Wrapping up December…on to a new month and NEW YEAR!

Have a mentioned before that I really don't like the month of December?  I really don't.  For the past 3 several years, December has been associated with bad news and bad things happening.  Both of my maternal grandparents died in December; Elise was diagnosed with Biliary Atresia in December; Elise had 2 of her 3 episodes of Cholangitis in December 2012 and December 2013, and she underwent her ureter re-implant surgery in December 2013 after which she did not wake up for 13 hours, and now, in December 2014, Elise had an emergency admission to TCH--which I will explain later in this post--and she has been battling elevated liver enzymes this entire past month. Despite it all, we have managed to have lots of good times too though.

On December 9, we went for routine labs at Texas Children's, just as we do every month, to monitor Elise's liver enzymes and liver function as well as her serum Prograf level. Elise wore her SuperGirl cape and proudly checked in for her blood work. I was shocked to hear back later that afternoon that her liver panel was abnormal and quite elevated.
AST 150,  ALT 140,  GGT 29

For comparison sake…Elise's baseline liver panel looks something like this:  

AST 45, ALT 44, GGT 17

Elise registering for her labs

                                   

The following week, on Wednesday, December 15, we repeated labs, hoping to see some improvement. The results were, however, worse:  AST 147, ALT 179,  GGT 35.  Of course, I started to panic at this point, but her doctor and transplant coordinator told me to try to relax; they said, "Don't worry; we're not worried yet." Ok well….sure….if you say so…..not.

On December 16, I brought Ethan and Elise to the TCH Transplant Holiday party. We had a great time visiting with other transplant families, including some of our sweet liver friends, and we also had the opportunity to visit with Elise's transplant surgeon, Dr. John Goss…..a  rare occasion aside from the times we cross paths in crowded elevators when he is clearly in a hurry to be elsewhere. I never know quite what to say to Dr. Goss, other than "thank you."  To which he always responds, "Oh,  I was just the plumber." We all know he is much more than that.  He is an amazing liver transplant surgeon, who saved Elise's live with her new liver.  I had no choice but to lay Elise's life in the hands of a surgeon on March 23, 2013, and I thank God every day that it could be Dr. Goss.

Transplant surgeon, Dr. John Goss, with Elise and me at the TCH Transplant Holiday party

                               

For the past 3 years, we have been getting together with our "liver friends" here in Houston for a holiday party and ornament exchange. This year's party was on Saturday, December 20. Everyone brought food and drinks, and we had a fantastic time visiting with our friends, and I found it amazing to see how much the kids have grown over the past years.  Ethan and Elise have been saying they want to go "back to our friends' house" ever since we left.

Rockin' Liverland "liver friends" Christmas party and ornament exchange…..

                                

On Monday, December 22, I brought Elise once again to TCH, bright and early, for 7:30 am repeat labs. And, once again, her liver panel was elevated; her liver enzymes were continuing to progressively increase.  AST 166, ALT 218, GGT 40.  The tone of our transplant coordinator's voice gave away the plan before she even said it. A liver biopsy would be needed to rule out or confirm rejection, and if not rejection to hopefully identify the problem, if any, causing the abnormal labs.  Our coordinator, Sarah, said that they wanted us to enjoy Christmas at home, and they would arrange a direct admission to the 12th floor on Sunday and liver biopsy on Monday. If the biopsy confirmed rejection, she said, we would be looking at 5 days inpatient for aggressive IV steroids to hopefully turn it around.

December 23. Despite everyone's best efforts to keep Elise at home for the holidays….she found a way to TCH. I'm reminded of one of my favorite poems from English literature, To a Mouse, by Robert Burns:

The best laid schemes o' Mice an' Men, 

Gang aft agley, 

An 'lae'e us nought but grief an' pain, For promis'd joy! 

(The best laid schemes of Mice and Men

oft go awry.

And leave us nothing but grief and pain

For promised joy!)

Around 7:00 pm, I set Elise's Prograf medication out on the counter and proceeded to bathe the kids and get them ready for bed. I believe it was while I while I was running bath water that Elise grabbed the medicine bottle and drank an unknown amount of it.  There was some spillage on the counter, and her dose has changed multiple times since I filled it, so it's impossible to determine exactly how much she drank.  BUT, she walked up to me holding the open bottle, I asked--as the blood drained from my face--Elise, did you take your medicine?" She proudly answered, "YES, I DRANK IT BY MYSELF!" I became frantic, and when I started crying and tearing apart the living room looking for my phone, she rescinded her claim, and changed her story, telling me that she spilled it.  I immediately paged the liver team MD on call, and while waiting for a response, I called David.  I was in such a panic, I don't even remember what I said on those calls. And I didn't pack any bag. I just stuffed the half-empty Prograf bottle in my purse, buckled Elise in, and raced to TCH as soon as David (who was already on his way home) turned in the driveway.

Upon arriving in the ER, Elise was immediately taken back to an exam room where a medical evaluation was completed and labs were drawn, including primarily a liver panel, chemistry panel to check her kidney function, and of course a serum prograf level.  Elise and I slept in the ER for a couple of hours before we were admitted to the 12th floor around 2:30 am on Christmas Eve.  Thankfully, later that day, the liver team rounded after consulting with the renal docs and toxicologists, and they discharged us back home, as Elise's kidney function was excellent, and serum Prograf level--although extremely high-- was dropping. To give you some idea of how her overdose affected the concentration of Progaf in her blood….her normal goal level is 3-5, but her Prograf level on the morning following this incident was 29.8. In any case, we happily returned home with orders to "hold" Prograf doses until further notice and bring Elise back in the next day, on Christmas morning, to repeat labs.

                                  

We woke up early on Christmas morning so that we could open presents and have a little fun before heading to TCH for labs.  I can't really talk about Christmas without sharing a few pics! We had so much fun, and it was a welcomed reprieve!

                             

                             

                           

December 25, 2014….repeat labs drawn…. AST 184, ALT 254, ALT 43.  So….her liver enzymes were still climbing.  I was sort of hoping that if Elise was in fact in rejection, the overdose on anti-rejection medication might knock her back into shape. But such as not the case. The good news was that with each lab draw, her kidney function was perfect. For those of you interested in the nitty gritty… BUN/ Creatinine 12/ 0.24.

                                

Friday, December 26, 2014. AST 203, ALT 263, GGT 43.  On this day, I was finally able to speak to our transplant coordinator, who had previously been out on vacation all week.  I felt SO much better after talking to her. She provided me with more accurate information than that which I received in the ER; she said that although Prograf is a renal-toxic medication, it will not "kill her kidneys" just from a one-time mega dose. She went on to say that they frequently encounter cases of patient's with serum Prograf levels shooting up into 30s and 40s even under their careful management, and they have had other cases of overdoses with levels going into the 100s, so while a level of 29.8 might seem scary to me, it's not really that scary to the liver team, and it's not going to cause Elise any serious problems.

Sunday, December 28.  Dr. Himes, the attending hepatologist on hospital service, admitted Elise to TCH as planned and scheduled a liver biopsy for next morning. Elise was super brave throughout her admission process, an it was a fairly uneventful afternoon and evening.

                

Monday, December 29. Elise and I were taken down to the OR just a little later than scheduled, around 10:00 am. Despite having not eaten in more than 12 hours, she was happy as a peach. Until it was time to separate and take her behind the double doors. Fortunately, we were blessed with an AMAZING Anesthesiologist who allowed me to "scrub in" and be with her through induction. I sat her down on the table and held her tightly against my chest as the anesthesiologist pushed Propofol, titrated with a little Versed, into her IV, and then once she was completely sedated, I laid her down….gave her a kiss…..and told her I'd see her in a bit.

                               

                        

There were no complications at all during the biopsy procedure. She drank apple juice and a sprite within about 10 minutes of waking up, and she was back in her room within an hour. Soon, she was "on duty," wearing her very own set of scrubs and taking care of the little patients she had with her in her room.

                               

                     

Tuesday, December 30.  I must say, it's hard to spend the the better part of a day waiting on liver biopsy results; it's even harder when previous two biopsy experiences have very negative and traumatic associations; now multiply that times 1,000 when it's your wedding anniversary…. the second anniversary you have spent inpatient at TCH in the past three years.  My depressed mood, compounded with anxiety and fear of the unknown, made for an unpleasant experience, but not one unfamiliar to me.  The dark cloud was definitely mitigated, however, by my husband, David, being there the entire day with me and by Elise's unwavering happiness and joy in every moment.

When Dr. Himes entered our room at 4:15 pm, I swear the weight lifted off my shoulders as soon as I saw his face and heard the light tone of his voice.  He informed us that he and Elise's primary hepatologist, Dr. Fishman, reviewed Elise's biopsy with the pathologist, and there was no evidence of rejection, and furthermore, no evidence of any serious viral processes that might be harmful to her liver.   He went on to say that there was no reason to start any steroids or other IV or oral medications….no med changes needed at all for that matter….with the exception of a new antibiotic ear drop medication to treat her ear infection which everyone agrees has not resolved. So, of course, I asked about the differential diagnosis.  If not rejection, and not a virus, what could it be that has been causing the progressive increasing liver enzymes? Dr. Himes and Dr. Fishman agree that the most likely explanation is an infection; very likely, the ear infection. Another possibly, though they do not think this is the case, is Autoimmune Hepatitis (AIH.) They ordered an additional blood test to screen for AIH markers out of an abundance of precaution, and we should have that result in a week or so. And the final possibility is that there is just no identifiable explanation.


According to Dr. Himes, the important thing is that her liver looks just fine on biopsy; there is no damage, and simply having elevated numbers is not going to cause any damage.  They will closely monitor her labs weekly, and they fully expect to see her numbers improving and returning to baseline soon.  With that, Dr. Himes gave Elise her walking papers, and we were able to return home……just in time for David and I to make our 8:00 pm anniversary reservation as planned!

Count on me to capatalize on picture ops!   Here's Elise and me with TCH liver center director Dr. Himes, just after receiving  excellent biopsy results and discharge papers!

Happy 9-year Anniversary to US!  :-)

                                            

Reflections…National Biliary Atresia Awareness Day 2014

Three years ago this week-- on December 6, 2011-- I walked into our pediatrician's office with Elise for her 2-month "well-baby" check.  She was actually only 7 weeks old, but I was trying to get all my ducks in a row so that I could return to work the following week.  As I recall, the appointment was on on Monday, because just 2 days prior, David and I had spent a wonderful evening at his company Christmas party. Lots of friends and coworkers wanted to know how our sweet baby girl was doing, and we were thrilled to tell them that she was amazing, and we were just so happy to be home after spending the first 14 days of her life in the Clear Lake Regional NICU. She was born 5 weeks early, but everything was perfect, or so I thought.

Elise was and always will be an amazing little girl…but there was nothing perfect about her health when she was born. Our pediatrician did not like her eyes at that 7-week appointment.  I thought her eyes were beautiful.  I was wondering if the would stay blue, but I thought they would probably turn brown. I barely noticed the yellow….and when I did, I just pushed it to the far recesses of my mind, assuming it was related to her prematurity.  The last thing a new mommy thinks is that her sweet baby has a life threatening liver disease. Ok…. I regress….our pediatrician didn't like Elise's eyes, so she requested a split bilirubin test.  I left the clinic with Elise, expecting a call back shortly that everything was fine.  I did get a call back shortly, but all was not fine.  We were told to bring Elise to Texas Children's Hospital that night to rule out or confirm a rare, pediatric liver disease called Biliary Atresia.

Today, December 1, 2014, is National Biliary Atresia Awareness Day.  Biliary Atresia, affecting approximately 1 in 15,000- 18,000 infants, is a disorder in which the common bile duct between the liver and small intestine is blocked, or absent, causing bile to back up into the liver.  The only effective treatments are the kasai procedure, and in 97% of patients, ultimately a liver transplant. Early detection and intervention is crucial to positive outcomes. The symptoms of BA are jaundiced after 2 weeks of age, light colored stools, dark urine, nutrient malabsorption/ lack of weight gain/ failure to thrive, and excessive sleepiness or fatigue.

So, today I have spent much of the day reflecting on Biliary Atresia and the affect it has had on our lives. Denial is such a powerful thing.  When the liver team reviewed the biopsy with us, we clung to and overanalyzed the terminology, such as the words "consistent with Biliary Atresia."  Surely, there must be a million issues that could be "consistent with" BA but not actually BA.  So that brought us to December 16, 2011, the kasai operation.  Elise was in the operating room for approximately 9 hours, but for at least the first 3 hours of that, I refused to believe that the the "kasai" operation was actually going to take place; I was hoping and praying that the surgeon would come into the waiting room and tell us that he had closed her up after confirming it was just a false alarm.  That, of course, did not happen.  An OR nurse called me in the OR and said to me, "Dr. Wesson has now completed the cholangiogram portion of the procedure and has confirmed Biliary Atresia...and he is now proceeding with the kasai portion."  When I heard those words,  I knew that when Elise emerged from the OR, our lives would be forever changed. It was the first and last time that I have ever really cried over Elise's condition.  I became a 'liver mom" on that day. This was our new reality, and I immediately knew that I would simply do what I needed to do….  day-to-day, month-to-month, year-to-year.

Now I can't lie; for the first couple of months, I hoped and prayed that Elise's kasai operation would work flawlessly, and that she would be in the 3% that would not require a liver transplant.  Initially, that scenario was my ideal, and my dream for Elise. Which brings me to my reflections today.  You all know, I am part of a huge online "liver mom" support group; actually, more than one group.  Already feeling emotional going into the day today, I was struck by a post of a kasai-only young man.  I am sure I over-reacted to the post and misinterpreted it, as I took the post to insinuate that because he is ___ years "kasai-only," those newly diagnosed in the BA community and others "kasai-only" should have hope. I couldn't help but think, "So, what about Elise? Does she not have hope because she has required a transplant to survive?  Is she not a "fighter" or a "winner" like those who fight BA without a transplant?

I've come to the conclusion, with the support and insight of my wonderful fellow liver moms, that there is no winning with Biliary Atresia; there is only living and surviving.  Those kids that are kasai-only right now fight every day to stay healthy; they battle the complications of liver disease, and many of their moms suffer with the fear of a "ticking time bomb." Those kids post-transplant, like Elise, also fight every day to stay healthy, and they face potential post-transplant challenges of infection, dehydration, rejection, or post-transplant lymphoproliferative disorder (PTSD) to name a few. Whether "kasai-only," awaiting transplant, or post-transplant, THERE IS HOPE, and all of our kids are fighters; they are superheroes….rockstars….winners.  It is not our role as their parents and advocates to commit them to any certain path; we must simply support them and lift them up as they continue to fight the fight…whatever their particular fight might be at a given time.