Biliary Atresia Awareness Day!

Today, December 1, is National Biliary Atresia Awareness Day. Where better to spend this day than at Texas Children's Hospital, where Elise's journey with Biliary Atresia and liver transplant first began.

Last night, Elise developed a fever, after several days of runny nose and cough, and upon further examination, I found that she was breathing approx 60 times/minute with a pulse of about 180, and she was "retracting," or working very hard to breathe. I called the TCH liver doctor on call to report her symptoms, packed a bag, and brought her in to the ER upon his request. 

Because I've been to the TCH ER approx 20 times with Elise having fever over the past 2 years, I am very familiar with the "typical" orders. Well, the ER doctor last night must not be familiar with moms who are familiar, because he came across as somewhat irritated when I questioned him about whether he had consulted the liver team yet.  He said, "No, this is clearly a respiratory issue, not a liver issue." I responded, "No sir; Elise is 8 months post- liver transplant presenting here with a fever; regardless of whether she has acute respiratory symptoms, it most definitely is also a liver issue, and I'm going to need you to call the liver team. It was the liver doctor on call who advised us to come here to the ER." I further explained to him my concerns that her body's immune system is obviously working hard to fight whatever infection she has, and so precautions need to be taken to evaluate her liver function and ensure that her immune system is not trying to attack it.  I asked for a complete set of labs including a CBC, chemistries, liver panel, EBV, CMV, blood cultures, viral studies, and a chest x-ray.  In a condescending tone, he told me he would draw a liver panel "if it makes you feel better." I literally begged for a CBC and blood cultures at the very least, citing previous admissions during which Elise had to have blood drawn repeated times to obtain cultures.  

At approximately 11:30 pm last night, Elise's condition worsened: her oxygen saturation dropped into the 80s,  and she was placed on oxygen.  Our ER doctor came into the room and ordered a chest  x-ray (yes, the same chest x-ray that I had requested 2 hours earlier.) The x-ray showed pneumonia. As he was telling me this, he said, "Ms. Babin, I know you're going to be irritated with me, but I need to order some more blood work; in order to start antibiotics, we need blood cultures, and the liver team also wants a CBC, chemistries, and EBV.  Of course, I consented, but not without sharing with him a few of my thoughts.  He then assured me that the additional blood work would be collected and antibiotics would be started as soon as possible.  This was approximately midnight last night.  Around this time, the nurse came in to administer a dose of steroids which I declined, and I indicated that I would explain my reasoning to the ER doctor when he could come to speak with me. He came in, and we discussed that Elise has a history of reactive asthma symptoms, but due to her known EBV issues, she is not to have any steroids for the treatment of respiratory symptoms. The intent of Elise's hepatologists has been to minimize her exposure to immunosuppresents as much as possible, and I assume he is aware that steroids are in fact an immunosuppresent. I noticed that he was standing there with his arms crossed, which is not surprising to me since he was"closed off" to everything I had suggested or requested since our arrival in the ER. I just don't feel that this is acceptable, as I will will not just sit there and take a passive role in her care. 

Imagine my surprise, when at 1:00 am this morning, the transporter came to escort Elise and me to our room on the Liver/GI floor....before any blood work or antibiotics were started. I expressed my displeasure but agreed to go, indicating that I would obviously be much more comfortable under the direct care of the Liver/GI team. Upon arriving in our room, I inquired regarding the results of the liver panel which had been drawn on its own hours earlier.  I was told that due to a "lab error," there was no result, but they would repeat it with the blood work they were preparing to draw.  Around 3:00 am this morning, after the additional blood work, Elise received her first round of antibiotics. Elise fought through the night with the oxygen cannula, and she eventually weaned herself off of it by simply refusing to keep it on. At approximately 7:00 am, I asked the nurse for the results of the repeated liver panel. She informed me that the repeated liver panel was also apparently canceled by "someone" who thought it was an error since it was already drawn.  I couldn't believe what I was hearing, and at that point, I called administration to request that a patient care advocate come to my room to take my report and write up a grievance. 

The silver lining to all this? The results of her liver panel, after 3 draws, was quite pleasing! There was no significant change from her labs last month, so her new liver is doing just fine, despite the assault of pneumonia. 

Unfortunately, the attending physician who saw us today was not familiar with Elise's case or history. I was happy to hear he was going to discharge us today, but I had to object to the changes in meds he proposed. It was almost the exact same scenerio that we experienced on her last admission in early November.  He suggested we "double her Prograf" because her blood prograf level was very low.  After the reassurance I received from the team last month that I always have the "all clear' to refuse this type of med change from anyone other than her primary hepatologist, especially in light of a perfect liver panel, I did just that; I refused, referring to Elise's known EBV problems. I assured him that the reason her prograf level is low is because she is sick, and he agreed this could likely be the case. I told him that we would be following up with Dr. Fishman at "liver clinic" on Wednesday, and if he wants to make any changes, he can do so then.

What I want others to take away from this post is not simply my "gripes," and I want to emphasize that Elise's care in general by the liver/transplant team at TCH has been extraordinary. As a clinical social worker, I am well aware that mistakes happen all the time in hospitals.  My intent is for those reading this post to feel empowered. If you don't know your child or loved one's medical history and treatment plan backwards and forwards, then who does?  Even the primary physician has other patients.  I make it my business to know everything; I know "when to call" the liver/transplant team; I know the "Dos and "Don'ts" of post-transplant life; I know what over-the-counter meds Elise can and can not have; I know her allergies; I know her routine meds and dosages; I know her baseline lab results from at least the past month as well as the comparable "normal" ranges; I know the normal consistency and color of her stools, and I'm not afraid to take pictures of that which I deem to be abnormal and email to to her transplant coordinator. Because I "know," I feel confident in advocating, and I have the peace of mind of knowing that I have power and can play an active role in keeping my baby girl and her new liver healthy.