What is biliary atresia?
Biliary atresia is a life-threatening condition in infants in which the bile
ducts inside or outside the liver do not have normal openings.
Bile ducts in the liver, also called hepatic ducts, are tubes that carry bile
from the liver to the gallbladder for storage and to the small intestine for use
in digestion. Bile is a fluid made by the liver that serves two main functions:
carrying toxins and waste products out of the body and helping the body digest
fats and absorb the fat-soluble vitamins A, D, E, and K.
With biliary atresia, bile becomes trapped, builds up, and damages the liver.
The damage leads to scarring, loss of liver tissue, and cirrhosis. Cirrhosis is
a chronic, or long lasting, liver condition caused by scar tissue and cell
damage that makes it hard for the liver to remove toxins from the blood. These
toxins build up in the blood and the liver slowly deteriorates and malfunctions.
Without treatment, the liver eventually fails and the infant needs a liver
transplant to stay alive.
The two types of biliary atresia are fetal and perinatal. Fetal biliary
atresia appears while the baby is in the womb. Perinatal biliary atresia is much
more common and does not become evident until 2 to 4 weeks after birth. Some
infants, particularly those with the fetal form, also have birth defects in the
heart, spleen, or intestines.
Biliary atresia is a life-threatening condition in infants in which the bile
ducts inside or outside the liver do not have normal openings.
Bile ducts in the liver, also called hepatic ducts, are tubes that carry bile
from the liver to the gallbladder for storage and to the small intestine for use
in digestion. Bile is a fluid made by the liver that serves two main functions:
carrying toxins and waste products out of the body and helping the body digest
fats and absorb the fat-soluble vitamins A, D, E, and K.
With biliary atresia, bile becomes trapped, builds up, and damages the liver.
The damage leads to scarring, loss of liver tissue, and cirrhosis. Cirrhosis is
a chronic, or long lasting, liver condition caused by scar tissue and cell
damage that makes it hard for the liver to remove toxins from the blood. These
toxins build up in the blood and the liver slowly deteriorates and malfunctions.
Without treatment, the liver eventually fails and the infant needs a liver
transplant to stay alive.
The two types of biliary atresia are fetal and perinatal. Fetal biliary
atresia appears while the baby is in the womb. Perinatal biliary atresia is much
more common and does not become evident until 2 to 4 weeks after birth. Some
infants, particularly those with the fetal form, also have birth defects in the
heart, spleen, or intestines.
Logan's Story...
On November 6, 2013, really early on a cold morning, Derek, Devin and I made our way to the
hospital. Logan was born by c-section at 10:10 a.m. He was a beautiful, healthy looking baby at
7 pounds and 11 ounces. Logan was surrounded by love from the second he was born and really before that! We had been waiting for him for a long time. He was a true dream come true for Derek and I and our family. Although he was slightly jaundiced at birth, we didn't have any major reason for concern. We were released from the hospital five days after his birth and started adjusting to our new life.
We celebrated Devin's 12th birthday shortly after we were home. It was so nice for everyone to be able to spend time with both our boys at home and Logan didn't seem to mind the loud craziness of our huge family! Logan's two week check up with his pediatrician went very well! When we asked about his color and jaundice, which had appeared to be gone at this point, the doctor said there was no reason to retest his blood and that he looked good! This was great news for us and so we carried on celebrating the holiday season with family gatherings for Thanksgiving including a trip to Richmond! Holidays are a busy time for everyone, especially with a large family, so on December 7, 2013 we had a family and friends Christmas Party! We rented a venue and had a great turn out, at the party my mom and Aunt Christina noticed that Logan's eyes had a slight yellow look. Derek and I had to agree with them so I called his doctor first thing Monday morning. The doctor's advice was to keep an eye on it and let him know if it got darker. So we figured nothing to worry about and continued on with everyday life.
Logan slept a lot on Christmas Eve, which had me a little concerned but I figured maybe a growth spurt? And he did wake up long enough to take pictures with Santa (Granddad) and make his first cookie! But that night he wasn't eating much and I mentioned to Derek that I felt like something was wrong. Christmas morning was great!! Devin got a new tablet and loved it! Derek and I were having a blast watching both boys check out their new stuff and things were pretty perfect. Logan seemed back to normal so we packed up the car and headed to Uncle Jarrod and Aunt Lorrie's house to celebrate Christmas morning with them, Caleb, Grandma and Nana. We had a great meal and were having a great time until I checked Logan's diaper and his urine was brown. I paged his doctor and was told to go to the emergency room right away. And that was the beginning of a whirlwind few days that would change our lives.
On November 6, 2013, really early on a cold morning, Derek, Devin and I made our way to the
hospital. Logan was born by c-section at 10:10 a.m. He was a beautiful, healthy looking baby at
7 pounds and 11 ounces. Logan was surrounded by love from the second he was born and really before that! We had been waiting for him for a long time. He was a true dream come true for Derek and I and our family. Although he was slightly jaundiced at birth, we didn't have any major reason for concern. We were released from the hospital five days after his birth and started adjusting to our new life.
We celebrated Devin's 12th birthday shortly after we were home. It was so nice for everyone to be able to spend time with both our boys at home and Logan didn't seem to mind the loud craziness of our huge family! Logan's two week check up with his pediatrician went very well! When we asked about his color and jaundice, which had appeared to be gone at this point, the doctor said there was no reason to retest his blood and that he looked good! This was great news for us and so we carried on celebrating the holiday season with family gatherings for Thanksgiving including a trip to Richmond! Holidays are a busy time for everyone, especially with a large family, so on December 7, 2013 we had a family and friends Christmas Party! We rented a venue and had a great turn out, at the party my mom and Aunt Christina noticed that Logan's eyes had a slight yellow look. Derek and I had to agree with them so I called his doctor first thing Monday morning. The doctor's advice was to keep an eye on it and let him know if it got darker. So we figured nothing to worry about and continued on with everyday life.
Logan slept a lot on Christmas Eve, which had me a little concerned but I figured maybe a growth spurt? And he did wake up long enough to take pictures with Santa (Granddad) and make his first cookie! But that night he wasn't eating much and I mentioned to Derek that I felt like something was wrong. Christmas morning was great!! Devin got a new tablet and loved it! Derek and I were having a blast watching both boys check out their new stuff and things were pretty perfect. Logan seemed back to normal so we packed up the car and headed to Uncle Jarrod and Aunt Lorrie's house to celebrate Christmas morning with them, Caleb, Grandma and Nana. We had a great meal and were having a great time until I checked Logan's diaper and his urine was brown. I paged his doctor and was told to go to the emergency room right away. And that was the beginning of a whirlwind few days that would change our lives.
We arrived at the emergency room in Williamsburg around mid afternoon on Christmas Day. My dad met us there and took Devin back to his house while Derek, Logan and I got into a small room and waited. They did an x ray, blood work, asked a lot of questions and we were worried but not too scared. Our friend Patricia, a nurse on the labor and delivery floor, that had taken care of Logan when he was born came down to visit us. She suggested we get print outs of Logan's blood work and pointed out that his "direct" numbers were still too high. Now honestly, Derek and I were confused. Why did the pediatrician not seemed worried about it? We were released from the emergency room that night and told to take Logan in to see his doctor the next morning. We managed about an hour of Christmas time with my family before it was time to go home to get some sleep.
And this is when things started to get scary. When I called the pediatrician to set up a visit they told me they had already consulted with a doctor at Children's Hospital of the King's Daughters and that they would be contacting us. It didn't take long before they called and let me know that they had scheduled an ultrasound for Logan first thing the next morning, Friday, December 27 and then we would be having an appointment with a gastroenterology doctor. I remember getting off the phone, telling Derek what they said and being confused and really concerned as to what this meant. Our parents and my grandmother, Derek's grandmother and Devin went with us for that visit. Logan did great during his ultrasound, he slept on and off and was very calm. We made our way upstairs for the appointment pretty early, fed Logan and they took us right back. Their was a lot of paperwork to do so it seemed like no time before the doctor was in the room. Dr. Atay was great from the start, very personable and friendly, open and intelligent. Looking back I'm really glad that it was him we saw that day because he was about to deliver the worst news we'd ever gotten. Dr. Atay explained that Logan's ultrasound was to look for a gallbladder but they couldn't find one. This combined with the jaundice led him to believe that Logan had biliary atresia, he explained very briefly what that meant and that things were going to start happening quick. He said Logan would go on medication over the weekend and then have a HIDA scan on Monday. On Tuesday, New Years Eve, Logan would be put under and have a liver biopsy and be admitted to the hospital. He would be monitored on Wednesday and on Thursday morning he would have the Kasai procedure done. At 8 weeks and one day old. He explained that after 8 weeks of age the odds of success drop by 50%. I tried to keep it together but broke down crying when when told our family what the doctor said. I couldn't imagine him having to go through that. I didn't really understand what all of this meant? What was wrong with our baby? And so we started to do some research and things started to get real.
And this is when things started to get scary. When I called the pediatrician to set up a visit they told me they had already consulted with a doctor at Children's Hospital of the King's Daughters and that they would be contacting us. It didn't take long before they called and let me know that they had scheduled an ultrasound for Logan first thing the next morning, Friday, December 27 and then we would be having an appointment with a gastroenterology doctor. I remember getting off the phone, telling Derek what they said and being confused and really concerned as to what this meant. Our parents and my grandmother, Derek's grandmother and Devin went with us for that visit. Logan did great during his ultrasound, he slept on and off and was very calm. We made our way upstairs for the appointment pretty early, fed Logan and they took us right back. Their was a lot of paperwork to do so it seemed like no time before the doctor was in the room. Dr. Atay was great from the start, very personable and friendly, open and intelligent. Looking back I'm really glad that it was him we saw that day because he was about to deliver the worst news we'd ever gotten. Dr. Atay explained that Logan's ultrasound was to look for a gallbladder but they couldn't find one. This combined with the jaundice led him to believe that Logan had biliary atresia, he explained very briefly what that meant and that things were going to start happening quick. He said Logan would go on medication over the weekend and then have a HIDA scan on Monday. On Tuesday, New Years Eve, Logan would be put under and have a liver biopsy and be admitted to the hospital. He would be monitored on Wednesday and on Thursday morning he would have the Kasai procedure done. At 8 weeks and one day old. He explained that after 8 weeks of age the odds of success drop by 50%. I tried to keep it together but broke down crying when when told our family what the doctor said. I couldn't imagine him having to go through that. I didn't really understand what all of this meant? What was wrong with our baby? And so we started to do some research and things started to get real.
The HIDA Scan. Bright and early on Monday, December 30, we made our way back to Norfolk with Grandma, Maw, Granddad and Devin. We were taken into a big room with a HUGE machine. This looked a whole lot more intimidating than it actually was, thank God! Logan spent most of this time sleeping or sucking at his pacifier while watching the overhead TV as the huge cameras and bed moved all around him. He looked so small up there. He did so good, he was just started to show how calm and sweet he was. Derek and I sat on either side and the bed and watched him, the overhead TV only seemed to be playing Baby Einstein or the special features DVD from Despicible Me 2. Total the scan took 6 hours but Logan got breaks to eat. The grandparents and Devin made do with cafeteria food, the Starbucks and the really bad WiFi in the waiting room. It turned out when we were done that they still needed a few more shots so we were asked to report back in even earlier the next morning, before the biopsy, to do those. So we went back home, its funny but I don't remember anything else from that night, not packing or freaking out or anything.
I know that we had to be back in the hospital at 6, which meant we had to get up at 3 am. Logan wasn't able to eat after midnight. We rushed him to the scan and got the pictures done quickly, then went to the surgery center and checked in. Then we went to one waiting room and then we got moved to another waiting room. Then Derek, Logan and I were taken back to a regular room and Logan got into his little Looney Tunes gown. And then we went back to the waiting room and were told that the doctors, three of them, would come talk to us. I was getting pretty upset because Logan was getting upset. He was tired and hungry and I felt awful that we couldn't feed him. We had a crowd in the waiting room, Devin, Maw, Granddad, Grandma, Mimi and Aunt Beth were all there waiting too. Slowly the doctors came out, first the anesthsiologist to explain how they would be putting Logan to sleep for the procedure, then the doctor doing the procedure, then we got moved back to another room and another doctor showed us how she would use the ultrasound machine to make sure they got exactly where they wanted to in Logan's liver, they would remove a piece and be done in no time. Two young nurses let us walk Logan about half way down the hallway and then they took him the rest of the way and we waited. We waited in the big waiting room where they have all the procedures going on listed up on a board so that you can see where you child is.. thankfully this was so quick they didn't have time to get Logan up on the board before he was done. It was such a relief to be back with him. I'm not a big fan of strangers having my boys. And then we got checked into CHKD for the first time, into room 803. This was a shared room which was a little crazy because we had so many people coming up to visit Logan. Dr. Tsou was on-call for the GI team, although we weren't as crazy about him as we were about Dr. Atay, he explained everything we needed to know. He was the one that delivered the news that the HIDA scan confirmed the diagnosis of Biliary Atresia. That Wednesday would be a day of monitoring Logan and that he would indeed have to have surgery on Thursday morning. I don't think I can put in words how horrible that felt. The whole world seemed to stop. Maw was holding Logan in the chair and I think up until that point she refused to believe that it was really going to happen because she started to cry and she never cries. I remember asking Dr. Tsou if Logan was going to die and he said no. It wasn't until much later that I realized he must have been feeling confident because babies die of this all the time. I don't remember a whole lot more of that day, it was a whirlwind of getting adjusted to hospital life, vitals being checked, doctors coming in, asking questions, filling out forms, taking phone calls, trying to keep family members updated. Logan was calm and cool through it all. We didn't know he was already a fighter. A super hero. He was waiting to show us.
January 1, 2014, New Year's Day was pretty uneventful for us. We spent the night before watching the ball drop, barely, because we were half asleep. Logan decided it wasn't that exciting and so he ripped his IV out. Scared me and Derek and the nurses to death! There was just blood everywhere!!! We met with Dr. Tsou and then Dr. Obershain came in to discuss the surgery. He explained that they would go in and scrape out what was left of his gallbladder, then attached a piece of his intestine to his liver to create a direct line for the bile. He also said that they would take another biopsy since they were already in there. When I asked how many times they had preformed this Kasai procedure his response was "no one has preformed a lot of them" " this isn't common" " we are going to give him his best chance". In writing this I realize there just are not words for how some of this felt. As a parent knowing something is wrong with your baby, its scary. Knowing there is no cure is terrifying. Knowing they are about to have a surgery that the doctors expect to fail and that they will likely go into immediate liver failure is life altering. I want Logan to know that we made the best decision we could. We believed that the Kasai procedure was the best thing to do, I don't think we fully realized until I talked to Dr. Obershain how close we were to them deciding it was too late to do the surgery because Logan was over 8 weeks. And because they didn't expect it to work. We got moved to room 802 shortly after the talk with the doctor, this is where Logan saw TV for the first time, where he drank Pedialyte for the first time, where we prayed that this would work, where we waited for them to come get us and take him down to surgery. Seeing him lay on that big bed when they wheeled him away was the worst feeling. I just wanted to hold him, I didn't want to let go. We went back to that big waiting room. They said the Kasai normally takes 4-8 hours. Expect about 6. They called when they started the procedure, all they said was "the doctor has begun, Logan is doing fine". They called an hour later and said "he's doing fine". I don't remember a whole lot of the waiting. I remember looking at Devin, he was so healthy and so beautiful and I was so grateful. How did I take that for granted for so long? And I looked at William and prayed one day Logan was going to crawl up next to him and steal his snacks. And I looked at Christina and Addison drawing a picture for Logan and smiled. And I looked at my husband and wondered what I would do without him. Our parents never left our side, my mom and dad, his mom, my grandmother, his grandmother, my brother Mike and his wife Christina, their kids, my brother Eddie and his fiancée Michelle, my aunt Beth, they were there. And a TON of other people visited. And Devin. Everyday Devin waited to see what was going to happen to his brother. At 3 hours they called, "he's doing fine". At 4 hours...no call. We waited. We stood by the phone. We paced. Finally the receptionist called the OR for us. She said the doctor is closing now. I thought something must be wrong. He said 6 hours. What if the damage was too bad? What if they couldn't "fix" him? The fact was we wouldn't know for months if this was a success. Less than an hour later we were in the PICU, staring down at our little hero. He looked like a fighter now. There were tubes everywhere. He was on oxygen. He was asleep still with classical music playing. He still looked beautiful. And he was ready for this fight.
We live our lives day by day. Logan fights everyday. This is just our introduction to his story. We are going to be using this site to do blogs to keep track of Logan's journey. We want to remember everything he has had to go through and one day when it get hard or if he needs a transplant, he can look at this and know he's already done so much. He can do anything. We are going to invite other people close to us to blog as well to share their stories of Logan. We will be using the "About" page to keep track of news on Biliary Atresia and Transplant Information.
Thank you for being part of our story.
I know that we had to be back in the hospital at 6, which meant we had to get up at 3 am. Logan wasn't able to eat after midnight. We rushed him to the scan and got the pictures done quickly, then went to the surgery center and checked in. Then we went to one waiting room and then we got moved to another waiting room. Then Derek, Logan and I were taken back to a regular room and Logan got into his little Looney Tunes gown. And then we went back to the waiting room and were told that the doctors, three of them, would come talk to us. I was getting pretty upset because Logan was getting upset. He was tired and hungry and I felt awful that we couldn't feed him. We had a crowd in the waiting room, Devin, Maw, Granddad, Grandma, Mimi and Aunt Beth were all there waiting too. Slowly the doctors came out, first the anesthsiologist to explain how they would be putting Logan to sleep for the procedure, then the doctor doing the procedure, then we got moved back to another room and another doctor showed us how she would use the ultrasound machine to make sure they got exactly where they wanted to in Logan's liver, they would remove a piece and be done in no time. Two young nurses let us walk Logan about half way down the hallway and then they took him the rest of the way and we waited. We waited in the big waiting room where they have all the procedures going on listed up on a board so that you can see where you child is.. thankfully this was so quick they didn't have time to get Logan up on the board before he was done. It was such a relief to be back with him. I'm not a big fan of strangers having my boys. And then we got checked into CHKD for the first time, into room 803. This was a shared room which was a little crazy because we had so many people coming up to visit Logan. Dr. Tsou was on-call for the GI team, although we weren't as crazy about him as we were about Dr. Atay, he explained everything we needed to know. He was the one that delivered the news that the HIDA scan confirmed the diagnosis of Biliary Atresia. That Wednesday would be a day of monitoring Logan and that he would indeed have to have surgery on Thursday morning. I don't think I can put in words how horrible that felt. The whole world seemed to stop. Maw was holding Logan in the chair and I think up until that point she refused to believe that it was really going to happen because she started to cry and she never cries. I remember asking Dr. Tsou if Logan was going to die and he said no. It wasn't until much later that I realized he must have been feeling confident because babies die of this all the time. I don't remember a whole lot more of that day, it was a whirlwind of getting adjusted to hospital life, vitals being checked, doctors coming in, asking questions, filling out forms, taking phone calls, trying to keep family members updated. Logan was calm and cool through it all. We didn't know he was already a fighter. A super hero. He was waiting to show us.
January 1, 2014, New Year's Day was pretty uneventful for us. We spent the night before watching the ball drop, barely, because we were half asleep. Logan decided it wasn't that exciting and so he ripped his IV out. Scared me and Derek and the nurses to death! There was just blood everywhere!!! We met with Dr. Tsou and then Dr. Obershain came in to discuss the surgery. He explained that they would go in and scrape out what was left of his gallbladder, then attached a piece of his intestine to his liver to create a direct line for the bile. He also said that they would take another biopsy since they were already in there. When I asked how many times they had preformed this Kasai procedure his response was "no one has preformed a lot of them" " this isn't common" " we are going to give him his best chance". In writing this I realize there just are not words for how some of this felt. As a parent knowing something is wrong with your baby, its scary. Knowing there is no cure is terrifying. Knowing they are about to have a surgery that the doctors expect to fail and that they will likely go into immediate liver failure is life altering. I want Logan to know that we made the best decision we could. We believed that the Kasai procedure was the best thing to do, I don't think we fully realized until I talked to Dr. Obershain how close we were to them deciding it was too late to do the surgery because Logan was over 8 weeks. And because they didn't expect it to work. We got moved to room 802 shortly after the talk with the doctor, this is where Logan saw TV for the first time, where he drank Pedialyte for the first time, where we prayed that this would work, where we waited for them to come get us and take him down to surgery. Seeing him lay on that big bed when they wheeled him away was the worst feeling. I just wanted to hold him, I didn't want to let go. We went back to that big waiting room. They said the Kasai normally takes 4-8 hours. Expect about 6. They called when they started the procedure, all they said was "the doctor has begun, Logan is doing fine". They called an hour later and said "he's doing fine". I don't remember a whole lot of the waiting. I remember looking at Devin, he was so healthy and so beautiful and I was so grateful. How did I take that for granted for so long? And I looked at William and prayed one day Logan was going to crawl up next to him and steal his snacks. And I looked at Christina and Addison drawing a picture for Logan and smiled. And I looked at my husband and wondered what I would do without him. Our parents never left our side, my mom and dad, his mom, my grandmother, his grandmother, my brother Mike and his wife Christina, their kids, my brother Eddie and his fiancée Michelle, my aunt Beth, they were there. And a TON of other people visited. And Devin. Everyday Devin waited to see what was going to happen to his brother. At 3 hours they called, "he's doing fine". At 4 hours...no call. We waited. We stood by the phone. We paced. Finally the receptionist called the OR for us. She said the doctor is closing now. I thought something must be wrong. He said 6 hours. What if the damage was too bad? What if they couldn't "fix" him? The fact was we wouldn't know for months if this was a success. Less than an hour later we were in the PICU, staring down at our little hero. He looked like a fighter now. There were tubes everywhere. He was on oxygen. He was asleep still with classical music playing. He still looked beautiful. And he was ready for this fight.
We live our lives day by day. Logan fights everyday. This is just our introduction to his story. We are going to be using this site to do blogs to keep track of Logan's journey. We want to remember everything he has had to go through and one day when it get hard or if he needs a transplant, he can look at this and know he's already done so much. He can do anything. We are going to invite other people close to us to blog as well to share their stories of Logan. We will be using the "About" page to keep track of news on Biliary Atresia and Transplant Information.
Thank you for being part of our story.