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.