Houston, you always have problems

Our Liver Clinic Appt:

1. Ws labs are more elevated than the last go round. Bili is normal. Maybe from being sick with a cold?

2. Currently, they are thinking that his vein scarring procedure may have helped and hurt him. While he needed to have something done to keep him from bleeding into his intestine, now there is more compromised blood flow.

3. Compromised blood flow to any organ/tissue causes damage. (Think heart attack process) This is probably why his numbers are still high.

4. The great bile duct blow out of 2012 has nothing to do with this vein/blood flow thing that is currently playing out.

5. "Portal Hypertension" keeps getting used. I need to review some info on this before I explain further.

6. A very brief, very much un-welcomed mention of a possible re-transplant years down the line if they can't get this sorted out. Mom started to get bat-shit crazy, so I was reassured it was nothing he needed NOW and the convo was quickly steered elsewhere.

7. Next step is a MRI over the holidays to study blood flow to the liver.

While we are not in a crisis situation right now, I sure would appreciate prayers and positivity sent his way. We'll keep you updated. Thanks!


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Lots of Sharp Objects

What a week.
Daddy and Weston went to Houston Sunday night for his summer check-up and follow-up biopsy.
That night they lived like bachelors in the hotel.

Since moving back, his liver panel has started to trend down. YAY!
Not sure why all of a sudden, but we are not questioning!

As much as 33% in the case of his GGT!
The biopsy showed no inflammation (!!!); no scarring (!!!); no bile ducts (???)
At least in that sample.
Because his labs are improving and his bili isn't elevated, hes got to have ducts somewhere. Needless to say, they weren't too upset about it and sent them home Tuesday night.
While waiting for results, he hung out with an "astronaut", stole a police car and chucked two cans of Sprite at the recovery room nurses. :-o

Annnnnd just because we're home doesn't mean that we're done with doctors and needles.
This afternoon, we had a DOUBLE kid check-up with back to school vaccinations.

Weston got 3, Emmy got 2.
o_O

We got some stats:
At 3 years old, Wes is 3' tall and 36lbs.

75th and 80th percentiles, respectively.
His pediatrician was very impressed at his growth and development for being a transplant munchkin!
At 5 years old, Emmy is 3'9" tall and 43.4lbs (50th%)
She'd be in the 95th percentile for height...if she were 6+.
Tall, tall girlie.
I'm going to have to cut this short, because I was just informed that she did her make-up for her wedding happening in two minutes.

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The Deal

We've just been going along with minor adjustments to W's ProGraf for the last month.
Weekly labs.
No real changes to the values.
Very jaundiced little boy.
All too frequent pale/less than acceptable poop.

Then I was a bit blindsided Monday afternoon.

He has to have labs drawn Friday (tomorrow) and if there is not significant improvement, they want us to come in next week for a new medicine infusion. 

Rituximab

Chemo.

I want to vomit. Cry. Smash things. Scream WHY?!

I had a hard time with the addition of Imuran.
Every time we start a new immunosuppresant I freak and worry myself sick about cancer.
Well, now he could get chemo. 

I am terrified and angry and so, so sad.

As I am understanding, he is still trying to reject his liver. So, with this Rituximab it will basically target all his B cells (part of his immune system)...pretty much wipe them out and "reboot" the immune system.

Lots of prayers for a miraculous turn around.
We should know by Monday.

His favorite bath time activity.

December?

1. Christmas Cards
Your Christmas card is going to be late...and, no offense, you might not get one.
First time ordering cards via Internet and they got our order wrong.
So, just in case, Merry Christmas!

2. Jack

Don't feel sorry for him, he totally deserves it

Ok, so the new puppy smell has worn off and now Jack is an accident having, biting and terrorizing little fuzzy demon.
Alright, he's not that bad. But, the honeymoon is definitely over.
Emily is still in love.
So, that's good.

3.Emily
She's been on a roll lately.
"I can't go to sleep; I'm allergic"
I wish I would write her words of wisdom down when she says them...I can't remember the rest, but trust me, they make you shake your head.

She had her Christmas Party at school yesterday.

"I had too much hot chocolate."

She's precious as ever and looking forward to Christmas.
She has asked Santa for a microphone so she can be the "hopstar" of Princess and the "Hopstar".
She is quite the performer.
We get multiple choreographed renditions of "Here I Am" from the Princess and the Hopstar, and "Extravaganzalorious" from Elf on a Shelf.

4. Bubby
Bubby and DaddyDaddy are both sick with icky colds.
They're so sad.
:-(

On the liver front, he remains a weirdo. While his enzymes have all improved (including his GGT by 1000 points), his bili level is up above 7.
What the heck, man?

He's almost as yellow as he was for his first Christmas.
:'-(
We're just going to watch him and see where it goes.

He crashed Emmy's party yesterday and discovered Doritos.
I guess it was the first time ever to have the evil cheesy chips and he couldn't get enough.

Check out those Doritos stains on his sleeves!

Poor little guy.

5. Me

I turned 30 last week.
I guess I am officially an adult now.
:-/
The DaddyDaddy was wonderful and surprised me with a dinner Saturday night with friends and family...even though I had to wear a sombrero and be the target of loud singing.
As a present to myself, I went and had a physical.
I mean,  guess that's what old people do?
Along with the realization that I am an adult, I guess I need to really start taking care of myself.
So, had to get all the baselines checked.
Blood pressure was good, blood sugar was certainly in range, cholesterol and LDL were NORMAL (shock) and even my HDL was in range.
Triglycerides were the only area I need to work on...and as I incorporate more fruits, vegetables and flax seed (like old people should), that should come down.

So, that's what we've been up to.

Can't wait to see what Santa has in store for us!

Pleased

This IS my happy face.

Actually, I'm over the moon!

So, the attending this week is the new Chief of Staff for the Liver Center and is the Biliary Atresia guru.

And the fellow is someone who has been with us through this whole fiasco starting in September.
Awesome team this week.

YAY!

So, Dr. S says that the biopsied tissue looks really good!

-Little to no inflammation

-BILE DUCTS ARE REGENERATING

CAN I GET AN AMEN??
TESTIFY!!

-"If a pathologist was just looking at random samples, he'd think this was a pretty normal finding."

OH EM GEE!!

BUT.

-No, Doc. No buts. Let's end it there and Bubby and I will ride off back to Stephenville.

I would say off into the sunset; but, you know, its after 5pm.

Its stupid dark so early!!

...

BUT..."we have no idea what has caused all of this. It's an anomaly."

There's that word again.

When they do the biopsy, they go into the right lobe of the liver. You are assuming that the left lobe is behaving the same way. You know what they say about assuming...

I guess they have a good reason why they don't biopsy from the left lobe. 

Needless to say, they don't.

The LIVER looks amazing in the biopsy. The labs, especially the GGT (bile duct) is still crazy high.

Weird.

Three hypotheses:

1. Chronic Rejection: Maybe he was starting an episode of chronic rejection and the steroids and extra immunosuppresant are starting to work at turning the situation around. Labs are just going to be the last thing to reflect that.

2. Medication Related: Medication theory that something he was taking was destroying the bile ducts. *Pretty much ruled out, because everything potentially hazardous has been stopped for quite some time and the GGT is still abnormal*

3. Hepar Sinister: (I totally made that up just now. Look at the big brain on me)-It is possible that there is a piece of liver in the left lobe causing all these problems and while the majority of the liver is functioning normally, that is the part being reflected in the labs. Which may explain the intermittent weird poo and the bile related itching.

One more test...

A MR Cholangiogram. 

I'm not going to even pretend to know why and how this is different from the cholangiogram they did during the biopsy yesterday. Suffice it to say, it is and it's what might be able to tell if the left side is running-a-muck.

Fine.

Whatever.

The important part of my story is
WESTON'S LIVER IS DOING WELL AND THE BILE DUCTS ARE COMING BACK!!!

He is scheduled as the first case of the day at 0730 (meaning 10am) and then after he recovers from the sedation, we can go!!

Waiting For White Coats

It's almost 5pm and we're still here...

This is how my nightmare starts...I go in for something simple, say "How to Rid Oneself of OCD Tendencies." They find out I'm bat sh*t crazy, commit me. The next thing I know, I'm having a lobotomy and drinking mashed potatoes through a straw. 

Ok. That was extreme.
But, its almost 5pm AND WE'RE STILL HERE!?

WHY?!

They came by earlier this morning to say that they are waiting for the biopsy results, but in the meantime, lets do an ultrasound.

Sure, why not?

And that, friends, is where we are.

It's almost 5pm and we're still here.

"They looked ok, I'm not messing with it"

That's what the Interventional Radiologist said about Weston's bile ducts, when I ran into him, wild-eyed and crazed for information. So, to me, that means he got in there and was able to shoot the cholangeogram
and everything looked "post liver transplant fine". Haven't talked to any other the liver doctors, so I don't know if this is actually true or not. No drain necessary. Yay!
I was really sweating that one...can't lie. What if he pulled it out??? What of the bag leaked at school, spilling bile all over?? Gross.

Of course, my pessimistic nurse brain is now wondering, if the plumbing is working, why is the GGT still high and why are there intermittent acholic stools?

BTW, as I write this, the CA just rolled in to do vitals on my JUST now asleep child...**primal scream!!!!!**




Waiting for the fun to start, watching Octonauts.

Now we wait for tomorrow afternoon when the biopsy results are in.




10 minutes ago...


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Location:Bates St,Houston,United States

Secret Basement Lab

I've been keeping this under my hat for a week or so now. Quite frankly, I've been trying to weasel our way out of it since the day the decision was made.

We are in Houston for yet another liver biopsy. Weston's labs haven't really changed. And now he's having intermittent acholic stools (pale, no color=no bile). There were not only pictures of said stools, but actual electronic transmission of the pictures. You gotta hope THAT was the email Big Brother decided to audit ^..^
Dr. F was concerned and after consulting with the new Liver Center Captain, they wanted to biopsy to see if any of the bile ducts have started to regenerate; why the acholic stool; possibly place a drain to help pull bile off.
Like I said, I've come up with all these fantastic scenarios NOT to have this done just now...included but not limited to bribing and promising our next pet will be named after Dr. F
They are immune to my shenanigans.



We got to Houston a little after 8 and went straight to the hospital's mysterious basement lab for pre-procedure blood work. His biopsy, etc is scheduled for noon tomorrow.
I know we will stay at least tomorrow night...I hope that's all.

On the home front:
Emmy and Daddy are adjusting to life without Baby Bunny. It was a little touch and go for a while, but the ever so resourceful Daddy made it all better with pickles...




Keep Bubby in your prayers tomorrow. He could be in the procedure room for up to 3 hours depending on what they do.
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Location:Dryden Rd,Houston,United States

Son of a B*SCREEEEEE!*

If you've watched Rango, you know what I'm talking 'bout.

Five glorious days at home.

Someone crashed out in the middle of the floor

 Emily and I had a date afternoon. She got her first mani/pedi. She picked sparkly purple with flowers. She did such a good job and the ladies were so nice and patient with her.

Bubby is not going to miss an opportunity to slide, no matter if its raining.
Checkout his wee little boots!!
Ostrich boots, courtesy of GD; admired by all.

 Granny and Grandpaw are visiting from Nevada!
Yay!
Talk about some serious baby spoiling.
Sunday, we went over to Billy's step-brother's house for a BBQ to celebrate his recent engagement to sweet Mindy.
We are so excited for you guys!!
Talk about a super cute couple!

My beautiful girl <3 br="br">(She may have received the coveted "Rapunzel dress" and matching accouterments despite technically "earning" it; even though she's been a sweet girl. Mother's guilt may or may not have been part of the decision making.)

Swinging in the rain

I don't even know.
He mad, bro.

 Which brings us to current events.
Monday morning, Bubby had blood drawn at our local hospital lab.
He went to school.
No biggie.
Until around noon.
His labs were significantly elevated and needs to come back to Houston now for another liver biopsy Tuesday morning.
*Insert blog title here*

So, we packed up.
Including our own straws, diapers, diaper trash bags and graham crackers. Oh, and emergency chocolate.
(This old dog learns pretty quickly)

Bubby was all smiles this morning before his biopsy. 

So much so, we are suspicious of the so-called "IV fluid".

Cutie pie is all smiles

 On his way down to International Radiology.

He loves to ride in wheelchairs.

Pre-procedure snuggles

 His biopsy went well and they also placed a PICC.
YES!
No more pokes for the time being.

Recovering
I don't know how he was able to sleep, as there was a screaming contest going on just behind that curtain.
I am so glad I don't work in pediatrics.
o-O

No news until tomorrow late afternoon.
An awesome bonus is that our beloved Dr. F (regular liver doc) is on hospital service this week!!

Thank the Lord!
Not saying anything negative about anyone else, but it is so nice and reassuring to see the familiar face.

He's doing well this evening.
A little crabby, but ok.

Liveration, Part 2

We are home!!

We were discharged from the hospital late Tuesday afternoon and spent the night locally because we had to have labs drawn in the morning. 

My uncle and aunt put us up and fed us REAL food aaaaaaand perhaps the best part, they live maybe 5 minutes from the TCH West Campus, which we were able to go to instead of traveling all the way down to downtown Houston.

I just heard the news.
I'm too cool for this place.

Tuesday, before we left, the wives of the Houston Texans visited and brought some goodies.
They were oohing and awwing over my Bubby, and he let out the biggest burp.

lol

Good. He just loves the Mommy.
And the Cowboys.

On his way out.

Houston traffic has never been so beautiful

I'm free!!

We got home to Stephenville Wednesday evening.
I was so excited to see Emily!!

She was a bit more excited to see Wes, but I'm ok with that.
I was pretty excited to shower in my own bathroom and sleep in my own bed.

Weston was so excited when he realized we were home.

Clapping. Cheering. Big smiles.

My big guy

These are my toys!!
I remember them!!

So, here's the plan:
Labs AGAIN tomorrow and then weekly thereafter.
:-(
We went from just one medication twice a day, to five all through the day.
It is going to take some time for the liver numbers to normalize (they are no where near normal, yet) and they are going to watch him like a hawk (hence the weekly labs).

He should be able to go back to school on Monday, though.
I think it's good for him to get out and get back into a routine.
We've got to go shopping for Bubby. Thanks to the steroids, all the new clothes he had for fall don't fit anymore.

We are all so happy to be back together and start getting back to the new normal.

Thank you again, for all your prayers and support.

:-)

Soon

We are getting so close to being able to go home!
Today, we got things lined up to get his new medications delivered to home; started Imuran; and had another dose of IV Lasix and pulled off a lot more fluid.

A lot of people have been asking for updates and a prognosis regarding the episode of rejection and the bile duct business.
While we are pretty certain the root cause of all this mess has been uncovered, the situation is delicate.
At this time, all I can say is that there was an issue with the medication at home.
The best thing to come out of this, is that this bizarre "he's been in rejection all year and hasn't shown it"; the amoxil ate his bile ducts theory; and he's a fascinating alien; have all been de-bunked.
He is a normal post-transplant baby, and under normal, compliant circumstances, will do just fine.
Although his GGT is starting to improve with just the ProGraf and Prednisone (oral steroid), they still want to start a low dose of Imuran to help punt it back where it should be.
Perhaps, once all his enzymes return to normal and he has a good result from his follow up biopsy, we can see about getting rid of that second immunosuppressant.

Over the last two days, they've been able to pull off about 800mL of fluid with the Lasix. YAY!
While his face and tummy are still very puffy, I can totally tell a major difference in his feet and boy parts. I bet that feels a heck of a lot better!
He feels better, too.
More running and playing.

More laughing.
:-)

I thought I'd share some of that precious laughter with you.
The Lorax is Bubby's new favorite movie.
He thinks the Lorax and the little animals are hilarious.
It's totally presh, I tell you!!

Sorry for the low quality. :-/

Just Plain Over It

I don't know if it is the lack of activity on the weekends that just make them seem to last forever.
GD came in to visit this weekend.
He's been taking Bubby on his walks, so I can nap during he day.

Poor Bubby.

He is SO SWOLLEN.
His clothes don't fit.
Shoes don't fit.
Hands are puffy.
Boy parts are swollen, too.
:-(

Can you believe this is the same kid??

His belly is so big and tight; he's taking shallower breaths and just can't get comfortable.
Think 9 months pregnant.
I am guessing this is an issue associated with the steroids...I also feel like I'm the only one that this is concerning.

He gets tired walking around and wants me to carry him on the way back.
Problem is, he's getting so heavy, I can't really carry him.
He's up over 5 lbs since admission.
Poor little guy can't sit up without a struggle and took 30 minutes to get comfortable in bed tonight.

That diaper is reaching its limits.

**WARNING**
I'm hosting a pity party for myself, please stop reading.
It's more for me to vent and get it out.

Sick of Tired of:

• Midnight and 4am vitals, followed by midnight and 4am snack fest
• Neighbors
• Being at the mercy of others for clean sheets, towels and snacks and diapering supplies for W
• The massive temperature swing between the arctic setting of 70 and the Sahara setting of 75.
• Not having my own broom/mop/vacuum to clean this floor
• People just barging in without knocking
• Volunteers
• Cafeteria and Fast Food
• Not having the appropriate sized diapers for my child
• The same 4 outfits 
• Having to risk getting cut to do laundry
• Cars and Toy Story 3; Bubble Guppies
• Having to use the main garbage can as a diaper pail

Missing my little girl and the DaddyDaddy lots today.

Is That A Light I See At The End Of This Dark Tunnel??

I think it is!

Yesterday they drew a special blood test to check for some enzyme that will tell them how to dose the Imuran to avoid the bad side effects.
Can't argue with the cautious approach.
The bummer is that its a "send out" lab...like California send out.
2-3 business days send out.
:-/

You're killing me smalls.
Monday? Tuesday?

In the mean time, he is finally off IV steroids and on oral steroids.
Can't wait till we are totally off steroids.
Check out little man's poor belly.

Poor baby.
The shirts that fit when he came in are too small and don't fit over his belly anymore.
He's gained over 2 lbs since we got here.
It is so tight and his little belly button is poking out.
Most is fluid; some might be chocolate milk.

My feeble attempt at reminding him how much he liked vegetables before all this mess.

We haven't slept well the last two nights.
Midnight and 4am vital sign checks are getting oooooooooold.
Not to mention, our new noisy neighbors that moved in last night.
Geez almighty.
As with any hospital stay, there is no rest for the weary. Someone is always waiting to mess with you; usually on the heels of the last person that was messing with you.
Just a bit of fair warning...the next person that says "I'm sorry for waking you up," gets a punch in the face.

Bubby concurs.

In closing, I've got to share something that our attending doctor this week told me.
Got to say, I really like this guy.

He's very into visuals and teaching; and actively involves me in their rounding.
Like, pulling up the labs and going over trends.
Maybe he realizes that I am a control freak and am impatient and demanding?
And it is better to just indulge that kind of crazy, freaky parent.
 

Today, as we were going over his labs, I noted that all his enzymes went up slightly.

He noticed I was freaking on the inside.
 

"You know, think of this as an airplane ride. The take-off is all tense and focused for everyone involved. Take-off is like the transplant and immediately afterward. Then, you get up in the air and you're cruising along. Most of the time, its a nice calm ride. But, every once and a while, you fly through a cloud and the plane shakes. The pilot knows that you're flying through a cloud and its a little shaky. The passenger thinks "OH MY GOD! WE'RE CRASHING!!
We, the doctors are like the pilots. We know he's flying through a storm. But, we know we're not crashing."

Well played, sir.

Well played.

I like that.
1,000 bonus points to you, Doc.

Anomaly

a·nom·a·ly  (-nm-l)n. pl. a·nom·a·lies

1. Deviation or departure from the normal or common order, form, or rule.

2. One that is peculiar, irregular, abnormal, or difficult to classify

3. Weston Wyatt Golden


After the re-review of his biopsy, the conclusion is Chronic Rejection.

 Probably. 

Most likely.

Surely?

I know what you are wondering...HOW can he be "chronically rejecting" when he's never rejected before?
Ah. That's the good part.
Evidently, he HAS been in rejection for quite some time, yet his liver panel never showed such evidence.
What?
Yep.
Is that possible?
Evidently it is now.
This doctor has only seen this sleeper chronic rejection in ONE other kid.

Of course there still is the off chance that he's in Acute rejection coupled with something weird and bizaaro, like the antibiotic thing.
Probably not, though, as he hasn't taken amoxil in over a week and his GGT continues to rise.

My diagram of what is wrong with Bubby.

What now?
Imuran.
Ugh.
Imuran is another immunosuppresant that works on another aspect of the immune system.
So, he's on Prograf and that is working at keeping most of his immune system from attacking the transplanted liver. But those bastard bile ducts have to so darn tempting.
The goal of the second immunosuppressant is to further "blind" his immune system so it can't see those bile ducts.
At least that's the way I understand it.
He did say that his liver tissue looked really good and had hardly any fibrosis or cirrhosis.
That is awesome news!
I have tried very hard to wheedle out of them a long term prognosis on this Chronic business.
To say they are not forthcoming, is an understatement.
What I gather is:
If they can get the disappearing bile duct act under control (with the added Imuran), he has a chance of letting them regenerate and he'll be just fine with the liver he has.

If they can't get it under control and they keep disappearing...we're in trouble.
What do you mean "trouble"?
Hey! Look at that bird!

Ok, maybe not that bad, but definitely not super clear.

Imuran starts tomorrow. Of course, they need to monitor him on that for a few days, to make sure he doesn't have a reaction (ie nuetropenia) and they get the dosing right.
MAYBE.

M.A.Y.B.E.

We can go home in a few days.

PS:

Victory is MINE!!
Even if it is at 0700 am SHARP!