How We Got Here
Ashton’s Battle with Biliary Atresia & Beyond
This is a timeline of events (told by Ashton's mom, Shayna) to give you a little insight on Ashton and his story.
January 28, 2016 - Ashton’s Birthday!
At 5:24 pm Ashton Cole Guerra made his debut, weighing in at 8lbs. 3ozs and 21 inches long! With the newest member born, our family was now complete!
February 2, 2016 - First Red Flag
Ashton had his hospital discharge follow-up appointment, where our pediatrician expressed a concern about Ashton being jaundice. She told us this was very common in breastfed babies but labs were in order. Later that afternoon we got a phone call that we should take Ashton into Texas Children’s Hospital- Katy Campus immediately because his bilirubin was elevated and that it indicated liver disease. A wave of panic came over as we took Ashton into the ER where our doctor had called ahead to inform them of our arrival. Once there, we were informed that his bilirubin levels were slightly elevated but not so much so that he would need to be under the UV lights. We were sent home with the instructions to continue breastfeeding and place Ashton in the sunlight as often as possible.
Little did we know, our pediatrician was right, something was very wrong with Ashton’s liver but several factors contributed to his diagnosis being missed at this visit: 1) BA is so rare affecting only 1 in every 16,000-20,000 live births worldwide that many professionals who do not specialize in GI conditions are not aware of the signs and/or symptoms of a problem; 2) our pediatrician was on the right track but did not adequately explain her reason for referring us to the ER.
February 12, 2016 - Taking a Better Look
Ashton had his 2 week follow-up appointment, with a new TCH pediatrician. This visit officially marked our journey to diagnosis. Ashton’s pediatrician was concerned because Ashton had not yet regained his birth weight and he was still very jaundiced. Labs were ordered once again and his pediatrician informed us that he would be asking the advice of a liver specialist. Panic once again set in as we wondered what all of this could mean.
Later that evening we received a call from a liver specialist at Texas Children’s Hospital- Main Campus, turning panic to anxiety. He explained elevated bilirubin and unconjugated vs. conjugated and asked that we have Ashton do follow-up labs each day of the weekend.
February 14, 2016 - Google is Not Your Friend
Ashton had undergone a weekend of labs and we got another call from TCH explaining that Ashton’s labs were going higher and that we would need to bring him in as early as possible the following morning to have a full evaluation to determine the cause of elevation in his conjugated bilirubin. We were told that there were a few possible diagnoses that would allow us time in planning treatment and one diagnosis that was extremely time sensitive. By this point, anxiety had turned into fear and I turned to Google (this was the worst decision).
February 15, 2016 - The Hunt for a Diagnosis
We met Dr. H, who was excellent at breaking down the plan to achieve diagnosis and possible outcomes and course of treatment and then we began testing… lots and lots of testing! Ashton had a series of physical examinations, x-rays, ultrasounds, and MORE LABS!!!
February 16, 2016 - Still No Answers
We were called back and told that all tests came inconclusive for any other possible diagnosis but ONE…the one we had been hoping and praying was not THE ONE, Biliary Atresia. It was the only diagnosis that could not be ruled out from the last testing but it also could not be proven definitive. We were told to be on standby for the next day or so to come in for a cholangiogram and liver biopsy.
February 18, 2016 - Biopsy Day
Ashton’s 1st admission to TCH. We came in for the ultrasound guided cholangiogram and liver biopsy and stayed for observation while the results were processed. The cholangiogram results were inconclusive, because Ashton was only 21 days old his organs were very small and his doctor’s didn’t feel comfortable saying they truly witnessed a blockage. So we waited for liver biopsy results. At the time it seemed we waited an eternity, wondering if this “chronic, life-threatening, no cure” disease could be cutting the life of our brand new, perfect baby short.
February 20, 2016 - The Verdict Is…
Liver biopsy results were in! We were excited for the results only to be left in limbo yet again! Ashton’s liver had scarring but it was not significant and therefore not conclusive to indicate BA. Our team of doctors were great about helping us to see the cup half full, they explained that worst case scenario this is truly BA but we have caught it before significant damage had taken place and worst case scenario our hunt for a diagnosis would continue. The plan for Ashton at this point was to have an operative cholangiogram and if a blockage existed the surgeon would proceed with the Kasai procedure.
February 22, 2016 - Surgery
Ashton went back for the operative cholangiogram and I was notified that there was a definite blockage, Biliary Atresia was his diagnosis and the Kasai was underway. After a 6 hour surgery Johnny and I got the best news, the procedure went well and Ashton was doing well. He had to have a transfusion after the surgery but otherwise he was doing well.
February 23-26, 2016 - The Road to Recovery!
Ashton did very well postoperatively, he began eating again on his third postop day which meant we were one step closer to discharge.
February 27, 2016 - Going Home
Houston, we have POOP! Poop had become a very important factor in our lives. We were documenting it, taking pictures of it and emailing about it but of all the poops we had encountered, this one was the most exciting because it meant we were going home!
March to May 2016 - Post Kasai
The next few months post- Kasai were great, we were told that a successful Kasai usually shows normal conjugated bilirubin results within 3 months of the procedure. While Ashton’s bilirubin remained high, he was gaining weight. We were repeatedly told that Ashton was breaking the mold for Biliary Atresia and that his doctor’s and dieticians would continue to follow his progress but the fact that he was gaining weight despite his elevated conjugated bilirubin was the positive we should focus on.
June 2016 - What Are You Feeding Me?
We hit our first post Kasai road bump when Ashton went from being breast fed with supplementation of formula to being formula fed only. His appetite decreased, weight gain slowed and his conjugated bilirubin remained high.
July 20, 2016 - Take the Good with the Bad
Between June and July Asthon had several weight checks with little change. It was decided at this point a nasogastric feeding tube needed to be place to help facilitate his weight gain. On the flip side for the first time in Ashton’s life his conjugated bilirubin was perfectly normal! Again we were told that this happened a little later than it does for most but Ashton is paving his own path of what it means to have Biliary Atresia so we would continue to follow his lead with intervention when necessary.
August to October 2016 - The Joys of Tube Feeding
Ashton continued to receive night time feeds through his NG tube which was absolutely no fun for any of us! Machines beeping throughout the night, tubes being pulled out and replaced and all the TAPE! Ashton was gaining at a very slow rate but he was gaining and his conjugated bilirubin remained normal so all of the effort was worth it.
November 23-28, 2016 - Cholangitis Round 1
Ashton was admitted to TCH with fever, diarrhea and elevated conjugated bilirubin. After labs, urinalysis, ultrasounds and nasal swabs no other infection could be found and cholangitis was our diagnosis. Cholangitis happens as result of bacteria from the intestine flowing back into the liver, each episode of cholangitis takes away from the life expectancy of Ashton’s liver. We spent Ashton's first Thanksgiving with the lovely staff at TCH on IV antibiotics and having his first central line placed. The silver lining was he was taken off of the tube feedings.
December 2-4, 2016 - Right Back In
Ashton spiked a fever while on IV antibiotics and we were again looking for answers, this time hoping to rule out line infection. So we were back having labs taken from his line and thankfully his fever was not due to the line and we were discharged.
December 14, 2016
This was a happy day because Ashton had his central line removed and his conjugated bilirubin was back to normal
January to February 2017 - Milestones
We officially have a ONE year old! Ashton had his 1st birthday and reached his 1 year post Kasai mark. Things had been going very well since his last admission and we felt relief and a sense of normalcy that we hadn’t had in months.
March 22, 2017 - Cholangitis Round 2
Ashton was again admitted with fever and elevated conjugated bilirubin and had a full workup to rule out other forms of infection, but once again cholangitis was the culprit. It was also discovered that Ashton’s liver had developed a collection of bile called a biloma which increases the risk of him developing cholangitis in the future. Ashton once again had a central line placed and was sent home for a round 2 of IV antibiotics.
April 16, 2017 - Cholangitis Round 2.5
We went in to have Asthon’s central line removed but discovered that his cholangitis had relapsed. Since cholangitis affects the life of his liver and he had already had 2 full episodes, Ashton’s doctors determined it was in his best interest to be evaluated for transplant.
April 18-20, 2017 - Transplant Evaluation
Ashton was admitted for a change in his antibiotic and to have his liver transplant evaluation. This was a detailed process involving several specialties and lots of labs, x-rays, ultrasounds, as well as physical and developmental evaluations.
May 2017 - TCH Medical Review Board
Ashton’s case was presented and approved by the TCH Medical Board and he was officially listed on the liver transplant list.
June 3-5, 2017 - False Alarm
Ashton was again admitted for fever and to have a full workup to determine the cause. IV antibiotics were started immediately because of his history of cholangitis. His doctors were both happy and confused when labs did not find a cause and his conjugated bilirubin was normal. It is suspected that we may have caught the infection (cholangitis) early enough that his bilirubin did not have the chance to elevate but we can’t be certain. Ultrasound showed that the biloma in his liver had increased in size slightly and his doctors considered having it drained, however it was determined that the risk of draining it did not outweigh the benefits. So Ashton was discharged fever and IV free!
September 27, 2017 - CheckUp
At his checkup today, Ashton's spleen is slightly more enlarged. It is soft though and soft is good news. His weight gain is good, but for developmental purposes he needs to increase his table food intake so that we can start to decrease his formula. His dieticians are considering occupational therapy if he doesn't start eating more "real" food. He also took his flu shot like a champ!
Some of Ashton's lab results have come in, most of his liver panel results are "his normal" which means they are high but that is part of his disease process. The lab result most important to Ashton's well being is his conjugated bilirubin. We like to see 0.0, but today he was at a 0.1. This is such a delicate number, 0.0 is what you want to see, 0.1 is the high end of normal and 0.2 is indicates active infection.
For now, we are monitoring him for any signs that could mean that number is going up. Another episode of cholangitis will bump him up on the list and we will be actively seeking a liver/accepting offers. Prayers that his conjugated bilirubin stays within normal limits are appreciated. We want to postpone transplant as long as possible. The bigger he gets before transplant the better.
November 8, 2017 - Taking the Good with the Bad
Despite his inconsistent eating patterns, Ashton has gained weight and is up to 27 lbs! His dieticians said we should stay the course and not stress how much table food he eats or the fact that he is still taking a bottle. Therapy will be considered after his 2nd birthday if needed.
Dr. H said that Ashton's spleen has become even more enlarged since the last visit. This makes 2 consecutive visits of spleen growth. He said the growth of his spleen indicates that Ashton's liver is scarring and hardening aka cirrhosis is progressing. He again reminded us of the potential for portal hypertension to cause Ashton to vomit blood and he also said more long term thinking is the need for ultrasounds to monitor the state of Ashton's liver. As cirrhosis progresses the abnormal cells produced through cirrhosis can multiply. Ashton will start getting ultrasounds at his next appointment.
Dr. H also mentioned that at some point next year we may consider being more aggressive in finding a new liver for Ashton to help avoid some of these potential issues. As always, we will monitor Ashton's condition and let his body be our guide.
Johnny and I are grateful for the current state of Ashton's health because we know it could be worse. We were taken aback by this "long term" news. We are still working through the surprise of it all, but we have faith that God is in control and that Ashton has the very best team in charge of his care.
Best news from this appointment - Ashton's conjugated bilirubin is back down to ZERO where it belongs! Some of Ashton's other levels are way out of range, but this is expected with his liver being diseased.
Johnny and I both had a lot of anxiety about how his labs would be because he was at a 0.1 last visit and we are coming up on the 1 year anniversary of his first episode of cholangitis, but the power of prayer prevails! After the news of cirrhosis and abnormal cells multiplying we needed this good news. Dr. H said he was "very pleased" with Ashton's results and so are we!
November 10th - Cause for Concern
Around 1:30 am Ashton was running a 101.8 fever, by mid-morning it had only dropped to 101.5. Because of his history, he was sent for labs to rule out cholangitis and seen by the pediatrician at determine if there were any other "normal" baby illnesses causing his fever spike. Unfortunately, labs came back and his conjugated bilirubin is up to a .3. We packed our bags and headed back to Texas Children's to be treated for cholangitis.
November 11th - My Fear
Ashton's fever had cleared and he was in great spirits. He threw the doctors for a good loop, when labs showed his bilirubin was back down to zero and was so BEFORE they started antibiotics. Leaving to question whether or not this was actually a case of cholangitis. An ultrasound showed that the biloma in his liver had gotten larger which also presents as a possible source for his fever and bilirubin spike. We were in TCH until the doctors made a decision based off of his labs as to whether or not we would go home with a PICC line or if Interventional Radiology could do a small procedure to drain the biloma.
November 12th - Labs at Zero
Labs were still zero so it was looking like we wouldn’t have 21 days of IV antibiotics. We were still waiting to hear what Interventional Radiology says about draining his biloma.
November 13th - Not Going Home Yet
As much as we hoped and prayed Ashton was told he would be staying another day so his team could observe him for 24 hours without antibiotics to make sure he continued to do well. We were also still waiting on a decision from Interventional Radiology as to whether or not they would drain the biloma. That afternoon we got our answer, the biloma was too difficult to get to and Ashton would possibly have to go home with a drain port if they were to attempt draining it. So we settled in for at least another 24 hours without IV antibiotics to make sure he stayed fever free and bilirubin stayed at zero.
November 14th - Another PICC Line
Unfortunately, his labs were elevated again and Ashton had to have a PICC line placed. It was a long day on the 12th floor of TCH having to be NPO before the procedure but we survived! Ashton stayed a bit groggy from having the PICC line placed. They gave him a med to help with his temper coming out of anesthesia because he was pretty rowdy before going back.
November 15th - Finally Going Home
We finally got our “walking papers” and were sent home on 3 weeks of round the clock antibiotics (every 6 hours) and hopes that this episode of cholangitis would be cleared ASAP so we could get back to our normal routine. Ashton was so excited to be going home that laughed and squealed as he ran the hallways and rode the elevator all the way to the car! Even though we had a long 3 weeks ahead we were so happy to go home!
January 3rd - 1st CheckUp of 2018
Ashton had another check-up, labs, and routine ultrasound done today. Ashton is growing! He is 2' 10" and 28 lbs 3.5 oz which puts him just above the 50th percentile. Mom, Dad, and Dr. H couldn't be happier.
Labs are stable (basically the same as last month), conjugated bilirubin in 0.0 which is what we like to see! We are still waiting on his Vitamin A results to see if us increasing him to 16,000 iu has helped. The only area of concern is that Ashton's platelet count it is a bit low. The low end of normal is 150 and Ashton is at 125. This is an indication that the liver cirrhosis is causing portal hypertension which can lead to the scary episodes of vomiting blood that we have been warned about.
His ultrasound results are the true show stopper for this update. The biloma that was 9.3 x 5.7 x 4.9 cm is down in size to less than 2 cm all in less than 2 months time. His spleen has also decreased in size by 1.3 cm! Overall, we are very happy with this news. Ashton has grown and the biloma and spleen have gotten smaller! We will watch his platelet count closely and enjoy this time of overall good health.
Many people do not understand, "if he needs it why do we wait". We wait for several reasons #1 the older Ashton is at transplant the easier recovery will be and the less risky the surgery is and #2 When we transplant, it does not mean "cure". We will be trading one set of worries for another. With transplant comes a medically suppressed immune system which means a common cold could mean hospital stay, we have risk of Ashton's body rejecting the liver not just once but multiple times, and we will have the awful EBV levels to watch because this can lead to PTLD which is a type of cancer transplant patients can develop.
Ashton and his fellow BA fighters are in this battle for life. They may look "normal" but their bodies are constantly working double time. Their families all adopt a "new normal" where daily medication, hospital stays and being prepared for life to change at the drop of a hat are the way of life. We are on our 2 year anniversary of the road to diagnosis.