Is this week over yet??

Hello friends… it's been a long week.  Let me give you a review.  We have routine news, bad news, and good news to share! What else would one expect from "Liverland?"

Last Thursday January 29, Elise underwent her scheduled operation for adenoidectomy and ear tubes-reinsertion.

The only little problem we had was separating her from me in the pre-op area.  But I quickly suggested that they allow me to scrub in and go back with her to the OR, so as to reduce the trauma [for both of us.]  

My husband, David, being an anesthesia provider, can't believe that they allow me to do this, but they do…..this is the second time in a row.  Thankfully, we have been having very reasonable anesthesiologists, who trust me not to "freak out" when Elise goes limp in my arms. Now, let me tell you….there is a HUGE difference between your child sleeping in your arms and your child falling unconscious in your arms.  But nonetheless, I know what to expect, as I have done it before and acquainted myself with the process of anesthesia, and it is MUCH less traumatic for her to fall asleep this way than in a cold room with strangers in masks after screaming for several minutes as she walks down the hall.

I digress….Elise did GREAT with the surgery… so great, in fact, that the ENT and liver team decided to discharge her home with pain meds and 3 days of antibiotics rather than keeping her inpatient overnight as originally planned. I personally couldn't have been happier to bring her home. She did struggle with pain for a couple of days, but nothing unmanageable.

This past Saturday, approximately 48 hours post-op, I noticed she started coughing.  I tried to rationalize, telling myself she just had her adenoids removed, and her throat is probably scratchy and irritated. Then on Sunday, I became a little more concerned, as her cough became worse, and she spiked a fever of 101.2. I decided that this warranted a trip to the pediatrician in the morning.

On Monday morning, Feb 2, I took her to see her pediatrician. Her o2 saturation was 97% at that time. She was coughing quite a bit, retracting, and breathing very fast, but after an albuterol breathing treatment, her symptoms improved, so we were sent home.  I asked whether antibiotics might be warranted, questioning whether this might be--or might develop into- pneumonia, as we have experienced several times in the past. But…you know…doctors are under pressure to avoid ordering "unnecessary tests" and to also avoid antibiotics whenever possible. And so….we went home and started up breathing treatments three times/day. 

Yesterday, the coughing was worse, but she didn't really have a temperature over 99-100, and she didn't appear to be in distress…..until later in the evening.  When I was getting her ready for her bath, I noticed she was breathing fast--approx 38-40 times/minute, retracting, and when I checked her o2 saturation, it was ranging 88-92%.  I gave her a breathing treatment, and it improved to about 94%.  I really debated at that point about whether or not to bring her to the ER right then and there.  I decided to just increase breathing treatments to every 3-4 hours over night, checking SATS frequently, and try to wait and bring her in to see the doctor again this morning. So that's what I did.  I checked her and gave her a treatment at midnight, and again at 3:30 am this morning.

At 8:00 am, I spoke to Elise's pediatrician as I was on the way to her office. When I gave her a run down of her vitals overnight and indicated that her temp this morning was 100.3, she told me to head straight to ER at TCH. She said she would notify liver team that I was on the way with Elise, in respiratory distress, and that I should not have to wait to be seen. And I didn't.  I never even signed in to the ER. Come to think of it, I don't think I ever signed a "consent to treat." I'm sure I'll get a phone call about this tomorrow. 

Anyway…chest x-ray and labs were ordered quickly after we met with the first set of doctors- the ER resident, fellow, and attending MDs.  Liver team quickly involved themselves in Elise's plan of care, and they consulted pulmonology as well, since Elise has been followed closely by pulmonology since last year when she had several  bouts of pneumonia and a case of RSV. The ER resident came in shortly after the x-ray and stated, "the x-ray looks clear."  That didn't sound right. I asked about labs. At that point, the only thing back was her CBC which showed an elevated white blood cell count at 15,000. I asked, "well, that WBC looks like an infection brewing….but your'e saying there's no pneumonia or bronchitis, etc?"  She responded with comments that pulmology should be on their way down, and they'll give their opinion.  Good.  Because I could hardly wait to hear it. 

The pulmonologists did see her shortly thereafter. They reviewed her x-ray and examined her, and without a doubt, they confirmed that Elise has "full blown left-side pneumonia." They indicated that they could hear it on her left side, and after reviewing her history, suggested this is likely a bacterial aspiration pneumonia resulting from anesthesia during her recent surgery.  They spoke with liver team, and then ordered a round of IV Rocephin antibiotic to start treating the pneumonia. 

The only question that remained was whether to keep Elise inpatient or discharge her to treat the pneumonia at home. The ER attending deferred to Pulmonology attending, who deferred to liver team attending.  I asked, "Well, who is the liver team attending on service right now?" I was told, "Dr. Doug Fishman. He said Elise is well-known to him."  To which I replied, "LOL, you think?  He's coming down here to see her and talk to me, right.?"  "Yes, I believe he is," said ER doc. 

I could hear Dr. Fishman in the hall several minutes before he actually came into the room.  And then there was "the knock."  I can't count the number of times I've heard that knock during liver clinic appts and hospitalizations over the last 3 years. That knock actually makes me happy though, because I know that things are under control when he's around. 

And so, Dr. Fishman examined Elise, and then asked whether I had seen her liver panel. Well, as a matter of fact, I had.  He assumed as much…he knows I'm all up on her numbers hot off the press. 

He couldn't help but grin.  That's right.  Her current liver panel actually warrants a SMILE!  SHE"S BACK AT BASELINE!  Even in the face of pneumonia!  

AST 68, ALT 61, GGT 40. 

Which brings me to the rest of my discussion with Dr. Fishman.  You know, I have already had a consult/ liver clinic appt on his books scheduled for next week, Feb 11, to discuss in detail the new diagnosis of DeNovo Autoimmune Hepatitis (AIH.) He acknowledged that upcoming appt, then offered to start the discussion now, while we were face-to-face.  

Bottom line… Dr. Fishman says he is actually no longer ready to commit to the diagnosis of DeNovo AIH. He said that he has not started meds yet to target AIH because it may be "something to watch for but not necessarily diagnose" at this point. We reviewed her biopsy report together, citing the fact that there is absolutely no evidence of any fibrosis or cirrhosis, or any damage to her liver whatsoever.  There was 1 single plasma cell identified, but Dr. Fishman says that in a case of true DeNovo AIH, he would expect to see a whole host of plasma cells. The ANA profile, he said, pointed to DeNovo AIH, but it is more like "consistent with," and not necessarily a conclusive.  In any case, he says, "in a Dr. Shepard-ism," "MUCH better to have a little touch of rejection or Autoimmune Hepatitis than to have a real post-transplant complication…. like post-tranpslant lymphoproliferative disorder (PTLD,) which would be a risk if we start throwing steroids and increased immunosupressive therapy at Elise to target autoimmune hepatitis."

I can't even tell you how much better I feel after this conversation with Elise's liver doc, Dr. Fishman.  I just wish it wouldn't have taken an entire day in the ER fighting pneumonia to get this face-time.  But now I feel even better prepared for our appointment with him next week. I won't be so much like a "deer in the headlights" going into it. 

Tonight, we are back home as a family.  Elise was discharged home after receiving IV antibiotics in the ER, and she is to continue oral Cefdinir antibiotics x 14 days at home.  We will see her pediatrician tomorrow morning, who will give her an injection of an additional dose of Rocephin, and then we will be following up with pulmonology and liver clinic next week. 

On Friday, Elise's Grammie & Paw will join us to attend the Houston Rockets' basketball game, where Ethan and his TOT basketball team will perform for the pre-game show.  I believe he will be receiving his first trophy, and he couldn't be more excited.  I couldn't be more proud. 

I just couldn't be a happier mommy.  We have our challenges, but doesn't everyone?  As they say, "what doesn't kill you makes you stronger."  And I know for  fact that everything we battle with Elise makes me a better mom, a better social worker, and a better wife.  

"Elise is a Trooper!"

Today, after several hours of labs, clinic appointments at TCH, and several conversations with various members of Elise's treatment team, I arrived home to begin my work day.  One of the doctors I worked with asked, "How'd everything go?"  And I bet he wished he hadn't, because I gave him an ear full.  "Well….Elise is a trooper!" That's what he said.  Why yes…yes she is.  That's all she's ever known how to be.

Let's start with the good news.  Elise's labs this morning were pretty good, relatively speaking.  Her liver enzymes are dropping, which is great!   AST 100 (down from 263,)  ALT 110 (down from 254,)  GGT 44 (no significant change.)  Her Prograf level is perfect 2.3, so no change in her Prograf dose.

                           

After labs this morning, we met with Elise's pulmonologist and ENT for post-hospitalization follow up. Elise has had several bouts of pneumonia and other upper respiratory infections over the past year, RSV in November, and she promptly developed a double ear infection last month after her tubes fell out. After some collaboration on her case, her ENT presented me with the recommendation that they schedule surgery to replace her ear tubes….and remove her adenoids, the reason being that adenoid removal might clear up her airway and help her to breathe better even when sick, thereby reducing risk of infection. And you can be sure…anything that equals reduced infection risk will get liver team support.  So I knew that would be a non-issue, other than of course the fact that this would-be day surgery is going to turn into at least an overnight stay at TCH under the liver team to be sure she doesn't develop a fever after surgery or experience any complications that could negatively impact her liver.  Okay.  Fine.  Sounds like a plan.  It's another surgery under general anesthesia, which I'm not crazy about, but the benefits seem to outweigh the risks, and we're on board. So, we scheduled surgery for January 29, 2015.

                                          

Now, if you recall, we were discharged from the hospital a couple of weeks ago, on December 30, after her liver biopsy showed no evidence of rejection. What wonderful news!  One of the last little tidbits we heard from our liver center director, Dr. Himes, before we left on our merry way was that Dr. Fishman would order "just a few extra tests" to look for auto immune hepatitis (AIH,) "just as a precaution," because they saw one single plasma cell in her liver biopsy which could be consistent with AIH. He said this condition is rare though, and that typically there would be more than just one cell…..anyone could have just one plasma cell hanging out, right??

De ja vu….."tests," "rare," "consistent with…"  I've heard this before. No matter how many times I hear these phrases though, I always assume it's going to turn out to be nothing.  And then it's something.

So, last week, these tests for AIH were drawn…specifically, they ordered an F Actin AB IGG, IgG, Liv/Kid Microsome AB, and an ANA profile.  After a couple of days, I emailed our coordinator regarding results and quickly received a response that "everything is fine….but the ANA profile is still pending."  GREAT!  This past Monday 1/12/15, I followed up again; I asked whether the ANA profile results were in.  And with that began the longest week I've spent in Liverland in quite some time. Sarah indicated to me that the results were "available" but "not released" by our liver doc; she said that once he reviews the results, she would get back to me.  I made countless calls and sent many emails to our team again on Tuesday, Wednesday, and Thursday (yesterday,) but to no avail. Sarah of course responded to each and every one of my outreach attempts, but only to say that her hands were tied; she repeatedly explained to me that she could not access the results to disclose them to me until they were reviewed and released by the ordering physician.

I was so upset by the end of the day yesterday….I was literally becoming ill. It's really interesting how such strong feelings of anxiety, worry, fear, and anger can manifest somatically.  I was able to get some sleep last night though, knowing that I would be at TCH today, and I was sure that I would be able to pin down some results one way or another. And that I did.

Around 1:00 pm today, I was informed that after a careful review of Elise's biopsy together with the ANA profile, it appears that Elise does in fact have "a mild case of De Novo Autoimmune Hepatitis."  I asked, "She has a component of Autoimmune Hepatitis?" Response: "She has Autoimmune Hepatitis." I asked, "She has DeNovo or just some other type of AIH?" Response: "She has DeNovo; it is De Novo AIH."  It's like I wasn't hearing it for a few minutes.

Of course, as I doodled some non-sensical notes on the paper in front of me, I asked question after question. The "take-away" is that Dr Fishman basically sees this more as "information," and obviously a complication, but definitely something they can manage. He said that we are not adding any meds or doing anything differently in her treatment plan at this point. I asked whether this AIH would lead to her needing another liver transplant in the future, and he said that it should not mean that.  He said they will simply monitor her labs, as they already do, and if it ever flares up to the point that additional meds are of more benefit than risk, they will intervene at that point. Right now, with Elise's liver enzymes actually trending down towards normal, the addition of steroids and/or other meds to target AIH would likely re-activate CMV (Cytomegalavirus) and EBV (Ebstein-Barr Virus) issues, and the last thing we need is for those viruses to again rear their ugly heads. Apparently, this AIH can be compared to other chronic autoimmune disorders (i.e. Chrons Disease, Lupus, etc.) in that there will be times that things are baseline, and there will be times that the condition will flare up and will look like rejection, so there will have to be a careful differential diagnosis made.

I felt like I asked a million questions, but I guess not, because I still have a million questions left, and I've scheduled a phone call with Dr. Fishman for next Tuesday to address these concerns.  I am also considering scheduling an additional "liver clinic" appointment with him and our team, because given this new information and diagnosis, I feel that some face time is in order…..and it can't' wait until the end of March.

By all accounts today, Elise looks GREAT!  And I have to continuously remind myself to look at HER, not her "problem list' on her paperwork at TCH, because frankly, it is a mile long.  Like we discussed today, Elise's identified problem is that she is a liver transplant patient.  Period.  Secondary issues such as EBV….CMV….rejection….and now AIH are just that…..secondary; these issues are just complications…..these are the "bumps in the road' that the team has been telling me we would encounter since Day 1.  And since Day 1, they have also been telling me, "We can manage any complication post-transplant."  Honestly, we would have no idea about this De Novo AIH had it not been for the team ordering the right tests at the right time.

And so….like I have done so many, many times in this journey…I am placing my trust in God and in Elise's liver/transplant team at Texas Children's, for I know she is in the most capable hands possible. God has blessed us by placing us here in Houston where we have access to this team….and I am sure that Dr. Fishman having me wait five long, excruciating days this week to hear the test results and plan is just God's way of teaching me patience.  Dear God, I HEAR YOU!

Wrapping up December…on to a new month and NEW YEAR!

Have a mentioned before that I really don't like the month of December?  I really don't.  For the past 3 several years, December has been associated with bad news and bad things happening.  Both of my maternal grandparents died in December; Elise was diagnosed with Biliary Atresia in December; Elise had 2 of her 3 episodes of Cholangitis in December 2012 and December 2013, and she underwent her ureter re-implant surgery in December 2013 after which she did not wake up for 13 hours, and now, in December 2014, Elise had an emergency admission to TCH--which I will explain later in this post--and she has been battling elevated liver enzymes this entire past month. Despite it all, we have managed to have lots of good times too though.

On December 9, we went for routine labs at Texas Children's, just as we do every month, to monitor Elise's liver enzymes and liver function as well as her serum Prograf level. Elise wore her SuperGirl cape and proudly checked in for her blood work. I was shocked to hear back later that afternoon that her liver panel was abnormal and quite elevated.
AST 150,  ALT 140,  GGT 29

For comparison sake…Elise's baseline liver panel looks something like this:  

AST 45, ALT 44, GGT 17

Elise registering for her labs

                                   

The following week, on Wednesday, December 15, we repeated labs, hoping to see some improvement. The results were, however, worse:  AST 147, ALT 179,  GGT 35.  Of course, I started to panic at this point, but her doctor and transplant coordinator told me to try to relax; they said, "Don't worry; we're not worried yet." Ok well….sure….if you say so…..not.

On December 16, I brought Ethan and Elise to the TCH Transplant Holiday party. We had a great time visiting with other transplant families, including some of our sweet liver friends, and we also had the opportunity to visit with Elise's transplant surgeon, Dr. John Goss…..a  rare occasion aside from the times we cross paths in crowded elevators when he is clearly in a hurry to be elsewhere. I never know quite what to say to Dr. Goss, other than "thank you."  To which he always responds, "Oh,  I was just the plumber." We all know he is much more than that.  He is an amazing liver transplant surgeon, who saved Elise's live with her new liver.  I had no choice but to lay Elise's life in the hands of a surgeon on March 23, 2013, and I thank God every day that it could be Dr. Goss.

Transplant surgeon, Dr. John Goss, with Elise and me at the TCH Transplant Holiday party

                               

For the past 3 years, we have been getting together with our "liver friends" here in Houston for a holiday party and ornament exchange. This year's party was on Saturday, December 20. Everyone brought food and drinks, and we had a fantastic time visiting with our friends, and I found it amazing to see how much the kids have grown over the past years.  Ethan and Elise have been saying they want to go "back to our friends' house" ever since we left.

Rockin' Liverland "liver friends" Christmas party and ornament exchange…..

                                

On Monday, December 22, I brought Elise once again to TCH, bright and early, for 7:30 am repeat labs. And, once again, her liver panel was elevated; her liver enzymes were continuing to progressively increase.  AST 166, ALT 218, GGT 40.  The tone of our transplant coordinator's voice gave away the plan before she even said it. A liver biopsy would be needed to rule out or confirm rejection, and if not rejection to hopefully identify the problem, if any, causing the abnormal labs.  Our coordinator, Sarah, said that they wanted us to enjoy Christmas at home, and they would arrange a direct admission to the 12th floor on Sunday and liver biopsy on Monday. If the biopsy confirmed rejection, she said, we would be looking at 5 days inpatient for aggressive IV steroids to hopefully turn it around.

December 23. Despite everyone's best efforts to keep Elise at home for the holidays….she found a way to TCH. I'm reminded of one of my favorite poems from English literature, To a Mouse, by Robert Burns:

The best laid schemes o' Mice an' Men, 

Gang aft agley, 

An 'lae'e us nought but grief an' pain, For promis'd joy! 

(The best laid schemes of Mice and Men

oft go awry.

And leave us nothing but grief and pain

For promised joy!)

Around 7:00 pm, I set Elise's Prograf medication out on the counter and proceeded to bathe the kids and get them ready for bed. I believe it was while I while I was running bath water that Elise grabbed the medicine bottle and drank an unknown amount of it.  There was some spillage on the counter, and her dose has changed multiple times since I filled it, so it's impossible to determine exactly how much she drank.  BUT, she walked up to me holding the open bottle, I asked--as the blood drained from my face--Elise, did you take your medicine?" She proudly answered, "YES, I DRANK IT BY MYSELF!" I became frantic, and when I started crying and tearing apart the living room looking for my phone, she rescinded her claim, and changed her story, telling me that she spilled it.  I immediately paged the liver team MD on call, and while waiting for a response, I called David.  I was in such a panic, I don't even remember what I said on those calls. And I didn't pack any bag. I just stuffed the half-empty Prograf bottle in my purse, buckled Elise in, and raced to TCH as soon as David (who was already on his way home) turned in the driveway.

Upon arriving in the ER, Elise was immediately taken back to an exam room where a medical evaluation was completed and labs were drawn, including primarily a liver panel, chemistry panel to check her kidney function, and of course a serum prograf level.  Elise and I slept in the ER for a couple of hours before we were admitted to the 12th floor around 2:30 am on Christmas Eve.  Thankfully, later that day, the liver team rounded after consulting with the renal docs and toxicologists, and they discharged us back home, as Elise's kidney function was excellent, and serum Prograf level--although extremely high-- was dropping. To give you some idea of how her overdose affected the concentration of Progaf in her blood….her normal goal level is 3-5, but her Prograf level on the morning following this incident was 29.8. In any case, we happily returned home with orders to "hold" Prograf doses until further notice and bring Elise back in the next day, on Christmas morning, to repeat labs.

                                  

We woke up early on Christmas morning so that we could open presents and have a little fun before heading to TCH for labs.  I can't really talk about Christmas without sharing a few pics! We had so much fun, and it was a welcomed reprieve!

                             

                             

                           

December 25, 2014….repeat labs drawn…. AST 184, ALT 254, ALT 43.  So….her liver enzymes were still climbing.  I was sort of hoping that if Elise was in fact in rejection, the overdose on anti-rejection medication might knock her back into shape. But such as not the case. The good news was that with each lab draw, her kidney function was perfect. For those of you interested in the nitty gritty… BUN/ Creatinine 12/ 0.24.

                                

Friday, December 26, 2014. AST 203, ALT 263, GGT 43.  On this day, I was finally able to speak to our transplant coordinator, who had previously been out on vacation all week.  I felt SO much better after talking to her. She provided me with more accurate information than that which I received in the ER; she said that although Prograf is a renal-toxic medication, it will not "kill her kidneys" just from a one-time mega dose. She went on to say that they frequently encounter cases of patient's with serum Prograf levels shooting up into 30s and 40s even under their careful management, and they have had other cases of overdoses with levels going into the 100s, so while a level of 29.8 might seem scary to me, it's not really that scary to the liver team, and it's not going to cause Elise any serious problems.

Sunday, December 28.  Dr. Himes, the attending hepatologist on hospital service, admitted Elise to TCH as planned and scheduled a liver biopsy for next morning. Elise was super brave throughout her admission process, an it was a fairly uneventful afternoon and evening.

                

Monday, December 29. Elise and I were taken down to the OR just a little later than scheduled, around 10:00 am. Despite having not eaten in more than 12 hours, she was happy as a peach. Until it was time to separate and take her behind the double doors. Fortunately, we were blessed with an AMAZING Anesthesiologist who allowed me to "scrub in" and be with her through induction. I sat her down on the table and held her tightly against my chest as the anesthesiologist pushed Propofol, titrated with a little Versed, into her IV, and then once she was completely sedated, I laid her down….gave her a kiss…..and told her I'd see her in a bit.

                               

                        

There were no complications at all during the biopsy procedure. She drank apple juice and a sprite within about 10 minutes of waking up, and she was back in her room within an hour. Soon, she was "on duty," wearing her very own set of scrubs and taking care of the little patients she had with her in her room.

                               

                     

Tuesday, December 30.  I must say, it's hard to spend the the better part of a day waiting on liver biopsy results; it's even harder when previous two biopsy experiences have very negative and traumatic associations; now multiply that times 1,000 when it's your wedding anniversary…. the second anniversary you have spent inpatient at TCH in the past three years.  My depressed mood, compounded with anxiety and fear of the unknown, made for an unpleasant experience, but not one unfamiliar to me.  The dark cloud was definitely mitigated, however, by my husband, David, being there the entire day with me and by Elise's unwavering happiness and joy in every moment.

When Dr. Himes entered our room at 4:15 pm, I swear the weight lifted off my shoulders as soon as I saw his face and heard the light tone of his voice.  He informed us that he and Elise's primary hepatologist, Dr. Fishman, reviewed Elise's biopsy with the pathologist, and there was no evidence of rejection, and furthermore, no evidence of any serious viral processes that might be harmful to her liver.   He went on to say that there was no reason to start any steroids or other IV or oral medications….no med changes needed at all for that matter….with the exception of a new antibiotic ear drop medication to treat her ear infection which everyone agrees has not resolved. So, of course, I asked about the differential diagnosis.  If not rejection, and not a virus, what could it be that has been causing the progressive increasing liver enzymes? Dr. Himes and Dr. Fishman agree that the most likely explanation is an infection; very likely, the ear infection. Another possibly, though they do not think this is the case, is Autoimmune Hepatitis (AIH.) They ordered an additional blood test to screen for AIH markers out of an abundance of precaution, and we should have that result in a week or so. And the final possibility is that there is just no identifiable explanation.


According to Dr. Himes, the important thing is that her liver looks just fine on biopsy; there is no damage, and simply having elevated numbers is not going to cause any damage.  They will closely monitor her labs weekly, and they fully expect to see her numbers improving and returning to baseline soon.  With that, Dr. Himes gave Elise her walking papers, and we were able to return home……just in time for David and I to make our 8:00 pm anniversary reservation as planned!

Count on me to capatalize on picture ops!   Here's Elise and me with TCH liver center director Dr. Himes, just after receiving  excellent biopsy results and discharge papers!

Happy 9-year Anniversary to US!  :-)

                                            

Reflections…National Biliary Atresia Awareness Day 2014

Three years ago this week-- on December 6, 2011-- I walked into our pediatrician's office with Elise for her 2-month "well-baby" check.  She was actually only 7 weeks old, but I was trying to get all my ducks in a row so that I could return to work the following week.  As I recall, the appointment was on on Monday, because just 2 days prior, David and I had spent a wonderful evening at his company Christmas party. Lots of friends and coworkers wanted to know how our sweet baby girl was doing, and we were thrilled to tell them that she was amazing, and we were just so happy to be home after spending the first 14 days of her life in the Clear Lake Regional NICU. She was born 5 weeks early, but everything was perfect, or so I thought.

Elise was and always will be an amazing little girl…but there was nothing perfect about her health when she was born. Our pediatrician did not like her eyes at that 7-week appointment.  I thought her eyes were beautiful.  I was wondering if the would stay blue, but I thought they would probably turn brown. I barely noticed the yellow….and when I did, I just pushed it to the far recesses of my mind, assuming it was related to her prematurity.  The last thing a new mommy thinks is that her sweet baby has a life threatening liver disease. Ok…. I regress….our pediatrician didn't like Elise's eyes, so she requested a split bilirubin test.  I left the clinic with Elise, expecting a call back shortly that everything was fine.  I did get a call back shortly, but all was not fine.  We were told to bring Elise to Texas Children's Hospital that night to rule out or confirm a rare, pediatric liver disease called Biliary Atresia.

Today, December 1, 2014, is National Biliary Atresia Awareness Day.  Biliary Atresia, affecting approximately 1 in 15,000- 18,000 infants, is a disorder in which the common bile duct between the liver and small intestine is blocked, or absent, causing bile to back up into the liver.  The only effective treatments are the kasai procedure, and in 97% of patients, ultimately a liver transplant. Early detection and intervention is crucial to positive outcomes. The symptoms of BA are jaundiced after 2 weeks of age, light colored stools, dark urine, nutrient malabsorption/ lack of weight gain/ failure to thrive, and excessive sleepiness or fatigue.

So, today I have spent much of the day reflecting on Biliary Atresia and the affect it has had on our lives. Denial is such a powerful thing.  When the liver team reviewed the biopsy with us, we clung to and overanalyzed the terminology, such as the words "consistent with Biliary Atresia."  Surely, there must be a million issues that could be "consistent with" BA but not actually BA.  So that brought us to December 16, 2011, the kasai operation.  Elise was in the operating room for approximately 9 hours, but for at least the first 3 hours of that, I refused to believe that the the "kasai" operation was actually going to take place; I was hoping and praying that the surgeon would come into the waiting room and tell us that he had closed her up after confirming it was just a false alarm.  That, of course, did not happen.  An OR nurse called me in the OR and said to me, "Dr. Wesson has now completed the cholangiogram portion of the procedure and has confirmed Biliary Atresia...and he is now proceeding with the kasai portion."  When I heard those words,  I knew that when Elise emerged from the OR, our lives would be forever changed. It was the first and last time that I have ever really cried over Elise's condition.  I became a 'liver mom" on that day. This was our new reality, and I immediately knew that I would simply do what I needed to do….  day-to-day, month-to-month, year-to-year.

Now I can't lie; for the first couple of months, I hoped and prayed that Elise's kasai operation would work flawlessly, and that she would be in the 3% that would not require a liver transplant.  Initially, that scenario was my ideal, and my dream for Elise. Which brings me to my reflections today.  You all know, I am part of a huge online "liver mom" support group; actually, more than one group.  Already feeling emotional going into the day today, I was struck by a post of a kasai-only young man.  I am sure I over-reacted to the post and misinterpreted it, as I took the post to insinuate that because he is ___ years "kasai-only," those newly diagnosed in the BA community and others "kasai-only" should have hope. I couldn't help but think, "So, what about Elise? Does she not have hope because she has required a transplant to survive?  Is she not a "fighter" or a "winner" like those who fight BA without a transplant?

I've come to the conclusion, with the support and insight of my wonderful fellow liver moms, that there is no winning with Biliary Atresia; there is only living and surviving.  Those kids that are kasai-only right now fight every day to stay healthy; they battle the complications of liver disease, and many of their moms suffer with the fear of a "ticking time bomb." Those kids post-transplant, like Elise, also fight every day to stay healthy, and they face potential post-transplant challenges of infection, dehydration, rejection, or post-transplant lymphoproliferative disorder (PTSD) to name a few. Whether "kasai-only," awaiting transplant, or post-transplant, THERE IS HOPE, and all of our kids are fighters; they are superheroes….rockstars….winners.  It is not our role as their parents and advocates to commit them to any certain path; we must simply support them and lift them up as they continue to fight the fight…whatever their particular fight might be at a given time.

And that's a WRAP....TWO admissions in 8 days

I see I left off on Day 2 of our admit last week??  Well, now I'm going to have to back-track a bit because this morning we are home from the hospital for the SECOND time in the past 8 days.  I would have updated throughout the admissions, but Elise had custody of my laptop for the majority of the time, and I choose my battles carefully.

Ok.  So last Friday, November 14, shortly after I posted my last update, the liver team came in initially with some less-than-great news but same as I had been told in the ER-- "not much to do except manage the symptoms of RSV and monitor and protect her liver." Well, I expressed to Dr. Sonny that it was so upsetting that there was no actual treatment to help her get over the RSV, and I asked whether it might be appropriate to consult infectious disease team, as I know Dr. Munoz has been very helpful in the past with Elise.   

Dr. Sonny Harpovat (attending hepatologist) with Elise

I didn't get the impression that they took my commentary during rounds really seriously, but….. ASK AND YOU SHALL RECEIVE!!  About an hour later, the liver team and infectious disease teams, led by Dr. Sonny and Dr. Munoz, descended on our room.  We were in an isolation room, so I could hear them in the dressing area right outside our door, and I was thinking to myself, "OMG, what else has come back positive that we're about to have to deal with.?"  Dr. Sonny came in w/ the hugest smile on his face….and he said, "Erin, this is important since we dressed out again…We have a new and very good plan….but a personal question too….are you pregnant or do you plan to become pregnant in the next 6 months?"  I almost wanted to LOL at him, but given the situation, a simple "No," and "No" sufficed. The plan?  RIBAVIRIN.  Ribavirin would be an inhalation medication administered in an isolation tent for 2 hour treatment sessions, 3 times/day, for 3 days.  Dr. Munoz stepped in at this point and answered my questions regarding the risks vs benefits.  They all agreed that this medication would not harm Elise and could only help. The only risk would be to developing fetus, so no one exposed to the treatment should be pregnant or become pregnant within 6 months. The team indicated that Ribovarin is rarely used at TCH because of the strict criteria to use it for RSV, and it is super expensive, so insurance coverage had to be verified. Dr. Munoz stated that although she has not used Ribavirin for RSV since 1995, they would be keeping Elise inpatient for at least 3 days at the rate things were going anyway, so at least the use of Ribavirin would allow us to be ATTACKING and killing off the RSV rather than just monitoring and treating the symptoms. And so I agreed to start Ribavirin, and the respiratory therapists came in to set up the tent treatment by 1:00 pm that day.

      

Elise began responding wonderfully to the Ribivirin almost immediately following her first treatment.  She started talking and smiling and showing interest in eating and drinking.  So much so that when she asked for chicken nuggets, I was motivated to run across the street in dress boots and sweat pants to get her a "Happy Meal."

"Happy Meal' makes things happier! 

On Day 3, November 15, I was starting to get "cabin fever" in that room.  Thankfully, David relieved me that evening so that I could go to our good friends' and fellow liver family's annual fundraiser event benefiting pediatric liver disease research conducted at Texas Children's Hospital and Children's Hospital of Atlanta.  I had a wonderful time visiting with our liver family….love them all so much…..they truly keep me sane much of the time in "Liverland." 

"Liver Family"… from left to right….me, Ben, Troy, Kelli, Tara, Hal, and Jennifer. 

LIVER MOMS!   Jennifer, Tara, me, and Harriet :-)

David had "tent duty" that night while I was away.  I was so anxious to see how he would deal with 2 hours inside that tent with Elise.  I was utterly shocked when he texted me the following picture.  Not sure if it's the advanced practice nurse inside him or the mechanical genius….but he managed to keep Elise calm breathing through the mask without being inside the tent with her.  

David on "tent duty." Notice he is not inside the tent. Good for him, I suppose.  I was not able to negotiate that. 

In other news, on November 15, I was pleased to lay my eyes on fastastic-looking liver panel.  And I could have sent a fruit basket to our nurse for actually printing it for me.  Because….let's be real for a second….I KNOW it CAN be printed.  But I seem to always get these nurses that tell me they can't print it, leaving me to wait until rounds to find out about Elise's liver numbers.  Anyway, if I haven't said it before, I LOVE our liver team….they are BRILLIANT.  When we went to liver clinic on 10/20/14, Dr. Fishman noted her elevated numbers but suspected a brewing virus.  Now, the virus was identified, and we were attacking it, and her liver was beautiful and happy!!! :-) 

liver panel, November 15

November 16…..Elise was doing so much better….continuing Ribavirin treatments but really ready to bust out of there.  

November 17….Day 5….our primary attending liver doc, Dr. Fishman, and Dr. Munoz with the infectious disease team rounded bright and early.  Elise got a thorough once-over by everyone.  According to Dr. Munoz, "Elise got our special treatment because there was the potential or things to go very bad….but she came in at exactly the right time for the Ribavirin to work."  And with that, were were DISCHARGED home, with instructions to follow up for repeat labs on Thursday 11/20.  And a final order by Dr. Fishman was to decrease Elise's prograf (immunosupresent) to 0.25 mg twice/day in order to allow her body's immune system to do it's job and clear itself completely of any lingering virus or infection.  

Dr. Fishman (standing,) and Dr. Munoz (kneeling) with Elise during rounds

Ethan and Elise were so happy to be reunited on Monday night.  They played together until about 10:00 pm.  Elise continued to look GREAT on Tuesday and Wednesday.  I resumed my work, with no reason to think that all was not well (remember, I do maintain a full-time job as a clinical social worker/ Care Advocate outside of "Liverland.") 

   

     

This past Thursday, November 20,  I woke Elise up at 6:00 am for our scheduled labs, and Elise sat up in bed and declared, "I not feel good." Her face was red, and her body felt warm.  I checked her temperature which was 101.7.  Surely, this couldn't be. I hoped that it was just a fluke….maybe she had a nightmare overnight and got overheated similar to what I was experiencing at that particular moment.   Since she really didn't have any other symptoms at that point, I gave her a dose of Tylenol and figured I would continue to monitor her, and if things got worse, we'd be at TCH for labs in a bit anyway.  So we got dressed, jumped in the car, and we were on our way. 

As we sat in the lab waiting area, I could feel that Elise's fever had not broken, and I noted that she was breathing hard. I checked her pulse and started counting her respirations; she was tachicardic and breathing very fast. Her labs were drawn quickly, and the phlebotomist also noted that she didn't look very good.  It was all I could do not to request that they draw an extra couple of vials, as I anticipated that we would soon be in need of blood cultures. As soon as we left the lab, I called Elise's transplant coordinator, Sarah, and asked if she could come down and "eyeball her" to see if there's really any concern or if I'm just overreacting.  Sarah wasn't happy with how Elise looked and re-routed us from the elevator lobby to a 3rd floor seating area and asked us to stay put there until she had a chance to review Elise's labs and speak with Dr. Fishman.  It seemed like just minutes later, my cell phone rang, and Sarah was on the other end telling me that Elise's WBC (white blood cell count) was 20--very high, indicating infection-- and so Dr. Fishman wantd us down in the ER for a full medical workup.  

Well, I sat there in disbelief for a second.  I had nothing with me in preparation for an ER visit or admission.  David was at work and would be stuck there until 8:00 pm, after daycare closes.  I had no plan for Ethan.  I was expected to be working that day too. So, I started doing what I could do to keep things together.  On our way to the ER, I stopped with Elise in the gift shop and let her pick out a toy to keep her entertained in the ER. And I got myself a coffee. I called my manager and team lead and told them I would not be able to be at work, and I called HR to report an intermittent absence on my FMLA case. 

We arrived in the ER, which was overflowing. Thankfully, Dr. Fishman had called ahead for us, and we didn't have to sit down waiting area or even in triage; we were taken straight back to an ER exam room, and following an initial exam,  Elise was promptly placed on "shock protocol." Several nurses came in and simultaneously began working to establish an IV access, draw labs and cultures, and to start IV fluids and antibiotics.  I pointed out that we had just gotten labs drawn within the hour, so although they did have to stick her again for additional labs needed, they didn't repeat anything unnecessarily. This was not my first rodeo, but I listened as the nurse explained to me that Elise was being treated w/ shock protocol to treat for or prevent septic shock based on her fever and elevated blood pressure, pulse, respirations.  

in the ER….again….

Liver team admitted Elise to the 12th floor again.  Apparently a fever and WBC of 20, being immunosupressed, gets you a ticket to a room with a view.  Chest x-ray looked good, urinalysis was negative, and ears/ throat looked good, so the plan was to try and identify the source of infection, and until then continue treatment with the IV antibiotic Zosyn and IV fluids.  Shortly after we arrived on the floor, Dr. Fishman came by and told me that her liver is still looking good, and that they would be monitoring her liver very closely to make sure it tolerating lowered Prograf doses. Also, he indicated that he'd be requesting some consults with other specialties such as pulmonology, immunology, and infectious disease, and possibly immunology to get their opinion and recommendations. By the time Dr. Fishman came to see her, she had already received a couple of rounds of IV Zosyn and fluids, and her temperature and vitals were stabilized, which was great news, and said this was very encouraging.

By the next morning, yesterday, November 21, Elise looked 100% better. There was still no answer as to the source of the infection, but whatever it was seemed to be under control.  For whatever reason, Elise was being kept on "contact precautions" meaning she could not leave the room, but she was making it clear that she wasn't having that.  Dr. Fishman said during rounds that he still wanted pulmonologist to see her, and he wanted her to get additional doses of IV Zosyn, but he felt that she could probably be safely discharged by the end of the day.

"gonna make a run for it!" 

The pulmonology team came in around 2 pm, and they recommended that we increase her daily inhaled steroid, Qvar, to 2 puffs (160 mg) twice daily. They also reviewed the previous pulmonology notes from April and said it might be appropriate to consider a bronchoscopy soon to evaluate whether there is an underlying airway issue secondary to her history of numerous intubations.  Dr. Fishman concurred but stated that if we were to take her to OR and place her under anesthesia for a bronchoscopy, they would want to get ENT on board to come in and re-insert her ear tubes at the same time, because Elise's ear tubes are noted to no longer be sitting in hear ear canals, and this of course places her at risk of ear infections again.  And Lord knows we don't need that.  David asked his routine questions about the anesthesia, and we all agreed that IF she were to put a brochoscopy on the books, it should be done along with another procedure that is actually medically necessary (i.e. ear tubes) rather than "just to look around." 

We are now back home again, and we are to follow up for labs at TCH on Tuesday morning 11/25/14. I swear…we're going to be in and out of there so quick, I won't even get charged for parking.  LOL.  Seriously.  I've had enough of the hospital routine for a while. So happy to be home, and we are so ready for the holidays!! :-)

Adios, TCH!!!  (take 2!) 

Jinxed

I'm not sure if it's the shirt I'm wearing on these days that we keep getting admitted to the hospital, or if it's the fact that I started my last blog post bragging about 6 months of no admission to Texas Children's…..but SOMETHING jinxed us.  Because I'm sitting here typing this from the 12th floor of the West Tower at TCH.

I probably should get rid of this shirt...

For the past couple of weeks, Elise has been coughing.  I've brought her to the pediatrician 3 times in the past 4 weeks; yesterday would have been the 4th time, but as I was sitting in the waiting room at 9:30 am, I emailed her primary liver doc, Dr. Fishman, and our transplant coordinator the following message:

Good Morning,

Elise became very sick again overnight last night.  This morning she has fever of 101.7 and o2 is ranging 90-92%…and she is very tachicardic, pulse in the 160s-180s.  I am at the pedi right now waiting to see doc, although we won't be seeing her regular pedi b/c she is unavailable.  I called earlier wondering if maybe I should just bring her straight to TCH.  I am pretty sure she has some type of URI.  This has been going on for 3 weeks now, and her liver enzymes were already elevated at liver clinic on 10/29 after she had only been sick for a couple of days, so i'm obviously very concerned about the effects that this all may be having on her liver.  Please let me know your thoughts.

Thanks,

Erin Babin

I sent that email at 9:58 am.  I kid you not-  at 10:01 am, I received a call back…Dr. Fishman saying to leave the pedi's office and bring her straight into the TCH ER, where he said they would call ahead for her and have her put directly into a room. So we left the clinic and were on our way.  I already had a packed bag in the car, but I suspected we might end up at TCH at some point. 

Upon our arrival to the ER, we were taken directly back to a room, despite the mob of patients who had been triaged and placed back in the waiting room, halls, outside walkways, and elevator towers.  Before I was able to even sit down, I heard the words over intercom, "MED EVAL IN ROOM 14 STAT." Attending and resident ER docs headed into our room, immediately suspected a left pneumonia, and ordered fluids, telemetry, chest x-ray, IV Rocephin,  LOTS of labs including liver panel, CBC, BMP, EBV & CMV levels, blood cultures, urinalysis, stool sample, and viral studies for Adenovirus, Flu, RSV, and parovirus.  I asked that they contact liver team to advise them that Elise had arrived and to consult with their regarding the plan.  Shortly thereafter, "liver team"--and I mean the "real" liver team, came in with a resident and a couple of students to eyeball her, and, according to the nurse "add a bunch more labs that I've never even heard of." They also took the liberty of requesting a room for Elise on the 12th floor/ GI/liver transplant floor and admitted her under their primary service.  

"I hate the ER…."

"let's order every lab that we can…."  And there were 3 more tubes after this pic.  

While in the ER waiting on results of chest x-ray, her o2 saturation plummeted into the low 80s, and she started looking worse and worse…right before my eyes. I was --and I am-- so thankful that we were already at TCH receiving the best of the best care when this happened.  Within a couple of minutes, she was hooked up with a nasal cannula for supplemental oxygen, and she was able to get some rest.  Liver team came back in to take another look. They told me that their main concern is the obvious infection in her body which could be a threat to her new liver if not appropriately treated.  Her initial liver panel yesterday was not significantly changed from the panel drawn on 10/29/14; liver enzymes still elevated but no alarm for rejection at this point, which is a very good thing, and her liver function is great! 

collecting stickers...

Last night, we were informed that Elise is RSV+ (Respiratory Syncytial Virus)  UGHH!  She had RSV twice while waiting for transplant, and it was terrible then, so hearing RSV now--when she is immunosuppressed-- was scary.  Liver docs said all they can do for RSV is treat the symptoms-- the coughing, wheezing, fever, and low oxygen saturation. Antibiotics will treat the pneumonia. 

So now, it' 8:15 am on the morning of Day 2 here at TCH.  They've just drawn another round of labs, and I've just made my coffee...we'e ready to see liver team and find out the plan for the day.  Oh… speaking of labs…..when resident came in last night, I specifically asked what labs would be drawn in the morning, and she confirmed that a liver panel would be drawn every morning in addition to chemistries and tacro level.  Well…this morning….nurse comes to draw labs, and I ask what is being drawn to compare.  Well….much to my displeasure, but not to my surprise, that resident MD that I spoke with last night did not actually order a liver panel.  REALLY?????   We are on the liver/transplant floor, and this is a post-liver transplant patient!   I insisted that the nurse collect an extra couple of vials of blood while the needle was in her arm "in case we have some labs added on" so as to save Elise another stick. The nurse did take the extra vials of blood, and I followed up with that resident MD. She immediately added the liver panel and assured me that the attendings would be rounding with the team shortly, and I can address all my concerns with Sonny (Dr Harpovat.)  We really like Dr. Harpovat, and I'm very happy that he's on hospital service right now if it's not Dr. Fishman. 

breakfast time…   it's a brand new day!!