in your liver as a reaction to an assault of some kind. Can be viral. Can be hystal (allergens) Can be fungal. Not sure of the origin at this point. They can be a clue to something or they can be nothing. But they were suprised by how numerous they were in Sean's (and now Jack's) liver. They asked Sean where he grew up. He grew up all over but they pinpointed Oklahoma and also his time here in Chicago as places where he could have breathed in stuff that would have caused this. They don't think it will affect Jack but they also don't know for sure. And they want Sean to see a liver specialist when recovers from surgery. Regina and Erma - don't panic. They said that probably 50% of people have these. They just were alittle suprised by how numerous they are and they can't ignore them because they can't ignore anything where Jack's liver is concerned.

3) He has a lip tremor that he has had for about 3 days. I never mentioned it. I chalked it up to the effects of the sedatives. He also hasn't been arousable much since yesterday so last evening I asked them to take down his methadone and adavan. His surgeon noticed the lip tremor and I told him about how unarrousable he is. They called for a Neurology consult. He came and he just left. Upon examination, he feels that Jack is slightly limp on his right side. They are sending him for a priority MRI to see if he has a brain bleed or a stroke. They are also going to hook him up to an EEG because of the lip tremors though they don't believe he is having seizures.

So - today - not going to the floor, obviously. They are going to give him an ultrasound and then tap his chest. They'll stick in a small needle and drain out the fluid between his lung and his chest wall. That should make it easier to breathe and to cough up the junk in his lungs. They think that his lungs may be the cause of his elevated temp. He'll be getting either a CT or an MRI or both on his head. Probably the CT first to rule out a brain bleed or something else they may need to intervene about.

I showed the Neurologist his head growth chart and asked what he thought. He said it's a definite concern. I asked if it could have been caused by Jack's inability to metabolize fat and fat soluable vitamins and he said - for the first time, people - drum roll - "Yes. of course." Thank you, universe, for that much at least. Of course, that may not be it at all - but he said it's a possibility which is more than we've gotten before.

ps - just to add a note - after the Neurology folks left, Sean said "Hey, Jack" and although he never opened his eyes, he smiled. Sean did it again and again. each time he said Hi to Jack, Jack smiled. I feel like that has to be a good sign.

Oh. MRI is ready for him.

THURSDAY, DECEMBER 11, 2008 09:57 PM, CST

Hi, everyone. This isn't really about Jack but this video (it's really short) was made about a little girl named Brooke whose mom is in my liver families support group.

http://news.medill.northwestern.edu/...aspx?id=109457

It is a good video and it is made even more significant because Brooke was in the hospital for rejection at the time of its release. It is just like the nurse who is the Biliary Atresia Research Consortium Study Coordinator said to us today. "One day he may be fine, the next day - maybe not. So today he looks good. Just today."

So we'll just be happy today. Each day he is on the mend.

Also, I am really embarrassed to say that even though my son was on the UNOS transplant list for 6 and a half days, I was not registered as an organ donor. You may not be either - even if you THINK you are. It is on the front of my license BUT many states now require that you register in other ways. You can go to www.donatelife.net to find out about the rules in your state. In Illinois, if you registered to be a donor with the state and put it on your license prior to 2006, it is not valid. It is really easy to register. I just did it online in about 30 seconds. There are children just like Jack whose parents are not a match and they are waiting on the list. There are children dying while waiting on that list. If half the population became organ donors, no one would have to wait any more.

THURSDAY, DECEMBER 11, 2008 11:44 AM, CST

Hey there-

So far so good today.

He still has effusion in his right lung so his resp. therapy is still Q4 (every 4 hours - dig my hospital lingo!) His liver numbers look good. His direct bili is actually 0 but that's a slightly misleading number as it can bond to albumin and read as indirect. Still - nothing that indicates to them that he is rejecting his liver at this point.

He continues on the methadone and adavan. It is all oral now which is necessary in order for him to be cleared to go upstairs to the liver floor and he needs to be on the liver floor for about a week before he goes home. Last night, he was having some major blood pressure issues that were not resolving even when given the adavan. Both his anti-rejection meds cause high blood pressure so many post-transplant babies have to be on blood pressure meds for awhile to counteract that. So the resident gave him a tiny dose of oral blood pressure meds and when it didn't fix the problem, she put him on a drip. Well, that helped his blood pressure BUT it keeps him in the ICU because he can't go upstairs until it is oral. Thank you! So now they are weaning him off his drip and onto the oral blood pressure meds again. His doctor thought that was a boo boo.

Still and all, to answer some of your posts, do I get mad at the residents? No. I don't. They are learning and Jack is teaching them. And so far, when there are questions they feel they can't answer, they call an attending. Or sometimes I say "Are you calling an attending now?" And they say "Yes. Right away." Jack's doctor is brilliant but he's old and someone will need to replace him so that other children with horrible liver diseases will be well taken care of. They haven't done anything terrible and they are always acting in what they feel are Jack's best interests. But they ARE the reason I don't leave here. I stopped them from putting an ng tube in him last night at around 10pm. They didn't realize he had taken 2 ounces from a bottle at 9pm. If I had gone home, I would've come back to find a very angry baby with a tube in his stomach. But they just want him to have nourishment. And they are young. And part of getting older is learning some patience. It is learning that sometimes it is better to sit back and watch the body work its magic than it is to intervene. Dr. Whitington has that kind of patience and maybe they'll learn some of that from him (and from Jack) and it will help some other little guy some day. The residents are sweet and they mean well and they all look about 16 to me which makes me feel very old. Well, that and the fact that everyone here calls me "Mom."

WEDNESDAY, DECEMBER 10, 2008 03:55 PM, CST

I don't want us to be here any more. We have been here 13 days - which I guess doesn't sound like much considering all that transpired in those 13 days - but it feels like forever. And we keep having these minor setbacks that are not a huge deal - they could be so much worse so I should definitely be grateful and not complain lest the universe really give me something to complain about (my mom, when we were crying about something she deemed inappropriate, would always say "You'd better stop crying or I'll give you something to really cry about." I said that to Lily once and she looked at me as though I had lost my mind and I said "You're right. That was silly." I digress) - but each tiny setback increases our stay by a day or two.

At around 2pm, while he was sleeping soundly, his heart started racing, his blood pressure skyrocketed, and his breathing became labored. There was a flurry of activity. They were afraid it was a lung problem so they got an x-ray. turns out now, we think he was just jonesing.

sigh. Baby is addicted to drugs. 6 days of heavy, heavy narcotics has caused him to form a dependency. He is now on Methadone and Adavan which they will wean him from slowly, you know, like a junkie. This morning a resident ordered all his pain and sedation to be cut off cold turkey. Smart. So, when he's off the drugs, he screams and twitches. And, of course, when he is on the drugs he spaces out.

This morning's labs show his biliruben down a bit but his other liver numbers were up a bit. Again a resident told me that his numbers might indicate rejection and they needed to talk about biopsy. Again I said "Mmmmhmmm" and yawned a little. Again his doctor said "These numbers are pretty consistent with what I would expect. Let's see what they're doing tomorrow."

He's not eating but since he is always either experiencing withdrawal or in lala land, food is the last thing on his mind. During rounds, after they talked about the fact that the resident cut him off his meds cold turkey at 7am this morning without taking withdrawal into consideration, she (the resident) suggested that they put an ng tube back in him to give him nourishment. I turned to Sean and said "That'll help with the anger." Then his doctor said "Why don't you just get his pain under control today so that he'll WANT to eat on his own tomorrow. Why agitate him further with an uncomfortable tube?" I really like him.

Meanwhile - I am beginning to know what it is to be a veal. I do nothing but eat, sleep, and loiter in a tiny tiny room. Well, every morning at around 6am, I put on my coat and take a walk through the ER, across the emergency driveway, and down the steps to the Starbucks to get my coffee. Then I go back. Sometimes the security guard recognizes me and waves me on. Sometimes I get to stop and dig for my blue wristband under my coat sleeve. Blue means I have a child in intensive care. I want to show it. My entire identity is wrapped up in his medical problem right now so if they don't even bother to see my band and recognize me as a mom with a baby in intensive care, then I am nobody at all.

TUESDAY, DECEMBER 09, 2008 05:58 PM, CST

So, my friend Nicky in Texas (Hi, Nicky!) asked me some questions and said that maybe others had some of the same questions. So here are the Qs and the As:

Will this new piece of liver grow with him?

Yes.

Is it possible for him to have this liver his whole life?

Yes. Theoretically. Liver transplant is relatively new. The first one was performed in 1963 and it wasn't until 1967 that a recipient lived a year post-transplant. There have been HUGE advances since then, obviously - including the use of immunosuppresents. There are people in my support group who are young adults who had transplants as babies. There is no data and there are no guesses as to what the average lifespan might be. That might be a good thing. I don't need to know about that. I'm assuming he can become an old man.

Will Seanís liver regenerate what was removed?

Yes. And it does so really quickly. Most of it will be back in a month.

I guess the answers to these questions will answer my last question which is why was it more ideal to have a donor liver instead of a piece of Seanís all along?

The short answer is that if you can keep a loved one out of surgery, you probably should. We were very lucky to have the option of living related donor. Not everyone does. But every surgery comes with risks. There have been two donor deaths since they started doing living related donor liver transplants. Also, although it is a really good option, the best option is to get a whole liver from a child of similar size because even though the liver Sean donated wasn't a bad size for him, the veins and artery from Sean's liver were MUCH bigger than the tiny veins and artery of Jack's that they had to be sewn to. It takes a lot of manipulation to get them to fit together without leaks or clots.

We would never have left Jack on the list and let him suffer. Sean was always our most probable option. But while we waited for our scheduled surgery day, we were listed and we had a tiny bit of hope that maybe Sean wouldn't have to have the big surgery

I think I remember something about size awhile back but I canít find it in the entries

Yes. That was a concern. The liver is naturally divided into 10 sections. The transplant team has to use two complete sections of the left lobe (skinniest part) of Sean's liver. It would have been possible for that portion of Sean's liver to be too big but we were SO fortunate - it was a great fit.

That's it. Now everybody knows a whole lot about living related liver transplant. Everyone should now make sure they are an organ donor on their drivers license. And talk to your families so that they know your wishes. Very important.

TUESDAY, DECEMBER 09, 2008 11:53 AM, CST

On New Year's Eve of 2006, I was bathing a 20 month old Lily. We were having a great time. She was in that stage where she wouldn't sit in the bath and I'd ask her to sit and she would laugh and laugh. We were putting soap bubbles on each others noses. I had one of those moments where you step out for just a moment and you think "Man, this is really good." And then the phone rang and it was my father and he told me my mother had passed away.

On the Saturday of Labor Day weekend this past August, we took Lily and Jack to the vegetable market across the street from the zoo. We spread out a picnic blanket and Lily - in full fairy regalia - danced to the music of the Beatles and Simon and Garfunkle being played by a live musician. We ate fruit and ice cream and again I stepped outside myself and I thought "This is exactly how I always dreamed things would be." We walked across to the zoo and Lily took her first paddle boat ride with her dad. I watched them paddle toward the middle of the lake and then checked my phone. 4 messages. They were all from a doctor I had never met. "Jack's blood test came back. Call us immediately."

So the universe has taught me that every time things are particularly good, it might be a set up for a cosmic kick in the tush.

With that in mind and without any celebration at all, Jack was extubated this morning and his blood gases remain great. They also removed his foley catheter which isn't particularly significant except to Jack. He's much more comfortable now. I sat in a chair and held him for a bit for the first time since Thursday morning.

His bilirubin is still on the rise. The transplant fellow (and I use 'fellow' in the medical sense of the post-residency/pre-attending step in the career of someone who wants to specialize. I was not about to refer to him as "dapper" or anything. Although, now that I mention it, he rather is) Anyhoo - came in and told me that his bilirubin had risen again and since it is day five, they have to consider it to be a possible sign of rejection and possibly biopsy his liver today. That's not the nicest thing to hear but thank goodness I have a decent learning curve. Anything a resident or fellow tells you PRIOR to conferring with an attending must be taken with a grain of salt. Jack's doctor said he has a hard time believing this could be a sign of rejection when the rest of his liver numbers are good. That doesn't mean that he's not concerned. He is. But he wants to wait and watch until Thursday morning before they decide to biopsy. It may be swelling at the site. It may be the blood transfusions and the dehydration. It's a scary thing - which is good, I think, because I know the universe now and if his bilirubin was normal I would have no choice but to feel relieved and wait for the sky to fall.

MONDAY, DECEMBER 08, 2008 05:51 PM, CST

So far, so good.

Jack is still on the vent. His body is still pushing all his fluid into his tissue. He is bloated and the fluid surrounding his lungs is causing the vent to have to work harder to keep him breathing. He can't do that on his own. Also, the patch of junk in his right lung went away and then immediately came back so he is still requiring respiration therapy every six hours. His doctors say that this is up to Jack now. They say that it is normal for kids to retain fluid this way post transplant - especially little babies. They say he will diurese when he's ready. Are you impressed? A week ago, I didn't know "diurese"was a word. Now I can use it in a sentence.

With all the fluid in his tissue instead of his blood, he is dehydrated. His blood is getting thicker. It needs to dilute but it won't until the fluid goes back into his bloodstream.

The residents all want to intervene. Today's rounds were all about the various things they can put Jack on to get him to pee and all the things they can do to decrease his hemoglobin (including good old fashioned blood-letting which is now called "phlebotomizing" in civilized society and is done with syringes and not leeches or knives and bowls but really, it's all the same thing.)

Jack's doctor said that they could do a bit of that if they want to (well, except the blood-letting. He nixed that one) but they'd be chasing their tails because it would be a very temporary quick fix and we have to be a little patient and let Jack start functioning better on his own. I tend to agree with that, myself. The constant balancing act with all these different drugs is scary to me and sometimes I think you need to step back and let him work through some of it himself.

We had a rough night last night because he is becoming immune to the sedation. He was awake from 2 to 3am screaming and crying - which is upsetting when he isn't intubated but is absolutely heartbreaking when he is because he can't make a sound. You can tell by his scrunched up face and the heaving of his chest and the way his blood pressure alarms start going off that he is crying but it is all completely silent. Besides his constant drip of pain meds which started at a dosage of 50 and is now at 200, they gave him morphine, versed (a sedative) and a tylenol suppository and he still cried for an hour. I rubbed his forhead and sang him songs. I told him about all the people who were reading about him and sending him good wishes. Eventually he fell asleep but was up again every hour and a half.

So, that's about it for today. He's doing well. The bumps he is experiencing are normal, expected bumps so far and those are the best kind. Of course, tomorrow could be a whole different story - that's what I'm learning. He's doing well right now - just right now. Tomorrow marks the first day when rejection of the organ becomes a worry. So I guess I'll need to get some sleep and then buckle up.

I hope everyone has a great week! Thanks, Neos, for the card and photo from the Christmas Party. I love everybody. So does Jack. I can't wait until he can smile that big smile again and prove it.

SUNDAY, DECEMBER 07, 2008 04:33 PM, CST

This is a roller coaster" they keep saying. "This is a long journey and there will be bumps in the road."

I have learned that roller coasters make me sick.

We hit a bump today. Jack is taking it better than I am but you gotta figure the sedatives help. Oh, for a sedative. I woke up this morning to the news that Jack's bilirubin has gone up. That's the wrong direction. Also his hemoglobin had skyrocketed from 8 to 13. They want it around 10 or else Jack is in serious danger of developing a clot. A rise in bilirubin can also be an indication of a clot. If he clots he could lose his new liver. So they called the ultrasound tech from home to rush in and give him an ultrasound. She showed up and his surgeon left because he has a tendency to stand over her and interfere with her work because he gets so excited. I stood behind her and held Jack's hand and tried to interpret an ultrasound even though i can't read ultrasounds. Where is Uncle Steve when you need him? So, I was more than panicked and crying and standing near to the trashcan because I might lose my coffee. I thought the ultrasound looked terrible. I thought it looked like there was no flow. I finally walked away because I didn't feel well enough to watch anymore.

The surgeons came back in and asked how the ultrasound looked. It was great. Blood flow into the liver still looks great. Blood flow out now looks good as well. It was his best ultrasound yet.

So - why the elevation in the bili? Why the higher ALT numbers? Both are liver function indicators. So far, we don't know. It can indicate rejection but generally the rejection danger is from 5 - 7 days out. We are only 3 days out. It could be because he peed out a ton of fluid yesterday - maybe he got a little dry. They don't know. They will run labs again tomorrow. If the bili is higher I will most likely lose my breakfast. I'm sure there are worse things that can go wrong with someone but bilirubin is my personal nemesis. For me, it is "the one who shall not be named" and every time it comes close to me, the scar on my forhead hurts.

So I cried when I heard his bilirubin was rising and then I cried when they said his ultrasound looked great. And I got the "roller coaster" speech again. The surgeon said that it is not until he is two weeks post transplant that we can start to exhale again. Even if we get past this bilirubin thing, we are heading into prime rejection season - and infection is always a dangerous possibility now as well. Maybe I relaxed too much, felt a little too relieved. No more of that.

On a positive note, the respiration therapy seems to have worked and the are working on weaning him off the vent. They have taken the settings down 4 times now and each time Jack has compensated and his blood gas readings now are great each time. If all goes well with this gradual weaning over night, they hope to extubate him tomorrow. The scary part of all that to me is that they will have to wean him off his sedation so he can fully breathe. That means he'll be upset and we can't put him under AND I can't pick him up. He is a crazy maze of tubes and wires. All I can do is give him a pacifier and stroke his forhead and sing and talk. I'm pretty helpless. He'll still be on pain medication so he'll be sleepier than normal but the sedative is what has been keeping him calm.

I am so tired I keep falling asleep sitting up. I am going to try to sleep a bit now because tomorrow will be hard.

SATURDAY, DECEMBER 06, 2008 11:49 PM, CST

Quickly - before I go to sleep - Jack's blood gases were bad again. They did a chest x-ray and discovered a pocket of junk in the right lung. Not uncommon when you are laying still after surgery and are not able to cough because of the chest tube. Respiratory Therapy came with this little vibrating massage thing and massaged that side of his chest and loosened things up. Then they did a bunch of suctioning. Not pretty or even remotely comfortable for Jack but very important. The ICU doc came in and said that it was great - they found the issue. Now they can fix it and Jack can move on.

Sean is doing well, I think. I have spies at home that tell me he is often to be found pacing the hallway - doing plenty of walking as prescribed and he took a long nap with Lily today and he was able to eat some food. He is smart about following orders and being diligent about his recovery. He'll do really well, I think. And his scar will be impressive.

I made a little collage tonight with photos of Jack and a copy of the gorgeous poem Genevra wrote for him. I put it on the post of his crib. I want people to see exactly who they're taking care of and how important he is to everybody.

Really really tired. Love to everybody.

SATURDAY, DECEMBER 06, 2008 01:59 PM, CST

They adjusted the vent this morning and the last reading of his blood gases is much better. His CO2 is down. yay! And did I mention before that he opened his eyes and they are white? WHITE - which is something we have never seen as he was jaundiced from birth. Amazing.

In the guestbook, my great friend Val mentioned that we should all get together some day - Jack and all his fans. I agree. You can rest assured that if we can get to an anniversary, there will be a huge celebration. Big. BIG. Everyone can come.

I also understand from the guestbook that Jack and Lily have some surrogate Grandmas in Lakeland. I think that's great as long as they realize that in a few years my children may be shipped off to them for a week so I can actually go to a movie and read a book.

The updates mean so much to me. You have no idea. I read them and reread them and some day Jack will read them, too. And if you haven't read Val's post about her pet duck, well, get on that because it should really be published.

SATURDAY, DECEMBER 06, 2008 11:00 AM, CST

We are about 41 hours post-surgery now. He had a pretty uneventful night and he is still so far so good. He had his morning ultrasound and the blood flow into his liver looks good and the flow out is still slightly compromised but not more so than yesterday. He is really swollen right now so they have started a lasix (diuretic) drip and his blood gases are off because his lungs are wet and can't work as effectively as they should. They think that once they reduce some of the fluid and dry out his lungs they can start to wean him off his ventilator - maybe tomorrow. They have to move very slowly and cautiously because once they start messing with the fluid balance while they are still giving him transfusions (his hemoglobin remains a bit low) there is an increased chance of clotting in his new liver veins and artery and that would be disasterous. Baby steps. It's all a delicate balance.

They have changed his pain medication. It has been a challenge to deal with his pain. They have raised the dosage, like, 5 times and he keeps fighting through it. ultimately I think that's a good thing - he's fighting - but it makes the pain hard to manage. He begins to struggle and we can't tell if he is angry about the oxygen and ng tubes of if he is in pain so they give him a boost of sedative in case it is the latter. His arms both have restaints on them to keep them from bending because if he is given half a chance he'll try to pull out his oxygen. So he flaps like a bird when he's agitated.

I am invited to attend Jack's portion of rounds when they do them. It is just like on Grey's Anatomy - everyone piles into the room and a resident reads off all of Jack's numbers and vitals and then they make a plan for the day. I have noticed that the main difference between rounds here and rounds on TV is that the residents on TV are all actually trained actors who know how to project and enunciate and use inflection. I would really like Sandra Oh to come here and do rounds.

I want to post photos of him now but I am computer challenged. He looked especially good when he first came out of surgery - before he got the vent and the maze of tubing. I sent out a photo on my phone last night to some of my friends with camera phones since I couldn't post any photos. Then I thought that they all must be thinking "How random. why was this sent to me?" and they probably think I'm losing my marbles. I'm not - I promise - there are just moments where there is precious little for me to do right now. Where is Rachel with her Sweet Valley High board game? If you have a book suggestion, let me know - though things will get much busier for me soon as they begin to wean him from the sedation.

There is this smell that babies have - you know the smell. The smell of sweet baby skin and Dreft detergent and milk breath and lotion. Have you smelled that smell? Everybody loves that smell. But for mothers, that smell has a physical affect. When mothers smell that smell, there is a tightening of the chest and a release of endorphins and milk starts flowing. I realized today that I have that reaction when I smell Jack's medication. For his first two months he smelled heavenly. For the past three he has smelled like a heady mix of steroids and bactrim and urso and aquadex and plastic. It has been contantly on his breath and seeps from every pore. So today, when I went over to the side of his bed where his IV bags hang (6 bags and lots of tubing) I breathed in and I had that mom reaction. And that can make you a little melancholy. BUT we are together - Jack and I - and we are warm and dry even though there's a wet snow outside - and he is still "so far so good" so that's enough to fight off the melancholy for awhile.

Have a great day everybody. Thanks for reading.

FRIDAY, DECEMBER 05, 2008 07:06 PM, CST

We have passed the 24 hour mark which is really significant. It is significant because we are now 1/3 of the way through the first 3 days and the first 3 days are critical. It is also significant because after a huge trauma like this surgery, the 24 hour mark is when the swelling really kicks in.

Jack hit a little bump this evening. Apparently, the spit in his mouth that sort-of bubbles up around the oxygen tube got the tape that holds the tube in place all wet - causing it to stretch. He started moving around and the tube fell into the right lung. There were lots of alarms and two doctors and four nurses and people saying things like "Stat!" and "Bag him!" which is very exciting on ER and really scary when you are standing 3 feet away and no one can tell you what's going on. They got a chest x-ray of him to see if his left lung had collapsed. That just came back and they said that it is the same as it was this morning so I guess everything is OK for now. The x-ray showed that they placed the tube a bit high so they are going to come down and adjust it soon. Jack is sleeping. I have body aches which I am hoping is exhaustion and not the beginning of the flu.

FRIDAY, DECEMBER 05, 2008 09:47 AM, CST

OK. So here we are 15 hours post transplant. Jack did really well last night. The exhaustion hit me shortly after I sent everyone home (thank you Erma, Rachel, Chloe, Jay, Diana) and went to the PICU. Jack would occasionally stir or grimace and i am really worried about his pain control. He is on a vent and can't cry or make any sounds. Last night he began to stir and then there were tears coming down his face. I feel like that is maybe my main job right now is looking for signs of pain and getting it taken care of. He is heavily sedated and medicated but with that tiny body it can cycle through quickly. My friend Mary recently had major GI surgery (Hi, Mary) and she described the post-op pain she had when the meds weren't quite keeping up. I can't let that happen to Jack.

Because of this, i felt guilty going to sleep last night but the walls began to look wavy - like when you are driving on the highway and you see that mirage up ahead. I went to bed around midnight and I slept until 6. I sat up a couple times when he was stirring but they seemed to be aware and acting on his behalf and I was having a hard time getting my eyes to open.

It's like walking on eggshells here. There is no success - only "so far so good" or "looks good right now". His doctor just reiterrated that this will be a long, long recovery that will be like a roller coaster. he said this is one of the biggest surgeries a person can have and we can;t expect him to not have issues. He does have a few issues they are watching. When they give him his pain meds, his blood pressure drops too low. They are giving him a transfusion right now that they think will take care of that and also his hemoglobin which was too low.

He had an ultrasound this morning to check the blood flow. They said the flow into his liver from the portal and anterier vena cava look really good. There is some compromise in the flow out of the liver through the hepatic artery. It is a concern but not uncommon. It could be due to some edema and his blood having to relearn how to flow correctly since it has had to reroute for so long due to the bad liver. They will look at it again tomorrow morning.

Cautious optimism is what we're allowed here. I feel like a bull in a china shop (is that the metaphor?) Like if I cough or bump his bed I will throw off his whole system.

Sean is doing well. He started walking around last night. This morning they took out a bunch of tubes and he will probably eat later on in the day. They are planning to release him tonight. I know it sounds like they are rushing him but actually, with a well person who becomes compromised by surgery this way, he is safer at home - away from all the staph infections and bacteria and viruses and all the stuff that could make a well guy not very well.

Lily is home with aunt Robin and uncle John. She is doing as well as can be expected. It is hard when both mom and dad are gone overnight. That hasn't really happened before. I'm so glad Sean will be able to go home and be with her.

Oh! One last thing - prior to surgery, Jack's total bilirubin was over 27. Right now it is 8. Kidneys are functioning fine.

THURSDAY, DECEMBER 04, 2008 08:06 PM, CST

Jack is out of surgery. It all went very well! There was a time for about an hour this morning when they thought the surgery might not happen. They were worried that Sean's liver might not match up with Jack's in a way where they could connect the hepatic artery. There were a number of phone calls back and forth between hospitals apparently. They told me none of this until after the surgery- which is wise because I'm not sure I could've taken that.

But they went ahead with it and it went very smoothly. "textbook." Jack remained stable the entire time and only required one unit of blood. He is in the ICU - heavily sedated of course. i was prepared for him to look horrible but he looks fantastic. His tummy is so flat. Flat - like Lily's. Like it is supposed to look. Beautiful. And his breathing is normal again.

The main thing they look for tonight and tomorrow morning is bleeding. That's the first big hurdle. That and the most dangerous thing they look for early on is the formation of a clot in one of the veins leading into the liver. If he bleeds or if a clot blocks off his blood flow, they would have to open him back up and try to fix it. We'll hope for that not to happen.

The surgeon told me to prepare for a roller coaster. It is not unusual for his recovery to have stops and starts, setbacks and advances. That's normal. We'll ride it together.

I am exhausted. Tapped out emotionally. I thought I'd have an emotional reaction today (aside from watching them wheel Jack to surgery which was very hard) - or cry from relief when the surgery was over. But I'm just so tired. And after the scares we had last night, sending him to surgery was the big relief. I feel a little like I'm through the looking glass. It all still seems so unreal.

So celebrate a little tonight. We've earned it, I think. We are not remotely out of the woods - we will never be totally out of the woods - never - but we can take a little breather tonight.

I love you all - everybody. Jack is so lucky that you've all got his back.

THURSDAY, DECEMBER 04, 2008 02:34 PM, CST

At 1pm I got a call that the liver was at Children's Memorial and they were preparing to begin Jack's transplant. Sean did well and his liver looked good to them. He is out of surgery and Steve has said that he will be allowed to visit Sean at 3pm.

Now comes the work of making the piece of Sean's liver connect correctly to the three main blood vessels and the bowel. They have to hook it in to Jack's anterior vena cava, his portal vein, and the hepatic artery and then they will attach it into the "v" they created to replace his gallbladder during the kasai procedure. The veins in Sean's liver will not match up. Sean's will be larger, of course. So they have to work to make it match up and possibly take some veins from Jack's leg.

There is a good possibility that they will not be able to close Jack after surgery. The intestines swell during surgery and Sean's piece of liver may be larger than Jack's. In this case, they would most likey close only the skin of his belly and they would give his organs an opportunity to go back to their normal size and settle in. Then they would go back in and close the muscles etc. Jack will be in the ICU after surgery for a couple days. He will be heavily sedated and possibly given a paralytic to keep him paralyzed from the neck down (if he isn't completely closed) Then he will move back to the floor and have a private room and his own nurse.

OK. Someone just came in. They have attached the veina and artery and allowed blood to flow through. It flushed his liver the way it should. So now they are going to connect his small intestine using the loop of intestine they used for the kasai. They said that Jack has been stable throughout and has only received one unit of blood so far.

I need to post this now because it is very late. Sorry about that.

THURSDAY, DECEMBER 04, 2008 11:02 AM, CST

1am last night marked a dramatic (in a good way) turn. They found the balance they were looking to achieve with Jack and everything started working. His kidneys kicked into gear and got a lot of the fluid out of him. That made his breathing easier and he was much more comfortable. He was sleeping soundly - a little too soundly for our comfort, actually, since he didn't rouse even when he was pricked every half hour for blood glucose readings. We got a little concerned so I rubbed his head and he stayed alseep. i petted his cheek and he didn't budge. I brushed my finger against his bottom lip and LOOK OUT. Jack thought he was gonna be fed. And he was up - alert and bright eyed and HUNGRY. But he wasn't allowed to eat due to the surgery. So he started to scream at us which lasted a couple hours. I was relieved that he was OK but also exhausted and really wanted him to go back to sleep so that Robin and I could rest. I got him back down at 3am and we slept until 6 or 6:30. So that was good. This morning he was a new baby. Still angry about the food but he could be distracted and he played with our fingers. I thought I might even get a smile but he wasn't quite that chipper.

Sean went into surgery at 8:14 this morning. Jack was taken down around 9am. We met his anesthesiologist and then they wheeled him away. That was difficult for me for a minute but really, we are all in good spirits today. This is good. This is the thing that can save his life so it's a very positive thing.

I'll continue to update the site throughout the day. My last update from Steve was that they would have the piece of Sean's liver at around 11:45 and would let Steve know when it was out.

I really believe that all the prayers and positive energy helped with Jack's rally last night. We appreciate it and humbly ask for more.

WEDNESDAY, DECEMBER 03, 2008 10:25 PM, CST

Alright. I think that was just a little scare. When they took his temp again it was 99.6 and about a half hour after that he felt cool to the touch on his legs and his head felt much cooler than it had. We think that the room got too hot. It is really impossible to regulate the temperature in these rooms. They are either cold or sweltering. Sean had gotten cold and turned up the heat. I generally don't feel hot unless it is REALLY hot - but poor little Jack is over in his bed working away to breathe - it's like he's running a marathon. So that plus the hot room made him overheated. That's our theory anyway.

Still, they took cultures of all the various lines in his body and a urine culture and they are putting him on antibiotics just in case. They are the same antibiotics that they would start him on tomorrow morning pre-surgery so it is really only one extra dose.

I was just looking back at some old pictures of Jack. (You know - waaaaaaaaay back to October when he was a baby) I love the photos of Lily and Jack and Daddy on the futon. I look at that and I realize that I have everything I ever dreamed of. That is the family I always wanted. And no, I never dreamed that liver disease and transplants would be part of that family. And there are times - pretty often - when I think "Why us? Why Jack? What did we do to deserve to be punished this way?" But when I look at those pictures I know that I am very lucky. Most people never get their dream. I did. My dream was Sean and Lily and Jack. If this is what we have to deal with in order to have the family of our dreams, well, we can do that.

WEDNESDAY, DECEMBER 03, 2008 07:30 PM, CST

Jack has not gone to the PICU. His kidneys are producing a little urine. He has a little fluid in his lungs but they can't do more to get fluid out of him than they were already doing anyway. I asked if it would keep him from getting the surgery and they said no because the only cure for it is a new liver.

We had to sign a lot of stuff today - about the possibilities of his not living through the first year post-transplant. They said about 15 percent don't make it. And because of the anti-rejection drugs he will be on, he is more likely to get cancer and it also causes kidney failure in many kids by 5 or 10 years post. I guess we can't really think about that. We just need to think that if we can get 5 good years, it's an enormous gift because without this surgery, I can't imagine he would make it another week. He is so incredibly sick. He is orange and swollen - so swollen - his face, his hands and feet, when he opens his eyes, they sort of look in different directions.

Sean just left to go home and put Lily to bed. I guess I won't see him again until after the surgery. And he won't see Jack.

Steve Mosqueda will be going with Sean to the hospital and will stick with him all day - giving me updates by phone. I'll be here at Children's with Jack sending updates back to Steve to give to Sean and I'll also be updating this site throughout the day. That'll give me some purpose. My sister will be here and so will my friends Chloe and Rachel and Sean's mom so we'll take over about half of the surgical waiting room.

I really really appreciate all of the prayers and vibes and wishes and lit candles. it means a great deal and we really do feel connected to everyone.

They just took his temperature and it was elevated to 100. They will retake it in a half hour and if it is still elevated, they will send cultures out to see if there is an infection. That is one of the few things that could stop tomorrow's surgery. Pray, please.

WEDNESDAY, DECEMBER 03, 2008 08:55 AM, CST

Jack's kidneys are failing. They are moving him to the ICU. They are hoping that they will be able to work on his kidney function and get rid of some of the fluid. This type of kidney failure is a direct result of the liver failure and the kidneys should be able to heal when the new liver is working.

WEDNESDAY, DECEMBER 03, 2008 06:51 AM, CST

He's finally sleeping in his crib but he's restless so I have to make this quick. He's so sick. He barely opens his eyes anymore and when he does, I don't really recognize him. His hands are so puffy he can't make a fist. He doesn't stare anymore. He makes a raspy "uhhhh uhhhh" sound when he is awake and we just try everything we can to put him back to sleep. His breathing is labored because the excess fluid and swollen organs leave little room for his lungs.

Last night they conferred with the kidney experts to find a new diuretic that would flush out all the fluid. They have given two doses now and he hasn't peed. But they have to keep giving him infusions of various things so fluid keeps going in. They think that if they can get him to pee out all of the fluid, they can get him back to the way he was the day before yesterday - clearly altered and sick but not as miserable as he is now. I think they'll have to think again, though, and find something else because this latest diuretic is clearly not it. I am terrified that something has damaged his kidneys or something.

OK. I have to go. 24 hours. In 24 hours, Sean will be heading into surgery and a couple hours later they'll take Jack. We just need to get him a little more stable and then hang on.

Thank you for all the love and support!

TUESDAY, DECEMBER 02, 2008 06:25 PM, CST

Surgery will probably be Thursday instead of Wednesday due to scheduling issues. Jack is on a steep downward spiral. I can't believe this is the same baby I fed cereal to on Thanksgiving. He is either crying, whimpering, sleeping restlessly, or staring at the wall (often without blinking for long periods of time.) There is no more smiling. He went downhill so quickly that I am scared that the 36 hours he has left until his surgery might be too long.

He is receiving blood now because his hemoglobin is low. His potassium level is low and will not go up in spite of all the potassium they are giving him. He is puffy because the diuretics aren't working any more. He was vomiting so often that they stopped feeding him through the ng tube and are feeding him only by mouth - except that he won't eat. Since he won't eat, they had to put him on IV fluid to hydrate him. Since they were already using his PICC line constantly for blood, plasma, potassium, lasix, in an endless loop, they had to start another IV in his other hand to give him the fluid.

I haven't really slept for 3 days so my sister is coming to stay here tonight so we can switch off holding him the way we did after his kasai in September.

We tried to explain to Lily that daddy would be in the hospital for a couple days. She became hysterical because he wouldn't be reading her stories and putting her to bed.

I am terrified he'll get too sick to go to surgery or will be so sick that he won't be able to recover.

He's sleeping so I'm going to lie down.

SUNDAY, NOVEMBER 30, 2008 03:00 PM, CST

Waiting. We're just waiting. Waiting for some family to receive the same kind of horrible news that we are trying desperately to avoid. We are all so precariously balanced, you know? All of us. And we have no idea how precarious until we get knocked down. I hope you saw your family over the holidays. I hope you hugged them tight. I hope that when your mom annoyed you you remembered how lucky you are that she's still around - even when she gets all up in your business. I know that I would really like my mom to be here right now and she could get as up in my business as she wanted. I hope that when your kids get all hyped up on holiday sugar and won't go to bed or they color on the walls or forget to use the bathroom on time you will laugh. It's awesome - all of it. It's funny and precious and you'll think back on it fondly some day. I can't wait for Jack to have normal kid problems. I hope he colors on the walls - freshly painted walls - with permanent ink. It's fine. I hope we get there. It feels far away right now.

They shut off Jack's continuous feed at 3am in anticipation of a 9am minor surgical procedure to install a PICC line. It is basically an IV that threads up his arm and through his heart and it will last for the entirety of his stay. It replaces the IV in his hand (which closed up this am) and - best of all - blood can be drawn through it so no more sticks! Labs are now pain-free!! It was a rough morning because he was starving and the procedure room got backed up so he didn't go in until 1:15pm. He was able to eat at 2:20 - so almost 12 hours without food. He was one angry baby. When they took off his old IV, the tape had caused a reaction on all his fingers. They look like they were dragged along a sidewalk. And he now has a bruise on his abdomen that I think is a small internal bleed from his inability to clot. HOWEVER, they are now giving him fresh frozen plasma every six hours and it has corrected his coag time to a normal level. That's really good. It's just a stop-gap measure - but it's good.

When we were waiting in the holding area for his PICC procedure, there was a little boy - maybe 10 or so - getting an IV. He listed off all his meds for the nurse and she explained to him that most of his veins were pretty tapped. He wanted the IV in a certain arm and she said the vein would blow if she tried it. He cried - not ill-tempered wailing but that kind of crying where you are trying hard to not cry and you just can't help it. And I felt tso sad. I told Sean that that is our best case scenario for Jack- that the best we can hope for is that he will be a kid who is such a regular here that they know his name. That he'll have a preferred arm for IV's and he'll be able to list off his meds to the nurse. And Sean said "And the rest of the time, he'll be a happy normal kid." He said he's done procedures on kids and the ones that can list their meds and position themselves correctly for x-ray are the ones that fare the best. And that they maybe are even more likely to develop an interest in the field. So maybe he'll be a doctor.

Sean Benjamin is awesome.

FRIDAY, NOVEMBER 28, 2008 11:52 AM, CST

Never get sick on a holiday. You won't even believe the ball of red tape Jack got tangled in. Jack's numbers continue to stay the same. The albumin and K they are giving him are not rectifying the situation though they are keeping the numbers from dropping lower for now. He is stable for now but in danger. This morning we were told that the liver team was ready to list him but Blue Cross/Blue Shield is taking a four day holiday and he can't be listed without preapproval. The business people have been trying to get the preapproval since Wednesday but could not get access to the right people. When he is listed. the hospital is required to give the most current set of labs for eval;uation so he can receive his PELD score. They can give him plasma and improve his numbers BUT then he will list lower and his chance of getting a liver in time will decrease. So they were holding him on the brink of disaster waiting for the insurance company to finish digesting their turkey. All I could think was that every minute he remains off the list was a possible missed opportunity. We started calling around ourselves, calling other nurses, speaking to doctors, Sean tried to get through to Blue Cross by pretending to be a medical professional. I began to hyperventilate. Just as I was starting to lose it, his doctor came in and said "We're listing him now. We'll deal with the insurance later. As soon as he is offically on the list, we'll come in with plasma and try to get his numbers back up.

So now I can come down to the 5th floor (we can't access the wireless network right now for some reason) and update the site.

Jack has pretty much stopped taking food by mouth. He occasionally seems to want some but then after 15 or 30 seconds, he's done. They put an ng tube in him last night (a small tube that runs from his nose into his stomach) and they put him on a continuous feed throughout the night. We both slept really well. Now they are putting him on a daytime diet and I am to try to get him to take the formula by mouth and then the rest will be put through the tube. I know him. He's not going to drink it. It's nasty. They also asked me to start him on solids so last night he had his first cereal which made him very excited. He liked it. Today he hasn't been in a very good mood so I haven't tried to give him cereal again but will try when I go back up to the room if he is still awake.

This just in: He is officially listed with a PELD score of 32. That's pretty high. I am pretending to not know that that number is high based on his poor chance of surviving for 3 months without a new liver. It doesn't matter. They are tentatively planning for a Wednesday transplant unless an organ comes in sooner or he becomes critical or suddenly recovers to the point that they could wait. They didn't say that could happen but, you know, he constantly defies odds.

Thanks to everybody for keeping up with all the news. Sean and I are keeping it together pretty well. It is actually an easier time than when he received his initial diagnosis becaiuse we have known for months that this would come. i mean, it is scary that in happened in this unprecedented manner, but as long as everything is going forward, I think I will be able to keep it together until the actual transplant day at which point I will most likely have to be sedated - especially if both my husband and my son are in surgery at the same time.

OK. It makes me antsy to leave him so I have to go back up.

ps - right now we are keeping his contact with other people at a minimum because any kind of germ could be devastating. Anyone who weants to come and hang out with me on surgery day (whenever that might end up being) will be more than welcome.

WEDNESDAY, NOVEMBER 26, 2008 10:54 PM, CST

One thing you can say for Jack Benjamin (besides the fact that he's a major flirt) is that he keeps everyone on their toes. They don't know why his liver is failing so rapidly. They are currently unsuccessful in getting his numbers back to where they should be. His specialist says that BA doesn't cause this rapid of a decline and that there must be some other injury on top of it - like possibly a cold virus or mild bacterial infection - that would be nothing for a healthy child but was enough to tip the scale for Jack.

We have gotten so much conflicting info today that they ended up coming in and apologizing. One specialist said he'd go home tonight and we'd watch him very carefully. His main specialist said that he is in acute liver failure and needs to be transplanted immediately. The surgeon said we should move forward but not rush it too much. Sean has now been evaluated at Northwestern Memorial and the preliminary findings are good. There is still a chance that the section of liver will be too big for Jack's tiny body - we'll know more tomorrow. They seem to want to wait a little to see where his PELD score places him and whether or not he might get a cadaveric liver really soon. It is always better to keep a parent out of major surgery if you can help it.

I have had a meeting with financial and with organ procurement. I think we will still have to meet with psych and social work but maybe they are skipping that because of the rush.

The one thing they did seem certain about by this evening is that Jack isn't going anywhere. He'll most likely be here until he is post-transplant - and so will I, right here in this chair - which could be a month or so from now.

Right now he is asleep. The diuretic that had helped him this morning has worn off and the ascites is back. his tummy is large and tight. They have started measuring it so they can track it. They may do more albumin and lasix tomorrow. His pt numbers are still rising - that's not good - he isn't absorbing K and his blood isn't clotting well. The albumin is obviously still a problem or the ascites wouldn't be coming back. Still - as far as he is concerned, he feels better today than he did yesterday so he was pretty happy. He flirted with nurses - smiling at them and then hiding his face in his hand when they smiled back. He got so into it that he smacked himself in the forhead with the IV shield. He didn't cry but he looked a little embarrassed.

We were in a shared room with a little girl whose parents don't stay overnight. I called the nurse 6 different times because she was crying all alone on the other side of the room. Then, today, her family showed up and they were all SICK - hacking and sneezing. If Jack catches a cold, they will cancel the transplant until he recovers. A cadaveric liver could become available and if he's sick, we would have to turn it down. So, you know, i pulled a shirley McClaine and got him out of there. When they didn't move quickly enough I unplugged Jack's monitors and walked the hallways with him and his IV tower until we got a private room. I felt like I was being high maintenance but all I could think was that if I stayed silent and he lost out on a liver and if something horrible happened - it would be my fault for being a coward. So, you know, I made a little fuss. It was pretty mild. i think they knew I was right.

OK. I'm going to try to sleep while he's sleeping.

I am really thankful tonight for all the people who have called or emailed or written in the guestbook. I am thankful for my fantastic husband who is an amazing father who went through an awful lot today and may be going through an awful lot more when all is said and done in order to save Jack's life. I am thankful for my healthy daughter who copes incredibly well with a mom who has TWICE gone away for a 2 hour doctor's appointment and not come back home and who has never once held any of this upheaval against her little brother whom she loves in a big way. And I am so so thankful for Jack who is teaching us every day - about priorities and about living in the moment and appreciating what you are given and strength and the power of a sincere smile.

Thank you thank you thank you.

WEDNESDAY, NOVEMBER 26, 2008 11:04 AM, CST

Jack is in acute liver failure. They are rushing around trying to get Sean evaluated to be the donor. They want to transplant in the next few days.

Jack is 5 months old today.

He is still smiling.

WEDNESDAY, NOVEMBER 26, 2008 08:47 AM, CST

Well, I'm afraid we're in the hospital again. We were admitted yesterday evening. Yesterday Jack had one of his worst days ever. Well, let me backtrack a little. He seemed a bit worse over the weekend but I figured we could wait until his scheduled appt. on Tues. Actually, he had such a bad night on Sat that I called the GI fellow on call on Sunday and told him how much pain Jack was in. He said "sounds like gas - nothing you can do but comfort him." Yeah. He's in trouble.

Anyhoo- by Tuesday he was just not feeling good. We had the beginnings of our transplant eval in the morning so I had to wake him up to leave the house by 6:50. They drew 9 viles of blood and he was very brave. Then we went to ultrasound and it took more than an hour. It was very uncomfortable for him - he was so miserable. During the ultrasound, his liver nurse, Cindy, came in. She said that some of his labs came back and his blood isn't clotting (vit. k deficiency) She gave him a shot of K in the leg. Then we had a completely worthless Genetics appt. "Don't know why his head isn't growing but he looks good to me. Maybe he's just small. Pay me $2,000." Jack had just fallen asleep and woke up to the Genetics guy poking him. Not happy.

Then we had liver clinic. Jack has ascites (fluid in the belly) and portal hypertension. His K and albumin levels were dangerously low. It could mean one of two things: either the synthetic function of his liver took a sudden nosedive OR something created a tiny imbalance in his levels that caused problems that affected his absorption abilities which caused greater imbalance etc. If it is the first thing, I think the transplant process will be sped up. If it is the second thing, they will be able to get him balanced again and we'll proceed as planned. They believe it is the latter scenario since he is still a feisty little guy. Last night he was given albumin and lasix (a diuretic to get rid of the ascites.) He had a pretty good night last night once people stopped poking him (they took 4 more vials and tried to put an IV in his foot, failed, and put it in his hand, then 2 of the vials had to be redrawn because "something happened to them." If you are keeping a tally, that's 15 viles of blood, 5 pokes, 1 shot.)

Today he is visibly improved. His belly has reduced in size and is soft - not rigid like it was. He had edema in his legs and lower back that seems reduced. And he's a smiley guy again. Everybody is getting big smiles when they come in. He had 4 vials of blood drawn this morning. We'll see what they say.

Regardless, his transplant is not far off. His total bilirubin is up to 21. That's very high. It is increasing at over a point a week so he can't wait that many more weeks. I would be amazed if we got to the end of February with this liver.

I'll let you know what the doctors say today.

OK. Someone just came in and said the vitamin k didn't work. I now think we're in for a longer stay. They may end up fast-tracking his transplant. More later.

FRIDAY, NOVEMBER 21, 2008 10:02 AM, CST

This entry is for everyone but especially Nicky.

I'll tell you what it's like - for me. It's like standing on the sidewalk and watching a car accident in super-slow motion. The crash is going to happen - and you know it's going to happen - and you're powerless to stop it. But it is happening so slowly that you have lots of time to think. And sometimes you think, "Oh, they'll swerve and miss each other." or "They'll slow down and it won't be more than a fender bender." But the more you watch, the more you see that isn't a possibility. And sometimes you think "This is going to end in a firey explosion" and then you feel guilty for envisioning that end because it feels like you are willing it to happen. And then, at the same time, when you gather the strength and focus to look around, the air has never felt more crisp or smelled so fresh and the colors of the leaves cause your heart to swell or the starkness of the bare trees against the sky is haunting and beautiful. Because you know that crash is coming and you know how fragile it all is.

End metaphor.

The worst part is not knowing. I'm a practical, sturdy girl and if you give me all the info I can process it and deal with it. But we don't know. We don't know the outcome of the transplant. We won't know until after the transplant if it was the BA that is affecting his head growth or if he has some other syndrome. And even if he has some other syndrome, we don't know if the effects will be mild or devastating.

There are moments when I'm angry that this liver disease has stolen his babyhood from me. I want to enjoy having a little baby - it is such a short time before they grow up and start smart-mouthing you. It is precious. Then, there are other times when I think that without this illness, I wouldn't notice the precious moments. A couple mornings ago, Jack got sick all over both of us at around 5am. I quickly got us both into a warm shower. And he was fascinated by the running water. And he loved how warm he felt. And he wanted to drink the water off my shoulder. And he held on to my neck and my hair really tightly to feel safe. And I took in all of it and I really enjoyed it. And I'm really grateful for moments like that that happen when I least expect them. And if he was well, maybe I would've been irritated to be up at 5am.

When I get new info about micrcephaly or his liver that makes things look bleak, day to day life is a huge struggle. That's why this has been so hard for Lily. I can't play princess while I'm straining to not cry. I can't be sympathetic about 3-year-old traumas like "I want juice and you gave me milk!" when I am fearing for Jack's life. But those moments come and go - and there are hopeful moments when going trick-or-treating or getting Lily to Tap class is more fun than it would be otherwise - if that makes sense.

So - that's my update. All about ME. Jack has a peds appt. today and his liver clinic was moved up to Tuesday because of the vomiting. So - there will be more updates soon, I'm sure.

ps - In this Thanksgiving season, I am thankful for all the friends offering help with Lily, for Pete and Steve and the Craft Brewers Association for raising over $3,000 for Jack's transplant, for the postcards (I put them in front of his car seat and they really do keep him from crying), and for Facebook for bringing Nicky and Angie and Ana back into my life. And for my sister who takes more than one tearful (even hysterical) phone call from me per week - half of which would have been taken by my mom if circumstances were different. Thanks to Robin and John for being Aunt and Uncle and the youngest grandparents on the block all at once.

MONDAY, NOVEMBER 17, 2008 09:36 AM, CST

Well, the BA is starting to affect him more. His tummy is huge. The last time we had liver clinic, they said that he didn't have ascites (collection of fluid in his abdomen) so the size of his tummy is most likely just due to the size of his liver and spleen. The more damaged they become, the more swollen they get. His spleen is not diseased per se but once his liver is damaged, the blood coming back up from his feet on the way to his heart has to have a route. If it can't get through the liver, it heads to the spleen. The spleen and liver get big and crowd the rest of his organs - kind-of like when you're pregnant. According to the other moms in the support group, this is pretty common. Their stomachs get squished and pushed around so now his eating has dropped off a bit because he feels full and he has started throwing up. This will not help his weight gain, obviously. His tummy is about 20 and a half inches around. To give you an idea of that size, his 3 and a half year old sister measures at about 19 inches.

His veins are starting to be very visible on his back and stomach. This is because they are stressed by the bloodflow issue as well. One thing that BA kids tend to get is called portal hypertension. If it gets really bad, the veins leak. We aren't there yet. But his tummy and back look like a road map now.

Jack and I went to Costco with my neighbor yesterday. When we got into the store, she said "Oh my GOD! His eyes!!!" He looks much better in natural lighting. Store lights are particularly unkind.

I am part of this whole other world now. I am on the Liver Families website several times a day - the liver disease support group. It's really helpful. And occasionally devastating - like when I find myself reading about a child who died in his mother's arms waiting for a liver. But mostly it helps - sometimes in a weird way. Many of them have already gone through the transplant process. They have always chatted about PELD scores but I had no idea what they were until the transplant info came. PELD stands for Pediatric End-stage Liver Disease. It determines your ranking on the transplant list and it is basically a number that indicates the chance that the child will die within 3 months. According to his Dr., Jack's score will be fairly low because he's developing so well (they don't count his head) The other moms believe he will score higher due to his total bilirubin count being almost 16 and the fact that he's so young. It is weird, but even though it means something horrible, all the liver families want high scores - because it also means first shot at a liver. I have a feeling that if Sean or I are a match, we'll go ahead and do the living donor thing because I don't want him to sit on a list once he starts getting really sick. And if he's not really sick, his PELD score will be lower. They did say that he has a bit of a better chance since he's B+. People with type O blood wait a long time because O is a universal donor and always goes to the top person on the list. B+ is a more rare blood type so if a B+ person dies, they skip down the list to the first B+ person. They say that gives Jack a better chance at a cadaveric liver. But I can't sit around and watch him get sicker. I just can't. He's still got a good quality of life right now. Except RIGHT now because he is sick of being in the excersaucer while I write this. Gotta go.

WEDNESDAY, NOVEMBER 12, 2008 10:23 PM, CST

Well...the transplant packet came in the mail today. I knew it was coming. I knew the evaluation was coming. I knew the kasai failed and that the transplant is inevitable. But nothing prepares you to sign the paperwork that says that I understand that he might die from the surgery. I understand that he could reject the organ. I understand that his kidneys could be damaged by the drugs. I understand that his appearance could change drastically.

I don't. I don't understand. He's a baby and this is not fair. He's a baby. He's a baby.

I still haven't signed it.

His evaluation is Dec. 2nd. Well, he has some labs and an ultrasound on Nov. 25th and then Sean, Jack, and I will spend the day from 9am to 3pm at Children's getting evaluated - by financial, social workers, psych, meet the transplant team, etc.

Another paper Children's sent is the survival rate of CMH patients versus the National Average and CMH is BELOW the National average - substantially. I put in a call to Jack's liver guy and am waiting a call back. When we were in the hospital, we asked about the survival rate for the liver transplant. The study nurse shrugged as if it was almost a silly question and said "Oh, like, 95 - 96 percent." We always took great comfort in that. Hmmmmm. The paper I got said that survival after 1 month is 91% and survival after 1 year is 89%. They lose 1 out of every 10 kids? Jack has a 1 in 15,000 disease. He doesn't play the numbers well. We need better odds than this.

Yesterday I was sad about his head growth. I was sad to think that he might be slow or he might look funny and other kids might pick on him. Now I'm really sad about far worse things. I'll take slow and funny looking. It's OK.

In the meantime, the person who suffers most from all the stress is Lily. She is the lovliest little girl and I snap at her often- for simple, regular, 3-year-old stuff. And I instantly regret it. But it happens again. The poor little thing lost her grandpa, gained a sibling, sibling got sick, started school, mom has seemingly lost her grip and she has handled it all with grace. She is amazing and brilliant and I am afraid I am going to ruin her by being so terrible to her. Her teacher told me that Lily is doing great in school and that she is a leader amongst her peers even though she is only 3. Apparently she organizes activities on the playground. She also apparently performs songs from Little Mermaid during circle time. This really should surprise no one.

I have started taking triple strength Omega 3 pills because I was thinking that maybe Jack was lacking Omega 3 and that that would help his head. We'll see. He has started rejecting the fortified breastmilk so completely that I now am back to only nursing. He also spits out most of the E I try to give him. I'm not sure how to fix this but he needs those nutrients and calories.

We can't lose him. I know you know that but I thought I'd put it out to the universe. We can't lose him.

FRIDAY, NOVEMBER 07, 2008 07:30 PM, CST

Went to the pediatrition. No head growth at all. Still 15 inches. sigh. WHAT is going on? Otherwise, he hasn't grown in length so his length is now right at the 10th% BUT height happens in bursts and not steadily so every two weeks is not an accurate measure. His weight is still moving steadily along the 25th percentile. He is still developmentally on track, though. And he's a cutie. He has a very round tummy that sticks out but otherwise he looks a little small.

It doesn't stop him from flirting, though. There were two little girl babies sitting with their moms outside of Lily's tap class (5 mos and 9 mos - older women) and Jack kept trying to launch himself at them...while grunting. Actually, I've met men who use that tactic. We're gonna need to work on Jack's technique.

THURSDAY, NOVEMBER 06, 2008 06:55 PM, CST

Not much to report, really. I had thought his head had grown another 8th of an inch since his doctor's visit 2 weeks ago but now when I measure it it doesn't seem to have grown. He's very wiggly so getting an accurate measurement is pretty challenging. His doctor will measure him tomorrow. I really need her to get 15 and 1/8th. I really really do.

It looks like we'll be doing his evaluation kind-of spread out from November 25th to December 9th. On the 25th, he has a Genetics appt, an ultrasound, and labs. On the 2nd, we have liver clinic and part of the eval and on the 9th we have Neurology and the other part of the eval. There's also an MRI on the 5th in case Jack was missing the nice doctors.

His eyes are so yellow now that they almost look brown sometimes. I think people are surprised when they see us, though, because I think they expect Jack to be bed-ridden or all sickly, weak, and lethargic. He's not. He's a normal baby - with green skin and yellow eyes. I'm worried that he's beginning to itch. It's called pruritis and it's from all the excess bile salts in system coming through his skin. I understand it is pretty torturous and causes kids to claw at themselves until they bleed and not be able to sleep - stuff like that. He keeps rubbing at his face and ears. It could be because of normal baby stuff - sleepy or conjested. I have no idea. He has made his ear bleed twice. I hope he's not itching yet. I hope he never starts.

They removed him from one of his meds. He was on a drug called Actigall that is supposed to promote bile flow but they took him off because it has never been proven to be effective once the total bilirubin level is over 15 (Jack's is 15.2) and since it can cause stomach problems, there's no point in him taking it anymore.

We are looking at joining something called COTA (Children's Organ Transplant Association) which helps people raise money for organ transplant. We have been told we should save up $30,000 for the initial transplant and transplant-related costs. Once we join, we will have to use their website and we won't be allowed to have our Caring Bridge site anymore - I think. There is a journal function on that site as well but no fishies. I really like the fishies.

FRIDAY, OCTOBER 31, 2008 10:22 AM, CDT

Dear, Jack-

Hello, my baby. I love you. I'm watching you sleep right now. You are such a sweet boy. I am struck, today, by how amazing you are. I love the way you always wake up with a smile. You should teach that to your sister. I was especially taken with you last night. You woke up at 4am ready to play and I whispered "Jack. It's still sleeping time" and I watched you quietly work on putting yourself back to sleep for over a half hour. And you did it. You are amazing.

It is Halloween tonight. I'm going to dress you up as a pumpkin. This year we'll carry you but next year, I want you to run. I want you to follow your sister from house to house and hold out your bag and smile that great big smile.

Jack, we love you so much. We are so sorry you are so sick. If I could take it away, I would - in a heartbeat.

You are one of the three great loves of my life. When we found out we were having a boy we thought "A boy? What in the world will we do with a boy in our princess-filled house?" Then you came and I can't imagine a world without you in it. Did I really live almost 39 years without you? How did I do that? Well, I have you now and you're not going anywhere. We're gonna fix you up and let you get on with the business of growing and learning and laughing.

You're awake and hungry now so I have to stop writing. I love you, buddy. Forever.

Mommy

WEDNESDAY, OCTOBER 29, 2008 01:13 PM, CDT

Well, no surprises at liver clinic. Dr. Whitington said his head size is in no way related to BA or malnutrition from BA or the steroid- no way. But he did speak to a Neurologist on Jack's behalf (which is a lot better than I've been able to do) and the Neurologist said that first and formost you have to look at development - is he on track. If he is, head circumference doesn't mean as much. Dr. W thinks Jack's development looks great and feels that's pretty remarkable for a chronically ill child. I am still unconvinced that there is no relation to the BA but only time will tell.

As for his liver, they are ready to call the Kasai a failure and we have begun the process to list him for a transplant. At this point, his ranking will be very low on the list because he is growing (except his head - though it did recently grow 1/8th of an inch) and gaining weight and he isn't feeling sick. Also, his liver is still functioning in many ways. His enzymes are currently OK. But - he has no bile flow. His direct bilirubin is nearing 10 - big jump from 3 weeks ago. So - yeah - we'll list him. We'll hope for a cadaveric donor but since Sean and I are both donor candidates, we aren't going to allow him to suffer. If his health begins failing, we'll do the living donor transplant.

So - currently they are talking with our insurance company - getting approval for evaluation. That will take a couple days. Then we will schedule a full-day appointment to do his evaluation where we will speak with financial people, social workers, the transplant team, have a psych evaluation, an ultrasound, bloodwork etc. Then we wait for him to be approved and listed which takes a couple weeks. After that, the potential donor - whomever we decide that will be - will have a big work-up done at Northwestern Memorial. Then we'll watch and wait for his growing to slow down. There's no telling when that will be. Dr. W said that babies are generally transplanted between 6 and 18 months with the mean being 14 months. Jack is currently 4 months old. He is also quite healthy considering he has no bile flow at all. I mean, he's not healthy compared to normal kids but Dr. W says he has a lot of infants in his care who are sickly and lethargic. Jack is not. He's very strong. It is certain that we will be doing this transplant by next Christmas. From the way the nurse talked to me about his bilirubin levels this morning, I think we can bet on it being in the next 6 to 8 months. The rapid rise in bilirubin means that he has 0 bile flow and that will mean that he will simply not be able to continue growing for that much longer. But they did tell me that the nice thing about liver failure (there's a nice thing about liver failure?) is that everything happens gradually. There will be no sudden - wake up in the middle of the night cause his liver just shut down - types of emergencies. We'll watch it happen over the course of weeks and we'll be able to act accoringly.

So - there you go. We are about to be on the road to a new liver - just not sure how lomng the road will be. We'll keep you posted.

MONDAY, OCTOBER 27, 2008 09:22 PM, CDT

Well, it's time for liver clinic again. We go in tomorrow at 2pm. Then we'll get the blood test results on Wednesday - but I already know the bilirubin will be even higher. He doesn't have any bile flow. His tummy is getting bigger. I'm not sure if it is his liver and spleen swelling or if it is ascites (fluid collecting in his abdomen) or maybe both. Both are pretty inevitable in his condition. I guess the deal is that not only do they want him to get bigger before his transplant, but it is a rule of UNOS that he has to be in end-stage liver failure in order to have a transplant - even if we donate the organ ourselves. It still falls under UNOS and their protocol. And end-stage liver failure is pretty rough, I think. So we are waiting around for him to get sicker and waiting around for his head to grow. Some days I am able to stop worrying about it and just be happy that I have the beautiful boy we were waiting for. And he is really beautiful and sweet and happy. But there's always the worry underneath. His cheeks are very pink tonight. He looks adorable - like a cherub - but I can't stop wondering if it is a sign of something awful. I keep feeling his head to see if he's feverish. He's not.

I found a paper written by someone on the faculty of Cornell. She's a psychologist and a nutritionist and she has studied the effects of malnutrition on brain development. I wrote to her about Jack. I hope she writes back.

That's about it tonight. I'll let you know what they say at liver clinic.

ps - thanks to everyone for all the support! The messages in the guestbook are really helpful. I often read them two or three times. And some day Jack will read them as well.

THURSDAY, OCTOBER 23, 2008 10:40 AM, CDT

OK. Enough already. This whole thing is insane. We met with the Neurosurgeon who had, really, nothing to tell us EXCEPT that benign external hydrocephalus is very common - especially in male babies - and is no big deal. It simply means that the process of absorbing the fluid needs to mature a bit. We looked at the ct of Jack's brain and they said it looks normal "but, of course, you really can't see too much on a ct. What you really need is an MRI." He also said "You know, you really need to see someone in Neurology - not Neurosurgery." We said "We know. We tried and they referred us to you." He said "I know. They're a mess over there. This happens all the time." sigh.

So I said "Doesn't it seem like - with his whole body growth slowing down after the surgery, couldn't it be related to the malnutrition and the trauma and the steroids?" And he said "Well, that sounds reasonable to me. We know how important nutrition is for proper brain growth. But the liver people would know better than me." And I said "Would they? Because they don't routinely track head growth. They are only concerned with his liver. So if a baby shows decreased brain growth when his liver is failing and then gets a new liver and his head catches up, no one would be tracking it." And he said "Yeah. Definitely brain growth that is slowed by malnutrition would catch up after the nutrition is taken care of. So...I don't know. ?The liver people would no better."

Meanwhile, my sister the research Neuroscientist said that they see this in mice all the time. They put them on a diet that is lacking an essential nutrient and they get tiny heads. They replace the nutrient and the head overcompensates (like what happened to the little girl whose mom wrote me.) So, if this theory is correct, why don't they know it/acknowledge it? Probably because it isn't tracked and no studies are being done and if it doesn't have lasting effects once liver function is normal, NIH would never fund such a study. So, we will do the MRI even tho it means sedation and I'm not thrilled about it but I really think that unless they see something crazy on the MRI, we need to chalk it up to malnutrition and concentrate on this horrible liver disease and all the really alarming complications and side effects we could be seeing soon as his bilirubin continues to rise.

In the meantime, he's still happy and smilin' and I plan to follow his lead.

TUESDAY, OCTOBER 21, 2008 03:35 PM, CDT

Well, yesterday's ct-scan went as well as it possibly could. They decided that shooting him with contrast was unnecessary and they also didn't need to sedate him. He liked getting the ct. He got to drink sugar water and he got all swaddled up. He thought it was nice.

Then, in the afternoon, I got an email from a mom on one of the support networks whose daughter was born with a head in the 35th%. She had BA and had a kasai that failed. Her head growth dropped to 20th%. Her kasai was revised and it worked and in the next year, her head grew rapidly until it was over the 99th percentile. This caused other concerns but 2 years later she's fine and normal. This made me feel much better. I just wanted someone to acknowledge that something like this could happen. So I thought "OK. If this ct comes back normal, I'll cling to this woman's story and assume he will catch up on his growth once we get his new liver." Then the ct came back - and it was ALMOST normal. Apparently, Jack has benign external hydrocephaly. This is generally not a big deal. It is something they see in kids with unusually LARGE heads. It is some fluid between the brain and skull and generally goes away on its own by the time the child is 2. But what the heck does it mean for a boy with a tiny little head? No one knows.

SO - last week our pediatrician got us a 10/22 appointment with a neurosurgeon which was to be cancelled if the ct was normal. Since it is not completely normal, we are going to meet with the neurosurgeon tomorrow. Our Dr. doesn't believe the neurosurgeon will recommend surgery but she thinks he may be our foot in the door to get people to really work to figure out what's going on with Jack.

I also wrote to a Pediatric Neurologist whom my sister was able to hook me up with. I told him Jack's whole story - maybe he'll have some insight.

This is so insane. I was so ready, after I heard that woman's story, to just say "OK. It's gotta be the BA - I don't care what the liver people say." But now, maybe there is something else wrong. I don't know. This is exhausting.

SATURDAY, OCTOBER 18, 2008 08:59 PM, CDT

Today was a very nice day - just hanging out in Andersonville. I want to make sure Jack has plenty of visual stimuli so I am getting him this cool mobile from the arts store that can hold postcards. I also want to tape different images on the car seat that he faces and change it all around periodically. So - we need lots of postcards. Bright vibrant ones with bold patterns, ones with stark contrasts, large black and whites images, ones with people's faces - stuff like that. You can write him a little message and I'll keep his postcards always for him. email me at noeykrimm@hotmail.com if you need our mailing address.

FRIDAY, OCTOBER 17, 2008 07:20 PM, CDT

Well, Jack and I visited the nice people at Cranial Technologies today. There was no big "a ha!" moment but I knew that would be the case. They took some nice photos of him from all angles (if he commits a crime we have his mugshots) and took a cool 3D image. The technologist said that she sees a lot of baby heads and this is what she could tell us: Could the dramatic drop in head circumference have been caused by the flattening of his head? Sure. Is the plateau in his head growth caused by the skull shape? No. She could tell me that the shape of his head is consistent with the effects of positional malformation (although she thinks the bridge of his nose looks atypical which is something I keep harping on that no other doctors seem to recognize.) She didn't see any reason to believe his skull was fusing early. That was pretty much it. She also said that she thinks the reason the Drs (except my pediatrician) aren't reacting with the panic I feel the situation deserves is that there is no way to assess what is really happening and the extent to which it is happening without more time. I think it also has to do with the fact that microcephaly is not curable or even really treatable so all they will be able to do is sit with a weepy mom and talk about how they have no idea how badly this will affect him or what his future holds.

So waiting is our only option and the only thing we can control is how we deal with it. The outlook we are trying to adopt is - he's our boy and we love him. He is what he is and whatever that is, we will love him unconditionally and take the best care of him we can. Right now I think the best way to do that is to stop carting him to a different doctor every three days. He needs to be playing and interacting and seeing new things - not strapped into a car seat and screaming - not poked with more needles. I think the brain can do miraculous things if you give it a chance.

I am reading about dealing with having a child with special needs. We can expect Jack to have delays - even if it isn't from the microcephaly, the liver transplant is inevitable and that sort of trauma and healing takes its toll. Also, he isn't absorbing vitamin E and long term effects of that deficiency are neurological damage as well. So I am working on getting past the grief of losing the child we dreamed we would have and making peace with the reality of it. That's our new job.

An observation about perspective: I see things more clearly now and have some insight as to what a "problem" truly is. I would like my neighbor to know that the fact that she saw two roaches in her apartment is not really that big of a deal and she should not be "more freaked out than she has ever been." And, if she is, she should not knock on my door and tell me about it.

The woman accross the street nneds to not complain to me about the fact that there's a woman at work who doesn't like her and not everyone RSVP'd to the party and why do they have to adjust their snacks for the one girl with the wheat allergy. These are not problems. Sheesh.

And finally, a look at the bright side:

We have not had to buy Jack any new hats.

TUESDAY, OCTOBER 14, 2008 11:11 PM, CDT

The panic is back. I'm sorry. I think this site is more about me sometimes than it is about Jack. I can tell you that Jack seems pretty oblivious to all of this. He is a smiley guy. His dad sings him Beatles songs and he laughs. One day, he got a particularly big kick out of the word "October." It's a funny word. He doesn't like to get a bath by himself so we take baths together and that seems to be a good thing and he especially likes to chew on the damp towel afterwards. God, he's so beautiful.

I found his vital stats from his 2 month appointment - the one where they decided to go ahead and give him a blood test for the jaundice. His head growth was already beginning to drop then. It was in the 5th percentile at that appointment. Since that is still on the chart and not too much of a drop from the 10th percentile, his doctor wasn't alarmed. But it does show, to me anyway, that the head was beginning to slow before the surgery ever took place - and before the flattening happened. At that time, Aug. 27th, his head was only one 8th of an inch smaller than it is right now. He's going on two months of essentially no head growth. And although the back of his head is flat, the shape is really not altered enough to make it impossible to track his growth. I think that theory was silly. They are all silly. The fact is that his head has stopped growing - because his brain has stopped growing. And we will probably never know why - which is consistent with most of the other parents in the microcephaly support group I joined. I joined it to try to get some answers - to find out who I am supposed to be seeing who I haven't seen. And they just patiently tell me that there really isn't anyone - that once you rule out genes and diseases, there are no answers. And most of them have accepted that and moved on, I guess. They say that only a select few ever find out why their children have microcephaly. And the future is completely unknown. If his head would just start growing, it would still always be microcephalic but it might track along its own curve and he could be OK. But it is still not growing. In fact, I think that the slight growth that was detected at his last pediatrician visit was not correct. I measured him 4 times today. 14 and 7/8. Every time.

I feel so sick all the time. Sick to my stomach - everything hurts. My jaw has been clenched so tightly that I'm having a hard time forming words sometimes. And I am so sad and frantic and sick that I may be missing out on the joy of the only time he may be seemingly well. Except for the jaundice, you would never know there was anything wrong. That will probably not always be the case. But I will miss it because I can't seem to pull it together. And I miss Lily, too. I used to put her to bed every night and now I can count on one hand the number of times I've done it since Jack was born. And I miss my old life. And I feel guilty for missing my old life.

MONDAY, OCTOBER 13, 2008 02:37 PM, CDT

Spent half the day trying to speak to the people at Cranial Technologies. The jist of what I was told is that although she is not an MD and she has not seen Jack, she can confirm that flattening of the skull can affect the measurements and if pressure to the flat areas continues, the brain will push other areas of the skull causing the brain to grow upward. Jack is being assessed by them this Friday. Then, on Monday he is having his CT and we are going to ask our pediatrician to adjust the orders to include a brain volume assessment so that we can know whether or not his brain is the correct volume regardless if what direction it's growing in. I am hoping that after all that we can feel like`we have some answers.

Still waiting on his liver to function. Poop color is not good. I think that if his growth and weight gain can stay on the track he's on, we should be able to get him well into next year before he needs the transplant. I am in a couple of support groups and there are children in them who are as much as 8 months old who weigh less than Jack does now. So I think on that front he is doing as well as can be expected.

Fortifying the breastmilk is a special challenge. While it takes breastmilk 4 hours to go bad, once it has been fortified, it is only good for one hour. If Jack decides he's not hungry, the milk goes to waste. Then, an hour and a half later he's starving and I have nothing to feed him. Our timing is particularly off today.

But, all things considered, not a bad day. I still don't really feel like I can stop holding my breath. Every time I do, I seem to get the wind knocked out of me. So I'll just wait until we get more info and work on being cheerful and positive in the meantime so my kids aren't scarred for life.

FRIDAY, OCTOBER 10, 2008 09:37 PM, CDT

OK. We are going to tentatively call this a good day. Jack saw his pediatrician today. She was really happy with his weight and length - he's actually gaining really well (this is a very good thing as he can hold off on the transplant if he is gaining weight) His head had barely grown an eighth of an inch. Not good. We got the test results and he was negative for all the viruses they tested him for (including toxoplasmosis) and his genetic micro-array came back totally normal. The only test results that are not back are the ammino acids but those were tested when he was born and were normal so we expect that those will be fine. SO - what is up with his head? We have no real idea. She measured our heads and remarked that neither of us have very big heads. She also said that she couldn't help but feel like it's not a coincidence that his head size dropped concurrent with the surgery. She said that maybe he just didn't get the memo that his head size shouldn't be affected by any of this. She also remarked that his development is not at all delayed and that that is really impressive since the hospitalization could easily cause noticeable delays. While she was examining him, he reached up and took the pacifier out of his mouth. She was really impressed. Sean asked her if it was possible that the measurements would be smaller since the back of his head became really flattened from all the lying around. She said that maybe it would but it would still continue to grow. She did add, though, that if his head is growing up instead of out - because of the new shape it is taking - that is not something she could really measure.

The craziest thing is that a brochure fell out of Jack's chart and Sean picked it up. It was about the importance of "tummy time." Our Dr. said - "Oh. Somebody stuck that in your file. You can have it if you want it." It was by a company called Cranial Technologies. I went home and looked up their website and there was a profile picture of a baby that looked just like Jack. There is a before and after photo and I just can't imagine that the measurements of that child's head circumference didn't drastically increase when his head got rounded out again. So our new theory is this - he started out with a small (but normal) head, it got flattened out during his hospital stay and the recovery time that followed so it measured too small for his age, now his skull is lengthening instead of growing in circumference. OK. It's just a theory. I'll be calling the people at Cranial Technologies to see what they think and I am going to measure him from the bridge of his nose to the back of his head and try to track growth that way as well as the regular way. Maybe I'm way off again like I was with the toxoplasmosis. He has a head CT scheduled for Oct. 20th to check for any calcifications that can be caused by infections and also to make sure his skull bones didn't fuse early - thus restricting the growth of his brain. But with all his tests coming back normal (which is the best news we've had in a long, long time) this seems like it might at least be plausible. And we feel so relieved right now. I mean, his liver is failing and he will need a transplant soon and his head is definitely still a concern but still... we are going to try to take a break from the constant gut-wrenching fear and worry and just enjoy our little family. We have a beautiful boy who smiles a lot and has the best laugh that comes from the back of his throat. We have a brilliant, adorable daughter and the weather in Chicago is gorgeous. We're lucky.

THURSDAY, OCTOBER 09, 2008 03:59 PM, CDT

Sometimes I expect everyone to be a liver expert. Bilirubin is bad - it is bile backing up into the bloodstream. His liver should be small and soft - not swollen and firm. But, like I said, I barely think about the liver stuff anymore. At least with his liver they know what's wrong and his doctors are experts and will know the appropriate steps and when to take them. His head a is a total mystery right now and no one knows what to do. And, in the meantime, while we wait for test results and cancel CT scans because he has a chest cold and speak with doctors who have no idea what to make of him, he gets older and older and falls further and further off the growth chart.

I am longing for the simpler times when all he had was a devastating liver disease.

WEDNESDAY, OCTOBER 08, 2008 09:56 PM, CDT

Jack was called in to see the specialist yesterday because of all the phone calls I made to various people on the liver team - trying to figure out if his head growth could possibly be attributed to the BA. His specialist says no but he also said he thought Jack seemed to be developing well and he didn't think everyone should get so bent out of shape about head size. He called in the Genetics specialist who said that he didn't see anything in Jack that would indicate any genetic problem that he has seen. I felt better yesterday long enough to eat. But here's the thing - these guys don't know general pediatric medicine any more. They are specialists and this falls outside their expertise. I spoke to my pediatician today and she was horrified by the notion that his head size is nothing to worry about. My biggest concern is that during all this time that we are running around trying to figure out what his head size means, days are going by and the head is STILL NOT GROWING. Not at all. He has gained weight - but no head growth. Some of his labs came back - rubella, herpes, and CMV were negative. thyroid and ammonia - normal. I have decided today that maybe he was exposed to toxoplasmosis. If I got the infection while pregnant, it could have passed to him. One symptom is unusually small head. I also found one article about a child who had both toxoplasmosis and biliary atresia. I also read that children with toxoplasmosis can be normal at birth and begin to display symptoms months and even years later which include eye lesions and blindness, hearing loss, mental retardation etc. There are drugs that children with the congenital infection can be given but I'm not sure if his liver can handle it and it won't fix any damage that has already been done. They did a toxoplasmosis test but the results haven't come back yet. I don't know what to hope for with these tests. None of these reasons for his head growth would be good but if the alternative is not knowing and watching his head drop further and further down the chart, I don't know which is better or which is worse. No one seems to be able to help us. And Jack rolled over today and laughed a belly laugh. And I don't know if we'll lose him or not.

I am so consumed by his head now that the liver disease has become secondary in my mind - but those numbers came in as well and the bilirubin continues to rise. Total went from 9.6 to 10.2 and direct went from 5.9 to 6.7. Not as huge a leap as the last time, but still rising. And his liver is larger and more firm than it was before.

MONDAY, OCTOBER 06, 2008 11:16 PM, CDT

Oh, man. Things just go from bad to worse. Jack's head has stopped growing. He was born with a small-ish head - in the 10th percentile of normal male baby heads - small but still "normal" Now his head has dropped below the 5th percentile - it is right around 1 percentile - which is concidered microcephaly. According to the liver specialists at Children's, Biliary Atresia would not affect his head growth so his pediatrician ordered a huge series of tests - genetic tests, tests to see if he was exposed to various viruses in utero, amino and organic acids, etc. If the head growth problem is not related to Biliary Atresia, then it is the result of some other rare birth defect - caused by genetics or by a virus - and it is incurable. I can't even write down all the implications of microcephaly. It makes me sick to my stomach. If it is related to the BA, then he should recover if we can get a working liver in him and although he will probably have developmental delays, he should be able to catch up. So we seesaw back and forth between relief and despair several times a day. My sister sends us articles that do relate head growth to the malnutrition caused by BA (relief) then one of the liver people shoots that theory down saying "Yes - but not in a 3 month old." (despair.) I wonder how long you can feel sick and anxious and desperate before you start rocking back and forth and talking to air conditioner units (one of my high school teachers used to do that.)

On the BA front, there doesn't seem to be any bile flow still. His appetite isn't too great, either. It takes a half hour or more to get 3 ounces of fortified breast milk in him. He's better with nursing - sometimes - but he doesn't get enough calories that way. They are talking about putting an NG tube into him (a tube that goes in his nose and into his stomach that I could pump fortified milk into to try to increase his calories. There is no indication of bile flow right now. His bilirubin will be taken again on the 14th and then I'll get another phone call on the 15th with the news. The results of the blood and urine tests should be in by Monday the 13th. That's all I know.

WEDNESDAY, OCTOBER 01, 2008 10:53 PM, CDT

I'm having a little trouble with his eating. His appetite seems to have slowed down. So has my milk production. I have been advised to pump and fortify the milk with special formula but the whole pumping thing is easier said than done. And then, getting him to drink from the bottle is a whole other battle. I would give him the formula straight except that he hates it. I keep trying and he keeps spitting it out. His color looks bad still. Sometimes I think his eyes look white and it makes me excited but it is just the lighting I'm in.

TUESDAY, SEPTEMBER 30, 2008 01:58 PM, CDT

I just joined an online support group and am working on setting up this site. There will be photos soon. One thing that is clear from reading posts from other parents in the support group is that we are on a long road to health that will include all kinds of changes and setbacks. This site is a more streamlined way to distribute info and you can always unsubscribe and I'll never even know.

We still have another 16 days til his next blood test. All we can do is wait and try not to obsess. One way to detect bile flow is the color of the poop. I obsessively examine his poop. I never thought I'd be doing something like that on a daily basis. Every day I am either heartened or discouraged by his poop color. Today it was a lovely mustard yellow which is normal for breastfed babies. Today I am heartened. Yay poop!