a little and a lot

Thursday, July 30, 2015

The 12 Weeks of Summer (Otherwise Known as: The Summer Everything and Nothing Changed)

By the 12th week of summer, my true love gave to me:

One new old house in midtown

Two AC unit repairs (including a brand new unit for one of them!)

Three fridge failures (still working on resolving that one--but it's under warranty!  Whew!)

Four family members heading to Chicago for a fake vacation this coming weekend/week.  (Brooklyn's next GI clinic checkup is next week, and since we haven't been able to stray too far from Chicago or out of phone range this summer in the event that we might get the call we've been waiting for, we figured if you can't beat 'em, join 'em...)

Five years old and ready to start Kindergarten in eleven days--that's Rhet!

And a partridge in a pear tree...and by partridge, I mean Brooklyn...and by pear tree, I mean a new diagnosis.

I'll expand on that one...
(And it's going to be long, sorry about that.  I owe you a long post!)

Back in May, Brooklyn and I made a quick 24-hr trip to Chicago for her GI clinic checkup.  While we we were there, they took labs, checked her out, and issued her a new PELD score.  To refresh your memory, the PELD score stands for Pediatric End-Stage Liver Disease.  The transplant list isn't a "waiting list" where the person at the top of the list gets a liver and you move up in line and take your turn.  Instead, your PELD score ranks your "priority."  The higher the number (up to 40), the higher your priority over lower numbers.  There are more factors involved when a liver becomes available, such as your blood type or whether the liver is a good "match" for your body, but the PELD gives you first dibs.  We've been on the list for 6 months, sittin pretty in the lower middle.  Someone could be listed today and jump to the top.  It all depends on your PELD score.

Brooklyn started out in February with a PELD of 13.  Her bilirubin (one of the numbers involved in determining her PELD score) had risen by the time we came in March/April for her hernia surgery--it had been steadily, almost rapidly rising at times since we had seen her first labs back in October.  Then, something strange happened in May.  For the first time, her bilirubin had dropped.  It was enough to make her PELD score drop, although the change wasn't significant.  They gave her a new PELD score of 12.

(They are required to re-PELD every 3 months.  We get labs done every 6 weeks--if the numbers are significantly worse, they will request a new ["better"/worse] PELD score.  If the labs numbers are the same or better, they just wait it out until they get worse or until they are required to re-score.)

So I came home in May a little bummed.  Why had we rushed with all that lightning speed back in the fall only to be sitting around six months later, stable and waiting?  Liver failure is so weird.  Compared to you or me, Brooklyn is a very sick little girl.  Compared to other kiddos in liver failure, she's not sick enough to take top priority.  It feels so strange to be hoping for a "better"/worse score.  Like, if she's going to be very sick, let's be very very sick really quickly for as short a time as possible so that she can get her transplant and be healed!  I know, this transplant world is so weird.

The doctors had also talked to me about something else.  They were questioning her biliary atresia diagnosis.  They wanted to take extra labs to do some genetic testing for a rare condition called Alagille Syndrome.  When they had done a CT scan immediately after her hernia surgery, they had found some abnormalities that had peaked their interest.  They would send in the genetic test and wait for the results--if it came back positive, that gave some answers.  If it came back negative, it didn't necessarily rule out the new diagnosis--the genetic test only tested certain markers.

I felt fear and discouragement when we arrived home in Memphis...what if this changed everything?  What if a whole new can of worms or just a new can of uncertainty was opening?  And Googling Alagille Syndrome (which is what every smart person should do when faced with a new diagnosis, right?) only made me feel worse.  Biliary atresia is congenital, meaning Brooklyn was born with it--it wasn't passed on to her and she wouldn't pass it on.  But Alagille Syndrome is a genetic disease...it would change the story about what had been and what will be...

A week later, Rhet finished up preschool, we moved to our new house, and we had plenty on our minds to keep us busy for awhile.  (Including Brooklyn's 6-month post-placement report...can you believe she has been home for HALF A YEAR?!  I am in awe.  And 2015 is and always will be a blur.)

In July, the results of the test came back.  Brooklyn tested positive for Alagille Syndrome.  They were officially dropping biliary atresia from her diagnosis and changing it to ALGS.

It changed everything, but it also changed nothing.

Alagille Syndrome can include other complications--some children's symptoms are so severe that they are inoperable or need multiple organ transplants.  Because Brooklyn had already received a heart echo, an abdominal ultrasound, and a CT scan as part of her transplant evaluation, the doctors had already determined that she had no additional complications.  Her kidneys and heart look fine, and this condition is not degenerative, meaning these complications won't arise later in life.  Her symptoms/presentation for ALGS include: some narrowing in her vascular system (not enough to cause heart complications), a few of her vertebrae are butterfly-shaped (super weird, but that is one of the symptoms), she has a broad forehead and triangular face shape (common in ALGS patients), she is small for her age (we had always attributed this to liver failure, but because she has ALGS, she will probably always be teeny tiny for her age), and of course, the bile flow in her liver is decreased/blocked.  The only symptom needing correction is the liver failure, which can be fixed with transplantation.

So, things are different, but not really.  She has a new diagnosis, but her current condition of health, the way they'll monitor it, and the way to fix it are essentially all the same.  It was all earth-moving and anticlimactic at the same time, if that makes sense.

The Alagille diagnosis clicked a puzzle piece in place.  We may never have known if she had biliary atresia--her liver is too damaged to tell with a biopsy or during transplant at this point.  But with a positive genetic test result, that mystery has been solved.  We know definitively what has caused the liver failure.  And we also know why we're sitting around in July without the same rate of rapid decline that was happening October - February.  Alagille Syndrome patients have no anticipated rhythm or pattern to their liver failure.  While biliary atresia patients tend to decline steadily and/or rapidly at different times, ALGS patients can plateau, decline steadily, plateau, decline rapidly, etc all at varying times and speeds.  This plateau that Brooklyn seemed to have hit this past spring was just that.  And it explains why she had seemed to be declining so rapidly in the fall--because she was.  She is still in liver failure and still needs a transplant, but her decline has transitioned into a period of stability for an unknown length of time.  (It won't be years, though--her body wouldn't be able to sustain that--it will be more like an unknown period of months.)  At some point, the decline will pick up again...steadily or rapidly, we can only wait and see.

So, that's the latest.  And we're about to have more of the latest after our next GI clinic next Tuesday.

Please pray for our family as we continue to deal with change and transition in many areas.

Pray for our trip to Chicago, that we can experience sweet time together as a family--we are really thirsting for some happy memories to be made with all four of us together.

Pray for Rhet's first day of Kindergarten!  I want her to feel brave and joyful and confident.  Actually, I want to feel that way too.  ;)

Pray for Brooklyn's liver...and for her new liver, however and whenever we will receive it.  It is a terrible thing to be waiting on that life-saving gift.  Honestly, I have trouble wrapping my mind around it and have to push that part away from my thoughts most days.

Pray for God to prepare our hearts and schedules and finances and physical endurance/health for liver transplant time, whenever it will be.

And thank you, friends.  We couldn't do this life without you.  We have chosen to follow the Lord in faith, and you have lifted us up and encouraged us every step of the way, even as we've felt tired and discouraged.  God is for us!  He is with us!  And He is using you to remind us...

Wednesday, July 01, 2015

Goodbye, Hello

We will call this place our home,
The dirt in which our roots may grow.
Though the storms will push and pull,
We will call this place our home.

We’ll tell our stories on these walls.
Every year, measure how tall.
And just like a work of art,
We’ll tell our stories on these walls.

Let the years we’re here be kind, be kind.
Settle our bones like wood over time, over time.
Give us bread, give us salt, give us wine.

A little broken, a little new.
We are the impact and the glue.
Capable of more than we know,
We call this fixer upper home.

With each year, our color fades.
Slowly, our paint chips away.
But we will find the strength
And the nerve it takes
To repaint and repaint and repaint every day.

Let the years we’re here be kind, be kind.
Settle our bones like wood over time, over time.
Give us bread, give us salt, give us wine.
Let the years we’re here be kind, be kind.
Settle our bones like wood over time, over time.
Give us bread, give us salt, give us wine.
Give us bread, give us salt, give us wine.

Smaller than dust on this map
Lies the greatest thing we have:
The dirt in which our roots may grow
And the right to call it home.

- "North" // Sleeping at Last

At the end of May, we said goodbye to our little house on Saint Nick.  It was the house where I began adulthood, where I began this blog!  It was the house I brought a husband home to, we brought our first baby home to, we brought our second baby home to.  So many happy memories made and lessons learned there.

And we began a new chapter in a different part of our same great city.  We love our new house, with its old bones and history and new paint and wide open spaces.  We love living in midtown with all its great food and its proximity to many of our favorite places.  We love a fresh start, with promise and hope and blank pages.

We've been "cracking the spine" of this new book this past month.  Turning the first page.  Thanks for being patient as we deal with the boxes and stress and change and newness.  (Because we all know "turning the first page" is a pretty way of saying you're unpacking box after box and figuring out what tripped the breaker for the air conditioner and eating boxed macaroni & cheese until you figure out how to afford curtains for every room! [laugh/cry emoji])

We're still waiting waiting waiting on that ever-important call.  I'll post more about Brooklyn soon.  In the meantime, thanks for your continued prayers for Brooklyn and this crazy 2015!

Photo courtesy of the one-and-only Stephen Jerkins

Tuesday, May 05, 2015

How You Can Be Awesome Today...

We've been making lists of what we need to do the second we get "the call."  And I finally read the "Liver Transplantation" guide given to us at the hospital--a medium-sized booklet full of info of the before's and after's of this new normal we're living in.  Oh man, do we have a lot of plates spinning right now, and while I'm eager for Brooklyn's health to be fully restored, I'm also able to be thankful on the days we don't get that call, because there's lots of TCB (taking care of business) to be done.

One of those pieces of business to be TC-ed is rallying our prayer warriors and sucker-punching the Enemy's attack.  Here's where you come in...

Would you commit to praying a verse of Scripture over us every day for 30 days starting with the day we receive "the call?"

1. Use an index cards or cut a regular sheet of paper in half (nothing larger please).
2. Write/type/paint/doodle/chicken scratch your committed verse onto both pieces of paper/cardstock.
3. Mail it to us in Chicago:
Brooklyn Faris
c/o April Clark
3539 W. Carroll Ave #2
Chicago, IL 60624

The verse can be primarily for/about Brooklyn (her health, her surgery, her life) or primarily for/about Nick & me (our family, fear & courage, strength, faith).  Whatever it is, when we say "Go!" you pray that verse over us every day for 30 days.  Write it on your calendar or set an alert in your phone.  And we will post your verse in Brooklyn's hospital room as a banner of prayer and protection and encouragement over our family.

We need this, friends.  God's hand through your prayers and encouragement have carried us through.  We know we can choose faith and courage over despair when you're standing with us.

Go ahead and send your verse NOW, because we have no idea when that call will come (it could be...now!  Or...now!  Well, I guess not, but it could also be...now!)  ;)  and we want your Scripture prayers to have gone before us, ready and waiting.

Feel free to send this to anyone and everyone--strangers-turned-new-friends are our favorite kinds of friends!  And thank you in advance.  You guys are clutch.  

Sunday, April 12, 2015

Telling the Story to Ourselves

I feel sad and overwhelmed today as the sunshine streams through my window on this pretty spring day.  And that's the way it is sometimes.

We'd like to think change is just good and not accompanied with stress and exhaustion and regression.  That healing is only triumph and will erase all trauma.  That progress means that you'll never get stuck again.  That you won't feel gloomy anymore once the sun is shining again.  

Last spring, we went through an intensive training program in order to become certified to teach a set of parenting strategies that we feel passionate about to others.  The program is called "Empowered to Connect," (ETC) and it was developed to support parents & caregivers of children "from hard places."  That phrase "from hard places," gets thrown around a lot as a popular phrase in adoption circles now, but in essence, it refers to a child who has experienced trauma of any form.  (That can include children who were involved in high risk/high stress pregnancies/births, spent time in the NICU/hospital, experienced childhood illnesses, or experienced any kind of traumatic event in their life.  That includes many more children than those who came to their families through adoption!)

We have loved and used this material since it was first presented to us when we were preparing to become adoptive parents.  We've read Dr. Karyn Purvis's book, "The Connected Child," multiple times, attended the Empowered to Connect Conference, participated in both the pre-adoptive ETC course as well as the post-adoptive parent Connect course, and then we were trained to be trainers.  We have had lots and lots and lots of practice with this material over the last six years.

And yet, I felt a heaviness yesterday as I attended a simulcast here in Memphis of the annual ETC conference.  No matter how much training I've received, how many books I've read, how many seminars I use to remind myself of the principals that promote connection and healing with my children...it's still really hard.  It's HARD.  Because trauma and pain and feelings and growing and BEING A PERSON is hard.  For everyone.

We've had a big year.  We're having a big year.  I joked the other day (in the context of forgetting to take a family picture on Easter) that "I don't do 2015."  So many plates are spinning and they're overwhelming and complex, and everything else has been pared down to the bare bones.  I didn't do my "12 Days of Christmas" this year or put ornaments on the tree or travel for New Year's or make Nick's chocolate peanut butter layer cake or compile Rhet's annual birthday video or do anything out of the ordinary for our 9th anniversary or hide eggs to hunt in the back yard.  We are keeping it so so SO simple.

And we're all feeling it, those big changes.  I've been honest with friends about how we're dealing with transition and regression with our kiddos.  But it struck me yesterday as I tried to hold the guilt and shame at bay while listening to experts talk about these parenting principles and values that I feel so passionate about...WE'VE regressed too.  Stress just does that.  It knocks you off your feet...pushes you back a couple steps.  I'm not peeing in my pants, but I might as well be...because I sure am regressing back to that yelling voice and angry eyes and lecture, lecture, lecturing...and so much despair that what I'm doing doesn't matter, that it's not making a difference, that I'm just spinning my wheels...

This is why the story matters.  This is why His Story matters.  We struggle to connect and and we repair our mistakes and we do our best, and then we do it all again the next day.  And through that mess, He is healing, restoring, redeeming.  WE are the WEAK ONES.  He is the strength.  We lose sight of the progress, and we feel like everything is sliding backwards, but He is writing the story.  And when we tell it, we see where we've come from and how we got here.

This whole time I've been thinking, "I need to tell them Brooklyn's story.  I need to tell them how God is at work in the world and in her life.  I need to tell them for His glory.  I need to tell them to strengthen their hearts and faith."  But this whole time...I've needed it.  I've been telling the story to myself.

He is not finished.  There is more to come.  And some of it will be so freaking hard.  And some if will be blindingly glorious.  But I'm going to keep telling this story...her story...His Story...to myself.  Because it's just what I need to keep doing this today. 

Thursday, April 09, 2015

Those Sweet Cheeks

I'm interrupting the crazy stories of how God worked to write a little "everyday" post of what life is like on a daily basis with sweet B.  Hoping I can satisfy some curiosity as well as educate a little on how best to pray for us and treat Brooklyn when you see us out and about.

First, I can't remember if I ever told you what "biliary atresia" is!  So for those of you who haven't asked Google yet, my most simple explanation is this: Everyone has ducts that lead from the liver to the small intestine.  The liver produces bile, and the ducts remove it from the liver and dump it into the small intestine.  This is what makes your poop brown.  (Gross, I know, but now you have your fun poop fact for the day!  I know you needed one of those, right?)  A person with biliary atresia (BILL-ee-airy uh-TREE-shuh) was born with ducts that are blocked, so the bile can't make it out of the liver.  Bile is toxic and causes liver damage and ultimately liver failure.  Sometimes a surgery called the "Kasai procedure" can be done, which reroutes the flow of bile and extends the life of the liver, but it needs to be done early enough in life (by 3 months old) to keep the liver from sustaining significant damage.  Without a successful Kasai procedure, a child cannot typically live past the age of 2 years old with biliary atresia.

So, there's my non-medical and probably not 100% accurate explanation.  But now you know.  {Cue shooting star with rainbow.}  In terms of Brooklyn's little body, she was given the Kasai procedure at around 4 months old, which is too late.  Her liver was already damaged and thus the older she got, the more that liver started shutting down.  We found out about her when she was 9 months old.  She weighed 10 lbs.  (The liver processes certain fat-soluble vitamins like A, D, E, & K, and they're needed for proper nutrition.  Since children with liver damage/failure cannot process those vitamins, they often have trouble putting on weight and getting the proper nutrition to help their little bodies grow.)  She had a big "buddha belly," caused by acites (ah-SITE-ees, which means there is fluid in her belly as a complication of liver failure), but her chest, arms/hands, legs/feet, and little booty were so, so tiny.  The doctors in China gave her a 50% chance of living 4-6 months.

PS: We are now at 6 months from that original prognosis!  {High five!}

Since October, Brooklyn has made it up to 15 lbs and grown 1 inch taller!  Yeah, girl!  And her development in all areas is just flourishing.  We're so proud of our Little Biscuit!  Lots of people (including us) are so surprised when they first meet Brooklyn, because she is tinier in person than she appears in pictures.  She has these sweet chubby cheeks and that big buddha belly, and they don't realize that her feet don't even fit in 3-6 month-size shoes!

(Her latest trick: saying "cheeeeeese!")
So, a day in the life with Brooklyn looks like this:

7-8a - She wakes up and we give her 3 vitamin supplements: a water-soluble combination of vitamins A, D, E, & K, a water-soluble version of vitamin D, and a water-soluble version of vitamin E.

When she first came home, she was gagging on pureed foods, but now she can feed herself finger foods and loves being fed with a fork or spoon.

More poop talk: B's poop is grey/white because it doesn't have bile in it.  Don't worry, it still stinks to high heaven.  ;)

9:30a - Brooklyn drinks a bottle of formula and usually takes a morning nap.
Normally, a 15-month old wouldn't still be drinking formula, but B drinks Pregestimil, which is specially formulated to be easier to digest for babies with fat-soluble digestion issues.  It's also $40/can at retail cost.  {Cha-ching!}  Totally worth it for the weight she's able to continue putting on!

11a - B wakes up, and we eat lunch and play.  She loves pulling up and cruising, she'll obsess over anything paper or plastic (or iPhone--ha), and she adores music.  (If she meets you, she will ask via hand motions if you know "The Itsy Bitsy Spider.")

1-2p - Time for another bottle and another nap!  I call the two-nap phase "nap jail" because you can only really get out of the house between those two naps.  But look at all the napping, uh I mean blogging, oops I mean housework I can get done!

2:30-4p -B wakes up and it's time for more playing!  We usually go pick up Rhet from school around this time.

5p - Dinner time for everyone--Brooklyn eats little bites of what everyone else is having.  Plus Annie's Cheddar Bunnies: her one true love.  ;)

6p- Brooklyn loves splashing in the water at bathtime.  She drinks one more bottle before going to bed around 7p.  I usually give her Benadryl, because hightened amounts of bilirubin (a result of bile) in the blood make your skin jaundiced (as you can see) and itchy.  (B's bili level is up to 23.  A normal level is 0.)  Especially when she's tired, Brooklyn scratches and scratches, and she will often scratch herself so much that she starts bleeding somewhere.  The Benadryl often helps alleviate some of the itchiness.  And thank you Old Navy, for making the only pj's B wears these days because the sleeves fold over and cover her hands in her little 6-9 month jammies!

10:30p - We wake B up to drink a bottle right before we go to bed.

2-4p - On a "good" night, B usually only wakes us up once to drink a bottle in the middle of the night.  (When we first came home, we were on "ever hour/every 2 hours" duty.  {Zzzzzzz})

Also, every minute of every day, have our phone turned on and nearby, because once we get "the call," we'll drop everything and follow our "Liver Call List."  We have an hour to return the call if we miss it, and we have 24 hours to get there after we receive it.

After transplant, Brooklyn will be in-patient at the hospital for about 2 weeks (barring complications) and then we'll stay in the Chicago area for another 2 weeks (totaling a month) so they can keep an eye on her.  Then, we'll come back every week for a month for check-ups, then every other week for a couple months, and then eventually once a month until we reach the one year anniversary of the transplant.  So far, we love Chicago, and we love Lurie, so we hope it stays that way!

One more thing: before transplant, we have to be diligent to keep Brooklyn from getting sick, because she can't be cleared for surgery if she is.  (And because transplants are so time-sensitive, that could cost us a liver!)  Post-transplant, she'll be on immune-supressing medications to keep her body from rejecting her liver, so it will be very very easy for her to catch illness.  That being said, we are being trying to be super vigilant about not exposing her to illness and germs (short of putting her in a bubble).  EVERYONE wants to touch those sweet cheeks of hers, but we're asking everyone (kids AND adults) not to touch her face or tiny little hands.  Thanks for using your super-human willpower!  (Because you guys, she's just so dang adorable!)

Thanks for praying for her and for us.  God is so good to us, and we are enjoying this sweet post-home/pre-liver time to grow our attachments nice and strong.  :)

Adopting Rhet: Click on the timeline above to read more