Wednesday, June 19, 2013

Rockin' Liverland

We've known since Day 1 that Elise is a little ROCK STAR!  But it has never been more evident than it is right now. Truly, Elise is rockin' liverland in every way!  Her liver panel is perfect, her liver looks great on ultrasound, she is gaining weight, thriving, and even beginning to throw some tantrums!  While tantrums are not pleasant, I remember a time when I was not sure we would see Elise go through the "terrible twos."  Thankfully, the "terrible twos" are in full swing now, and those ominous thoughts are fading from my mind. 

Okay, so back up a couple of weeks.  Two weeks ago, Elise had a set of labs drawn between clinic appointments.  Her liver panel was perfect; there seemed to be no worries; however, last week, on our way to Louisiana, Elise's transplant coordinator called me to tell me that her EBV level (which takes several days to process) was a bit high. "But don't worry.." she said.  HA!  I wasn't worrying; I was having a small coronary. An elevation in Ebstein-Barr Virus (EBV) level needs to be closely monitored, and intervention should take place as soon as possible,as uncontrolled EBV levels can lead to a very serious complication called Post-Transplant Lymphoproliferative Disorder (PTLD), which can turn into a full-blown malignancy, lymphoma.  So, like I said, early intervention is critical, and as always, our liver/transplant team jumped on it immediately. After consulting with her liver doctor, our transplant coordinator instructed me to decrease Elise's Prograf (anti-rejection) from 3 mL twice/day to 2 mL twice/day. Based on all the research I had previously done related to EBV levels and PTLD, I know that this is the most appropriate initIal course of action. I was just really concerned that the lower Prograf dosage would upset her new liver, and we might face rejection issues.  I asked, "Should we have labs drawn while we're in Louisiana...you know, just to check in on her liver?"  Per her doctor, "NO! She has a perfect new liver and it will be fine."  If you can call her team at TCH anything, you can call them "confident." And "amazing." 

So this all brings us to where we are...TODAY!  Today, June 19, was Elise's 3-month post-transplant liver clinic appointment.  Her regular liver doctor was unavailable, and we couldn't push the appointment out any further, so we were seen by our liver center director, Dr. Shepherd.  I've talked about Dr. Shepherd previously-- he is WONDERFUL, and he is a real expert in the field of Biliary Atresia and pediatric liver transplant.  He has worked in hospitals all around the world including several of the top liver transplant centers in the United States. Anyway, I just love that he is never short on words.  As you might imagine, I like doctors to talk until I've had enough, and then talk some more.  THAT, my friends, is exactly what he does.  He's been through this so many times, that he actually foresees my questions and answers them in a way that demands my trust.  He talked to us for about 45 minutes today, and I LOVED the sound of every word that escaped his mouth.  He walked in with a huge grin on his face and said, "Her numbers look perfect...all of them...and not just her liver panel....ALL of her labs." He went on to explain that Elise's new liver has obviously tolerated the lower Prograf dosage very well, which is great! He informed us that it is now time to begin carrying out their plan to wean Elise off most of her meds, leaving just a small dose of Prograf.  And eventually, he was sure to add, "Elise will be an ideal candidate to wean off ALL of her meds--including the Prograf-- completely, though that last move will be at least 3 years down the road. 

So, the PLAN is as follows:  Elise's steroid dose was cut in half as of today.  Over the next 10 days, we will be phasing out 4 meds completely-- the steroid Prednisolone, the antacid Prevacid, the ant-viral drug Valcyte, and the anti-fungal med Nystatin.  She will remain on Prograf 2 mL twice/day, Ursodiol 2.5 mL twice/day, and Bactrim 13 mL three times/week. She now weighs 23 lb 15 oz and is 31.5 in long, placing her in the 50th% percentile for weight and the 15th% percentile for height.  She no longer needs quite the amount of calories that she has been taking in, so she is to begin weaning off the Pediasure formula and transitioning to whole milk!!  MUSIC TO MY EARS!!!  

We will go back to TCH for repeat labs in 2 weeks, and assuming everything still looks great and there are no more nasty EBV issues, next liver clinic will be in 6 weeks, on July 31. 

Based on the recommendation of Elise's liver/transplant team at TCH, she returned to her normal daily routine at daycare this week. Dr. Shepherd explained today that she is not more at risk of catching normal illnesses, but it might just take her a little longer to get over illnesses that she does catch...and despite our best efforts...she WILL get sick sometimes. We should expect this and know that her team will monitor her closely and manage any issues that come up. Dr. Shepherd emphasized the importance of not keeping Elise in a bubble.  He said, "If our transplant kids needed isolation, that's where she'd be, in isolation."  Elise needs to go out and live life like any other kid, with just a few special precautions in place." 

For those interested, here's Elise's liver panel as of TODAY (***Albumin-higher is better;   all others- lower is better***) 

      AST 38 ALT 34, GGT 14, direct bili 0.0, Albumin 4.9

Just to compare,  here's Elise's "old liver" panel from March 2013:
      AST 129, ALT 111, GGT 536, direct bili 0.0, Albumin 2.9

As always, thank you all so much for following Elise's journey in "Liverland." We love you all and appreciate all the prayers and support so very much!!