A month in review...

Today is June 8, right?  Yes, well then I have a little catching up to do….

Exactly one month ago,  Elise seemed to have a "cold." She frequently develops "reactive asthma" or "reactive airway" symptoms when ill, so I decided to get myself an early Mother's Day gift….a pulse oximeter. Well….within 12 hours of my coming home with this new gadget, it showed a significant decline in Elise's respiratory status…..so concerning that I paged the liver/transplant doctor on call and left for the ER rather than waiting on a call back.

Upon arriving in the ER, her vitals were critical, and she was placed on "shock protocol." Believe me when I say….there was no waiting around.  She was immediately triaged, and as soon as the triage nurse uttered the words "she's shocky," we were put in an exam room and quickly greeted by the ER attending physician, all available nurses who were simultaneously completing assessment, drawing labs and cultures, and starting IV fluids, the radiology tech w/ a portable x-ray machine, and a respiratory therapist who was taking a nasal wash sample for viral studies and starting Elise on oxygen.

                     

Her WBC (white blood cell) count was 17,000, and her chest x-ray showed pneumonia with a collapse in the left lower side of her lung. Her urinalysis and urine cultures were also positive for a urinary tract infection. She spiked a fever, which was > 102 by the time we arrived in the ER, and her temperature did not respond to the oral Tylenol given upon admit, so she was given IV Tylenol, approved by the liver doc. IV antibiotics were also started.  Given her immunosuppressed status, she was admitted by the liver/transplant team critical care team to the PCU.

Here is the blurb that I wrote on Facebook on May 12…

Waiting on docs to round, so no "official word" yet. Between not eating in 24 hrs and doing my crunches all night to see her monitor each time it alarmed, I'm tired and very sore. Lol. Elise is still on 10 liters o2 high flow with o2 sats sitting at about 92%, occasionally dropping to 90%. Heart rate and temp are much improved though. She's still breathing fast, in the 40s. They are monitoring her closely- we are still in the PCU. Labs from this morning are still pending. None of her cultures or viral studies have come back positive to my knowledge. So currently we have the pneumonia, collapsed bottom left lung, and possible UTI. She is on 2 IV antibiotics including Vancomycin and fluids. Her output seems adequate though I am interested in her actual kidney function on her labs.

Later in the day on May 12, I was able to meet with the liver team, and we reviewed Elise's liver panels. Her liver enzymes showed significant improvement since beginning the IV antibiotics, so my thought was that maybe this respiratory infection has been brewing for several weeks, and this has been the problem with Elise's labs!  Liver team agreed. 

Mother's Day came and went while Elise and I remained at TCH.  David visited with Ethan each day, and my Mother's Day present was a small/portable size Keurig coffee maker and K-cups!!  I mean….I have to say this is BRILLIANT!  I have frequently complained about the coffee available on the floor….and it's not always easy or affordable to take a walk to the Starbucks. I planted this idea in David's head, but I must credit my "liver-mom-friend," Dorothy, with this notion.  I remember visiting Dorothy and her baby boy, Jason, and noticing the Keurig machine in their room.  I was literally stunned and could not believe that I had not thought of this after more than 20 admissions to TCH in the past two years.  

Here is the blurb that I posted on Facebook on March 13….

Worst night we've had in a long time last night. Elise was extremely agitated, ripped out her IV and repeatedly pulled off leads and o2... She kept de- satting and took a few steps back on her o2 wean... Around 2 am after second back to back round of albuterol, she really went nuts. At that point I requested to speak to the attending ICU doc and discussed my concern that this was all being caused by the albuterol, and that we should switch to Xopenex which is what she is prescribed at home, and that's in her medical record here. He agreed and changed the order. Since 4 am she had been doing much better... Much calmer and down to 5 liters o2. ICU team says she is better, but not out of the woods, and probably several days or a week from being able to return home. ICU doc took a second look at the chest X-ray on my cell phone from March (when she swallowed the penny) and had me email the film to him so that he can do some comparisons and possibly consult pulmonology to look into whether there was a pre-existing issue with her left lung that could have exacerbated this whole situation. Waiting on today's word from liver team which will include a review of her labs. 

March 14….Elise started the day being given a trial without supplemental oxygen.  And she ended the day discharged home.  Prior to discharge, I mentioned to the resident MD that I was suspicious that Elise was about to spike a fever, as her face appeared flushed.  I was assured that this was probably just a side effect of the IV antibiotics.  Hmmmm……

On May 17, we were on our way back to the ER at TCH. Elise's temperature spiked to 102.2,  she was not urinating, and her oxygen saturation was again dropping. Liver team wanted her back in. Her chest X-ray  showed more inflammation than it did previously, so she was re-started on the IV Vancomysin and Zosyn….and an additional antibiotic was started….Azithroymsin, which treats mycoplasma pneumonia. She was also of course put back on supplemental oxygen.

                             


May 18…. David took over with Elise at TCH, and I went home to spend time with Ethan.  I hated leaving the hosital…ahem….I hated giving up control of the situation….but I really didn't have another choice.  David happened to be scheduled off the next day, and I need to work whenever possible to preserve my available PTO days for when I really need to take them. So I swapped off with David, returned home with Ethan, and brought Ethan out on a Mommy-Ethan date to Chuck E Cheese!!

                         


On May 19, Elise was discharged in stable condition, to continue oral antibiotics, and Daddy brought my baby girl home!!

Since Elise has been home, she has been doing GREAT!  During her hospitalization, one doctor mentioned the possibility of acquired tracheomalacia as possibly triggering Elise's respiratory issues. My first though was "no," but then I am prone to denial….so after a little research and discussion with Elise's primary hepatologist, Dr. Fishman, transplant coordinator, Sarah, and pediatrician, Dr. Pocsik, I decided I would feel most comfortable with a pulmanology consult…..as soon as possible.  So we have an outpt appointment scheduled with a TCH pulmonologist, Dr. Farber, on June 10.

"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.

Elise's primary hepatologist, Dr. Fishman, during his portion of the presentation  

Dr Himes captured this SELFIE
from left: 1/2 of Dr Fishman's face, me, Harriet & Eleanor (liver friends), and Dr. Himes

Thank you all for continuing to follow Elise's journey. We appreciate the support and prayers so very much!! 

Not Stressing...

Our transplant coordinator, Sarah, and I spoke several times over this past week. Today, we spoke again on the phone and exchanged several emails. Sarah says, "Erin, don't stress." I can't imagine why she thinks I would be stressing.  Well, there might be a couple of things…

Last month, for Elise's annual transplant review clinic, her labs were great;  she was essentially at her baseline:  AST 56, ALT 52, GGT 24.

This past week, however, her liver panel has been elevated, and there is no clear explanation. We have had labs drawn three times within the past 7 days. Following the first draw last Wednesday, after confirming her CMV result to be negative, Dr. Fishman increased her Prograf (anti-rejection med) from 1 mL twice daily to 1.5 mL twice daily. Even with this med adjustment, her liver panel is not exactly back to baseline, which worries me.  BUT, the good news is that her numbers are trending down, even if it is just slightly. 

Here is a review of her liver panels the last week:

Wed 4/16/14:  AST 94, ALT 139, GGT 43

Fri 4/18/14:  AST 86, ALT 118, GGT 42

Tues 4/22/14:  AST 80,  ALT 114,  GGT 38

I also inquired about her kidney function tests today. Prograf is notoriously hard on the kidneys, and since her Prograf dose has been bumped up a little, I just wanted to be sure everything looks good there and that she is well enough hydrated, etc.  Her BUN is 14, Creatinine is 0.24-- both very normal! 

Today Elise's WBC (white blood cell count) was 5.99, which is low (normal is 6-20), so Dr. Fishman suspects she may be fighting off a little bug in her system.  I also pointed out that her Prograf level is still 4.4, even with the increased dosage, and historically, she does not absorb Prograf as well when she has some sort of acute illness such as a cold. She does not currently have any symptoms of a cold or other virus, but I will be watching her carefully and will bring her to the pediatrician at the first sign. It is very important to be proactive and treat acute illnesses, even minor issues, because any infection can stimulate her immune system and possibly trigger a rejection episode. That being said, her doctors say that they are comfortable with the fact that her numbers are trending in the right direction, so we will just continue to monitor very closely.  We willl repeat labs on Sunday 4/27/14, and we are officially cleared to then proceed with our vacation plans, which will take us home to Louisiana for a few days, and then to Orange Beach!

So....repeat labs on Sunday morning.  Which means I will have to wait until Monday for results. Sarah gave me a friendly reminder today, "You don't need to call the liver MD on call to get results on Sunday; I will call you on Monday." PSHHH.... like I would ever do that!  lol.  Oops.

Anyway, this Saturday, April 26, 2014, TEAM ELISE will be participating in Liver Life Walk Houston 2014 for the 3rd consecutive year to benefit the American Liver Foundation. Elise will be leading her pack...probably in sparkly pink high heels, if I had to guess.  

Thank you all for the tremendous support you have all shown our sweet girl. She is so brave, and she amazes me every day. I don't know that I've ever had six tubes of blood drawn at one time ever, much less multiple times/week. But Elise endures this and more, and she does so with a smile on her face. 

One final note.... April is National Organ Donor Awareness month!  Elise was proud to be featured on the TCH Liver Transplant Center display board for the donate life day held on April 10. We attended the event, and we were very excited to meet the captain of Team Texas for the Transplant Games of America. He recognized Elise as soon as we walked up to the table....she is the youngest member of Team Texas, and she will be proud to participate in honor of her hero, her organ donor, Julia.

Elise checking herself out out on the TCH Liver Center display board!!

Elise and me with our team captain (TEAM TEXAS) for the Transplant Games of America

Elise's 1st Transplantiversary

We celebrated Elise's 1st Transplantiversary, March 23, at our house with our amazing friends and family. Thank you to everyone who came out to celebrate with us. And most of all, thank God for Elise's donor angel, Julia, and all organ donors, who make days like this possible.

1 YEAR ANNUAL Transplant Review Clinic

Can you believe this week makes 1 year since Elise received her liver transplant?  It seems surreal to me.  I vividly remember this time last year. I was living minute by minute, day by day....and I could not imagine that on March 19 of 2014 we would be at our 1 YEAR ANNUAL transplant review clinic. But here we are.

Elise and I spent most of the day today at Texas Children's Hospital, and I must say it has been a wonderful day! We started out having her labs drawn at 7:30 am.  We then went to the West Tower 12th floor inpatient unit and visited with a new BA family--yes, we have a new baby boy in our "liver family," who is 5 months old and awaiting a liver transplant. It seems like only yesterday, WE were the new BA family at TCH. Anyway, the emotions they are feeling right now are all too familiar, and it just tugs at my heart. Anyway, Elise and I spent about an hour talking to them and sharing our experience and hope.  

At 9:30 am, we arrived to clinic. We first saw our transplant coordinator, who reviewed all of Elise's lab results with me. She walked in the door saying "Her numbers look great!" Which immediately put me at ease. In fact, her "numbers" are some of the best she's ever had! 

AST 54, ALT 56, GGT 24, conjugated bili 0.0, total bili 0.2, Albumin 4.8

Her CBC (complete blood count) and CMP (complete metabolic profile) are normal!  Her Prograf level is 4.4, which is perfect (goal range 3-5.) 

Dr. Fishman came in saying "She looks FANTASTIC! And BIG! What's she been eating, besides pennies?" Elise was seen in the ER this past Saturday after swallowing a penny. He was happy to hear that I "confirmed" the penny came out on its own in her diaper the next day. "Better you than me," he said. Haha. Apparently, Dr. Fishman routinely fishes foreign objects out of toddlers via endoscope. So yes, better it was me than him.

Anyway...moving on.  Dr. Fishman had a medical student with him, so we reviewed Elise's entire history from age 6 weeks, when Elise was first diagnosed with Biliary Atresia.  It was nice to hear Dr. Fishman's summary, although I'm quite sure I could not begin to summarize our experience in 3 minutes. Dr. Fishman then completed his exam and reviewed her current labs again with me. He and our coordinator emphasized the importance of this milestone-- being 1 year post transplant. They indicate that the worst should be behind us now, although there will always be the possibility of bumps in the road. 

Near the end of our clinic appt, our dietician, Stacey, came in to review Elise's current "growth trends" and "dietary needs." Elise's current weight is 29 lb 15.4 ounces (75th percentile,) and her height is 2'11" (50th percentile.) Stacey advised us to switch Elise over to skim milk rather than whole milk, no more than 20-24 oz. per day. She needs to have a well balanced diet high in protein and veggies and low in sugar and fat, with fruit for snacks.  These recommendations are meant to keep her new liver nice and healthy and functioning at its highest potential. 

After clinic, Elise had her LAST pentamidine treatment. She was required to take inhaled pentamidine treatments once/month for 1 year post transplant to prevent PCP, a very dangerous type of pneumonia. She never really fought the treatments, but it's nice to be relieved of them now.

We ended our day at TCH by meeting up with our very good liver friends, Tara and Abby.  Elise and I waited with them for Abby's labs, which gave Tara and I lots of time to chat and catch up while Elise and Abby built a lego choo choo train. Then we all went down to the food court for lunch. Dr. Fishman and Stacey would be proud to know that Elise ate grilled chicken and fruit with no complaints! 

AND NOW....we are officially free to celebrate Elise's 1st Transplantiversary this Sunday, March 23. We've planned a special gathering of family and friends. We'll have a balloon release in honor of Elise's donor, Julia, and organ donors everywhere. As we know, "Without the organ donor, there is no transplant, no hope, no gift of life." 

Thank you all for your continued prayers and support and for continuing to follow Elise's journey.

Bombshell

Things change so quickly around here in Liverland. As a result, I have been putting off writing this post for quite some time.  I do want to keep you all up to date though, so here it goes.

Last month, on January 17, I received a phone call from our transplant coordinator that rocked my world. You see, I was under the impression that the biopsy which Elise underwent in December was the "ultimate" test with definitive results. Elise was taking her oral steroid medication for what doctors assumed was acute, mild rejection. David and I reviewed the biopsy results with her doctor, and all evidence did point to rejection, so I had accepted this as matter of fact, and we were dealing with it.  So, you can imagine, when our coordinator called with the following information, my brain froze; it was like "does-- not-  compute." She said, "Erin, I have good news and not-so-good news.... we know why Elise's labs have been so elevated, and it's NOT REJECTION....  the other thing is that she needs to be admitted again...tonight...to start IV Gancyclovir for the treatment of cytomegalovirus (CMV). After about 15 minutes of Sarah repeating herself and explaining her directions, I understood that she would call back when a room on the transplant floor was ready, and we would then go over to TCH for Elise to be admitted.  

Elise was admitted on that Friday night, the night before David's birthday, and we spent the next 5 days in the hospital. True to our fashion, we were admitted on a weeked, when liver team is not on service in the hospital. Thankfully, Elise's primary hepatologist documented admitting orders as well as a very specific treatment plan. Since we were no longer treating for rejection, the steroids were immediately discontinued, and her anti-rejection medication was decreased to the previous dosage which had done well with her for almost 9 months prior to this whole episode.  Elise was started on IV Gancyclovir (anti-viral) medication twice daily. Prior to discharge, Elise had a PICC (peripherally inserted central catheter) placed, and we agreed to a plan to continue IV Gancyclovir at home for 4-6 weeks to ensure that the virus is completely eradicated.

Elise has been home now for the last couple of weeks, and the improvement in her labs is astounding!  

Her AST has dropped from 250, to 78, to 53.

Her ALT has dropped from 287, to 104, to 52.

Her GGT has dropped from 181, to 61, to 27. 

NEXT MONTH will be Elise's 1st annual transplant review clinic.  I can not believe it's almost been a year since Elise received her gift. The improved quality of life she enjoys now is just amazing. I think about where we were at this time last year--and where we might be now had she not received her gift of life when she did-- and I just cry. 

As we gratefully anticipate Elise's 1st "transplantiversary," we know very well that there is another family--Julia's family-- still grieving. Every single solitary day, this weighs heavily on my mind. I hope that Elise will always be a living testament of Julia's "gift" though. With this in mind, "TEAM ELISE will be participating in Liver Walk Houston 2014 on April 26, 2014.  Elise is also registered for the Transplant Games of America, which Houston is hosting this summer. 

Elephant in the Living Room

Those of you who follow my blog and Facebook page may have noticed that I have been MIA from the blog lately, and I have neglected to address the "elephant in the living room." Call it a busy schedule...or denial....or exhaustion....or a little of all of the above. I think I have done a decent job of holding everything together over the past month, but the thought of sitting down to write about it did not appeal to me.

Today, I'm in a better place, so here it goes.

On December 19, 2013, I brought Elise for "routine labs." Typically, I wait excrutiatingly long hours following lab draws to get a call with results, but this day was different. When my phone rang with the TCH number at 9:30 a.m.-- about 15 minutes after I arrived home-- I knew something was very wrong. Our transplant coordinator said, "Erin, don't panic (yeah, right!) but please bring Elise back to the hospital right away to redraw her liver panel. She gave me "the numbers," and if I had had a chance to eat that morning, I would have probably been sick. Of course, I did bring her back, but the results were the same; these "numbers" were real. 

AST 531 (up from 64,)  ALT 417 (up from 66,)  GGT 72  (up from 17)

Elise was admitted to TCH for a liver biopsy the next morning, December 20, which confirmed  mild rejection. Of course, we received results of Elise's biopsy on a weekend, and I'm pretty sure this was the first time this resident MD ever presented results of biposy showing rejection.  She was using those vague terms that I despise such as "consistent with" and "suggests...."  I let her finish her review, and then I asked, "So, is this rejection?" She said, "It shows inflammation of the liver that is consistent with rejection." I asked, "So you will treat for rejection?" She responded, "Yes." Finally, I asked, "On a scale of 1-10, "1" being the mildest rejection, and "10" being the most severe rejection, how do you rate it. Our resident MD looked at the attending physician, a GI doctor, who said he would rate it a "4," but he suggested I discuss the results more thoroughly with the hepatologist, Dr. Fishman, on Monday.....as if I wasn't already emailing him in my head. 

Shortly after the biopsy results came in, Elise was started on high dose IV steroids while hospitalized at TCH. Thankfully, the side effects were minimal, other than a largely increased appetite. Since Elise had no physical symptoms of illness whatsoever, she and I made the best of our 3 days there.  We took walks to Starbucks and to the ice cream shop; we walked around in the gift shop and bought Elise little "treats" and "surprises" to reward her for being such a trooper; we visited the child life specialist; we read books in the library; we saw Santa Claus; we did "arts & crafts;" we played with toys in our room. Grammie & Paw visited, and so did Daddy and Ethan. Although I was terrified on the inside, I did my best not to show it or reflect that on Elise. Also, since Elise had no symptoms, I advocated for her to be left alone at night--no q 4 hour vitals, or even q 6 hr vitals. The team was very reasonable and did issue an order, "do not disturb between 9:00 pm and 5:00 am."

While Elise was in-house at TCH, our sweet little liver friend, baby Madelyn, was also inpatient...in the critical care unit....fighting complications of her liver transplant. She was briefly re-listed for a second transplant and did actually receive an offer for a new organ; however, she was too sick and therefore unable to undergo the second transplant. At each opportunity--when David was there and able to stay with Elise--I ran down to visit Madelyn and her mommy, my dear friend Amanda. We talked, we prayed, we hoped, we pleaded with God for Madelyn's miracle. In all honesty, Elise's situation was put into perspective by the  blatant direness of Madelyn's predicament. 

On December 23, 2013, after a thorough review with our hepatologist and liver team, and after Elise received her 3rd dose of IV steroids, we were discharged home....with an increased dosage of anti-rejection medication of course. 

On December 26, 2013, I had to bring Elise for repeat labs at TCH.  Normally, I would have texted our friend Amanda, to ask what kind of coffee she would like. It was always "our thing" that I would bring her a a vanilla latte or a caramel machiatto when we were there, and we would visit. But on this particular day, I just didn't want to intrude, and I was heading towards the elevator when I ran into Amanda's mom, "Coco." She hugged me, and she said she was going to go get Amanda, because she would want to see me.  Amanda came out, and the raw emotion, and primal mother instincts were evident. As we talked, she told me, "We're going to let her go today." She talked about how hard Madelyn had fought and that as her mother, she had to support her if she needed to go; she did not want Madelyn to feel that she was disappointing her.  I didn't know what to say, so I just lifted her up. A mother has to do what she has to do, and let me tell you, Amanda was never a better mother than she was on that day.  Around 3:15 pm, I received a simple text, "Madelyn smiled when she saw Jesus."  Heartbroken. That's what I felt. But when I spoke to Amanda later that evening, we talked about how Madelyn did in fact receive the "miracle" we prayed for; Madelyn is healed now and free of pain; she earned her golden angel wings and is playing with the angels. I attended her memorial service, which was beautiful. Her grandpa, "Roro," pointed out that Madelyn's life was not measured in number of days but in the number of lives she touched. How true.  At the end of the ceremony, we all released muti-colored balloons for her, and I know that she enjoyed them.  

In regards to the treatment for Elise's rejection, Dr. Fishman did not initally prescribe oral steroids for her to take at home due to her known EBV issues. Steroids are a type of immuno-suppressive, and they typically cause EBV blood levels to rise. For about 2 weeks, it appeared that Elise's labs were improving, which was wonderful. We celebrated a wonderful Christmas at home as a family; David and I went out for our anniversary on December 30, and then we brought in the New Year at our fellow liver family's NY Eve fundraiser for liver research. 

This past Monday, January 6, however, things went off-course. Who was I to expect things to go just as planned?  And whose plan was this anyway? I was reminded of a line from John Steinbeck's To A Mouse, "The best laid schemes o' mice an' men gang aft agley, an' lea'e usnought but grief an' pain for promised joy."

Elise's "numbers" were elevated again:  AST 250, AST 287, GGT 97

Fortunately, when our transplant coordinator calls with bad news, she always follows it up with a specific plan. In this particular instance, the "plan" was to start Elise on the oral steroid medication Budesonide. The tricky part was that Dr. Fishman ordered the inhalation formula of this med, which is designed to be administered via nebulizer/ breathing treatment. The pharmacist and I both spoke to our coordinator multiple times to clarify and ensure that the order was indeed to take this medication by mouth. When I went to the pharmacy to pick up the medicine, I was only given 2 boxes--or approx 6 days supply--and I was informed that our insurance was only covering the  2 boxes, and that the remaining 12 boxes was "outside of the appropriate clinical dosing parameters."  Of course, I called our insurance company, only to be told, "Yes, your coverage guidelines only allow Budesondide up to 2 boxes/month, for the treatment of Asthma or other respiratory conditions."  I responded, "Okay, great, well what does our plan cover for the treatment of rejection of a solid organ transplant?"  The response?? "Ma'am, I would suggest you file an appeal."   HA!  I'm one step ahead of you, buddy.  OF COURSE I am filing an appeal. 

The next day, I had Elise's hepatologist, Dr. Fishman, call in with the clinical rationale, and the entire quantity of Budesonide was approved. For anyone interested, the rationale was the fact that Elise can not safely take "cheaper" steroid meds such as Prednisone, or even Presnisolone, because of the effect on EBV; Budesonide is a newer steroid having less severe side effects, and is not likely to elevate the EBV significantly at all.  She obviously requires a larger dose by mouth for the treatment of rejection than she would require if prescribed for respiratory conditions such as Asthma.

Today, January 10, 2013, Elise had repeat labs done again, as well as a CT of her abdomen, chest, and neck. The labs were ordered to ensure that the steroids are doing their job.  The CT was ordered mostly as a precaution, "to take a look around," to look for any suspicious areas, and most importantly to provide a baseline CT in case we encounter any more serious EBV or PTLD (post-transplant lymmphoproliferative disorder) issues.  I am thrilled to say that Elise's current labs are very promising; her liver "numbers" are definitely going in the right direction, and this new treatment plan seems to be working very well.

AST 136 (down from 250,) ALT (down from 287,) and GGT 78 (down from 98.)

We are instructed to keep her steroid dose exactly the same for now, and we will also be keeping her anti-rejection med (Prograf) dose the same, as her blood Prograf level is 4.4 (goal range 3-5.)  Repeat labs are ordered fo Tuesday 1/14/14. 

I'm not really sure how many of you are still following this blog and/or Elise on Facebook, but if you are, or if you ever have, I thank you. We appreciate your support, prayers, and encouragement.