THE CALL…at the Zoo!!

^{Since I'm running a little behind on this post, many of you probably already know this, but Elise received THE CALL on Saturday 3/23/13.  When we left home that morning, it was to go to the zoo... we were not exactly expecting that we would bring Elise home from the zoo with a new liver!!  Actually, I shouldn't say that; call it mother's intuition, but I put the hospital/ transplant bag in the van when we left home... "just in case."   So like I said, we were at the zoo when our transplant coordinator called to tell us that they had an offer for a liver which appeared to be a perfect match for our sweet Elise. Obviously, we were overwhelmed with emotion...mainly anxiety... and we left the zoo in a hurry to drive three blocks to  Texas Children's Hospital.} 

^{Upon arriving at the hospital, they expedited our admission through the ER, and we were led straight to the room which was awaiting us on the GI/liver floor.  The nursing staff collected lots of blood for lab work, and a chest x-ray was done to rule out any illnesses which would preclude transplant.  She was of course cleared for surgery fairly quickly; however, we still faced the wait for the surgeons to harvest the donor liver, transport it back to TCH, and examine it to ensure that it was indeed a perfect match and size for Elise and that it was in good condition.  Surgery was tentatively scheduled for 6 pm. And so, we waited.}

^{At 7:15 pm, the OR nurse and anesthesiologist came to take Elise to the OR.  It was A GO!!  I then handed our baby girl over to the transplant surgery team for the liver transplant, calling upon every defense mechanism I could to avoid the immense panic related to the lengthy list of potential life-threatening complications. As the OR nurse took Elise from me, I also handed her my cell phone number and a disposable camera as well as instructions to take a picture of Elise's old liver once it was removed.  She said she would do whatever she could to get that picture for me.  I just felt that I needed to see the organ that has caused such a crisis in our lives.}

^{Shortly after 9:00 pm, I received the first update from the OR: "Dr. Goss has made the first incision; the operation itself has begun."  I said to David, "I guess now there's really no turning back."  David responded, "When they told us she had BA and that her liver was cirrhotic, there was no turning back;  when she had that bleed 2 weeks ago, there was definitely no turning back.  " Of course he was exactly right.  At 10:35 pm, we received the next update: "the old liver is out, and the new liver is going in..."  Wow.  So, that was probably the point at which things started to hit me pretty hard.  This surgeon had just taken out one Elise's most vital organs, and if this new liver didn't turn out to work, we could lose her. It was then not until 1:09 pm that we were informed that the surgeon was closing the incision and the operation would soon be over. Elise would be taken straight to the pediatric intensive care unit, where we would finally be reunited with her about an hour after surgery.  While waiting to see Elise, her surgeon, Dr. Goss, came out to the waiting area and spoke with us. He informed us that the surgery went very well with no significant complications.  I thanked him, and he said, "This is what we do."}

^{Yesterday, on Sunday 3/24/13, at about 2:30 am, we saw Elise post-op for the first time post-op.  She looked so little and helpless, although in reality, she was about 2-3 times her normal size as a result of fluid retention from surgery.  She was intubated on a ventilator, meaning there was a tube inserted in her throat to breathe for her until she could do so on her own. She was also covered in monitors and had multiple peripheral IV lines as well as an arterial line which measured her blood pressure internally, a central line placed in her neck, a NG feeding tube, and a foley catheter. While it initially hurt me to see her like this, that feeling quickly began turning to triumph as Elise began to remind us who she was under that puffy skin.}

^{Just 7 hours after coming out of surgery, Elise was obviously taking her own breaths, so the decision was made to remove the ventilator (breathing tube).  The ventilator was taken away, and the rest of the day went fairly well without incident.  Then this morning, less than 36 hours post-op, the liver and transplant teams rounded and made some additional surprising and exciting decisions.  As the surgeon, Dr. Goss, walked into the room, Elise was screaming.  Like music to my ears, Dr. Goss said, "Would someone get this kid a bottle!? Who has a bottle?!  Let's let her eat...I bet that will fix her all up.  The nurse quickly provided a small bottle which Elise latched onto while Dr. Goss continued his orders: "OK, she looks great...lets get rid of all this "stuff" and get her to the floor. To clarify, by "stuff" he apparently meant the foley catheter, the peripheral IVs, the NG feeding tube, oxygen, and heart monitor.  I was stunned...but ecstatic! I asked whether he meant that she was really going to go to the regular floor today....and he said, "Yeah, why not! She's a rock star!"  I reminded myself that Elise now has a perfect liver, matched and custom fit just for her by our amazing liver transplant team, led by this one Dr. Goss; HE is a rock star, in my opinion.}

^{This afternoon, since arriving on the 12th floor at TCH--the GI/liver floor--Elise has been drinking bottles of formula, and late this afternoon, she wet her diaper for the first time!!  For a short while, she gave us a little scare though, as she approached six hours without wetting any diapers at all. There was a short sighting of "momma bear" during the time that I felt there was a serious problem, and my concerns were not being addressed.  Her catheter was removed at 10:00 am.  Around 4:00 pm, she had still not wet any diapers at all, and she began refusing bottles and oral meds and appeared highly agitated. Her swelling appeared worse, and her abdomen was more distended. I told the nurse, who called the resident, who called the fellow, who said he was busy and asked her to call the resident again.  STOP. WHAT?  I was livid when I heard that the fellow was actually requesting the person one level behind him make a decision after that said person had just escalated the issue to him!!   I went out in the hall and exclaimed, "Does that even make any sense???  You can go ahead and make that resident's day, but not at Elise's expense.  I want the attending physician to see her within the next 10 minutes, or I am activating the RRT (Rapid Response Team), which is basically a patient/ family-powered "code" designed to alert medical staff and administration when a family feels the patient is getting worse and there is no action plan in place.  Well, like magic, the attending MD came in and quickly ordered a "in and out catheter" to drain and measure urine.  As soon as the word "catheter" was mentioned, Elise soaked her diaper!!!   Potty training she go well now that I know the magic word.}

^{Elise is now resting comfortably.  She is receiving pain meds as needed as well as multiple other meds, some of which she will be on indefinitely. Her primary attending hepatologist, Dr. Fishman, came to visit her this afternoon, although he is not on hospital service this week. He's obviously very pleased her her progress and expects that she could conceivable discharge home by this Friday 3/29/13, barring any major complications of course!  What a miracle have received this Holy Week, and the blessings seem to continue moment after moment. This Easter we are so deeply grateful for the unselfish decision one family made in their grief and darkest moments, to donate or donor angel's organs, thereby giving Elise "new life"-- her "gift of life!"}

^{Please always keep our donor angel and his or her family in your prayers!!}

^{Thank you all for love and support you all have shown to Elise and our family! We are overwhelmed and appreciate it all so much!!} 

Upping the Ante

For the past several weeks...maybe months...Elise's doctors at Texas Children's Hospital have said that we would soon begin to see significant complications of Elise's liver disease which would "stack the cards" or "up the ante" and ultimately pave the way to Elise receiving the liver transplant that she needs. True to their fashion, they were right, and that became very clear this past Saturday morning, March 9-- my birthday.

On Saturday morning, as I woke up to Elise screaming into the monitor, I said to David, "we should go to the rodeo today." As I walked into Elise's room to get her up, I knew what I was about to find just based on the smell. Then it was confirmed. I picked up my baby, whose diaper, clothes and sheets were soaked in dark black bloody stool. She was obviously having a MAJOR GI bleed. For the past two weeks, I had been noticing some intermittent black, tarry stools. But each time, I brought her in to the hospital for labs, and her blood counts were confirmed to be within normal limits, so we were sent home.  I knew this time would be different though. Strangely enough, I didn't really panic; we would not be going to the Houston Rodeo like we planned, but THIS was certainly "not my first rodeo."  We called the GI/liver team at TCH and drove Elise straight there.  With ziplock bags of the bloody diapers in tow, I marched Elise straight in to the ER and insisted that we go through the "rapid treatment area," as this was obviously an emergent, high-risk situation.  For the first time ever, we actually bypassed the medical students, residents, and fellows, and we were immediately seen by the ER attending physician.  She wasted no time in ordering  a slew of lab work including a "stat H&H"- hemoglobin and hematocrit- i.e. blood counts. Her hemoglobin had dropped to 6.8, which was critically low. As a result, Elise was admitted to the hospital under the care of the GI/liver team and received a blood transfusion. She was also started on IV meds including a Protonix drip to help with acid in the esophogus and Vitamin K to help with clotting. They drew labs ever 6 hours on Saturday, Sunday, and Monday, monitoring Elise's blood counts very closely.  

Yesterday, on Monday March 11, the director of our liver center and current attending hepatologist, Dr. Shepherd, took Elise down to surgery to find and treat the cause of the bleed, which he suspected would be esophogeal varices--basically, varicose veins in the esophogus that can burst and bleed.  As David and I sat in the OR waiting area, I didn't pray that they would find a beautiful esophogus; I prayed and trusted that Dr. Shepherd would indeed find the problem and FIX it!  The procedure took about 1 hour, and then Dr. Shepherd came out to explain the findings to us, and he brought pictures. He said that he found three large varices in the esophogus, including the "big bleeder" which was causing all of the problems this past week. In the pictures from the endoscopy, we could see the protruding veins and bloody residue. He explained that the "sclerotherapy" was successful- he injected a medication into the offending veins, which caused them to constrict and collapse, thereby stopping the bleed...at least temporarily.  PRAISE GOD!!  Of course, this sclerotherapy would need to be repeated at least every few weeks to prevent additional bleeds, but according to Dr. Shepherd, he feels that Elise will receive her new liver before additional sclerotherapy is necessary. He told us that as a result of this procedure, they have documented the esophogeal varices, which is the "last and most severe complication of portal hypertension secondary to the Biliary Atresia; it is absolutely life-threatening and will not be cured without a liver transplant." This has given the team the clinical support needed to increase Elise's PELD (pedicatric end-stage liver disease) score and attain a national listing status, which will allow Elise to receive liver offers from regions other than our own.  

This morning, prior to Elise's planned discharge, I was sitting with Elise on the sofa in our room, ruminating over incidental issues. Just as I saw the shadows of feet at our door and wondered which of the bright new enthusiastic residents was coming to complete his/ her assessment prior to the team's rounds, I was stunned....my thoughts momentarily stopped....in walked the transplant surgeon, Dr. Goss, and he came in like "a man on a mission. His objective was to take a look at Elise's abdomen. He commented, "Nice...she's a good size!" Then he asked whether I had any questions.  Bad idea.  I decided to narrow down my questions to just one: "Are you here to 'eyeball' her because you have an offer on a liver for her??"  He said "No, but we're going to find a perfect liver very, very soon." After he left the room, Dr. Shepherd and several other members of the liver/transplant team came in and talked to me for quite a while.  They said several times, in several different ways, that "it won't be long." I even got a much better timeline than I have ever been given before.  When the transplant coordinator scheduled our follow up appointment for March 27, 2013, Dr. Shepherd chimed in, "But Elise should actually be inpatient post-transplant by then; you probably won't need that appointment, but it is a formality." 

So, over the past 3-4 months, Elise has encountered several bumps in the road: an episode of "acute on chronic liver failure," cholangitis, an unexplained high fever, and now esophogeal varices resulting in a major GI bleed that dropped her blood count to a dangerous level. She is definitely "upping the ante."  While this all scares me, and it hurts me to see her getting so sick, I am also much more at peace with the plan for her.  I have  struggled with the idea of handing Elise over to the transplant team for surgery, not knowing what the complications may be, and whether I will ever hold her giggling little body again. It has now become very clear that there is no question of making the right or wrong choice; transplant is the only option.

Okay, well, I could go on and on, but I am officially exhausted.  Here's to hoping and praying that the subject of my next blog update is very good news.

Dry Run

Well, yesterday on 3/6/13 at 10:30 am, we got THE CALL for which we have been waiting for the last nine long months...a donor liver was available which was a match for Elise. We left home in a hurry and got to TCH at about 12:00 pm. Elise was admitted to the GI/liver floor, labs were collected, x-rays were done, and IV lines were started in preparation for surgery.  I was told that our transplant surgeon, Dr. Goss, had flown out 3 hours away harvesting the liver himself and would be returning around 8:00 pm to proceed with the transplant.

I ran into the attending hepatologist, Dr. Shepherd, while pushing Elise in a little pink car up and down the halls.  He indicated that he had just received a text that the donor liver appeared to be "pristine," a perfect match for Elise as far as blood type, size, and condition of the organ. In fact, he told me that although Elise needed a left lateral lobe of a liver and could not receive a whole, the surgeon was bringing the entire liver back to TCH with plans to split the liver and transplant the left lateral lobe into Elise and the right lobe into another 13 y/o patient awaiting a liver transplant at TCH.  What a miracle!!  This one liver had the potential to save two lives. 

Around 9:00 pm, I learned that the donor liver had arrived at TCH, and the surgeon was taking it to the OR to begin the process of splitting it into the two segments. There was such a mix of emotions: lingering shock, excitement, fear, anticipation.  At about 10:00 pm, we received news that added immense disappointment to the rollercoaster of emotions. Apparently, as it was being split, this organ became "unstable" and therefore unsafe to be used for transplant into Elise or any other patient. I literally could not believe the words that I was hearing, and it had to be repeated multiple times.  The nurse came in to tell me that discharge orders would be written and Elise could return home....without the new liver that she was expecting.  David and I sat there in the room for the next 2 hours basically in silence.  I was numb.  I didn't know what to think or feel.  I methodically typed in a status on FB: "Unfortunately...bad news...the donor liver became ustable and can not be used for Elise. So the surgery is cancelled." Then I proceeded to respond to texts, emails, and FB comments, which were all very encouraging and uplifting; however, I continued to struggle with the disbelief that this was actually happening.  Eventually, I realized what so many other "liver moms" and others were saying was SO incredibly true; that is, it's okay, because we  obviously don't want this particular liver, and God doesn't want this liver for Elise....it's defective, certainly not "pristine" as it initially appeared, and it means that it was not in fact the perfect liver for Elise.

I'd like to quote our dear friend and fellow "liver dad," Bruce: "Organ transplantation is a modern miracle because it's hard to do.  Tonight, after a long day of waiting, the donor liver became unstable...and Elise will have to wait for another wonderful family to give the gift of life under the worst of circumstances.   This illustrates how important it is for us all to be donors should we die in such a way that it's possible to use our organs and tissues.  Tell all your family your wishes now. Wills and directives to physicians can be overruled by uninformed family members and usually are not accessible at the time. We need lots of donors because not all organs are suitable." 

Just wanting answers…and coffee…and...

The only thing worse than weekends at Texas Children's Hospital is weekends at Texas Children's Hospital without decent coffee and without an attending hepatologist (liver doctor) in-house or even on call. That being said, we spent almost this whole weekend in the ER at the mercy of the ER physicians, fellows, residents, and interns…

Let me start with yesterday, Saturday, March 2.  Around 4:00 pm, Elise suddenly spiked a high fever of 104.4.  The only symptoms were the fever, rapid breathing, grunting, and of course the past two days of bloody  stools. I called TCH and had the GI/liver doctor on call paged.  A GI fellow responded and recommended that I bring Elise in "to be evaluated by the GI/liver" team," so that's exactly what we did.  Once we were registered, triaged, and admitted to the ER, the ER doctors ran some labs, a urinalysis, and viral studies to try to determine the source of the fever.  Her ears, throat, and lungs were clear.  Preliminary results on viral studies were negative. Flu & RSV tests were negative. Her urinalysis was "questionable," but yesterday the ER doctor was not ready to call this a urinary tract infection and/or prescribe any antibiotics. And last but not least, her liver panel was not much changed from her baseline.  So the logical thing to do, apparently, was to discharge Elise with an unresolved fever and no plan, other than to "return to ER for worsening symptoms." Wrong. 

At 2:00 am this morning March 3, I woke up to Elise screaming and grunting, breathing rapidly, and appearing beet red and listless. Her rectal temperature was 104.9. I gave her a dose of Tylenol followed by a 1-hour sponge bath. She eventually went back to sleep with her fever down to about 101-102.  At 8:00 am, I gave her a second dose of Tylenol and called the GI doc on-call again at TCH.  I noted him to be giving me the "run around...not-sure-what-to-say-because-I'm- a- fellow- and- my - attending- is- not- a- BA specialist." I was very frustrated and called Elise's pediatrician at home on her cell phone.  She was immediately very helpful and was angry to find out that we were sent home from the ER last night with no treatment plan. She called down to the TCH ER and then called me back and told me to bring Elise back there, that they were expecting us.  So, again, that's what we did.  

We registered, triaged, and were again admitted to the ER.  I swear, within 20 minutes, the doctor tells me, "we think it's probably a UTI based on the urinalysis taken last night, so we can probably send you home on an antibiotic, Suprax."  Wrong. I explained to this doctor that we  were going to need Elise to have a complete work up to rule out ALL possible sources of infection rather than just blaming it on a UTI. I reminded her that in January, Elise was suffering from full-blown cholangitis, while everyone was claiming it was "just an ear infection."  After approx another hour of arrogant ER staff run-around, I emphatically stated my expectation: I want a HEP-A-TOL-O-GIST (liver doctor) down here to physically examine Elise and her test results and make some recommendations.  And no, it was not sufficient for the ER to speak via telephone to a GI fellow who has never seen Elise or reviewed her case and probably knows as much about Biliary Atresia as I know about football....just the basics. At this point, I was told that the liver doctors at TCH are not always "on call" or assigned to hospital service on the weekends...and this was a weekend where they were not available.  Wrong again.  THAT, my friends, is not my problem...certainly not Elise's problem...and Elise will not suffer because of it.  Not long after this, the GI fellow came in to proudly annouce that he had contacted Elise's primary hepatologist at home, and as a result, now had orders for a complete abdominal ultrasound to r/o signs of cholangitis, peritonitis, or other possible sources of infection. FINALLY....a plan that makes sense!  So, we spent the next few hours following through with that plan.  

Around 4:00 pm this afternoon, the ER doctor came in to review the results of the abdominal ultrasound.  She informed us that there was no evidence of infection in the abdominal cavity which would suggest cholangitis or peritonitis; however, the ultrasound did show that her "right portal vein is smaller."  I asked, "WHAT DOES THAT MEAN?"  the response?  "You'll need to follow up with the liver team this week."  Well, I'm trying very hard not to jump to conclusions, but the only thing I can think is that she meant that her portal vein appears to be narrowing, which is not usually a good thing and can cause big complications for transplant. 

So in the end, we left the ER this afternoon for the second time this weekend with Elise still running fever of 101.9 and still more unanswered questions than resolutions.  At least, this time we have started Elise on an oral antibiotic to treat the infection, wherever it may be.  

Now, while I don't have a very clear idea of what THE plan is for Elise as far as addressing her ongoing bloody stools and apparently "smaller right portal vein," I do have my own plan.  I have already emailed her primary hepatologist, outlining my concerns. Tomorrow morning, at 8:00 am, I will contact the transplant coordinator and liver center director regarding the events of this past weekend, that of which I have documented a tamed version here in this blog.

I just want answers.....and decent coffee....and effective treatment by Elise's attending LIVER team....none of which seems available on the weekends.  

Pins & Needles

I meant to update last night, but I obviously didn't get around to it. Yesterday, I brought Elise back to TCH for another hemocult. Unfortunately, the results are still positive for blood in her stool. In the absence of Elise's primary hepatologist (liver doctor), the director of the liver center reviewed the results and states that Elise likely has a small, slow bleed high up in her esophogus. Because her hemoglobin levels are still within normal range, her condition is not critical. However, she will go back for more labs next week, and if her blood counts drop significantly, she may require admission to the hospital for a blood transfusion. In the mean time, I'm supposed to monitor her closely and "bring her in right away if she vomits blood or produces bright red blood in her stools."  As if they had to tell me to bring her in to the hospital in such a case.  

Anyway, I normally like to post cute, sweet pictures of Elise. But for the purposes of this blog entry, I am including a picture of her which shows her protruding belly, which is becoming more and more distended these days. She is about as physically comfortable as I was when I was 8 months pregnant. Even still, she is almost constantly smiling, which really amazes me. I worry about her developmental milestones though. For example, she is still not walking at 16 months old. Her doctors and physical therapist tell me that it is likely her big belly on her small frame which is to blame; they say she will catch up quickly post- transplant.

Transplant. The primary focus of my daily thoughts. Considering the most recent developments and progression of Elise's liver disease, her treatment team is planning to request additional exception points on her PELD (pediatric end-stage liver disease) score, thereby heightening her placement on the transplant wait list. With her current score though, she is already "next" in this region for her type/size liver, so the increased score will not actually change her status at this point. 

So towards the end of my conversation with the doctor yesterday, I asked, "So, regarding this esophogeal bleed, I just have to sit around and wait for it to get worse before anything can be done?" In not so many words, the response was "yes." Actually, what I was told is that these problems that Elise is now incurring are very concerning but not unexpected with Elise's diagnosis. These are the very reasons that she has been listed for a liver transplant. The team has prepared well for his, which is why she is now sitting at the very top of the transplant wait list at the time when she is becoming more ill. Again, I heard the words that I have heard time and time again. That is, Biliary Atresia is a progressive liver disease which is fatal if not effectively treated; her liver will not get better; in her case, the only effective treatment is a new liver. So that is what we await and pray will come very soon.