A little perspective

Life isn't always fair.  Things don't always make sense.  And when you think that your struggle sucks and your situation feels very heavy, I can promise you that someone else is going through something worse than you. Sitting in a children's hospital, watching kids fight for their lives - shows me how true these statements are.

For the past 3 weeks, my life has been filled with watching kids fight to stay alive.  It just doesn't seem fair.  I've spent a lot of time talking to other parents that have been in the hospital for twice or three times as long as we have and who have no end in sight.  It makes my heart sad.  And honestly, if I'd allow it, this could make me really angry.  So I've had to learn to see beyond this sadness and be thankful that God's allowed me to experience this part of life.

I began to learn, at the young age of 16, how precious life is and how quickly it can be taken away- when my mom was diagnosed with brain cancer.  After my mom died - I kinda thought that I had a golden ticket to get through the rest of life because our family had gone through enough.  And then Addie was born and I knew my theory was void.

So, I've learned to be thankful for every thing that life throws at me.  To hold our good days tightly and find value in the bad ones.  To trust that God's allowing every single I've walked through into my life for a purpose.  I may not always be happy about the situation I am in, but if I hold tight to Him - He will carry me through it.

To be very honest, there were more days than not, since we transplanted Addison, that I wondered if we made the right decision.  We watched her fight like a little warrior in battle.  We watched her cry from pain and were not able to do anything to make her feel better.  We watched her feel crazy in her head because of the narcotics she was taking.  We watched her body shake uncontrollably from the new meds she needs to take to keep her new liver happy.  This journey has been hard.  In fact, about a week after Addie's transplant, I was walking to the hospital from the Ronald McDonald house (it was my night to get some sleep in a bed) and just feeling angry with myself for making the decision to transplant her.  Almost regretting our decision.  And as I walked into to Addie's hospital room, the surgeon that transplanted Addie followed me into her room.  He told us that he finally got to see Addie's old liver.  And it was ugly.  Not only was it 4 lbs (a normal 5 year old liver is around 2 lbs) and huge - it also had scar tissue all the way through it and was beginning to cirrhos.  He said "you choose to do this transplant at the perfect time.  If you would have waited any longer, her arteries and ducts would have also started to cirrhos - making her transplant an extremely hard procedure."  Not to mention, that she would have started to become a very sick little girl.  I looked at Ben and shook my head.  So we really had no choice.  We made this decision because God knew this is what her little body needed and this was how He was going to save her.  Her metabolic doctor's told us from the time she was a baby - that she would need a transplant some day.  They just wanted her to be older before we went down this path.  So we made this decision several month ago because this was the path that was laid out for Addie.  And not because WE said so.

So the icing on the cake was as we actually got to see Addie's old liver this week.  We got to see first hand how awful her liver really was.  And just how much she needed a new one.  Thank you God for your faithfulness!

Look at the size of this belly.  It was ALL liver.

We truly have no idea how it all fit in there.  The surgeon said he had to cut her belly 60% bigger than what he initially thought, just so he could get it out.

This the entire liver 

The liver on the left is a normal, healthy 5 year old liver.  The liver on the right is Addie's (its actually  just a slice).

Addie touched it!!

And when Ben held her old liver up to her, it becomes clear how much space that thing must have taken up.  

Even Alivia got to join in the fun

Discharged

February 20, 2015

After Addie's biopsy and the insertion of the ND tube - Addison hit a wall.  She started to withdrawal from us.  She stopped talking and would barley look at us.  She was telling us (without using words) that she was done.  We rocked her world and she had no say in it, so now she is showing us her anger by withdrawing.  We were not prepared for this.  We were prepared for the pain and for her not wanting to eat and for her to feel like crap for a few weeks.  But we were not prepared for Addie to get angry and withdraw from us.  But as we've done with every other step of this journey, we did our best to just be what Addie needed.  Even if that meant, letting her alone and giving her a tiny bit of space.

Her team also saw a difference in Addie and heard our concerns so their solution was to discharge her. Her liver numbers where looking so much better since we started treating her sinus infection and we had the ND tube in place to give her nutrition and hydration so there was honestly no reason to keep her inpatient.  Especially because their experience showed that kids do much better recovering outside of the hospital. And they were only discharging her to the Ronald McDonald house which is directly connected to the hospital.  So if we needed anything, they weren't too far away.

Ben and I certainly weren't feeling ready for this.  We figured after the set back this week- they would want to keep a close eye on her.  So we were a bit surprised as they started pushing to get her out of the hospital.  We were very nervous about working the ND tube and administering all of the new meds (she was on 8 new meds at the time of discharge) on our own.  And we debated whether or not this was truly the best decision.  But we knew, at some point - we'd have to put one foot infront of the other and learn how to walk this path.  We knew that because we were doing this together, we would be perfectly fine.  And this would feel just as scary if we waited a few more days to take this step.  So we did it.  We walked our little girl out of the hospital just 16 days after a liver transplant.  We needed to try to find our new normal and to give Addie the chance to finally feel like she could just heal and recover in a safe place that only included her family.

 

Is this rejection?

February 18, 2015 - Addie's liver numbers are not behaving very well.  Starting on Saturday, February 14th - Addie started to have a very sore throat.  We first noticed her discomfort because she started gaging and throwing up all of the liquids were were putting in her mouth.  Prior to Saturday, she was taking small sips of formula throughout the day with no problem.  So we thought it seemed quite strange that she was refusing her formula.  Around the same time this throat issue started, her LFT's plateaued.  For the past week and a half, her liver number's were trending down and almost in the normal range.  We didn't think much of this plateau because her liver numbers right now are better than they ever were pre-transplant.  But her team wasn't thrilled by this.

We drew labs Sunday morning (we draw labs every day) and her team's inkling was correct - her liver numbers started to elevate.  Both of her LFT's bummed up about 50 points.  Defiantly not in the "scary" range - but we didn't want to see them trend this direction already.  Dr. Kyle wanted to take a proactive and aggressive approach - thinking she was starting to reject and he wanted to stop it, so he gave her a big bolus of steriods. But until we knew for sure what was happening to her new liver, the plan was to watch and see what her LFT's were gonna do over the next day or two.

The bolus of steroids didn't help.  By Monday, they continued to elevate.

Addie also continued to feel more and more sick.  Her throat hurt her so badly that she could only cry and moan from the pain.  Poor sweet girl.  The doctor's did all kind of cultures to see if she was sick with a cold, flu, or other type of virus.  But everything came back negative.  Our next thought was strep.

By now, the doctor's were thinking that her body was starting to reject this new liver.  They weren't AT ALL concerned about this because in the first 6 - 8 weeks after transplant, about 80% of patients reject.  They actually expect it when a healthy kid gets a transplant because it simply means their immune system is doing its job.  This doesn't mean that Addie will need a new liver - it just means they need to use some heavy hitter immunosuppression drugs to stop her immune system from attacking the new liver.  This is our new battle - the fine line between giving her enough of an immune system to fight against infection but not to fight her new liver.  Thank God we have a team of doctor's that have given their entire life to learning about liver transplants and rejections and have written research papers and have lead the transplant community in successful liver transplants.  We trust them.  And we trust that God's driving this ship.  So we knew that whatever they wanted to do with Addie, they could fix what her body was doing.

The recommendation was for a liver biopsy.  This would show us exactly what was happening in her liver and exactly how they needed to fix it.  So Tuesday, February 18th, Addie had her first liver biopsy.  Since Addie would need to be under anesthesia, again - we also asked ENT to do a scope so we could figure out what is going on in Addie's throat (which included a strep culture).  We also asked them to insert an ND tube.  This would allow us to feed Addie without her needing to eat or drink.  This was not at all what we expected Addie would need, but she has gone for about 3 days with barley anything to eat or drink.  By now she was extreamly dehydrated and had barely any energy.  She needed help - and this is the only way we knew how to help her.

 
Everything went well in IR.  ENT did their scope, the ND Tube was place, and the liver biopsy was done.  It took about an hour and a half and she was finished.  Back in her room on 7B and resting comfortably.

It took 24 hours for the results of the biospy to come back- and it showed NO signs of rejection.  Thank you God.  So what, then, made her LFT's respond the way they did...... A sinus infection.  Addie has low muscle tone (a direct effect from her initial crisis when she was 3 days old and her ammonia hit 2100) which has also given her a very weak cough.  During her transplant, the surgeons pumped Addie full of fluids and also inserted a breathing tube.  Both scenarios left Addie with a bit of congestion that she needed to clear.  So combined with Addie laying in a hospital bed and her weak cough - the congestion hung out and grew bacteria and became infected.  So the treatment was easy, start ammoxicillian.

Sounds easy enough.  But I'm honestly so tired of watching her fight so hard to feel better.  This journey is harder than I imagined.  It is a very helpless feeling for your mommy and daddy!

Valentine's Weekend

February 14, 2015 - We had a lot of visitors this weekend.  Alivia finally had someone to play with and keep her distracted from the heaviness of what she's been walking through the past couple of weeks.  Alivia has such a strong desire to be with us and help Addie to heal.  In so many ways, she is mature beyond her years - especially when it comes to Addie.  So the distraction that Teagan brought this weekend was much needed and allowed Alivia to feel 9 again.  We also got to spend time with our family from Ohio, which means Alivia also got to see her cousin, Sarah.  We are so thankful for family!

Our special vistor

February 13, 2015 - Today, I had one of the greatest surprises of my life.  My very sweet, loyal, and exceptional friend from Boston surprised me with a visit.  I had no idea that Michelle was coming and I cried like a baby when she walked into Addie's room.  I consider Michelle to be a living, breathing angel.  I was introduced to Michelle 5 years ago, before Addie's 1st birthday, by the President of the National Urea Cycle Foundation.  I was looking for someone to connect with.  Someone that understood what life felt like to have a child with Addison's disorder.  And God answered my prayer.  He sent me Michelle.  Michelle has a little girl with the exact same disorder as Addie.  Her little girl is 6 and was transplanted last year in Pittsburgh also.

Over the past 5 years, Michelle and I have become more like family than friends (and this is someone that until today, I've only actually met 1 time, all of our communication has been over the phone).  She's held me up when I was scared out of my mind.  She's given me advice to help me through some of my worst days.  And she's understands what I am feeling without having to say a word.  So as our family is walking through one of the scariest journeys of our lives - she flies to Pittsburgh from Boston, because she knew how much I needed her.

Michelle spent the weekend with us.  She ended up getting stuck in Pittsburgh an extra day because her flight to Boston was cancelled due to another blizzard that New England was getting hammered with.  See, God really does give us exactly what we need -even when we don't ask for it.

Our first day "out"

February 12, 2015 - Since Addie hasn't had much of an appetite since her transplant, the PA on the transplant service suggested trying some of Addie's old formula.  Since this was such a "normal" thing for Addie pre-transplant, they are thinking it will bring comfort to Addie and will allow us to get nutrition in her this way.  Addie did fairly well with the formula.  Again, we are taking it slow - but at least she is taking it without too much fuss.

The pulled her IV fluids down for 4 hours this evening and they gave Addie a pass so we could go to the Ronald McDonald house for a little.  They have found that when kids are discharged from the hospital, they actually do much better and progress much faster.  So, since we have a room (more like a small apartment) at the Ronald McDonald House - we took Addie to our room for a few hours.  We were so excited to be out of the hospital room for a little and to feel a little more relaxed!  Addie still doesn't have a lot of energy, so by the time we got there (even though we pushed her in a wheel chair)- she was exhausted and just wanted to snuggle on the couch.  Which was fine by me!

Post op - Day 7

February 11, 2015 - Addie is one week post a LIVER TRANSPLANT.  This is just unbelievable!  It blows my mind that Addie has a liver in her body that is working and doing exactly what it is supposed to be doing- that one week ago was in the body of another person.  Crazy!!

Today wasn't one of Addie's best days.  She developed another gas bubble and has a lot of discomfort and pain.  She had a terrible night sleep!  The treatment plan this time was mylicon (to try to pass the gas on her own) and taking walks.    She isn't allowed to eat or drink anything until the gas bubble dissolves because the bubble is blocking the bottom of her stomach and won't allow anything to pass. So we took a lot of walks today.  Little miss wasn't too happy about us keep dragging her out of bed - but we did it anyway  Believe it or not, sometimes mom and dad do know what is best.  I guess we wore her out because she ended up falling asleep on daddy's lap.  We did have to continue giving a few doses of mylicon - but eventually, we got the gas bubble moving!!

Post op - Day 6

February 10, 2015 - The word for today was "eat".  This was our focus.  Since her NG tube came out, she needs to eat.  Today Addie tried her first bite of chipped ham.  I was actually eating a ham and cheese sandwich and she asked for a bite.  Without hesitation we gave her a tiny bite.  Addie's reaction...... "I love ham".  Haha, all of these years without meat - and the kid loves it.  She eat a few more bites until she was done.  She also tried string cheese for the first time - and you guessed it, she loved this too.  We are trying to encourage small bites and small portions since she's gone for so many days without food in her tummy.

The doctor's are trying to wean down Addie's TPN so she starts to develop hunger cues - so they took her off her IV's for 2 hours today.  Addie was very excited to be "free" from her IV's.  We ran through the halls without dragging the IV pull behind us!

Post op - Day 5

February 9, 2015 - Addie had another busy day.  She is starting to transition from an IV to taking things orally.  The goal is to get her off of her IV so we can head toward discharge.  Although Addie doesn't have a great appetite, she is eating a few bits of things here and there.  She had some cheerios for breakfast, a few bites of yogurt for lunch, and a few bites of banana's for dinner.  She is also drinking small sips of gatorade.  These are all things she ate pre-transplant, but we figured we start with foods that she is familiar with so we don't turn her off from eating all together.  The exciting part was that we didn't have to weigh ANY of these foods on a scale or try to re-caculate what she didn't eat so we could adjust the amount of protein from her daily intake.  This is a big deal for us because this is all we've known for the past 5 years.

Addie's goal in PT today was to walk.  The PT got Addie out of bed and had her walk around the room a little.  We started to head toward the door to go down the hall and Addie very much declined. She told us "I want my BED.  I DO NOT want to take a walk".  With a little encouragement, we ended up in the playroom.  It was a beautiful site.  She did great walking.  A little unsteady on her feet and we supported from both sides - but she made it, all the way down to the play room.  And once we got there - her face lit up and she was a happy girl.  Addie has true meaning of "bed head" - and yes, there are big knots all through her hair (we tried keeping braids in her hair, but she got a bath and the braid came out).  We woke a beast.  Once she started walking and had a little more confidence in herself - she asked to keep taking walks.  Yay Addie!!

Addie is still experiencing night terrors.  Not quite as bad as yesterday through the night - but she continues to wake up very scared and yelling out in fear.  Poor thing.  We did discontinue one of her very strong pain meds (dilaudide - similar to morphine) and started oxy-codiene.  We are hoping the transition will minimize these night terrors and help her little body to get some much needed rest.

The child life team stopped in for a visit today.  They brought dolls that the girls could play pretend doctor with.  They got to start IV's (without the needle) and put band-aides on and listen to their heart beats and give shots to.  Both girls really enjoyed this activity!  Before Addie "inserted" the baby's IV - she knew she needed to put a tourniquet around the baby's leg first and she removed the tourniquet after the IV was placed.  The kid knows how to start and IV.  I'm not sure if this is really cute or really sad.  We will go with really cute!

Snuggles with sissy

Can you tell we are excited about her walking?!

Post op - day 4

February 8, 2015 - Narcotics are no joke.  They are like a double edged sword - they do their job at managing pain, but they come with some nasty side effects.  Addie is experiencing night terrors.  A combination of all of the narcotics that she is getting and everything she has gone through and being in the ICU and Dr's and nurses waking her and poking at her.  She didn't get much sleep last night because she would wake up screaming, trying to climb out of bed, and looking terrified.  I literally had to restrain her because I didn't want her to hurt herself.  It is very scary for us to watch.  She is also starting to have some jerking movements and jerks herself awake.  Sadly, these are normal side effects of this surgery.  During one of her episodes throughout the night, she ripped her NG tube out of her nose.  Not a huge deal - but this means she needs to start eating and drinking on her own.  We were able to start with small sips of gatorade and few bites of jello and a few bites of ice.

On a positive note, Addie finally stood up today and took a small walk.  She was very shaky and very scared (we expected nothing less, she hadn't used any of her muscles to walk in 5 days).  Daddy helped her stand and they walked from her bed to the couch in her room.  Baby steps!  But a very big accomplishment for the day!

Addison enjoys doing crafts.  She loves to do them when she is not in the hospital, so right now - they are comforting for her!  Today she worked on coloring and playing play-doh.  These things are very good distractions for her.

Post op - day 3

February 7, 2015 - Today was a good day for Addie.  She is still in quite a bit of pain and fairly uncomfortable, but the pain today seemed better than yesterday!  Addie had her very first poop today post-transplant.  This is such a silly update for me to report but for us - this was a big thing!!  Pooping means her bowels are finally starting to work properly and the gas bubble is passing through which should give Addie a bit more relief.  We honestly cheered for her!  Haha!  We are crazy!  She did need a unit of blood today because some of her blood counts were low and not coming up since her transplant- but this is very common after such a major surgery.

Addie started Physical Therapy - to help get her moving in a safe manor.  The goal for today was for her to sit in a chair.  Although she wasn't thrilled about this idea, once we got her in the chair, she did just fine.  She also was able to sit on the edge of the bed.  Although sitting in a chair may seem a bit more advanced, sitting on the edge of the bed takes a bit more core muscles and was actually a more difficult task.  She again, wasn't happy about this task - but she did it like a champ!

Alivia has been waiting very patiently to be able to play with her sister.  She prays every day that Addie gets better enough to play today.  She also talks frequently about the cute things that Addie does or says on a day to day basis that she misses right now, while she's feeling like crap.  So the fact that they got to play play-doh together today, was a highlight for Alivia.  She jumped right in bed with her and they played for a long time.  

We are praying for less pain and a desire and willingness to be able to get out of bed and walk tomorrow!!  You are making such great progress Addie and We are so proud of you, sweety!!

Post op, Day 2

February 6, 2015 - Addie had a very rough night.  She was uncomfortable most of the night and had a lot of belly paid.  Since Addie isn't eating or drinking anything by mouth, she formed a fairly large gas bubble in her intestines (an x-ray showed us this bubble).  In order to get this bubble moving, we needed to get her up and moving - and since she barely wanted to be touched without feeling discomfort, it didn't look very promising to get her walking.  So Dr. Shindi decided to put her NG tube back in to help keep things moving in her belly.  This gave Addie so much relief.  A gas bubble after surgery is very common.  Since Addie seemed to be progressing so well, Dr. Shindi felt that pulling the NG tube when he did would be ok.  Addie was just telling us that she needed a bit more time and we needed to slow down.  And that was totally ok!!

Pittsburg hospital has a team of professionals that help the patients and their siblings understand what is happening (medically speaking) to them and why there is so many wires and lines hooked up to them.  They also bring crafts and other fun toys to the bedside to help keep the patient comfortable and distracted from everything that is happening around them.  Today, Addie made an Valentine ladybug and Alivia got to make a different craft which used different things found in a hospital and turned it into art.  It helps to make things seem less scary!

Alivia needed to step out of the room while Addie was having a test done.  This is how I found her

The big news today was that Addie moved from the PICU to the floor (7b).  We are hoping that things are much more relaxed and comfortable on the floor than in the PICU.  Another step in the right direction!!

Post Op, Day 1

February 5, 2015 - Addie is awake!  She is breathing on her own and she is awake!  Thank you God!  We got a good night sleep too, and were up and ready to go to see our brave warrior.  We had a quick breakfast at the hotel and headed back to the hospital.

Unfortunately, Addie was uncomfortable most of the day.  She is also terrified of the nurses.  We experienced this with other hospitalizations prior to this one.  She thinks anyone coming into the room is going to hurt her.  And in all honesty, they are.  But a 5 year old, hopped up on pain meds and just coming out of anesthesia can't understand they are only trying to help.  Dr. Shindi asked for her NG tube to be pulled in hopes that she would be more comfortable with it out and this would allow her to start drinking.  She was able to take a few sips of apple juice but she was very resistant.  In fact, Ben was the one giving her sips and encouraging her to drink and when he walked out of the room, she looked at me and said "don't let daddy give me anymore apple juice.  I DON'T want it!"  Haha, my first glimpse of our Addie's personality.  We were able to get her out of bed a few times to use the potty - but the pain was entirely too great and she screamed every time we moved her, so we decided to put her back in diapers until some of the pain subsided.  We were sure how Addie would feel about diapers since she is potty trained, but I think they were a welcomed relief knowing that she wouldn't have to get out of bed.

An ultra sound was ordered for first thing this morning.  We were able to see how that new liver was looking and how well the blood was flowing.  And everything looked beautiful.  Blood flow was excellent.  Her LFT's were still very high, but certainly trending in the right direction AST was 869 and ALT was 882.  Her ammonia was 31!!  Without any ammonia scavenger or arginine - her ammonia was normal!  What an amazing feeling.  We can finally have comfort in knowing that her brain is safe!  Thank you God!

Hours After Transplant

After a full 24 hours of waiting for Addie's Transplant to take place, we thought we were finally done waiting.   But we were wrong - we were still waiting.  Except this time, we were waiting for Addie to wake up.  We were waiting to see how Addie's new liver was going to work.  We were waiting for Addie to be able to breath on her own.  We were waiting for Addie to be able to talk to us, and walk, and fight with her sister.  We were starting to become expert waiters (or maybe someone is just trying to teach us patience).

In the operating room, Addie was given a 1/2 dose of thymo (a drug used to suppress and nearly stop her immune system from working so her body wouldn't instantly reject the new liver).  Unfortunately, Addie was running a fever and her blood pressure was low - both side effects from this drug- but we were anxious to see this side effect diminish.  One of the way's to watch how well her new liver is doing is by liver function tests (LFT's).  Addison's ALT is 1,837 and her AST is 3,382 (to put this into perspective - normal ALT is 14-54 and normal AST is 22-63).  It was expected for Addie's LFT's to be elevated immediately after transplant as her body adjusts to the new liver.  After all, she has a new liver that just a few hours prior was in the body of another child.  How in the world.......

Addie's doctor's and nurses were watching her closely.  We felt very content that they were taking very good care of her and were doing exactly what they needed to help her recover.  As for Ben and I, were were exhausted - feeling that we could finally take a deep breath, our bodies were ready for some rest (I think we were going on 36 hours).  We headed back to the hotel that my dad reserved for us for some rest with Dad, Linda, Ang, Katie, and Alivia.  Just before we left the hospital, Addie was extubated (her breathing tube was removed and she was breathing on her own)!!  YAY Addie.  A step in the right direction, sweet girl!!


Mommy and Daddy are so proud of you, Addison Grace!  We are thankful for such a fantastic team of doctor's.  We are thankful for the surgeon's, doctor's, nurse's, and all other "hands" that took such good care of you today!  We are thankful for God's protections, guidance, and unconditional love for our family throughout the day.  We are thankful that you are such a fighter.  You are truly our little warrior.

Some silly pictures of our "night at the hotel".  Thankful for family!!