To B A "Liver Mom": "This is a Marathon, Not a Sprint…"

This post may be long-winded, so I apologize in advance. Part of the reason I blog is to process information and experiences, and I took away a great deal from my impromtu conversation with Dr. Fishman this morning. Actually, I suppose it wasn't really "impromptu,"since I had every intention of requesting a moment of his time following the TCH Liver Center breakfast this morning.

As of yesterday, Elise's liver panel remains elevated, so I emailed Dr. Fishman last night with a few questions and issues I wanted him to consider; I requested his thoughts on whether Elise's elevated liver enzymes could be an adverse hepatic reaction to Augmentin antibiotic, and/or whether we had any evidence to rule out auto-immune hepatitis. After spending most of yesterday evening thinking, looking over dates, talking to other liver moms, and reviewing Elise's medication record, these were the two possibilities that really scared me.

So, when I arrived at the TCH liver center breakfast this morning, I was greeted by Dr. Fishman, and he immediately acknowledged my concerns, as he had already read my email. We spoke briefly before the breakfast and presentation and then again for about 45 min afterwards. I explained that I realized last night that Elise's last normal liver panel was 3/19/14, and she was started on the antibiotic Augmentin on 3/24/14 by her pediatrician. Last night, I discovered that Augmemtin can cause negative hepatic reactions including elevations in bilirubin, AST, and ALT, and findings of liver biopsy can be consistent with that of hepatitis. Dr. Fishman gave me a "kudos" for my discovery and stated that it will in fact need to be considered in Elise's differential diagnoses, but he also said that he does not feel this is the issue at hand, as this reaction occurs only in approximately 3% of patients. In regards to auto-immune hepatitis, again, he said this is very rare, but it is indeed a diagnosis that will need to be ruled out.

So, then, I asked Dr. Fishman about his gut feeling; I explained that I need to know how concerned I should be and what to expect now. His response? "This is a marathon, not a sprint." That's what he said. This is so true, and I have known this from the beginning, but hearing him say this today put things in perspective. As he explained, Elise will be a transplant patient for the rest of her life. While they realize that we want answers [yesterday,] a resolution to this one hurdle will not be the end; really, there is no finish line; there is only pushing on. As he pointed out, "you have to look at the patient," and Elise looks great! Yes, her liver enzymes are elevated, but her liver function and other labs are normal! He said that we will re-check Elise's liver panel next Tuesday 5/13/14, and if there is not significant improvement, he will want a liver biopsy. His "gut feeling" is that a biopsy will confirm a viral infection in her liver such as EBV (Ebstein-Barr Virus.) Possibly CMV, but since her CMV blood tests have been negative weekly, probably not. Elise's labs have shown copies of EBV in her blood for 8 months though, so there it is reasonable to believe that EBV may be present in her liver. For this reason, he hesitates to increase her immunosuppressent medication in an effort to bring her liver enzymes down; if she does have a viral infection of the liver, increased immunosuppression would cause the virus to flare up which could potentially cause a severe complication such as PTLD (post-transplant lymphoproliferative disorder,) or essentially, cancer. So, okay then....a biopsy it is! Definitive answers please!! OR, a miraculous decrease in her liver enzymes!!!

Now, just for a moment, I have to go back to the TCH liver center breakfast this morning. What a great presentation! Five of our hepatologists (liver specialists) spoke, as well as several special guests, and most of the liver center staff attended, including some of those who work behind the scenes with our liver kiddos, such as the interventional radiologist who has inserted both of Elise's PICC lines, and our pathologist, whose reports I have read many times in Elise's chart. It was simply amazing to meet a cardiologist associated with TCH who is a 57 year old survivor of Biliary Atresia--he is "kasai-only," no transplant as of yet, and living a relatively healthy life. Apparently, he had the "kasai operation" before the operation was even really named, and he showed us the back cover of a magazine from the operating room where his surgeon illustrated what exactly he did during the surgery. "This is my medical record," he said. Wow!!! Well, he certainly carried the message of hope for medical miracles home.

The presentations by the physicians this morning covered various liver diseases, several different ongoing research studies at TCH, and the statistics and outcomes related to liver transplant at our center. I was impressed to see that TCH led the nation last year, in 2013, in the number of pediatric liver transplants, completing 98 liver transplants--Elise was of course one of them. TCH also exceeds national averages in success of the liver graft and patient survival post-transplant. There are also more participants in the iWITH research study (complete withdrawal of anti-rejection medication) here at TCH currently than at any other pediatric liver transplant center. As I looked over the numbers in black and white....particularly the sheer number of solid organ transplants completed at TCH... I was struck by the fact thatfor every transplant, there is an organ donor. Thank God for organ donors, and God bless our donors and their families.