Where to Begin? A Summary of Recent Events

I admittedly wait long periods of time between my posts recently, and the farther apart they are, the more overwhelming it is to try and sit down and write out the most current events. I thought that was just due to trying to remember what happened, the farther in the past it goes. Now I think it's also more about how time keeps moving, more things keep happening, the list of items to blog about and update grows longer and - recently - weightier.

My last post was about getting ready for "Plan B," an upgrade to my PTBD biliary drain - to do angioplasty of the bile ducts with a balloon and open up one of the main ones that has serious scar tissue narrowing. I had that procedure done at the beginning of April.

Here I am, ready to go... little did we know it would be about six more hours wait ;)

I came out of it with a French drain tube doubled in size. The doctor exchanged the 8 gauge drain tube I had to a 16 gauge. Wooey, the first look I got under that bandage made me grateful I'm not a wound care nurse, but much more capable, not as easily grossed-out people are in that profession! :)

I was in a groggy state for a day and a half afterward, but it was so good to be home!

That means it has been a whole month with this larger PTBD, and unfortunately, no changes. I tried capping the drain on two different occasions, following doctor instructions. It went well overall; right now it's capped. I was losing way too many electrolytes in the fluid output of the drain, so I had to. I'm still battling those low levels of magnesium and potassium, as well as working really hard (it's not easy, for some reason!) to keep drinking water and other wholesome beverages, and eat good meals. It's a bummer that there were no changes to my lab numbers after this larger drain; even when the drain bag was attached, my bilirubin was still up at 19. Yikes. And the jaundice has stayed the same, and itching has returned WORSE. Ah if I could pick pain or itching, I'd choose pain. Itching is slow torture!

As I mentioned, there haven't been significant or really even noteable changes, improvements, to my lab numbers. This is the reason for my next big piece of news...

I'm glad we got this news the week of Easter. It made everything easier. I cherish the Hope of and in the Resurrection.
Always - Only - Jesus.

I've been referred to the University for another liver transplant.

(:sigh:)


One of my liver doctors sent over the referral, and I'm just awaiting their call one of these days to set up all those crazy pre-transplant evaluation appointments. It's possibly going to be a busy summer... if only it will be full of things like swimming lessons and dance classes with my Victory bear, rather than doctor appointments and uncomfortable procedures?? I will keep hoping and believing.
It's something that's come up in conversation numerous times since about four years ago when my team started noticing that the PSC (primary sclerosing cholangitis) may have recurred. But it was never so definite or concrete as now.

This is a picture of the imaging from my second-to-last procedure, when the first drain was "installed." They got it in and were able to inject dye to illuminate the ducts (the darkened, tree root looking things).
There should be so many more, especially on the left...

That is one mass of sharp, irritating to my skin blue stitches. And one big drain! I flush it with saline like this once or twice a day.

Just for the record, heading into another twelve hour surgery is not what makes me take so many deep breaths, and need time to process these developments, and soak in the Word and worship to be fueled up for those inevitable Mind Monsters. It's not that that scares me. This time I would be heading into a transplant not as a light-hearted teenager, but as a wife and mom to a toddler. I want to be here for them. Always. And a waiting list of approximately, at all times, 17,000 people needing a new liver, versus only the 6,000 per year that receive one... well, do the math. (Source)
Of course this is a fear that I don't dive in too deep to, because my heart and that Holy Spirit voice thankfully always tug me back to earth and say, "but Emily, BUT GOD. This may look daunting, BUT GOD. He can do anything, and He wouldn't have brought you this far to leave you or let you be done living now."

So anyway, that's the biggest news I had. My days are so full of joy and memory making, it's not derailed me too much, just mostly made me live all the more intentionally to soak up the everyday moments. Hobby Lobby had this painted sign I wanted to get for my living room at one time (coincidentally, it's not there anymore). It said, "There is always always ALWAYS something to be thankful for." How true that is!

I had a glorious nap last week, later than usual, and woke up to a gorgeous sunset...

A couple Sundays ago, we took a short drive to the Peninsula and found a secret children's garden. Dreamy looking mature trees, and little fairy gardens someone's made. Miss V was on the hunt for Peter Rabbit :)

The Secret Garden

Momma and Miss V made it to Costco all by ourselves the other day! What a fun time and a big accomplishment :)

I experienced a huge glimmer of hopeful progress the other day. My eyes became more and more white throughout the day, and by evening they were whiter than they'd been since Christmas! Unfortunately back to very golden the next day, but that event sure was encouraging. Looking for more of those in days and weeks to come!

We always enjoy the beautiful garden at the clinic. What beautiful flowers and trees we get to appreciate because someone works very hard at planting and tending.

I've been marveling at God's grace and sustaining me to be able to keep up with Miss V around the house, and I'm on a baking roll this week as well! V and I made grain-free "puffy oven pancakes" on Monday, and I whipped up a batch of sugar cookie dough from a gluten free Hodgson Mills mix. We never got to make Christmas cookies.... or football/Superbowl cookies... Valentine cookies... or shamrock cookies... or Easter cookies. Despite me getting everything including the appropriate cookie cutters out on the counter, every time I've been too tired. Well we are going to make our Easter/spring cookies, no such thing as too late :)
Late last night I couldn't sleep and made coconut flour blueberry scones. (Yep we're still very much gluten free around here. We added back grains last year when I needed to put on weight desperately. Phasing them out again little by little, because all three of us do better without.)
Then today I made a rhubarb crisp, grain free, vegan, refined sugar free, with local rhubarb from a farm down the road. Delish! I have missed baking very much, and can't believe how much I've been able to do including cleaning up afterward, doing multiple loads of dishes and laundry each day, hanging with Miss V and watching a movie a day and doing crafts with her. Every day I have strength and energy to keep up and make life fun in my home, I am SO thrilled and grateful!

Speaking of energy levels and miracles...
Another hurdle I'm in process of jumping is some wonky blood and bleeding issues. My exhaustion level didn't fade enough once I capped my drain last week, and I had a hunch about not just electrolytes but also my blood counts. Sure enough, my doctor reported back to me that my Hemoglobin was at 8 and Hematocrit 26. My Prothrombin time and INR (both indicators of the time it takes for your blood to clot) are double what they should be. I actually had to wait a few hours while I was infused with three units of Plasma before my procedure earlier this month, because my numbers were past their cut off for a safe procedure.
It has to do with the challenges my liver is fighting through, and the state of sickness it is in; PT and INR rise. Not much you can do, I am now taking Vitamin K but really I just need healing! I'm really starting to feel it and noticing that it's not really improving.
Oh more blood drama! It's been almost a full year since my intense war against Autoimmune Thrombocytopenia ended and [I believe] God healed me and put that nasty disease into remission forever! But I'll never forget those trips to the ER hanging on as I was on the verge of passing out and my skin was so white and cold.
I got my blood drawn yesterday evening to check Hemoglobin, Hematocrit, and to do a Type and cross for a blood transfusion. It's inevitable that I'll need one within a week (actually, it's been a week since my labs were drawn last and my doctor informed me of the electrolyte and blood situation and my need for a transfusion.) but the Infusion Center where they do blood is so booked up, I can't get in until Monday. I do NOT want to end up in a critical situation ever again due to low red blood cells, so I figured checking today would be a good idea, we'll see where my numbers are at (knowing I'm still having bleeding issues - dumb gut ulcers!) and if needed, the team will go ahead and put me in the hospital for a short stay to receive a blood transfusion that way. I'd much rather give up a weekend day doing that then have to rush to the ER late Sunday night because the numbers got out of control.
Please say a prayer for clear results and a great plan of action. And as always, a blood match for me that's clean and healthy!

I think that is the summary of the major things going on. It's been a busy month - anyone else feel like April just FLEW by? But I am excited for May; it's my "happy month." :)
I will celebrate thirteen years since my liver transplant on Cinco de Mayo the 5th, my third Mother's Day on the 10th, and my 30th birthday at the end of the month! It's a good life. Never let go of your vision or your fight.

And don't take life or yourself too seriously! ;)

Right?? :)
Interesting to me, my hair is getting back to its curly, wavy state. It was like this before my liver transplant in high school, and changed to being mostly straight when I was pregnant with Miss V. Now, the curls returneth!

Here is a group of statements I wrote out to myself last week when things were feeling overwhelming, lonely and anxious. A declaration:

"I choose to live.
I choose to keep moving.
I choose to take one day at a time.
I choose to not quit.
I choose to trust Jesus.
I choose to believe the enduring, unfailing promises of God.
I choose to look high and low, far and wide, to find any joy I can find in the most difficult of days.
I choose to hope.
I choose to stay in the fight.
I choose to be fully present in the moments for myself, my daughter, and my husband.
I choose to thank God in every moment.
I choose life.
I choose joy."


And here is a beautiful prayer I discovered; I've heard and read parts of it before, but wanted to see it in its entirety. I want to print it out and carry it in my purse with me to remind me of these truths.

St Patrick's Breastplate

Christ be with me, Christ within me
Christ behind me, Christ before me
Christ beside me, Christ to win me
Christ to comfort me and restore me.
Christ beneath me, Christ above me
Christ in quiet, Christ in danger
Christ in hearts of all that love me
Christ in mouth of friend or stranger.

(390-461 A.D.)

Good News, Not as Good News, and a Plan

I had another appointment with hepatology on Wednesday. I have three liver doctors overseeing my "case" just in that clinic, as well as a gastroenterologist (guts doctor) and three nurses/medical assistants working with me to communicate messages and test results, med changes, appointment requests, and so on. I'm in great hands!
This particularly hepatologist is the newest to my team, but the most experienced. The more we learn, the more impressed I am - he has been a part of or in charge of boards locally and around the world for GI, liver, and more that I can't remember. It amazes me to look back and see the path that curved like a zig zag up a mountain for a while, years back, when trying to get through to my team was near impossible and I felt uncomfortable and stressed - to now, when I have world-renound specialists that study this rare disease PSC and other like it, for most of their career. God is always a few steps ahead, working things out, isn't He? Awesome :)

Leaving my appointment- WITH supplies, woo! -wearing my new "SheIsStrong" tee

It was a great appointment. Dr checked my biliary drain site and said it looks just like it should. I got three spare drain bags and three new "stat locks" - the special sticky-backed plastic locks that tack ever-so-uncomfortably to my belly and hold the drain tube in place just below the stitches, so as it continues down to the Luer lock connecting to drain bag, it won't pull out of my abdomen and gut that it's in, if it gets snagged on something. (Which definitely would have happened by now if i didn't have the stat lock... but the one that had been on there for almost six weeks was so ready to go!)

Much of my time is used up resting, resting, and resting. I have a list now: things I wish I could successfully do lying down. Sew, paint nails, write thank you's, bake, cook, ...  Thank you for the daffodils Ry :)

The not-as-good news of the week was that my bilirubin, although dropping initially after this drain was put in February 14th, has slowly climbed back up to a number that has brought back even the terrible itching that comes with liver disease and severe jaundice. Ah so uncomfortable!!! I'll wake up in the middle of the night scratching my arms intensely and realize- wow my skin is just on fire, sleeping doesn't matter! I can't wait for the bilirubin to drop back down to a good level so that will go away too!

Last weekend I got to sit down for some ME time and made this "CHOOSE JOY" tee :)
I'm loving my growing collection of meaningful shirts!

My bili from last week was 16.8, and my doctor was saying it needs to be down around 10 before they'll look at removing the drain bag and capping the tube to just keep as an access point into my bile ducts. Thus, we discussed, it is time for the next phase of Operation Open Bile Ducts! (not an official title ;)  My doctor shared with my at last appointment that he just had a patient receive a liver transplant last year, after TWENTY years of being on the waiting list, but successfully having a PTBD drain that they would use to access the bile ducts and put in balloon stents and shunts as needed over those two decades. That regular routine keep him going for twenty years; that amazed me! He has more than one patient like that; one was just in before me on Wednesday.
Hearing about that as a very viable option makes me feel really encouraged! My dream and prayer is still to be healed, miraculously, completely, instantly one day! God is in the healing business! But this is another way God can continue to sustain me and restore health to my body, and if it does go on for years, who knows what other technology will be discovered that can help even further! Can't put God or science in a box- or the way we think our prayers will be answered. You never know what God is planning and setting up a few steps ahead :)

Ok sorry about that slight rabbit trail - back on track now. That will be my next step - PRAY the scheduling staff calls me Monday morning and has an opening for Tuesday or Wednesday morning, because I am so uncomfortable and yellower and itchier, and my liver really needs a lower bilirubin level to function well. I am seeing signs of the same communication challenges we ran into last time, and I hope they clear up tomorrow! Nothing like knowing what you next step should be and not being allowed to set it up. {Frustrating!}
Whenever it does end up being scheduled and done, the procedure will be like an ERCP but going through the PTBD drain in my abdomen instead of down my esophagus, and they will use balloons to inflate the scarred, obstructed ("strictured") ducts to an open state, and get those major bile ducts, especially on the right sided liver, draining through again. I wish I could share the pictures from my MRI and fluoroscopy, they are so cool in 3D and animated and everything! I asked- not an option yet.

So if you would, pray for an opening to have this Interventional radiology procedure done SOON, for it to be a "slam dunk" as my dcotor said he expects it to be, and for great function and drainage flow to be restored to those strictured ducts as soon as they get in there with the balloons to inflate the roadblocks of scar tissue and stones!

Here are two photos from today after church. You can't always tell the severity of jaundice on film, but it's been pretty severe! Today at the store I think I scared a few people- my eyes were glowing golden, and next to the undereye concealer I was wearing (regular skin tone concealer - because where do you buy 'jaundice' colored makeup? Lol!) the contrast was just unreal!

I find that if anyone is going to stare at my golden glow, it's not kids--- 

---It's adults! Adults stare at me in public places, and I just want to say, "didn't your mother/teacher/brother/grandfather tell you, 'If you can't say or do anything nice...'?"

I'm thankful for a good week, energy to make memories and laugh with my two sweeties,, and some wonderful doctors with an encouraging, positive plan that we all feel comfortable and hopeful about :)
God is faithful

Little Bit Forward, Little Bit Back (or, Stinky Bag of Bile on my Hip)

It has been just over a month since I had the procedure to put in my PTBD (biliary drain), and I have not known how to write an update post. There is so much to communicate, or so little.

Some days I feel like writing out a post the length of a book chapter. Other days, I just want to write a sentence and post a photo, and leave it at that.

Things haven't gone quite how I expected. In several ways. 

I am always seeking a healthy rapport and clear communication with my doctors, and usually we all achieve that. But in the days before this procedure and also in the recovery time, that did not go as usual. Working with some new doctors who do not know me and my medical history quite so well, and don't realize that I know my way around my chart, my health and the way things work...

About to go into the procedure room.
I think this was right before the nurse gave me a dose of IV Benadryl.
Yikes that stuff is trippy! :

 Based off the first explanation of the PTBD back in January, I had different expectations of how the procedure would go. I was sitting there in the pre-procedure room, after it took the nurse twenty minutes and two painful attempts at putting in an IV (I'm normally a perfect IV and lab poke patient, but she picked an odd area of my arm and I was so dehydrated from the NPO/fasting before the procedure, it turned into a not fun time.) Once she got a line in, she brought in consent forms to sign, and in reading them I started to think I was in prep for the wrong procedure. It did not sound like what my hepatologist had explained to us. I had to wait almost a half hour, on top of the half hour we'd already been waiting, for the doctor to come in and answer my questions. He reassured me enough by his answers to my questions that he and I were on the same page.

Turns out, we learn afterward, not quite. I won't get into the details. At least it was a successful procedure and I now have a drain that can be used in ERCP to access bile ducts for things like putting in a stent, etc. Also, it's been allowing some bile to escape through the exterior line to a drainage bag connected at the drain tube end, which I have attached at all times, flush twice a day and empty about as often.

Sunday morning, first morning at home after my procedure Friday. I felt great that morning!

Upon discharge from the hospital the next day, it quickly became apparent that we weren't given much information that we actually needed to care for this new appendage I had, and hardly had the supplies to do the physical care, or the knowledge to know what to look for as a concern. I gave it some thought for a few days, as I wasn't in the mood to urgently drive up to the hospital to see the same doctor and receive more vague instructions, or be sent to the ER by my much more concerned doctors, plus I was in a LOT of pain. (The pain, I figured out on my own in week two, came mostly from the drain going into my abdomen right above my diaphragm - with every breath in, it would push on the tender area and hurt so much! Also, I discovered, if I ate more than a small amount of food and did not space out my meals into tiny ones that took all day to eat, my guts full of food, which run right under the exit wound, would push on it and cause quite a lot of pain. Ouch!

The drain was put in the middle of my abdomen because it could not go on my right side like they normally put them. I got to see this on fluoroscopy film later, it was pretty cool to watch the moving images in 3D of the right biliary tree spinning around on the screen. It was not cool to see how bad things look - my right sided bile ducts are nearly defunct. Perhaps a more blunt doctor would say they are defunct. They are shrunken, twisted, knobby, narrow tubes that look like knarly strings of yarn that a cat or child got to and twisted or tied knots in repeaedly. Or, they also made me think of a compacted, spiral strand of DNA gone very wrong. Basically no bile gets through them. 

What amazes me is, that they could get so bad without much indication. It must have happened over several years, and the only sign I've had that something was funny is the dull ache I sometimes had and have in my upper right side.

The main point is, it's been nearly five weeks since the procedure, and we're just now figuring out that the site may be infected. My recovery took much longer than the doctor expected. I have been the most proactive I could be, I just kind of fell through the cracks on this one. The good thing is that Ry and I spoke with the hospital administrator in charge of patient care, and we were able to give great feedback.

I'm not a whiner, I'm not a complainer, and I hate raising any negative point without having a solution to offer as well. But in this case it just happened that multiple things were dropped, all for the same patient (me), and it can at least be a learning experience for all of us.

The good progress that was made is that for several days after the drain was put in, what seemed to be stones (bile stones? I don't know what they're called when you don't have a gallbladder) were coming out into the bile bag several times a day. They were not large, but it was good to see that they were coming out, especially since I didn't know they existed! Also, my bilirubin (albeit slowly) started dropping almost right away. My eyes looked less green/yellow, and my skin as well. Some days would look worse and some much better.

Me and Ry doing an eye/skin color comparison.
On the left: Jaundice / Right: No jaundice!

Unfortunately, though the docs said it can take six to eight weeks for jaundice to clear after a gradual build up over time like I had, and things were slowwwwwly but surely improving... things have now plateaued, and my Bili number actually went UP last draw, and my skin and eyes are not looking too good. A little girl at the park came right up to my face as I sat on a bench the other day, and said, "are your eyes green? Why are they green? Is your skin green??" in disbelief. I told her it is because I'm extra special, and she said, "Oh! ok." And ran off to play.
 A few minutes later though she came back to stare at me. Lol! I asked why didn't she go play, and she said, "Because I want to sit by you, you're extra special. And really pretty!" So, what she lacked in personal space awareness, which really comes later in life anyway, she made up for in sweet compliments :)

It's just funny, I don't notice when the jaundice gets severe, or I tend to think I look better than I do, then we go out somewhere and people give me strange looks, and then I remember and feel bummed that, darn, I must not look too good after all, or be making progress after all.

I have a couple appointments coming up with liver doctors in the folllowing weeks, as well as more labs to keep tabs on my counts. I had to get a blood transfusion about two weeks out from the procedure, as my hemoglobin isn't staying up where it should. My hematologist thinks I am bleeding somewhere; we don't know where. Probably in my guts, though, because I had to switch back to Tacrolimus for my liver transplant immune suppression, and that tends to make my colitis flare, which would cause bleeding in the guts somewhere. The Cyclosporine which I was most rcently taking needs bile to be digested probably and keep the liver transplant safe... couldn't keep taking that when my liver and bile aren't working right. If I keep requiring transfusions, we will probably have to start discussing colectomy and an ostomy bag again as we were last spring, before the ITP went away. Hmm... carrying around not just one stinky bag of yellow green bile, but two bags, the other filling up with gut waste. Oh boy! I don't even know if I'm strong enough to go through that colon surgery right now, but we'll probably discuss it soon.

[So, what was the toughest decision YOU had to make today? Lol! I hope it was something fun like which pair of new shoes to buy, not which stinky waste bag you want to carry around with you for the next decade... or for life! ;) ]

I don't envy my doctors, that's for sure. What a balancing act they are doing, with all the body systems that are acting up, needing to stabilize and help those, while wanting to keep the still-healthy, quiet things that way. Pray for their wisdom and for perfect solutions to be thought of. I have some amazing doctors (the best team I've ever had in all these years of medical care).

I had a liver ultrasound on Thursday, to check on veins and do the biannual check for liver lesions or suspicious bile duct changes...
(Yeah I'm 29, and they were looking for pre-cancer or cancer. That's one part of my life.)
This is an every six month thing, probably more often if my liver doesn't shape up soon, and I get placed on the transplant list.
They alternate between ultrasound and MRI checks, because I opt for NO radiotion, NO CT scans.

If this is all pretty technical medical jargon and you don't understand it but would like a summary version, just leave a comment. Feel free to ask questions too. Like I said near the top, I don't even know where to start with everything that's been happening. I just started typing and here's where I ended up this time.

The main thing is, things aren't going as well as it seemed they would be, as presented to us before the procedure. But no one can control that. I got through it, and at least doctors now have an access point to my bile ducts, whereas before they did not, and were not going to put me through a third ERCP attempt to try that again.

I am taking care of this bile drain the very best I can, I am taking my meds and drinking my veggie-fruit smoothies (I crave them now when I miss a day!) to help my liver the most I can. And I am hoping for the best, declaring God's promising, and leaning on His truth! He's never failed, and He won't start now!!!

Thank you so much to the friends-that-are-family who brought us dinners for the first two weeks of this challenging time. You were our angels!

Thank you for those who continue to pray with us believing for the miracles we need, those of you who speak affirming words of health and agreeing words of healing over me, those of you who sent cards, letters, or very meaningful gifts like the custom made bracelet that says, "she chooses joy." Talk about motivating to keep up this fight! And some days boy do I need that motivation from those of you so dear to me.

Onward, forward, moving along... life goes on! I'm still here still breathing still very full of purpose and spunk - God's not done with me yet! ;)

Finally made it back to church last week, and this week I was even stronger!
These two lovies in the photo with me are my posse.
They make it all possible - their flexibility and kindness and selflessness and faith.
Yep, that all applies to Ry AND our two year old. She is amazing.
Love you two so much!