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.
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.
Elise is very sweet and this is a great article
ReplyDeleteThanks Julie!!
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