Fever, Fever, Go Away!

Elise is currently well into day #2 of a persistent fever.  She suddenly spiked the fever around 10 am yesterday. I picked her up from daycare and brought her to the doctor yesterday afternoon. Her pediatrician ruled out ear infection first, and then she ordered some blood work. Elise's white blood cell count was a little elevated at 14,000 (normal range is 6,000-10,000.)  To me, an elevated white blood cell count is a sign of infection somewhere in her body, and my first thought--and major fear-- is Cholangitis, an infection of the liver. Historically, her doctors have always admitted her to the hospital for fever because it is so critical that they protect her liver and treat for Cholagnitis once other sources of infection are ruled out.   

This time, however, her pediatrician spoke with a doctor on the liver/ transplant team at Texas Children's Hospital and decided to just treat the fever with Tylenol and Ibuprofen "and see what happens." I think the main rationale for taking a more liberal approach this time is that, as Dr. Pocsik put it, "She is the healthiest looking sick baby I have ever seen." I'm obviously praying that this fever disappears, but that hasn't really happened yet. The fever breaks every time I give her medicine, but then it returns after about 6-8 hours. 

So, anyway, we'll continue to watch her over this weekend. I'll be bringing her back to the doctor if she's still battling fever on Monday.  At that point, if she still has fever, I'm almost positive that I will have to bring her to Texas Children's for further testing.  

Well many thanks to all of our wonderful family, friends, and those we don't even really know who are praying for our little Elise and sending positive thoughts our way.  The support means so much to us!! :-)

Cautiously Optimistic

So, Elise's doctors at Texas Children's Hospital (TCH) have repeatedly told us that it is critical that Elise gain as much weight as possible prior to transplant. Ideally, they would like Elise to weight at least 20-22 lb at the time of the operation, as this ideal weight seems to correlate with a decreased risk of complications such as clots in the portal vein. When they first placed Elise's NG feeding tube, Elise was far behind her goal, not even on the growth charts, weighing less than 12 lb at age 6 months old. Today, she is 8 months old and weighs exactly 17 lb 0 oz, putting her in at least the 25th percentile for weight. Rather than being ecstatic, I am cautiously optimistic, because just yesterday, I realized that her tummy is more distended than it has ever been previously.     (click "read more")

We have already been old that she has "Ascites," and her doctors predicted that it would soon be getting worse. So all this begets the question, is her current weight gain true body mass or is it fluid? Well, we have an upcoming "liver clinic" appointment at TCH, so I'm hoping to get a doppler ultrasound to check things out. In fact, I may insist on it.  

On the Road to Transplant...

So, as of this past Monday, 6/18/12, Elise has been formally placed on the liver transplant waiting list.  I got "a call" on Monday...not to be confused with "THE call" from the transplant coordinator--Julie-- saying that Elise is officially listed with a PELD score of -3. The PELD (Pediatric End-Stage Liver Disease) score basically reflects priority on the wait list, or risk of mortality without transplant. So considering this, it's not a bad thing that Elise's score is low right now. Her doctor believes that he she will be granted exception points within the next 30 days or so, which will increase her PELD score somewhat. We'll see. Bottom line]is that we now need to be prepared to get called for transplant at any time. When it's a match, it's a match!"                     (click below to read more)

Over the last few days, I have noticed that I'm obsessing over every little thing. I have the urge to call the doctor's office with all of these little questions I have, such as "What's Elise's vitamin-D level?" and "What's her platelet count?" and "Are her bile salts high or low....or normal?" I've now had a revelation:  I'm directing my anxiety and energy towards all of these little, unthreatening questions that can be easily answered rather than confronting the BIG question on my mind which no one can answer: "When is this transplant going to happen, and will my baby be okay??"

Family Fun!!!

I just LOVE weekends!!  Although I have been fighting a severe headache for the past several days, which is actually not very unusual for me, I have managed to keep up with the rest of my little crew this weekend!  We lounged around on Saturday and tried on the kids' swimsuits....then today, we went to Galveston to spend the day at Pleasure Pier!!  We had such a good time, although it was very hot!!

Elise was able to enjoy the day without her NG tube since she conveniently pulled it out during the night last night. Actually, we have woken up the past two days to Elise covered in formula, bed basically flooded, andNG tube out.  She used to cry and cough when she would pull out the tube, so I would wake up and put it back, but now she's pulling it out silently. Sneaky girl!  Can I blame her? No.  I certainly wouldn't want to sleep with the thing.  Maybe this is her passive-aggressive way of proving she can gain weight without the overnight NG tube feeds.  We shall see!  All I can do is continue setting up her tube and pump each night, hoping she keeps the tube in and gets the whole 12-hour feed.

Anyway,  unfortunately this weekend is just about over....but it was a great one!!

"...I think this is the beginning of a beautiful friendship."

I LOVE my big brother!!

Off to Galveston!!  Big sunglasses are in these days, right???

Ethan is careful not to use of all of his energy on smiling for the camera!!      

Daddy & his baby girl!!

 

BAMN!!  got 'em Mom!!  

Mmmmmm.....where has this stuff been my whole life??

the Tea cup ride!!   

Stopping for some lunch....but Elise is ready for more!!  

The End

Giving Up the Wheel...

It's SO nice to be back home this evening.  It was a long day at Texas Children's Hospital today, as it is every time we are there for clinic, labs, etc.  Anyway, clinic went very well today!  Dr. Fishman said he was really impressed with Elise's weight gain. She currently weighs 15 lb 14 oz, 25 in long. That puts her now in the 25th percentile for weight!!  He still stands by his belief that she has ascites and portal hypertension. Oh, and the other problem that Dr. Fishman repeatedly refers to is her extremely high GGT level.  Her GGT is currently 451.  He says it actually doesn't make much difference to him whether it is 451 or 2000 because it's still entirely too high and means that her liver is not functioning properly, and she is not absorbing nutrition as she should. So, the plan is still to present her case to the Medical Review Board (MRB) with the intent to have her listed for a liver transplant. The MRB actually met today, but Elise apparently got bumped until next week's meeting due to there being two children who were more urgently in need of being reviewed and listed today.

Here's her current LIVER PANEL, for anyone interested in the details, like me:

AST (range 20-60)-            58

ALT (range 6-50)-              45

GGT (range 10-160)-         451

Albumin (range 2.8-5.0) -   3.9

Bili, direct (range <0.5)-      0.0

platelets are also normal- 370

Elise is just looking so wonderful these days.  It makes me crazy to think about the "what ifs"....but of course I do anyway.  I mean, I have read tons of kids' stories of transplant and the aftermath of transplant, and so often, I read about horrible, scary complications.  Some have even required a second and even third transplant. Just last week, one of our little "liver friends" went through 3 liver transplants in 10 days, and she's still not out of the woods right now, although she's much improved.  So when I look at Elise with near-normal labs, good weight, normal vitamin levels, no history of GI bleeds, and only "suspected" ascites and portal hypertension, it makes me think, "What if I consent to transplant and she dies or ends up in much worse shape than she is now??"  I mean, it wouldn't take a whole lot to be in worse shape than she is now, because unless I'm missing something big (which I very well could be), she's looking pretty darn good.  Except for the fact that... she does have a liver disease-- Biliary Atresia-- and there are cirrhotic nodules on her liver.  Those are the words that haunt me.  

You know, a few people have asked me recently whether I think I should get a second opinion at another center, since I seem to be so ambivalent with regard to my feelings about the plan for Elise. There's a really long answer, and a short one....here's the "in-between" version...

We love and trust Elise's liver team and transplant team at Texas Children's Hospital. We have had disagreements and have not always liked what they had to say, but ultimately, their rationales have always made sense. I've made no secret of the fact that I was very much against the NG tube when they placed it in April, but as it turns out, it has helped her to gain weight and to thrive. When I have argued with the doctors, they have argued back;  her doctors have consistently made great cases to support their treatment plans, which have in turn pacified me and put me at ease....relatively speaking, of course. The bottom line is that I would take Elise to another hospital in a NANO-SECOND if I didn't believe 100% that they are doing what is truly best for Elise, all things considered, even if their plan is at times based on gut instinct.

So, all of this brings me to a resolution tonight.  As hard as it is for me to give up control, I think I'm going to let God and Elise's doctors take the wheel.   But I'm still in the back seat.  :-)

Our little tiny baby girl, Elise, just beginning her battle with Biliary Atresia

Our big girl, 7 months old, fighting BA with a smile on her face.

Liver Clinic- 6/6/12