Great weekend, Great friends...

This past Memorial Day weekend was SO much fun!!  We had our friends over from Dallas....that would be Donnie and Martha, and their kids David and Maria.  My husband David and Donnie have been good friends since they were little kids.  And now, we have baby girls who are just about the same age-- Elise was born exactly 7 weeks before Maria!  They were both born 5 weeks early.  David is Maria's godfather, and Donnie is Elise's godfather.  Anyway....they spent the weekend here at our house, and we had a fantastic holiday weekend together!  

Elise & Maria...best friends forever!!  

My sweet girl!!

HAPPY baby...out at the Kemah Boardwalk!!  

the usual suspects...David, David, and Donnie

Ethan sleep any time, any place....just like his daddy. 

YAY for unch at Wings-N-More!!

Elise loves her Uncle Donnie!!  

Elise and Maria....so sweet!!  

In other news....

Elise's pediatrician, Dr. Pocsik, called me unexpectedly today just to check to see how Elise is doing.   Dr. Pocsik is just the best doctor in the world....hands down.  It was so thoughtful of her to call.  And it's not even just that.  First and foremost, I feel like I owe the world to her for following her instinct, ordering tests, and discovering Elise's liver disease early, before it was too late for the Kasai operation. I can't even say how many times I've been told by specialists at Children's Hospital, "You have a great pediatrician." Then Dr. Pocsik has literally made herself available to us 24 hours a day.  She has given me her cell phone number, and believe me, I have used it.  So anyway, we had a nice long talk today about this "transplant path" that we have found ourselves following at this point.  Basically, all things considered, she agrees with the plan, although she admits that she did not see this transplant referral in the cards a month ago. 

My Thoughts...Restructured...

I have mentioned previously that I am working on getting into a new mindset....the transplant mindset.  Well, it's still a work in progress....restructuring my thoughts. I have come to realize recently that I have been taking a very negative approach when considering transplant.  I was actually equating the need for "transplant" with some sort of failure on my part; For some reason, I think I was under the impression that  Elise would not need a transplant as long as I took care of her in exactly the right way.  So, when her doctor told me he was referring her for a transplant evaluation, it felt like a slap in my face.  Well, my friends, I should never have allowed my beliefs to become so distorted.  

I believe now that the referral for transplant evaluation at this point is a blessing. It is a fact that Elise has liver disease. It is a fact that there are cirrhotic nodules visible on her liver. Dr. Fishman believes that Elise will need a transplant before the age of 2, definitely before the age of 5, so listing her for transplant now will hopefully save us the trauma of watching her get really sick and suffer unnecessarily. I never want to be scrambling to get her listed for transplant, wondering if a liver will be found before it is too late.  

All this is not to say that I don't still wish I could tell the future.  Oh, how I would LOVE to be able to see into the future. I hope and pray that Elise's future is one of health and one free of liver disease post-transplant. Of course, she will always have special medical needs for the rest of her life, but I hope that she will just grow up accustomed to the meds and doctor visits and will always treasure her gift of life. 

Transplant Evaluation: Play-by-Play

Wow...where do I start??  Okay. Let me start by saying that Elise's transplant evaluation is complete.  It has been two very long, exhausting, somewhat overwhelming days....but now it's done.  The next step is for Dr. Fishman (Elise's GI/liver doctor) to present her case to the medical review board, at which time a decision will be made regarding whether or not to actually list her for a transplant.  If she is in fact listed as her doctor expects, she will be given a PELD (pediatric end stage liver disease) score to determine her priority on this list.

So, here's the play-by-play of Elise's transplant evaluation:

Wednesday, May 16--

9:00 am-  Meeting w/ Dietician- Stacey

Yay!  Elise actually got a GOLD STAR from Stacey-  she has been gaining approximately 35 grams/day since her last appt 3 weeks ago, which is an excellent trend! Elise now weighs 14 lb 6.5 oz.  She is 25 in long, and has a head circumference of 43.5 cm.  From a weight-age perspective, she is now in the 15th percentile!!  So, I suppose the NG tube is doing the job....but that doesn't mean I have to be a fan.  As it was explained to me, her nutritional status is so critically important to her recovery post-transplant, that the best plan is for her to keep the NG tube in place until the time of transplant. While we're on the subject, her overnight feeds via NG tube have been increased to 40 mL per hour x 12 hours....just in case anyone is interested in the details.

9:30 am-  Financial Counseling
So apparently there are a lot of transplant- related expenses not covered by insurance, such as copays for at least 10 medications each month, frequent office visit and possibly ER visit copays, daily hospital parking fees, and percentages of medical equipment and supplies that may be needed for such things as home IV meds and/or enteral nutrition. Mary, the financial counselor, suggested we look into COTA (Children's Organ Transplant Association), which is a fundraising organization which basically assists in raising funds for uncovered transplant-related expenses and actually acts as a trustee and manager of the funds, ensuring that the funds are always available throughout Elise's life.  As it was pointed out, she will be a "transplant patient" for the rest of her life, and her insurance coverage and financial status may not always be what it is now. I'm still not sure if we will actually get involved with COTA, but it's definately something to think about.

10:00 am- Visit w/ Child Life Specialist- Mary
Our child life specialist seems wonderful!! Basically, her role is to help Elise and her brother, Ethan, understand and adjust to medical procedures and the transplant surgery. She will asset with coping positively with medical experiences and reducing the emotional trauma.

10:30 am-  Psychosocial Assessment w/ Social Worker- Barbara
Well, it was sort of interesting being on the "other side" of this social worker's psychosocial assessment.  I'm more comfortable being on her end, but it was fine.  She asked all the basic questions. I may have gotten a little irritable with her when she began acting as though my anxiety was something unexpected.  I  pointed out to her that any anxiety I am experiencing is what we call "situational anxiety," which is related to Elise's chronic illness, and quite normal.

11:00 am-  Appointment w/ Infectious Disease physician - Dr. Munoz
Dr. Munoz reviewed Elise's medical history, particularly her history of infection and effective treatment. We discussed Elise's history of Cholangitis, urinary tract infections, RSV, Adenovirus, and Pneumonia, as well as the antibiotics that were effective vs ineffective. She recommenders that Elise have her 12-month immunizations early-- at 9 months-- since "live" viruses are involved and will be contraindicated post-transplant.

12:30 pm- Sedated Echocardiogram & EKG
Well, this was the major stressor of the day, for multiple reasons.  First of all, Elise had to be put on NPO status, meaning she was not allowed to eat from 6 am until after this test, due to the sedation.  She was allowed to drink clear liquids such as Pedialyte until about 10:00 am, but still, Elise was NOT a happy camper. She was sedated with an oral sedative, Chloral Hydrate. About halfway though the echocardiogram, Elise's oxygen saturation dropped to about 80-85 for several minutes.  The nurse had to try to wake up up somewhat and was preparing to put her on oxygen when she happily resumed breathing normally again.

2:00 pm-  Lab Work
Can I just say that "Carole" in the 3rd floor lab at TCH is AWESOME!! One stick and done...and it was 
several tubes of blood!! Elise didn't even cry!!! :-)

Thursday, May 17

8:00 am- Abdominal Ultrasound w/ Doppler
Well, I received the results of this ultrasound this afternoon.  It actually showed no evidence of Ascites or Portal Hypertension. Despite these results, her doctor says he still suspects that she is suffering from at last mild portal hypertension, and properly a little Ascites as well.  He didn't really have an explanation as to why it is not showing up on doppler ultrasound.

9:30 am- Chest  X-ray
As far as I know, the chest x-ray was normal.

10:00 am- Lab Work
I requested the same phlebotomist that we had on Wednesday, because she was really great.  Again, she got Elis's blood quickly, with just one stick.  We will be requesting "Carole" from here on out.

10:30 am- Meeting w/ Transplant Coordinator-  Julie
Very informative meeting. She reviewed a very handy, dandy little book which attempts to predict and explain a multitude of questions regarding the transplant process. I have a copy of the book for reference at home. It's quite good...and very well-written...however, there are still SO many questions/ answers that it does not even begin to address.

2:00 pm - Meeting w/ Hepatolgist- Dr. Fishman
Dr. Fishman reviewed the test results which are in at this point and then went over the "informed consent,"  which I signed.  He patiently talked to me for over an hour and provided very good responses to all of my questions. It was a good visit...and it was the most that I have ever heard him talk.  He obviously feels very strongly that listing Elise for transplant is the best treatment plan at this point....and I bet that if he presents his case to the medical review board like he presented his case to me this afternoon, they will vote to list her, despite her decent labs and ultrasound. Also, he pointed out that Elise will need a "split liver" transplant, as opposed to a "whole liver" due to the fact that she has bowel malrotation, a condition related to Biliary Atresia, as is "polysplenia," another condition found with Elise. I was not very happy to hear about this, as "split liver" transplants are a little more complicated due to being associated with a higher risk of bile leaks, etc.

Just What the Doctor Ordered....

So we had a wonderful visit from my parents a.k.a. Grammie & Paw this past weekend for Mother's Day,  and it was just what the doctor ordered! We had the very best visit ever!!  Seriously...I've been so nervous about Elise's upcoming transplant evaluation, that this visit with my mom and dad was just perfect--- a great distraction!  Ethan and Elise were SO happy to see them and play with them!  We all had a blast!

Here's some pictures from the weekend...

I don't think Grammie has ever had a bigger smile!!  LOL!! 

Grammie & Elise....you can really see in this pic that Elise has grown quite a bit!! :-)

Ethan & Elise... play time....I think they were both interested in Paw at this time...

And....she pulled the tube....PARTY TIME!!!  I really believe she pulls it out on purpose....as evidenced by her expression in this pic.   HA!  Caught her in the act!  LOL!!  

Lunch at Landry's Seafood for Mother's Day.   YES....that's a wine glass!!

Mommy getting some kisses!!! 

Sleepy time...  so glad she has no cares in the world.   Mommy gets to do ALL the worrying.  And that's the way it should be.  

Ethan has started cutting the grass in an effort to earn money for new pants....LOL!!  

Seriously....he doesn't usually wear pants w/ holes in them....but he was going to get them all dirty anyway, so fine!  

Anyway,  tomorrow begins Elise's transplant evaluation, and the last time I was this anxious was probably  during the week leading up to her diagnosis of Biliary Atresia in December 2011.  I just can't wait for this week to be over. Anyway, tomorrow--on Wednesday-- we meet with the dietician, child life specialist, social worker, infectious disease doctor, and her liver doctor followed by labs and a sedated echocardiogram and EKG.   On Thursday, we have more lab work, a visit with the transplant coordinator, and then an abdominal ultrasound and chest x-ray. 

Here's the link to the Texas Children's Hospital Liver Transplant Center.. just to provide some additional information on reason's for liver transplantation, types of liver transplantation, and the TCH liver transplant team.

"Click" HERE to visit TCH Liver Transplant Center

Okay, well, please keep all the prayers and positive thoughts coming....we appreciate the support SO much!  It is truly a blessing!  

A new mindset...

So, over the last couple of days, I've been working on getting myself more into a new mindset-- the "transplant mindset."  The wonderful support from my "liver family," my real family, friends, and coworkers has really helped me along with accepting the whole idea.  I mean, of course I want Elise listed if she needs to be listed....but I think it's the "needing to be listed" part that was giving me a problem.

Anyway, we are now awaiting Elise's transplant evaluation at Texas Children's Hospital.  I spoke with the care advocate on her case with the insurance company on Friday, and everything is approved, so now we're just waiting on the dates/times of all of her eval appts to be scheduled.  It will all probably be taking place the week of May 14th. This "transplant evaluation" consists of assessments by the pediatric hepatologist (liver doctor)- Dr. Fishman, the transplant surgeon- Dr. Goss, the dietician- Stacey, the transplant coordinator- Julie, the social worker- Barbara, the Child Life Specialist- Mary, and a  psychiatrist. She also needs lots of blood work done, a chest x-ray, EKG, echocardiogram, and an abdominal ultrasound.

Needless to say, Elise's NG tube is not getting pulled any time soon. Well at least not "officially" by the doctor.  That's not to say that Elise doesn't pull it out herself every day....because she does.  But back in it goes every time she pulls it!  So if she has to have this undesirable accessory, you can count on me to do my very best to make it a little more tolerable.  That's where these amazing "feeding friends" comes in.  One of my fellow "liver moms" introduced me to these little sticker creatures which do wonders for holding onto the tube, keeping it in place, and at the same time making it a little more child-friendly.

Elise taking a nap with her feeding friend, Mr Fishy--not to be confused with her liver doctor, "the Fish" Dr. Fishman...LOL!!

Elise showing off her little party dress, BA/transplant awareness bow (made for her by our "liver friend" Denielle), and her new feeding friend, "T-Turtle." 

And for any fellow liver moms who are interested in these little "feeding friends," here's the link to the website where they can be purchased:  

Click HERE to purchase some "Feeding Friends" of your very own!!

what a day...

Elise had "liver clinic" today.  I was actually really looking forward to our appointment today, since I feel like Elise has never looked better in her life.  Her skin is pink, her eyes are clear and white, she's gaining weight every day, and I was very optimistic that her labs would be looking good too.  And I was right... her liver panel has never looked better.  Here are the results:

ALT: 145
AST 52
Alk Phos: 317
GGT 496
Albumin 4.7
Conjugated Bilirubin:  0.0

Her weight today is 13 lb 8 oz; her height is 25 in.  She is now in the 10th percentile!!

So, as Dr. Fishman settled into the the exam room, I couldn't wait any longer to ask, "So do we get to lose the NG tube????" To make a long story a little shorter, his answer was "no."   And he went on to say, "You're probably going to like what I have to say next even less."  Really?  I said, "Let's have it."  Then "BAMB!"  The next words out of his mouth felt like a kick to the gut....

Dr. Fishman said, "With your permission, I'm going to refer Elise to our transplant team."

He let those words sit with me for a second, then went on to say that I need to hear him out so he can explain his rationale, and that he would then be happy to answer any and all of my questions.  But first, he opened the door and let the "transplant coordinator"-- Julie-- into the room.  Wow.  This was all happening very fast.

So, as I sat in the room, holding Elise, and talking to Dr. Fishman and the transplant coordinator, Dr. Fishman explained that he suspects Elise has portal hypertension and may be starting to develop Ascites.  These conditions can cause very serious complications such as varices, GI bleeds, respiratory distress, and right-sided heart failure. I challenged him, pointing out that Elise's ultrasound earlier this month indicated "no ascites" and showed no evidence of portal hypertension.  Well, Dr. Fishman responded by admitting it is very early, and he's just beginning to notice some tell-tale signs that this is the direction in which Elise is headed....not necessarily that she already has these diagnoses.

Dr. Fishman went on to say that he wants Elise thoroughly evaluated by the transplant team and listed for transplant so that she can start accumulating time on the list.  The more time she has on the list, the more "points" she can earn towards a higher PELD (Pediatric End-stage Liver Disease) score, which will be critical to ensuring that she can get a new liver when the time comes that she really needs one.  Dr. Fishman said that he would rather have her listed too early than wait too long to list and have her get so sick that she might not tolerate the transplant as well as she could.

Well, I suppose that rationale is about as good as I could have asked for.  It makes sense. I can't really argue with it.  Dr. Fishman made himself a little more comfortable in his seat before asking, "So, do you have any questions."  "Well, as a matter of fact I do...."

about 45 min later.....

I emerged from the exam room, sat down on a sofa in the clinic, and fed Elise a bottle just like I have done every day since she was born. It was like I didn't even just receive information that will quite literally change our life.

While transplant will certainly change our life, I have tremendous faith that it will be a change for the better.  We will no longer have the thought of the "t-word" looming over our heads.  It will be done...over with.  Elise will be a "transplant patient....not a "BA patient." She will have a brand new liver rather than the cirrhotic liver that is currently trying to kill her.

Our precious little Elise is one happy, very strong little girl and will face these upcoming challenges head on....and with a big, bright smile on her face!!

Liver Clinic 5/2/12