Back & Forth

So, obviously I forgot to update this blog last week when we went home. We were discharged last Thursday 1/24/13. Elise has a PICC (peripherally inserted central catheter) placed, so we were sent home with 21 days of IV antibiotics to be administered at home every 8 hours. Things were going fairly well, until Monday, when I began to notice that she was a bit "off." As the day went on, her face became more and more drawn and grayish in color. Her eyes were dark and sunken in, she was having a lot of watery stools, and I was certain that she was dehydrated-- at the very least. I called the transplant team at TCH, and her doctor told me to bring her in to the ER immediately for a full evaluation.

In my experience, an ER visit for a "full evaluation" almost always equals an admission, at least overnight, so I packed a bag, and we were on our way.

Upon my arrival to the TCH ER around 5:00 pm, I speed- dialed our transplant coordinator to make sure she had called ahead to the ER staff so that we would not have to wait. I'm sure you can imagine the discouraging scene that greeted us in the lobby of the ER at that time in the afternoon. Anyway, one of the "perks" of being a "transplant patient" is that ER triage is expedited, and we go straight back to a exam room rather than waiting in the lobby, minimizing the infection risk. The ER doctor took one look at Elise and stated that she appeared severely dehydrated. This was confirmed with labs--the results being quite alarming. Her sodium level was extremely high at 169. Normal limits for sodium is 135-145. Any level over 158 is considered critical, and a level of 180 or above would pose risk of coma, seizure, and/or death. The liver team was consulted, and doctors began working on a plan to bring down her sodium level; however, it was imperative that that the level be lowered very slowly, as a sudden drop in serum sodium can cause swelling of the brain. I felt that the medical staff was handling the situation approriately, but I still insisted on obtaining copies of all the results and normal ranges, and I was doing my own research for most of that first night. If there's one thing I can't stand (and believe me, there's more than one thing), it's having doctors just summarize everything, assuming that I might not understand the details. I am very number-oriented related to labs, and I want to be fully informed....of every detail. Her attending liver physicians, and even a couple of the fellows, know this about me, but I'm still training the residents. So, Elise was admitted to the GI floor at TCH on this past Monday night, 1/28/13. We have been here since then, and they have been drawing labs at least twice/day in order to closely monitor her electrolytes--particularly sodium and potassium, complete blood count, and liver panel. On a good note, her liver enzymes and liver function tests are trending closer to her baseline as compared to last week; however, her numbers are, of course, no where near normal levels.

This morning, Elise had a consult with the TCH dentist. She was referred for a dental eval by the transplant team to rule out any cavities or brewing root infections that could cause additional problems pre- or post-transplant. Biliary Atresia is known to cause dental problems due to the associated nutrient malabsorption issues and vitamin deficiencies. Anyway, the dentist noted that Elise has no cavities currently or serious dental problems; however, she does have a defect in one tooth, which is likely related to her low vitamin D level. I was told to use regular tooth paste with fluoride to brush her teeth rather than the baby tooth paste without fluoride. The dentist did seal her baby teeth today though to strengthen them and hopefully help to prevent cavities from forming.

And now, finally, we are going home again today. Her labs have stabilized and she has been able to maintain hydration without IV fluids for 24 hours. We will continue the IV antibiotics at home for the next 2 weeks, and we are scheduled to follow up with outpatient "liver clinic" on Monday 2/4/13.

Long Days

The days in the hospital seem so LONG! And yesterday was no exception. It ended up being a good day, but it certainly did not start that way; "Momma Bear" had to make another appearance. Elise was scheduled for to down for placement of her central line at 8:00 am yesterfday morning In preparation for this, the nursing staff kept her NPO- "nothing by mouth" starting at 2:00 am. Around 8:05 am, the nurse came in and tells me that I can go ahead and feed her because she has been bumped until tomorrow for the procedure. I'm not sure of my exact words, but it was along the lines of, "No, that's not happening; I will feed her after she gets her central line placed, which WILL happen today as planned." 

I demanded to speak face-to-face with the attending physician, patient advocacy representative, and a hospital administrator. Of course, the GI resident pops in, and I may or may not have asked him whether he had become an attending physician overnight, and if not, he needed to go and get an MD who makes actual decisions. So then the coordinator from Interventional Radiology came in asking if I wanted to "discuss my concerns." I told her, very politely, that the next person who comes into our room needs to be able to fix the obvious problem. Finally, a hospital administator showed up, and this guy was actually ready and able to take care of business. I explained to him that I could not even fathom the idea that TCH would allow a 15-month old child with a known history of failure to thrive and compromised nutritional status to be kept NPO for 6+ hour in preparation for a procedure, only to "bump" the procedure until the next day intending to repeat the whole routine again--THAT, I emphasized, is unacceptable, irresponsible, and in fact detrimental to her health. He took notes and then stated he would make some calls to see if he can resolve the problem. He returned within minutes, with our attending physician in tow, and I was told that "a breakdown in communication" had occurred, but it was taken care of, and Elise would go for her procedure at the next available time slot. Literally, within the hour, she was on her way down to the Interventional Radiology department for placement of her central line.

Okay, so moving on…

The liver team rounded as usual, and we reviewed Elise's curent labs, which look terrible; in fact, her labs have never been worse. On a positive note, Dr. Himes informed me that she is currently NEXT in line in this region to receive her type liver!! We discussed the fact that Elise's deteoriating condition is part of the normal disease process of Biliary Atresia. They reminded me that they have told me from Day 1 that she would likely require a liver transplant within the first 2 years of life. They also reviewed the fact that this situation is exactly what they have been expecting and planning for; it is the precise reason that they moved to list her for tranplant "early" 7 months ago, and as a result, she is now sitting right at the top of the wait list at the moment when she is becoming really very ill.

I also had the rare opportunity to speak with our transplant surgeon, Dr. Goss, yesterday. I had mentioned to our attending physician that I had a "list of questions" specificially for Dr. Goss, but I was not sure that he even heard me much less that he would actually facilitate a visit. Apparently, this "momma bear" is being heard these days!! LOL!!

Anyway, included in my conversation with Dr. Goss was the following:
a) Elise needs a "left lateral lobe" of a liver rather than a whole liver due to her transversed organs and malrotated intestines.
b) Related to any expected complications, Dr. Goss said, "Just forget everything you read on Google; those are national statistics, not TCH statistics." While I do appreciate his confidence, I still probed further, asking about specific complications such as Hepatic Artery Thombosis (HAT) and primary graft failure. He responded, bluntly, "Those things do happen--rarely- maybe once/year, and when they do, it's catasrophic." He then went on to review measures that they take in order to avoid these complications in most cases.
c) Living Donor tranplant as an option for Elise. Dr. Goss says he does not feel we need to aggressively pursue this route for Elise, as he feels fairly sure that they will be able to obtain a deceased donor liver for her, which will work wonderfully.. Of course, he said it can not hurt to have testing done and line up a living donor "just in case." Also he indicated that he and his partner, Dr Mahoney, would be doing both surgeries if we needed to move forward with a living donor transplant.
d) I asked whether they ever go into transplant and find the native liver to actually be in better condition than they expected based on the labs and imaging. He stated that "old livers" in patients with Biliary Atresia are cirrhotic or otherwise not compatible with long-term survival in 100% of cases that go to transplant. He further indicated, specific to Elise, that he suspects her portal vein may be in worse shape than it appears on ultrasound; he stated that he reviewed her many ultrasounds and is not sure that the vein identified as the portal vein, which does show blood flow, is actually her her portal vein. Without going into more detail on this, I will just say that I am very relieved that tranplant will likely be happening sooner rather than later.

So, it is now the early morning on Thursday, January 24, and it is a brand new day! I hope and pray that it is a good day which brings us good news. And as always, we would welcome the best news possible, "the gift of life."

NOT Just an Ear Infection

As much as I hate when my son, Ethan, is sick, at least I can assume it's just normal "baby stuff." Not so in Elise's case. This past Saturday, Elise woke up with a fever of 103.2. She was not acting like herself, and I thought she appeared a little jaundiced. I brought her to the pediatrician, as I was previously advised to do prior to calling the hospital, to look for any "normal baby" explanations for the fever. Her regular pedicatrician was not in on the weekend, so we saw someone else, and this covering pediatrician stated that Elise had an ear infection and sent us on our way with a quick script for an antibiotic. As the day went on, Elise's condition seemed to get worse; her fever would not really break with Tylenol, her skin was clearly yellow, and she seemed in pain- grunting, breathing fast, and screaming every time I would put her down.  

At the risk of possibly seeming neurotic, I called the liver team at Texas Children's Hospital and brought her in to the ER.

Almost immdiately, I was thanking God that I did follow my intincts; all of her liver enzymes were double and triple their baseline for her, and her liver function tests were significantly decreasedr. An ultrasound of her liver showed dilation of her biliary ducts and enlargement of her liver. She was admitted to the hospital to be treated for a seriousl liver infection, Cholangitis. Obviously, this was much more than just an ear infection.

On Sunday, things got a little worse, as we were told that she was possibly septic; bacteria was found in her blood, and her vitals were consistently elevated. I spoke with several liver doctors as well as the director of the TCH liver center and was told that all evidence indicates that her liver has incurred further damage. They also said that it appears there is very little "reserve" left in her native liver, so time is becoming more and more critical. They indicated that they were requesting an increase in her PELD score to take it from 28 to 32, placing her at very high priority for a donor liver. However, due to the malrotation of her intestines and transversed organs (related to her liver disease), she needs the left lateral lobe of a liver, which will take longer to find. Considering all this, the team informed me that they were now beginning to entertain the thought of testing for living donors.....just so that we might have a "back up plan" in case time runs out and there is no other choice. Of course, I completely agree with this plan, and I will be the first one tested to determine whether I am a viable match to be a living donor. I know that I am the same bllood type as her, A+, so now further testing will be done at St Luke's hospital.

A "living donor" transplant means that, assuming they find a willing live person who matches Elise's blood and tissue type. they would remove a small percentage of the donor person's liver and place it into Elise. As previously stated, this would be a last resort, although the prognosis would be at least as good if not better.

I can certainly say that the last two days here at the hospital have brought "momma bear" out of me. I have learned time and time again that I am Elise's best advocate, the one who knows her best. I've also learned to challenge and question anything and everything that doesn't seem 100% correct, as mistakes DO happen. I won't go into all the nitty gritty, but suffice it to say that a resident MD here made an order yesterday which could have potentially caused Elise to be inelligible to receive a tranplant if an organ had become available. This was based on a completely inaccurate and unresponsible assessment of her, noting that she was to be on "contact and droplet precautions due to suspected respiratory infection or virus." I am not a doctor, but I immediately questioned this. as her oxygen saturation was perfect. her viral studies were all negative, and she had no symptoms of a respiratory infection...other than a nasal sound that is part of her baseline presentation when she is upset or worked up in any way. I could have explained this if he has bothered to take a history on it before running off to make his orders. Anyway, I demanded that the attending physician, the supervising infectious disease doctor, come to evaluate her. She was thoroughly assessed from a respiratory standpoint, as I requested, and the situation was rectified. All special precautions were promptly removed, and it was documented in her chart that she is clear for surgery should it become necessary.

So, today she is scheduled to go down to interventional radiology for placement of a central line, a peripherally inserted central catheter (PICC) which will allow her to go home on 21 days of IV antibiotics once she is stable; this is the normal protocol for treatment of the Cholangitis liver infection. Her doctors are anticipating that she will be ready to go home by the end of this week.

Once again, thank you all for the continued prayers, love, and support. I know that this is all part of God's plan, and I have faith that Elise come through all of this just fine, hopefully with a brand new, healthy, left lateral lobe of a liver very soon!!! :-)