Liver check

Keller rocked her labs this month.  It has been over a year since we saw her liver doc… And she is doing awesome. The doctors were very happy with her progress and growth. Her liver is smaller than ever and they can no longer feel any enlargement of the spleen. They decided to take her off of her medication! Praise God! 

She will have an ultrasound and more lab work in May. But, we are so thankful that she continues to improve!

liver check

We went to Children's today for Keller's liver check. She did awesome and only fussed a little bit during her blood draw. She is so brave. The liver team couldn't believe how much KG had grown. They (as always) treated her like she was their favorite patient (because let's be honest) and then got spoiled with hugs and kisses. We are so blessed to have such a wonderful team.

To make the day wen brighter we found out Keller's levels were the bet they have ever been. While her liver enzymes (ALT and AST) are still abnormal and considered high, they are down to right around 100. Which is low for KG!  Her docs were so happy to see this. Her liver and spleen remain enlarged, but we don't have to have another ultrasound and blood draw for six months. This is great news!  The doc even mentioned that if she continues to be stable, we may go to annual appointments!  Praise God!

 

#liverkid

we are beginning to get in a routine/rhythm being a family with liverkid.  we took kg to children's hospital last week for labs/GI appointment.  dr. reyes and dr. palomo were happy to see how well keller was doing.  although her liver enzymes were still high...they are trending down.  one of the best pieces of news came this.  we were waiting on a couple more results...specifically her AFP level.  when keller was first diagnosed her AFP was in the THOUSANDS.  today, our favorite nurse Sarah called to tell us her AFP is only a FOUR.  yes, that's right.  single digit FOUR. AFP is a protein that is present in patients with liver disease...so given the number is so low...we are singing our praises!

prognosis has not changed.  we will return for another checkup in february.  after that, if things remain stable we may only have to have appointments every six months or so.  she will continue on her meds (ursodiol) and we will continue to live a normal life.

she was a real trooper at the hospital. it is always a long day, but having our day interrupted by this guy is a real treat!  thankful uncle jason has been working at children's so many different days we have visited!


















keller grace.  we wouldn't change anything about you.  we have experienced difficult days and sleepless nights worrying about you - - but you are perfect.  just the way you are.  we are thankful for your boisterous, loud and charismatic way.  we love your "i can do anything i want" attitude.  i love your snuggles and slobbery kisses.  but most of all, i love knowing you are mine.  forever and ever. love, mommy.

biopsy and results

Last Tuesday we took KG to children's to have a liver biopsy. We arrived bright and early and got some good play time in (for 6am).

playing with the mask before procedure

At 7am they let us take her back to the procedure room and the anesthesiologist put her under anesthesia before we left. 20 minutes later, Dr. Palomo came to our room to let us know all went well. Keller was back in my arms by 7:35 after a brief stop in recovery. She was very fussy at first, but slowly got back to her smiley self. She didn't nap for one second the entire 5 hours we stayed at children's. Needless to say, she took a great nap that afternoon and was completely normal that evening. Nothing can slow her down!

This afternoon, Dr. Palomo called us with the results from the biopsy. They ruled out autoimmune hepatitis (Praise God). In fact, they believe that her inflamed liver is simply due to alpha-1. They have prescribed a medicine called ursodial. While she does have some mild fibrosis (which can lead to cirrhosis of the liver) they say that it can be reversed given how the liver fights and regenerates. We are thankful that her liver has handled being alpha-1 antitrypsin deficient so far and pray that the damage and inflammation stops. 

Dr. Palomo will continue to see her every 4-6 months but he did say we are "far far far far away from needing a transplant consult."  We hope and pray that this is something that will never happen yet trust God's hand and direction. 

We again thank you for your prayers and support over the past year. Given that this condition is chronic we still have a journey ahead of us. **welcome to parenting :)

Love you all!

snuggling after procedure

"resting" with daddy

we loved our nurse, linda!  she took great care of KG!

going on a walk around the C.A.R.E.S unit with aunt kinsey

headed home!

heavy heart

on tuesday we took keller up to children's hospital for her checkup.  we always get a little anxious before we go, and this day was no different.  we did the normal routine...went and had her blood drawn (horrible experience every time...but luckily she recovers quickly)...and then headed up to our appointment with our GI docs.

dr. reyes came in and updated us that her liver enzymes had increased.  in fact, they were higher than they have ever been.  she consulted with dr. palomo (our attending GI doc) and between the two of them they recommended that we have a liver biopsy.  they want to take a closer look at the liver tissue to see what is happening.

so, now we wait.  on tuesday we will make a trip up to omaha again to have the procedure.  while the biopsy itself is pretty quick, it will take a few hours of observation afterward to make sure all is okay.  it's never easy watching your children go through things like this...and our hearts are weary.  i know we are surrounded by the best medical team and they will take great care of our sweet baby.  it will take a couple weeks to get the results.

it has been almost one year since we were admitted to children's hospital.  and for that entire year, we have known she has alpha-1 antitrypsin deficiency.  we knew the day would come that they would request a biopsy given this is a chronic disorder.  but, i was shocked to hear that her numbers had increased.  our sweet keller grace is happy.  she bounces all over the place.  she's loud, charismatic, smiley, curious, and appears to be extremely healthy.  you would never know that her liver is aggravated.

we covet your prayers.  please pray that erik and i can get rest.  that our families can get rest.  that evie can be protected from the emotional roller coaster.  please pray for dr. palomo and our team of doctors as they try to come up with more answers.  but ultimately, i beg you to pray the Lord wraps his arms around keller grace and protect her body and liver from any additional disease or damage.

we are humbled.  every time we walk through the doors of children's hospital, we watch parents with children who go through daily struggles that we cannot even imagine.  we are blessed.  we know that the Lord loves our children and will care for them.  but that doesn't change the fact that we feel weary and distraught as we wait.

Lord Jesus, please stick with us.  i beg you.

April GI update

We spent our day at Children's today. Keller is doing great. While some of her labs are a tad higher than normal, our doctor was feeling great. We don't return again until the beginning of July!

And while we were at Children's Hospital....Evie was at Children's museum with Peyton!

Ultrasound results

Last Friday we took KG to Children's for an ultrasound. She did awesome! It took about an hour and she was so patient considering she was hungry and had to sit still (which seems like an impossible task for an 8 month old!).

Much to our relief, the results came back normal. The liver tissue has not been damaged. This is incredibly great news as this is a major concern for people with alpha-1 antitrypsin deficiency (1 in 20 children with alpha -1 deficiency need a liver transplant due to liver damage, disease, or cirrhosis). Because the alpha-1 protein virtually protects the liver and lungs, not having enough (deficient) of these little warriors (alpha-1) in her liver can cause damage to the tissues (I hope you are enjoying my parenthetical commentary). Since she showed symptoms so early in life, it has caused the doctors to be extra cautious and mindful on the condition of her liver (which this mama is thankful for). Things continue to improve and they think her numbers will continue to trend downward.

We will take Keller for labs in mid-February but we don't need to return to see her GI docs until April (I think we might miss seeing them! We love her doctors! However I'm not complaining!) Praise God!

So today we can breathe easier and thank God for His continued protection over my sweet babe.

GI Checkup

It's been three-ish months since we had an appointment at Children's. We made the trip up to Omaha to meet with KG's GI specialists. As fate may have it, Uncle Jason, who's in his third year of med school, was doing a rotation at Children's. Even better...he was in GI. And even more exciting he was working with OUR doctor!!! So we got to see him for lunch before KG's appointment AND he sat in during our appointment!! So needless to say...we had extra special care today.



KG's numbers overall continue to improve however the liver enzymes are still a bit high. Her doctors are extremely happy with her growth but want to be aware of everything. So since it has been almost six months since she had an ultrasound they want to get another look at her liver and spleen. So next week we will make our way back up to Omaha for that appointment. We are remaining positive and believe that happy baby also means healthy liver.

I will update when we get the results her ultrasound. Surprisingly I'm feeling calm about it all. But as always we covet your prayers!!!

KG labs

Last week the infectious disease doc called us to give us the results of a another test they ran to learn more about CMV she contracted. One of their concerns was to try to figure out whether she contracted the virus in utero or after she was born. There can be severe complications (including vision and hearing loss) if she contracted the virus in utero.  However, the ID doc found that the counts for the virus were extremely low and we could therefore rule out congenital CMV.  Thank goodness!

However, the virus + alpha-1 together were a bad combo and caused the inflammation of the liver (hepatitis).  Yesterday, we went for more labs and today I got a call from the GI doc with the results. All good news!   Keller's liver enzymes, bilirubin, and a few other counts have all dropped! They still are a bit high but this is still really positive news!  We were anticipating good news because KG is looking so much better!  When we were in the hospital one of our doctors actually showed me how to feel for the liver.  And even though I'm not a professional, I can tell that the liver has shrunk in size significantly!  The whites of her eyes are almost completely white and her little belly is not nearly as distended. She's eating great.  Sleeping well and pooping like a champ!  Go KG! Go!

We will go see the GI doc again in a couple weeks for a checkup and more labs.  So one step in the right direction of healing from this battle with hepatitis.  We are thankful for our doctors!  We can totally feel your prayers and are very thankful!  We will update you all again when we know more in a few weeks.  So, today...we celebrate more good news.  And we give thanks for this beautiful baby girl.

say what?

alpha-1 antitrypsin deficiency.

huh?  excuse me?  say that one more time please.

alpha-1 antitrypsin deficiency.

what the hell is that?

"The alpha-1 antitrypsin (AAT) protein protects the body, especially fragile lung/liver tissues, from the damaging effects of a powerful enzyme called neutrophil elastase that is released from white blood cells. In AAT deficiency, a genetic mutation reduces levels of the protective protein in the bloodstream. AAT deficiency can lead to chronic obstructive pulmonary disease (COPD), specifically emphysema, and liver disease. A small proportion of children born with AAT deficiency are jaundiced in the early days and months of life. Between 15% and 19% of adults with AAT deficiency develop cirrhosis of the liver after age 50."

you have got to be kidding me right?  did you say genetic disorder?  you told me she JUST had a virus.  how can we be talking about metabolic problems?

deep breaths...  Lord, are you there?  what the hell are you trying to do to me?  can you hear me?  i'm so freaking mad you at you right now.  how could you do this to my sweet baby girl?

lots of tears.  lots of reading about this crazy deficiency.  lots of angry talks with God.  lots of tears.  lots of encouraging words and notes from family and friends.  lots of tears.

and now the good news.  

her liver enzyme levels are decreasing.  she seems to be healing.  she won't necessarily have ANY of the scary symptoms that we have had to read/talk about...but she could.  but if she does get liver damage/disease or cirrhosis...i know we have the best doctors who will recommend the correct route.  if that is a liver transplant, praise Jesus we are an hour away from one of the best transplant hospitals in the world.  praise Jesus that our doctors are taking this seriously.

she just happened to contract the CMV virus as well...which showed its ugly face by inflaming her little liver.  and because of the deficiency, it's slower to heal the liver.  so what now?  we will take KG for labwork every two weeks to continue to monitor the enzyme levels.  as long as they keep dropping we are on the right track.  every two-three months we will go see the GI specialist at Children's.  if the enzymes don't improve...we will talk about a liver biopsy to get a better look at the liver tissue to see what's going on.  then we reassess.  

for now, the whites of her eyes are no longer as yellow.  her skin is pinking up.  her spleen has shrunk in size.  and all of her levels have come down some.  just keep on dropping!!!!

so today, our daughter has a protein deficiency.  she does not have a disease.

tomorrow, i'm going to wake up and kiss her sweet, chubby cheeks and thank the Lord for my perfect baby.

the day after that i will probably yell at God a little bit more.

in two weeks, we will get labs and i will want to punch the phlebotomist in the face for sticking that needle into her little arms.

and right now, i'm going to fix my eyes on Jesus and beg Him to continue to heal my daughter from this virus and protect her little body from any further damage or disease.  please continue to be prayer warriors alongside us.

diagnosis

Well we found out that Keller has the CMV virus. Although she seems to be doing great, the doctors have scheduled another appointment for us to meet with one of the infectious disease doctors. There could be side effects including vision and hearing loss from this virus so they want to keep a careful eye on her. So in addition to the other appointments we will make another trip to Omaha to meet with another specialist.

We continue to beg for your prayers. Specifically that the Lord would continue to protect Keller's body. In particular, her ears and eyes.

Tomorrow is our appointment with our pediatrician which will include more labs.

Please also keep Erik in your prayers this week as he has officially opened his restaurant, Honest Abe's. You can check them out at http://eatabes.com

dearest family & friends

As we begin to find some normalcy I have been trying to process all that has happened over the past week. I am convinced that without our family and friends love, support, and especially prayers we would not be where we are today.

Four days ago we had a doctor tell us he had no idea what was wrong with out little girl. He said she may have a wide range of things including biliary atresia (which would likely require a liver transplant) or a fatal metabolic disorder.

Both of those have been ruled out and her body has begun to heal. And for that I thank the Lord and all of our for praying for healing.

I begin to question why this had to happen. Keller is fine. No medical intervention was needed whatsoever. So what was the point of it all? Why did my sweet baby have to go through that? God obviously had a message he wanted to send to us. So we try to hear his voice through the experience.

I can not adequately thank people enough. But here's a start.

Ashley. There are no words to express what your support this week meant. I called you hysterical begging for help getting our bags packed. You dropped everything for us. Your presence in the hospital with us...having you there as we fell broken in disbelief. Your words of encouragement. Your relentless compassion. We are blessed. I couldn't have done it without you. And Mark, thanks for supporting Ashley to drop everything to be with us. We know it was a sacrifice for both of you and we could feel your love through that. Evie and Keller are sure blessed to have you two.

Gus & Janie. I don't tell you enough how thankful I am to have you as my in laws. Janie, you were by my side (literally) from the very first day of this craziness. Thank you for how much you love us. Especially your granddaughters. Thank you for taking Evie no questions asked. Thank you for driving to Omaha every night. Thank you for being the unstoppable prayer warriors that you are.

Mom & Dad. Thank you for packing up and leaving right away no questions asked. I appreciate that I can count on you no matter what to come through for us. I know that you will always be there for me and your granddaughters and I'm so glad that I have you to help whenever I need you. Love you to pieces.

Melanie. No explanation needed. I love you.

Alexis, Marcia, Amelia. Thank you for playing words with friends. I needed the distraction and am grateful for it and so thankful for you and such a simple game that helped numb the mind.

Nicole & Andrew. Thanks for loving on Evie while we were gone. Your empathy was beyond comforting. Your text messages lifted us up and were sent when we most needed them.

Gabe & Emily. Thank you for handling the restaurant without Erik. And for not making him feel any pressure for being away. We knew we could count on you to handle it and you did great. Thanks for adamantly praying and showing your support in so many ways.

Jason & Kinsey. Thank you for being there for us. Jason having you at the hospital to help me try to digest all the medical lingo was so comforting. Kinsey, Evie was so stoked to play with you every night. So thankful you were in Lincoln this week to help her during such a confusing time.

All of our family & friends who sent messages, texts, Facebook wall posts, and emails. I can't name everyone individually because this post would be ridiculously long. God used your prayers and words to heal our daughter and calm us during the most horrendous week of our lives. Some of which were so perfectly timed during our lowest lows. I remember waking in the middle of the night crying as I nursed KG. And reading all of your messages. Somehow one of you would always say just what i needed to hear right when i needed to hear it. You helped me to breathe and have hope. Thank you.

Erik. I am so in love with you and so glad you are the father of my children. We are forever changed by this. Thank you for holding me tight as I sobbed. Thank you for ignoring your work responsibilities and giving 150% to your family when we needed you. I know I can always depend on you. We will hold KG and Evie tighter. We will love our loved ones and each other harder. We will be more grace filled toward one another. May we look back on this experience and be reminded of how we desperately had to cling to Jesus. And that He came through for us. I'm so glad I get to do life with you. I only pray that as we dive back into our chaotic lives that we learn to appreciate one another more. And be reminded how precious life is. I love you.

My God How Great Thou Art.

day 5: home

Home. Sigh. Thank you Jesus. Waking up in our own home was such a relief this morning. I cannot believe how emotionally and physically drained I am. I can't imagine what little miss K is feeling. Poor sweet baby.

We plan to spend the day lounging around and watching the Olympics. Much needed rest for all of us.

So what now? I plan to take it pretty easy and keep Keller home over the next few days. We have an appointment with our pediatrician on Tuesday afternoon. KG will get labs to check her bilirubin levels. Although its already obvious that they are dropping because her skin and eyes are looking so much less yellow.

Assuming that all is good and her levels continue to drop we won't see another doctor until aug 7th. That day she has a follow up appointment with the GI specialist at Children's. By that time we should also have the results from all of the tests and hopefully will have an answer. And I guess if they dont give us answers...as long as she is 100%!!

So thankful to be home. Your continued prayers for healing are appreciated!

day 3: more tests

we woke up this morning and took KG to have the HIDA scan.  i never knew what nuclear medicine was until today.  they gave KG an IV and then injected some sort of radioactive material into her bloodstream.  insane.

it took about an hour or so for the machine to take the various images of her organs.  she was calm and content just sucking on a paci.  she had a few fussy moments, but overall she did awesome.  the tech told me that her "plumbing" appeared to be in tact.  which gave us huge hope.

we had to go back at about 2:30 this afternoon for another scan to get a few more pictures.  at about 4pm one of the dr's came in to talk to us.  she share some great news with us expressing that KG does NOT have biliary atresia.  PRAISE GOD.  the scan also showed us that her liver is taking and excreting bile the way it is supposed to...but the "plumbing" is just a little slow.

the slowness is likely caused by some sort of viral infection.  the infection is causing the swelling of the liver and spleen.  this also is the reason so many of the enzymes are high, iron is high, etc.  the liver just isn't 100%.

an hour or so later, the GI team came in and shared the same good news.  at this point, we still don't know what virus.  and they want to figure that out.  so the game plan, as of now, is to run a series of tests.  KG will need to have some more blood drawn and they will send it out for more testing.  not sure exactly when/if we will be discharged...but they did mention we don't necessarily have to wait for results here in the hospital because her vitals have been good.  we just need the other numbers to drop down and for her yellow color to GO AWAY.

so again...we wait.  but today, we are refreshed and renewed and reminded of God's perfect provision and love.  we are thankful for our sweet keller grace and God's protection over her.

i have to share one email with all of you that i received from my best friend's dad.  i was so broken and this note from him helped to restore my faith...

Just a note to tell you how sorry I am about the uncertainties with Keller right now. I know that Erik, Evie and Keller mean everything to you; they are your life. 

And now, one of them is having some very real struggles. 

I hope this next sentence doesn't sound preachy or sound like there is even the slightest hint of criticism in it (there is no criticism here). You are a person who is used to being in charge; if there is a problem, you fix it. Now you are in a situation where you cannot fix the problem. For those of us who are problem solvers, this is a difficult place to be.

The God who knit Keller together and gave her to you knew what He was doing then; and, He knows what He is doing now. You may not know what is going on; but He does.
 
You are not entrusting her to the doctor's hands, you are entrusting her to God's hands. His loving hands are holding her. His loving hands are holding all four of you. His grace will sustain you - always remember that.
After all, before she was even born, God gave you the wisdom to place Grace in her name.

this is not the only note i received.  i have to say a huge thank you to all of you.  the text messages of encouragement, the emails of empathy, the facebook posts of support...words can not describe how grateful we have been for all the support we have received.  many of you i haven't talked to in years.  you have helped us to keep breathing and hoping.

day 2: update

today has been a roller coaster of emotions.  we are still left with uncertainty and lots of unknowns.  the ultrasound did not show any anatomical abnormalities (which would include biliary atresia) however, they want to do a HIDA scan tomorrow morning to rule that out for sure.  blood work is leaving a lot of question marks...but we are blessed with a wonderful doctor who clearly knows what he is doing.  the liver specialist here at Children's is nationally known and recognized...so KG is receiving the best medical care possible.  we are thankful for all of the love, support and prayers as we continue to wait and take things one day at a time.

what we don't know...

• what the diagnosis is
• what the long term/short term effects are
• how long we will be here

what we do know...

• keller is still acting like her normal self
• liver enzymes are slightly high
• her liver, gall bladder, etc are still working
• she is pooping normal and healthy looking stools
• bilirubin (both conjugated and unconjugated) levels are high
• she is eating like a champ
• iron and ferritin levels are high
• she is still smiley and content
• her liver and spleen are enlarged...but so is her smile

what you can pray for....

• that the scan would give us answers tomorrow
• that keller continues to eat, sleep, and poop normally
• that the doctors wisdom so that they can continue to narrow down the list of possibilities and find a diagnoses
• that erik and i can get some rest
• that we can feel a sense of hope with so many unanswered questions
• for evie as she is confused and concerned

what we are are certain of...

• no name of any disease, disorder, or illness can stand up to the name of Jesus

day 2: waiting game

KG slept pretty well last night. They didn't wake her for vitals or anything so that helped her. At 6am a member from the GI team came and chatted with us. They ordered more blood work for some more extensive tests and an ultrasound to try to get a better picture.

We just finished up at te ultrasound and will be waiting to hear from the doctor hopefully soon. Keller did great. Only fussed a little even though she's probably pretty hungry (didn't let her eat so they could get a better picture).

Strangely enough, she took a paci and it has soothed her really well this morning.

I'm really impressed with the hospital. The ultrasound tech was great. They warmed the gel...have a cute mobile on the bed...and sweet twinkly lights that look like stars on the ceiling here in the ultrasound room. The little touches that make all children and their parents feel more comfortable in a very uncomfortable situation.

So we wait...