New Developments

So, since my last post, a lot has transpired related to Elise's condition.

On December 13, I noticed Elise's abdomen was more distended than usual, so I put a call in to the transplant team. The transplant coordinator immediately called me back and asked me to bring Elise in to the ER at Texas Children's for further evaluation by the liver team. Well, after 8 hours in the ER which included lab work and an ultrasound, the liver "fellow" on call apparently did not think it necessary to see Elise in th ER and examine her.  I was LIVID, so say the least, since it was the primary liver specialist who instructed us to come in to the ER for examination.  If I wanted an ER doctor's opinion, I would have taken her to our local hospital. After I expressed my concerns....very emphatically...and refused to sign discharge papers until she was examined by the liver team, I got a phone call in the ER from this liver team "fellow," who stated that he had carefully looked over all of her labs and ultrasound, and he felt that to admit her to the hospital would do more harm than good, as there was nothing that could be done to intervene at this point.  Basically, he indicated that she would have to get worse.  Not exactly what any mother wants to hear....and especially not over the phone.  So at this point, I signed out of the ER and took our little Elise home.

Now....most of you who know also know that it wouldn't end there.  I got home around 11:00 pm, sat on the edge of my bed, and typed out an email to Elise's primary hepatologist, with a copy sent to the director of the TCH liver center. I included pictures of Elise's abdomen and a detailed explanation of my concerns.  Her doctor called back early the next morning and explained that he received my email, and he apologized, stating it was never his intention for us to leave the ER without being physically examined by one of the liver docs.  In any case, he further stated that he and several other liver team members reviewed Elise's labs and ultrasound from the night before, and assessed her recent trends.  He noted that it appears blood is being pushed away from her liver, which is contributing to some of the tummy distention and will ultimately lead to cirrhosis taking over her entire liver.  He said that Elise seems to be declaring that "it's time" to move forward and pursue transplant much more aggressively; the paperwork was being done to request a higher PELD score/ placement on the liver transplant wait list. 

Several days passed, and on December 19, we saw the liver/transplant team at TCH for "liver clinic." According to her doctor and the transplant coordinator, her PELD score is now 28, placing her at fairly high priority....meaning we could literally get "the call" any day now, and Elise could get a new liver....although it could still take several more weeks.

We have faith that the the perfect liver will come for Elise at the perfect time. 

Back Home Again

We just returned home today from another stay at Texas Children's Hospital. We were there for the past 3 days following Elise's bilateral re-implant surgery--the operation recommended by her transplant team to correct a kidney reflux which threatened to cause problems post liver transplant. We were expected to only be there 1 night....but of course that turned into 3 days.  (click "Read More")

Following the surgery, urology quickly signed off the case, and the liver team admitted us to the GI floor at TCH in order for them to closely monitor Elise's condition and take good care of her liver. Unfortunately, Elise's liver didn't handle the operation itself or the medications during and after surgery all that well. It took Elise more than 24 hours to become fully awake and alert after her last dose of Morphine. As the liver doctor explained, her liver just had a really hard time processing those narcotic meds. They also had to discontinue her IV fluids sooner than they would have liked because Elise's body was having trouble getting rid of it.

And then came the lab work. The doctors said that her liver function tests are dropping lower and lower, which essentially means that her liver is "packing up." Dr. Ross Sheperd, the director of the TCH Liver Center and widely respected expert in Biliary Atresia, commented that they would have expected to see her GGT spike immediately following surgery, which would have indicated that her liver was working harder in an effort to recover. But, in Elise's case, her GGT just continued to drop following this surgery. On a good note, the doctor did say that, through this "test," her liver has proven that it has a a little fight left in it....that a small portion of her liver appears to be doing all the work and getting the job done, which gives us some time to get our ducks in a row, etc.   In response to my question about a "timeline" as to what we should expect, the doctor said that when the GGT reaches the point where it is lower than the Alkaline Phosphatase, it is usually a marker that the patient will need a transplant within 3 months, and that is where Elise is right now-- as of today, her GGT is 278, and her Alkaline Phosphatase is 325. 

Weekend Sleepover

So, this past weekend, we had a sleepover....at beautiful Texas Children's Hospital....with all of her friends that we've gotten to know very well over the past year or so.   It all started Saturday morning when her "spitting up" turned into profuse vomiting after ever feeding. I was very worried and brought her to the pediatrician that morning, at which point we were immediately referred to Texas Children's Hospital.  By the time she was evaluated in the ER at TCH, she was very sick....very low energy, not interacting. At the ER, they collected labs including cultures of her blood, urine, and stools. She was found to be severely dehydrated. She had lost over 2 lb (11%) of her body weight, and her chemistries panel showed very high sodium and low potassium.  She immediately started on IV fluids and potassium as well as Zofran for the vomiting.  (click "read more")

She was admitted to the GI floor (12th floor) of TCH on Saturday night with the expectation that she would be discharged in the morning.  Her electrolytes were still abnormal though on Sunday morning, so her stay was extended until her sodium came down from 170 to a normal level of 140.  We are home now, and Elise seems to be doing much better.  

We are going back to TCH tomorrow for a repeat VCUG test in preparation for her surgery scheduled for Dec 4, which has been recommended to fix her kidney reflux issue. Elise has grade 4 kidney reflux and has had at least three urinary tract infections despite being on prophylactic antibiotics twice daily.  The transplant team has been worried about the post-transplant implications of this issue, and so the urologic surgery team was consulted. We met with the urologist/surgeon last week and came to a decision.  They will be doing the bilateral re-implant operation, which is the most complicated of the three options; however, it apparently has the highest success rate.  The surgeon explained to us that we don't want to find out that one of the "easier" procedures didn't work by her being post-liver transplant and developing a kidney infection, with immuno-supressents on board......we would have big problems.  So that's the plan.  They expect her recovery to be fairly easy, and it should have no effect on her listing for the liver transplant. Sot  that's that.  I will keep everyone updated prior to and following the surgery.

Again, thank you all for the wonderful prayers and support.  We appreciate it all so much!

A BOOLOOZA of a Week

Wow...what a week this has been!  I'm thrilled to report that "Transplant-BOO-looza, our first major fundraiser benefiting "COTA for Elise" was a huge success.  We raised over $12,000 for uncovered transplant-related expenses, which is phenomenal. We are so very appreciative to the many people who came out to show their support, the individuals and businesses who donated items for the silent auction and prize drawing, special in-kind donations such as the announcing/ entertainment provided by DJ Greg Dumas, all those who helped tremendously "behind the scenes"....and especially Nicholls Education Association of Teachers (NEAT) who sponsored this fundraiser.  (click "Read More")

On Monday, she saw her pediatrician for her 1-yr check up and vaccinations.  The appointment quickly became very complicated when her pediatrician began to second-guess whether or not she should give her a second dose of her live vaccines (MMR and chicken pox).  Elise already received these vaccines (which are normally first given at 1 yr old) early, per the recommendation of her transplant team, to ensure that she would have at least one dose prior to transplant.  Since she is still pre-transplant, her pediatrician felt that she should give her a second dose to further build up her antibodies.  We resolved the matter by making a call to her transplant physician, Dr. Fishman, at TCH, who recommended she go ahead and receive "round 2" of the live vaccines. 

On Wednesday 10-24-12, Elise was seen for "liver clinic" at Texas Children's. She now weighs 20 lb 11 oz and measures 28 1/2 inches!  She has grown quite a bit and impressed the doctors and dietician!! This brings me to the announcement of a major milestone...she is switching to a new formula for "big girls!" Starting this week, we are transitioning her to Peptamen Jr formula. They are saying that if/when she is able to take in at least 30 oz/ day of the Peptamen Jr by mouth and refrain from losing any weight, they MIGHT consider giving her a trial without the NG feeding tube...which would be the most fantastic news ever!  

So, now for the not-so-fantastic news...

Elise has apparently not outgrown her kidney reflux problem.  She is past 1 year old and still apparently has grade 4 kidney reflux. The transplant team has again consulted the pediatric urologist to devise a plan address this kidney issue prior to transplant. The kidney reflux places her at high risk of urinary tract infections and/or kidney infections, which could potentially cause big problems post-transplant when immuno-suppresents are on board. It seems that the recommendation might be for her to undergo a minor operation in an attempt to resolve the problem now, prior to transplant, and prior to her kidneys incurring any damage. The very LAST thing we need is for her kidneys to be damaged. So, I am now waiting for the urology office at TCH to call me and schedule an appointment, hopefully very soon. 

I will certainly continue to keep everyone updated!!

Thank you all for following Elise's journey, and thank you most of all for all the prayers, love, and support!  It means so much to us!! :-)

Happy 1st Birthday Elise!!

Wow!  Our sweet Elise turns 1 year old today!! With all the uncertainty related to Elise's diagnosis of BA in December 2011, there have been times I wondered if we would ever see this beautiful milestone. We thank God for Elise's early diagnosis and Kasai operation at 8 weeks old which has helped to get us to this point, as we are reminded that babies with Biliary Atresia who are not effectively treated usually do not survive the first year of life.  (click "Read More.")

As I think back of this past one year, it's hard to avoid thinking of the negatives: six hospitalizations, ER visits, clinic visits, four medications twice/day, a 21-day episode of home IV antibiotics via PICC line, NG tube feeds over night every night, incessant blood work, ultrasounds, x-rays, CT scans.  But then I think of how fortunate and absolutely blessed we are to be living here in Houston, a few minutes away from one of the nation's leading pediatric liver transplant centers-- Texas Children's Hospital. I still remember one of the first consultations we had with her treatment team at TCH, when they predicted that she would require a liver transplant before age 2. At that time, and every month since then, they have stated their "ideal goal" for her to reach one year old and 20-22 lb prior to transplant, which dramatically reduce the risk of surgical complications. Today, my friends, Elise is one year old and 21 lb 7 oz!!  With a smile on her face, she says to her transplant team, "Bring it on!"

This next year will undoubtedly present challenges for our family, but we are ready!

Copyright La Vie Photography

Liver Clinic 9-19-12: Impromptu Consult

 

 Well, although stressful as usual, Else's "Liver Clinic" appt today was quite productive. We had a guest appearance/ consult by Dr. Shepherd (new director of the TCH Liver Center & renowned expert on BA) who Dr. Fishman brought into the room after I challenged him [again] on the issue of Ascites, since it does not show up on her fourth consecutive ultrasound. Well, Dr. Shepherd examined Elise and said he agrees that she does have ascites, but the fluid is likely hidden on ultrasound by her enlarged liver, "multiple spleens" and malrotated intestines.  Furthermore, he said that looking at her ultrasound and veins which are visible on her stomach, he can see that blood is being diverted away from the liver which puts stress on the vessels and further damages the liver. He said that it is clear that her liver disease is progressing and that transplant being required within the next 6-12 months is inevitible.

He went on to explain that she will do best with a left side of a split liver due to the way in which her intestines are malrotated, and it will likely take longer to find an exact match to what they want for her, which is why they have listed her 3 months ago, ahead of the game, and she is accumulating time on the list so that once her PELD jumps up, she will get offers over others with same PELD who have less time on the list. Makes sense, right?? I feel like I understand everything so much better after hearing it from the mouth of Dr. Shepherd. I like his accent too!  Anyway, it's wonderful that it seems everyone on our treatment team is on the same page.  

 

 

So now to get to the nitty gritty details....

 

 

 

Elise has not gained any weight.  In fact, she lost a tiny bit of weight since last month-- 5 grams to be exact.  In her defense, she did wet her diaper shortly before clinic. Anyway, needless to say, the team is not letting go of the NG tube. They said that they will be looking very closely at her weight gain (or loss) next month though to evaluate for the possible need for TPN/ lipds intravenously. In regards to labs, all of her liver enzymes are elevated from last draw, except her conjugated bilirubin which remains 0.0 (great!)  Her GGT has increased this month, and her Albumin is lower indicating poor absorption of nutrition. Her PT (clotting time) and INR are within normal limits though, which is good.

 

 

 

 

 

 

 

 

 

 

 

OH, and I want to be sure and point out that the "kasai' operation Elise had shortly after birth has not yet failed....which is the reason that her conjugated bilirubin is still at 0.0, and she's not jaundiced. For now, bile is still managing to drain out of her liver. Her doctors commented that she would be in a much different place right now if her "kasai" had failed. The transplant surgeon Dr. Goss, Dr. Shepherd, and Dr. Fishman are all in agreement that her liver still has a good bit of fight left, and more importantly, her little body is in good shape to fight. And she's apparently going to do it all with that precious smile on her face!! :-)

 

 

Home Sweet Home

Well, this has been one LONG week.  Elise was admitted to the Texas Children's Hospital on Monday 7-10-12 with a high fever of 105.4.  The major concern was Cholangitis a serious liver infection. She was admitted under the care of the TCH liver team and immediately started on IV antibiotics Vancomycin and Zosyn to treat for Cholangitis while doctors ordered tests including labs, viral studies, an ultrasound, and an x-ray to rule out other causes of the fever.  Upon admission, the attending physician explained that they could not send Elise home on the bet that this was not Cholangitis and risk further damage to Elise's liver.  We agreed of course.    (click "Read More")

Once we got settled in, I was told that they would consult the liver transplant surgeon, Dr. Goss, to see Elise while she was in the hospital.  I was hoping he would indeed come to see her, but based on previous experience, I suspected that maybe some other surgery resident or member of the transplant team would come to see her in his place.  We have been  seen by everyone else on the transplant team during the evaluation process, but not HIM--the liver transplant surgeon--the "wizard" as we sometimes refer to him.  So imagine my surpise yesterday when Dr. Goss walked into our room, accompanied by his entire team....quite the "posse!"  It was actually very overwhelming and somewhat intimidating to see Dr. Goss himself, in the flesh, and the entire liver transplant team standing before me, all at one time.  For the first few moments, it seemed like all I heard was noise, but I believe he said, "Hi, I'm Dr. John Goss, and we are here to meet Elise and answer any questions you might have."  Well, he certainly came to the right place if he was looking for questions.  And the answers I was looking for???? I got them.  Dr. Goss made several good points. To summarize, he agrees with the liver team physicians that Elise needs a liver transplant at this time, primarily due to failure to thrive, but also based on her suspected portal hypertension and history of Cholangitis. He also notes that although her PELD score (pediatric end-stage liver disease) score currently remains low, the team could get at least 24 exception points for her at any time based on her presentation and history, which would place her at high priority to receive a liver. However, Dr. Goss recommends they just "hold the cards" for now and wait to request those additional points, thereby affording him the opportunity to "shop around" more for a pristine organ--an absolutely perfect liver for Elise.  And, now that Dr. Goss has seen Elise personally, he knows exactly what he is shopping for!!  So anyway, Dr. Goss left me with the feeling that they are going to see to it that Elise gets her liver transplant when she needs it, while she is still healthy enough to do well through the transplant operation...and he has no reason to suspect any negative outcome of complications. In regards to complications, I started to ask about the potential complications that all "liver moms" fear such as rejection and Post-Transplant Lymphopfoliferative Disorder (PTLD), but Dr. Goss sort of cut me off and reminded me not to get ahead of myself.  I then pointed out that these are very serious and anxiety-producing fears over which I can't help but obsess, especially since just a few rooms down the hall, one of our little "liver friends" was being diagnosed with atypical rejection, possibly chronic rejection--how devastating for this family who who has been celebrating as he thrived more than a year post transplant!!  According to Dr. Goss, what he knows for sure is that Elise will die without a liver transplant, and any complication that arises post-transplant can be treated and effectively resolved. 

So, after a few days in the hospital, Elise is now back home with us. She was discharged now that her blood cultures have been negative x 48 hours, her fever is gone, and her liver enzymes are back down to her baseline.  She will be on oral antibiotics for the next 10 days at home, but at least we're home!  

Liver Clinic 8-15-12

Yesterday, we had Elise's "liver clinic" appt at Texas Children's Hospital. It went pretty well but was generally uneventful.  She had an exam, doppler ultrasound, and labs.  The doctors said that on exam Elise's liver felt enlarged, and it appears that there is some fluid in her abdomen.  The ultrasound remains essentially unchanged from last month; there are cirrhotic nodules present and visible on Elise's liver, which does appear enlarged on ultrasound, as does her spleen.  Her liver panel is slightly improved from last month, but still abnormal, as most of her liver enzymes are elevated.  Her conjugated bilirubin is still at 0.0 though, and her INR and platelets are normal, so that's great news!  

Here's her liver panel for those interested in the details. 

Conjugaged Bilirubin:  0.0    

(click 'Read More")

ALT 85; AST 77; GGT 598; ALK PHOS 346; Albumin 4.2

Platelets 200                     

GOOD NEWS that we received is that Elise weighs 18 lb 13.5 oz now and is 27 in long, which puts her over the 50th percentile!!  The sort of bad news is that the doctors won't let her lose the NG tube yet.  Their rationale makes a lot of sense though.  They explained that the bigger she is at the time of transplant, the bigger her portal vein will be, which will reduce the risk of portal vein thrombosis (clots)--one of the most threatening complications immediately post transplant. So, I can't really argue with that. 

Crawling Right Along...

I can't believe it's almost been a month since my last blog entry.  The days go by SO fast.  Anyway, Elise is now crawling, even if it is sort of "army crawl" style.  She's definitely getting around....and fast!  I hope she's not burning up too many calories because we've been working really hard at packing in the calories and fat!!    (click "Read More")
Currently, Elise weighs 18 lb 6 oz.  The transplant team goal is at least 20-22 lb at the time of transplant, so I feel fairly confident that she will get there....probably very soon!!  She is now taking about 25 oz Pregestimil formula mixed with Polycose (30 cal/ oz), and NG tube feeds 8 hours/night (45 mL/hr).  OH, and she's increased the amount of baby food she is eating each day now too!!  She usually eats about 1-2 containers stage 1 or stage 2 food mixed with Polycose powder and MCT oil.  I'm really looking forward to our next "weigh-in" with the dietician to see what she has to say!!
In other news, Elise is finally sleeping every night in her nursery!!  For the longest time, I couldn't stand to leave her upstairs in her room because I was afraid that the NG tube would cause some sort of hazard, and I wouldn't be there to intervene.  But, I'm pretty sure now that the worst "hazard" I will encounter is the occasional soaked bed due to Elise having pulled out the NG tube in her sleep.  You would think those pumps would have some type of sensor which would enable them to stop pumping if the tube is pulled.  But that makes too much sense.
So we are hoping to see everyone at "Transplant-BOO-looza"-- the fundraiser planned for October 20 benefiting COTA for Elise.  Please check out the "Activities & Events" section of this website for more info on "Transplant- BOO-looza" as well as all the other great fundraisers currentl in the works!!   And don't forget about our "PRAYERS for ELISE" silicone bracelets!!  Please email me at ebabinlcsw@gmail.com or contact me through Facebook if you'd like bracelets.
Finally, I'd just like to say "thank you" to everyone who is following Elise's journey and sending prayers and positive thoughts our way!  The support has been overwhelming, and we are most appreciative.  Finding ourselves in this particular situation has been quite humbling, and we have really been touched by the care and concern of so many people, including family, friends, and even people we do not even know.  

Liver Clinic 7-11-12

Hmmmm....well....Elise had her "liver clinic" appointment today at Texas Children's Hospital.  We had lab work done and met with her hepatologist (liver doctor) Dr. Fishman, the transplant coordinator, and dietician.  

The good news is that she has continued to gain weight well, currently weighing 17 lb 11 oz.  She is 26.5 in long. Her growth chart now looks perfect, showing she is in approximately the 55th% for weight: height!! Her doctor and dietician agreed that we can now decrease her NG tube feeds to 8 hours/day...she had been at 12 hours/day since April.

The bad news is that her liver enzymes are going in the wrong direction, according to today's labs;  almost all of her numbers are up from last month.      

                                     June 5,  2012                                 July 11, 2012
AST: (normal 20-60)                    58                                           99
ALT: (normal 6-50)                      45                                           104
GGT: (normal 10-160)                 451                                         665
ALBUMIN: (normal 2.8- 5.0)        3.9                                          4.5
DIRECT BILI: (normal < 0.5)        0.0                                          0.0

So, anyway, her doctor is supposed to call me tomorrow to let me know if we need to move her doppler ultrasound appt up and get it done sooner rather than next month.  Other than that, there's not much else we can do differently or as an intervention. I mean, she's already listed for transplant.  

I remember when Dr. Fishman dropped the "bombshell" on me that he wanted to refer Elise to the transplant team....he said that he expected her liver enzymes to start climbing, and that he wanted to "get all the ducks in a row" and be prepared for when her liver declares that it's done.  After looking at today's labs, I'm starting to think he may have been right.   Imagine that.   I swear, my denial never ceases to amaze me.

Proud Mommy!

Today, I brought Elise to Texas Children's Hospital for her psychological testing appointment, which is typically part of the transplant evaluation. In Elise's case, there was no grave concern by any of the physician's regarding her physical, social, and/or emotional development, so we didn't get in for the testing until now.  Anyway, I initially thought this was going to be a waste of time.  I mean, really, psych testing on an infant??  It actually went very well though.  The psychologist was great with Elise, and very knowledgeable about pediatric liver disease too, which was nice.  Of course, she probably evaluates all the babies waiting on transplant there, so I'm sure she's made it her business to be well-versed on the subject.       

Anyway, using a combination of direct play/ interaction with Elise and the answers I provided to her questions, the psychologist completed several different infant and early childhood psychological and developmental assessments....and Elise "passed" with flying colors!! Elise is currently 8 months old, and she scored between 8 and 10 months old in all spheres! Her highest scores were in fine motor skill development and social development.  I'm having her mail me a copy of her complete formal report once it's done.  

So, despite Elise being born 5 weeks early, spending a total of 7 weeks thus far in a hospital bed, and having an NG tube since April which complicates activities such as "tummy time," Elise has managed to meet and even surpass the developmental expectations for babies her age!  That makes me one happy and very proud mommy!!  

After Elise's appointment, I enjoyed visiting with a fellow "liver mom" Jennifer and her kids. Jennifer's middle child had Biliary Atresia and had a liver transplant a little over 1 year ago....she looks so wonderful!!  It was great to see them! 

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

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!