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!