Waiting to hear from UNOS

Dr. Alonso wrote UNOS yesterday asking for Shelby's PELD to go from a 25 to a 30.  Dr. Alonso also said that she thinks we need to a get a living donor worked up.  I said to the nurse "That means Dr. Alonso is saying Shelby is very sick."  She said "Yes." :-(   I will fill out the living donor packet and send it in and wait to hear from Northwestern on when my testing will take place.  Somehow, somewhere (soon) we need to get this kid a liver.

O2 all the time :-(

Tuesday we had a heck of a time keeping Shelby's oxygen levels up.  They were in the low 80's to high 70's range with just a tad bit of movement.  We kept having her sit when her lips turned purple and her hands turned dusky only to have her sats go up to 88-90%, then immediately fall again when she started moving.  I called this info into Chicago the next morning and the pulmonologist ordered her to be on 2 liters of continuous oxygen and upping it to 5 liters (if needed) if her sats fell below 88%.  We found out after a few minutes of her being on the O2 and then checking her sats that 2 liters was NOT enough to keep her above 88%.  Since then she's mostly been on 3 1/2 - 4 1/2 liters.  :-(  Monday her doctor will be writing another letter to UNOS asking for more exception points in light of Shelby getting sicker so quickly.  Prayers, please, for a "new" liver soon.  As much as I love the comforts of home, I'd rather be sleeping in an uncomfortable hospital chair if it meant my daughter having a chance to be healthy.

Sat monitoring update

Shelby did the 6-minute walk test…twice.  The first time (which I’m sure is standard for the test) they told her to go at her own pace and there was a chair if she felt she needed to sit.  Shelby was not thrilled about the test at all so she sat…3 times.  She admitted to me later that she did not want to do the test so she didn’t try very hard.  I got on the phone with one of her liver nurses and expressed my concern that we were not caputuring her daily activities and the low sats that accompany them….so what was the good of this whole admission?!  She got the test reordered.  Shelby did the whole test without sitting.  I let her pick out a stuffed animal from the gift shop for her effort.  My LF friend, Noelle, was visiting and was a FABULOUS cheerleader.  Thank you Noelle!  So the lowest she went during the test was 81%, but she has had sats as low as 78%.  For now, we are in the Brown Family Life Center to keep her moving and we keep spot checking her.   We should be discharged just in time for rush hour traffic…on a Friday…yay…not!  Thank you all for checking in on us!

Actually I wrote this and then Shelby’s tummy started hurting so we headed back to the room.  She’s pooped; done for the day.  I’ve asked if they can expedite our discharge so we can get a little ahead of the traffic.  They’re working on it.

Fun with Funny Bones Improv that performed at the Brown Family Life Center

Met Minnie & Mickey (again)

Mickey taking interest in Shelby's Headbanz game

Shelby and one of the dj's from Radio Disney

Another Radio Disney dj

Shelby and Noelle, Jack's Mom, from Liver Families

Shelby and the respiratory therapist doing the 6-minute walk test

Planned Admit

Shelby will be admitted to CMH tomorrow for the monitoring of her sats.  They will most likely have her continuously hooked up to an oximeter and she may possibly have a blood gas drawn.  We pray that the doctors get the data they need to be able to get more exception points for her from UNOS.  Just the two of us will be heading in as Daddy's school/work schedule is VERY full right now.  I'm so lucky to have Scott to tag-team during hospitalizations and I know this isn't a "sick" admit, but I would appreciate prayers that I handle everything well.  I remember how lonely it was being in Chicago with Shelby in 2009 when she was taken in by ambulance and waiting (gasp! making fun of myself here!) a day-and-a-half before Scott could make it in.  Please pray for strength for her if she has to have a blood gas drawn (arterial stick in wrist - yuk!). 

RILEY TURNED 12 YESTERDAY!!!!  Here are some pics of our beauty!  Love you Ri!

Decline

Shelby's sats have gotten worse lately.  :-(  Her baseline sats will go to 90-91% if she sits for a while, but while she's on the move (just regular movement around the house) they drop down to the 75-79% range.  NOT GOOD.  Chicago is trying to work out an admit for next week so they can monitor her sats and have them on record to ask UNOS for more exception points.  Her lips will turn very purple and her hands have been turning dusky.  She is still our Shelby; pushing everything to the limit, not backing down.  In private, though, she'll admit to me that she is having trouble breathing. Trouble playing at recess, going up and down the stairs, etc.  Tonight is Riley's birthday slumber party and Shelby asked me to bring her oxygen down to the living room so she can have it when she sleeps tonight.  :-(  Please, new liver, its time.  We NEED that call.

Home, but....

Sorry, I didn't realized that I forgot to update this with "HOME!"  We were let go late Thursday afternoon.  Friday went well until 6:15 p.m. when Avery began vomiting.  About 3 hours later she had vomited about 18 times and there were flecks of blood in what was coming up.  The on-call Pediatrician said "take her to the ER."  Scott was at work, so Dad came and stayed with the big girls while Mom drove us there (and stayed ~ THANK YOU!) and I sat with Avery and caught more vomit.  She was miserable.  Long story short, we didn't get home until 4 a.m.  It was determined to be a version of what Shelby had.  They gave her IV Zofran and fluids.  Poor thing had a urine culture collected by catheter :-(  She was VERY brave.  She slept well once home and we just woke up less than an hour ago.  We have a script for Zofran to keep the vomiting away.  She is running a very low-grade temp.  Despite being given the IV fluids and drinking Gatorade this morning, she still has not urinated since before this all began last night.  I need prayers that she starts going and that the fluids she's taking in stay in.  Also, please say a prayer that this virus stops with her and doesn't taken down Riley, Scott or me. 

Shelby all ready for her "princess" ride out of the hospital.

Stopped on the way home from the hospital at Montessori to see Mrs. Lundgren

Sick baby in the ER.

Putting on such a brave smile after her IV was put in.  Daddy was working, but snuck down to be there for the icky IV and urine catheter.  He needed to wear gloves and a gown because his patient had all sorts of contagious stuff. 

Her IV.  It took 2 pokes. :-(

Shelby and the awesome Colette from RMH's Child Life.

Avery and Grammie having quite the deep conversation.  Avery kept saying "this is taking so long!"

Shelby and Mrs. Marshall, a volunteer at RMH and my *old* 4th grade teacher!

Of course we decorated Shelby's hospital room.  This time it was a Halloween theme.  If you can't make out her sign, it says "Anti-Boo-Otics."  Funny kid.  She came up with that all on her own!

Discharge?

1:34 p.m.
Waiting on the final o.k. from Dr. Deutsch - he is supposed to be here this afternoon - that we can go.  Sigh....

Wednesday

Shelby is looking much better, eating and still fever-free.  They want to give her a full 48 hours on IV antibiotics and make sure the cultures don't grow anything - 48 hours will be around Midnight so hopefully all will go as planned and we'll get sprung tomorrow morning :-)

Better Today

Fever is gone.  Tummy not hurting as much.  Yay!  Still getting IV antibiotics.  Maybe a discharge tomorrow evening?  She is smiling again.  We had a visit from Grammie and Avery this morning and then Daddy, Riley and Avery will come later this afternoon :-)  Looks like "just" a virus.  Let's hope it stays that way.

Admitted

Last night Shelby had tummy pain and it was bad enough that she didn't eat supper, made it hard for her to fall asleep and woke her at 5 a.m.  I called right away at 8 a.m. to get her in to see her GI.  He saw her at 3 p.m. and by then she had a low-grade fever of 100.  He ordered labs and an abdominal x-ray.  By the time we got home her temp was up to 101.5.  We called our GI again and he said to give it an hour.  Her temp was still up then.  He wanted her to be able to go to immediate care and get an antibiotic shot, but she's allergic to too many meds for that.  So...she's been admitted to Rockford Memorial for IV antibiotics.  She's feeling rough and looking a little jaundiced.  I'll update again tomorrow.

She got 'em!

UNOS awarded Shelby the 25 points.  Game on, people, game on!  Anytime, probably (and hopefully and optimistically) sooner than later, we will get THE CALL.  Until then, if all's well, I will most likely be quiet on here so no need to worry if you don't see anything. 

Take care~

Tomorrow...

....will be the 21-day deadline for UNOS to give an answer on Shelby's exception points.  I do know that Dr. Alonso, in her rebuttal asked for 25 points instead of 28 because that is what some centers said they would grant.  I've been told, too, that Dr. Alonso was very direct in her letter asking for Shelby's points to be reconsidered.  It was explained to me that the center's in our region (Region 7 - North Dakota, South Dakota, Minnesota, Wisconsin & Illinois) are not all pediatric centers.  Sometimes the voting party at those centers need to be reminded that they are looking at a pediatric patient's situation, not an adults.  Please, God, let Dr. Alonso's plea have been convincing enough to get Shelby these exception points!

Shelby is doing well.  She had a virus last weekend that she passed along to Avery who passed it along to Riley.  Riley missed school today because she felt so rotten and, as I type, Avery is coughing up a lung.  Shelby, surprisingly so, is resting peacefully on her oxygen.  Scott and I have talked about, but have not decided if we will keep Shelby attending school if she gets these exception points.  We hate for her to be home in "isolation," but would be devastated if they had to turn down a liver for her because she was ill.  We haven't made a decision yet; we're waiting on hearing from UNOS.  We'll see what the team thinks, too.

Scott is still plugging away at nursing school.  It is about half-way through the semester for him so he will wrap up his Renal clinicals and start on Pediatrics soon.  That means "finals" for him, though, next week.  He is one stressed-out, worried Daddy.  Please say some prayers for him.  That he can concentrate on his schoolwork until she gets THE CALL.

Tonight I packed up my toiletries and some odds-and-ends for my long upcoming stay in Chicago.  I will so miss sitting here on my couch, snuggling with my Ri and Baby A....the list goes on.  I tell myself it will be temporary, but it doesn't make me feel much better. 

I will update when we hear on UNOS' decision.  

Exception points DENIED

Yes, I found this out while driving in for Shelby's cardiologist appointment.  First of all, her heart looks great.  We just need to keep it that way.  UNOS denied the exception points because they wanted an arterial blood gas reading.  It has to be below 60mmHg for them to give her the exception points.  A LOT of strings were pulled while we were there Tuesday and it was worked out that she could have the ABG drawn.  An ICU attending was available to do it.  They had her walk on the treadmill for a few minutes and her sats fell to 78%.  Then she sat down and they started the draw.  They didn't get it the first time and by the time they did get it she had been sitting long enough that her sats were back to her baseline of 90%.  She was such a trooper through such a painful procedure.  Sadly her ABG was 64.5 - low, but too high to submit to UNOS.  Her team is talking and trying to figure out what to do next.  They feel it shouldn't be this hard to get her exception points.  We agree.  Today is the first time she's gone to school this week.  She's just been too tired the other days.  Her appetite has been poor and she has lost 2 pounds in 2 weeks.  This is something we need to keep a close eye on.  I had a thirty-one open house Tuesday night and she was at 110% because she was so excited to see a favorite person from her old school, but promptly dropped on the couch when the last person left.  Poor kid.  I am getting mad at her liver.  I am thankful it has held together so many years, but angry to see what it is doing to her now.  C'mon exception points...

Cardiologist

We have to be in Chicago tomorrow morning for Shelby's annual visit with the cardiologist.  Usually these are long, hum-drum clinics, but we actually have something to talk about now - her Hepatopulmonary Syndrome.  We're looking for more direction on how much/how often with the use of the oxygen.  Shelby's sats have been dropping even lower.  We've had her on as much as 4 liters of oxygen at night.  That's up quite a bit from the .5 liters we started at about a month ago.  I'm afraid her HPS is progressing rapidly.  :-(  This is such a scary time, but I try to focus on all the good that will come for Shelby when she gets a healthy liver.  We still have not heard on her exception points being granted.  She's only been listed for 11 days; UNOS has 21 to respond.  Sigh...time for my girl to get healthy.  She went to a birthday party Saturday afternoon and it took her until this afternoon to get her strength back.  That's not good....

Tuesday Ultrasound

Yesterday Shelby had an abdominal ultrasound so the surgeon could see what's going on in there - a little pre-planning and sneak-peak for transplant.  All went well, I think.  There was some question about whether or not her portal vein was seen.  Its pretty darn important.  We'll see what they say.  It doesn't change her need for a transplant, but it could change what type of graft she can get.  Right now (before the ultrasound) it was a whole or split liver.  That might change if there's problems with her portal vein.  

We walked - well, 3 of us walked, 1 was in a wheelchair and 1 in a stroller - around the Lincoln Park zoo.  My Mom had not been there and throughly enjoyed it.  She took tons of pics, but I'm too tired today to put them on here.  We also stopped by Build-A-Bear because it was very important to Shelby that she make her own bear.  She still loves Lovzey & Keisha (her other 2) very much.  I figured the kid deserves another if she's going to have to go through a transplant.  'Nuff said.  She only made it half the day at school today.  Too tired.  After she eats lunch, I'll have her put on her O2 and lay in our bed.  I'm ready for her to feel better.

ON the waiting list

Just got a call from Chicago that Shelby is ON the waiting list. Right now her PELD is -5, but they are appealing for a new "score" of 28 due to the Hepatopulmonary Syndrome. Amazingly calm at this point.

The Evaluation

We do a lot of waiting!

Shelby with Dr. Jill & the flower she brought her from Grammie & Papa's garden

Yesterday was incredibly l-o-n-g, but a lot was accomplished.  We met with all the members of "The Team."  The Transplant Team, that is.  From 1:15 - 5:45 one after another would come and talk to us; when they left, the next came in.  It was exhausting.  Shelby did really well.  We still have to go in for an abdominal ultrasound before transplant, BUT I should hear today or tomorrow that she is on the waiting list.  This is a scary time for us.  Exciting, too.  Scary because transplant is such a big thing.  Very risky.  Exciting because Shelby will no longer have liver disease and will feel better with a successful transplant.  Because of her Hepatopulmonary Syndrome, the doctors will be asking UNOS for 28 points on the waiting list.  We shall see what she gets....I will let you know when it is "official."

She only made it about 45 minutes at school today before she needed to come home.  Yesterday exhausted her.  Today she is resting in our bed, wearing her oxygen.  Hopefully she just needs this "recharge" time and we pray that she can return to school tomorrow. 

***Scott just informed me that he could not comment on here.  I forgot to change that setting back to how it was.  I think I have it fixed now. :-) ***

They know

We realized that Scott didn't have to work tonight AND heard the final word from Chicago so we told Riley & Shelby after school.  They took the news amazingly well.  Shelby started to cry & wanted Riley (we had taken her to our room to tell her on her own).  Once she hugged Riley & knew that her sister knew, she completely changed her tone and was joking.  Both of them seem o.k.  Better than Scott & I.  We let them know that it was o.k. to ask any question.  Then they wanted to go play.  Wow, wish I was that resilient.

The ball is rollin...

I got a call late this afternoon that Chicago wants to see Shelby Tuesday for her transplant evaluation!  I think they sped things up after I called today letting them know that the past few days she has been purplish on the inside of her lips and her sats have quite often been only 84%!  The pulmonologist said to have her wear her O2 more at home and today when I got them from my Mom's after school, she was so tired she wanted to ride in the wheelchair (my parent's have one) home.  Umm...we only live next door to them.  I hate seeing her like this!

Scott & I are so nervous to tell her!  We're going to do it Saturday morning over donuts and we'll tell Riley & Shelby separately.  This sucks....

Shelby WILL be listed

****What you are reading the girls are NOT aware of.  We have not told them yet.  Please be sensitive to this****

Today I heard the "official" word from Chicago that they WILL be listing Shelby for a liver transplant and appealing to UNOS for exception points due to the Hepatopulmonary Syndrome.  Exception points are points given for certain complications.  Specific labs & information are used to give a person a "score" that determines their place on the waiting list.  When you have complications that are not represented by that score then your doctor can ask for exception points.  Your case is then reviewed by UNOS and either they give the points or they don't.  HPS (Hepatopulmonary Syndrome) gives you automatic exception points.  From what I'm reading, 28 to be exact.   We knew this day was coming at some point, but now that its here we are scared.  We are very optimistic that Shelby's transplant will make her feel better, but sometimes you are just trading in one set of problems for another.  There is really no choice in this situation, though.  HPS is not reversible any other way.  Liver transplant is the only way to make it stop.  Oxygen was delivered yesterday by home health.  Shelby is to use it for comfort.  It doesn't help her oxygen saturation, just can make her more comfortable.  We are not ready for this, yet have been preparing for it for 9 years.  I just want my Shel-belle to be better.  I will keep you all updated on when she is listed.  We would appreciate prayers.  Thank you.