Liliana Jay Moore was born at 07:30 on October 4, 2013 at Yale-New Haven Hospital in New Haven, Connecticut. She came into this world after five hours of labor. She was a healthy 8 lbs 10 oz and everything seemed to be in order. We counted her toes and fingers and were in complete “awe” by our baby girl. By her third day the doctors gave us the ok to take her home and we couldn’t be more excited…. And nervous as any new parent would be. When we left the hospital it was brought to our attention that her bilirubin was still elevated but within a range to where the hospital staff felt comfortable sending us home stating “It would decrease over the course of the next 2 weeks.” (1)
When we arrived at home we began experiencing all of the exciting new things that newborns do; like eye rolling, sneezing, feeding, napping, making weird faces and pooping of course.
It wasn’t until 3-4 weeks after we came home that she began appearing a little on the tan side. We discussed this concern with our pediatrician who assured us that it was something called Breastfeeding jaundice.(2) Since Lili was only receiving breast milk at the time we took this diagnosis at face value and went on our way thinking that over the course of the next few weeks the jaundice would resolve. At this time, Jayme and Lili had been attending a lactation group as well and Lily was able to receive transcutaneous bilirubin (3) checks when they attended. This allowed us to keep track of her levels. A rotating group of pediatricians also attended the group to answer any questions breastfeeding mothers might have. They expressed some concern in her bilirubin levels and jaundice appearance but didn’t take any farther action because we told them that our pediatrician was aware . As time passed Lily’s levels slowly climbed and her jaundice never subsided (although some days were better than others). When Lily was approximately 10 weeks old we began to notice that her stools were very pale in color. Upon hearing this new sign the pediatrician at the lactation group advised us to bring her to her primary pediatrician office right away. After a very short visit at the pediatrician office, we were sent to the emergency room.
It wasn’t until 3-4 weeks after we came home that she began appearing a little on the tan side. We discussed this concern with our pediatrician who assured us that it was something called Breastfeeding jaundice.(2) Since Lili was only receiving breast milk at the time we took this diagnosis at face value and went on our way thinking that over the course of the next few weeks the jaundice would resolve. At this time, Jayme and Lili had been attending a lactation group as well and Lily was able to receive transcutaneous bilirubin (3) checks when they attended. This allowed us to keep track of her levels. A rotating group of pediatricians also attended the group to answer any questions breastfeeding mothers might have. They expressed some concern in her bilirubin levels and jaundice appearance but didn’t take any farther action because we told them that our pediatrician was aware . As time passed Lily’s levels slowly climbed and her jaundice never subsided (although some days were better than others). When Lily was approximately 10 weeks old we began to notice that her stools were very pale in color. Upon hearing this new sign the pediatrician at the lactation group advised us to bring her to her primary pediatrician office right away. After a very short visit at the pediatrician office, we were sent to the emergency room.
While in the emergency room they drew blood from Lily to run liver function tests and they explained to us that there are many causes of jaundice and that breastfeeding jaundice was most likely the cause. This seemingly common assumption was dispelled when a number of the liver tests came back extremely high. Over the next 12 hrs in the emergency room she received additional blood tests and an ultrasound; which all confirmed her liver was enlarged and there wasn’t adequate bile1 drainage. As stated before, there are many conditions that can cause jaundice. One of the more time sensitive diagnoses is biliary atresia (BA) , which led to the next step in the process: a liver biopsy. The presence of excess bile ducts may indicate biliary atresia. This is exactly what was found but to confirm this diagnosis an intraoperative cholangiogram (4) would need to be performed. The procedure took place 5 days later on December 20th 2013 and was to be immediately followed by a Kasai procedure (5) if biliary atresia was the finding and this is exactly what happened.
I must say the moment we heard that she had Biliary Atresia we were in a state of denial followed by a mixture of anger and sadness. Our thoughts were not only is BA a life threatening disorder but it would also put our daughter at a significant disadvantage, in multiple aspects of life, from the start. Regardless, our love for Lili was unconditional and we began digging in for the long haul, knowing we would get through this together.
The Kasai procedure took 6 hours and went well. Lily recovered fast and we were released on Christmas day. It was the best Christmas present to say the least!
When she was 6 months old she had back-to-back bouts of cholangitis (7), being hospitalized for 5 and 7 days respectively. After the second episode it was decided that she should have a peripherally inserted central catheter (PICC) placed to receive a 6-week course of antibiotics at home. We named her new friend “Pixie”. The line was removed in mid June 2014 and since than we have been fortunate enough to not have a repeat episode.
I must say the moment we heard that she had Biliary Atresia we were in a state of denial followed by a mixture of anger and sadness. Our thoughts were not only is BA a life threatening disorder but it would also put our daughter at a significant disadvantage, in multiple aspects of life, from the start. Regardless, our love for Lili was unconditional and we began digging in for the long haul, knowing we would get through this together.
The Kasai procedure took 6 hours and went well. Lily recovered fast and we were released on Christmas day. It was the best Christmas present to say the least!
When she was 6 months old she had back-to-back bouts of cholangitis (7), being hospitalized for 5 and 7 days respectively. After the second episode it was decided that she should have a peripherally inserted central catheter (PICC) placed to receive a 6-week course of antibiotics at home. We named her new friend “Pixie”. The line was removed in mid June 2014 and since than we have been fortunate enough to not have a repeat episode.
Over the course of the last 9 months Lily has done great. She has been gaining weight and meeting all of physical/developmental milestones. Currently, she is only taking two medications; which is significantly less than the 7-8 medications she was previously taking.
Currently her PELD (Pediatric End-stage Liver Disease) (6) score is -5. This time, however, has not been without its struggles.
There is no telling if this situation has or is affecting her psychologically. Stress during early development may have such consequences, however, she is a very happy baby and smiles more than any other baby we have seen.
During the evaluation for placement onto the liver transplant list, an ultrasound discovered that she has a lesion (8) on her liver. The transplant team/ board accepted Lili onto the transplant list on July 23rd, 2014.
She received an MRI on August 8th 2014 to confirm size and location of the lesion. Her transplant team decided to hold off on any invasive procedures at this time and reevaluate the lesion in three months by another MRI or ultrasound.
Currently her PELD (Pediatric End-stage Liver Disease) (6) score is -5. This time, however, has not been without its struggles.
There is no telling if this situation has or is affecting her psychologically. Stress during early development may have such consequences, however, she is a very happy baby and smiles more than any other baby we have seen.
During the evaluation for placement onto the liver transplant list, an ultrasound discovered that she has a lesion (8) on her liver. The transplant team/ board accepted Lili onto the transplant list on July 23rd, 2014.
She received an MRI on August 8th 2014 to confirm size and location of the lesion. Her transplant team decided to hold off on any invasive procedures at this time and reevaluate the lesion in three months by another MRI or ultrasound.
1. Almost all babies experience elevated bilirubin levels. Bilirubin is a product of the breakdown of red blood cells and is a necessary process. Bilirubin and other products come together to form bile and this is then excreted out of the liver into the small intestine through bile ducts. Bilirubin is normally elevated in newborns for a few reasons; 1. Newborns have a lot of fetal red blood cells, which are different from adult red blood cells. Their life span is much shorter than adult red blood cells and because of the quantity and short life span there is an increase in breakdown products of red blood cells, which produces more bilirubin. 2. The removal of bilirubin is slower in newborns because they have lower levels of a certain enzyme which aids in this process. 3. In a normal adults bilirubin/bile would be dumped into the small intestine and broken down by bacteria (this will give stool the characteristic brown color) but in newborns there is not that much bacteria to breakdown the bilirubin so it is not broken down. It takes the newborn system roughly two weeks to ramp up these pathways so that bilirubin can be cleared more appropriately.
2. Jaundice or a yellowing of the skin is caused when bilirubin is not cleared from the body appropriately and eventually deposited in the skin. This results in the yellow color. As discussed earlier, many newborns can have an elevated bilirubin level and thus be somewhat jaundiced. If levels do not come down after 2-3 weeks than further tests may need to be done to establish the cause. One such common cause may be seen in women who exclusively breastfeed their child. It is thought that breast milk may have an inhibitory effect on enzymes that are responsible for the breakdown bilirubin. It is harmless to the child but levels should be monitored. Bilirubin levels will usually decrease in 3-12 weeks and the process may be aided by discontinuing breast milk for 24 hrs.
3. Trancutaneous bilirubin checks are done as an alternative to sticking your child with a needle to draw blood. It is a good way to non-invasively monitor levels, however, there is a larger margin for error than blood tests. If results are consistently elevated it is a good idea to have then rechecked at a lab.
4. An intraoperative cholangiogram is a procedure that is done by placing a catheter into the gallbladder or the duct that drains it (cystic duct) and injecting a radiopaque dye. Imaging (x-ray) can than be taking to see if there is any narrowing or obstructions of the bile duct system.
5. The Kasai procedure also known as the biliary roux-en-y or a Heptoportoenterostomy is a surgical procedure done to relieve the effects of cholestasis (blockage of bile). It is performed by removing the gallbladder and all of the obstructed/damaged ductwork. The surgeon will then dissect into the hilum of the liver (this is the area where the bile ducts exit) in an attempt to establish good bile flow. Once this is done the small intestine will be cut and connected to the liver.
6. PELD score is a method used to measure the likelihood of liver failure in children. This measurement can than be used to place a child on a transplant list pertaining to their current need. It is calculated using age, bilirubin, albumin, INR and history of growth failure. It is not an absolute measurement and there are other things a physician can take into account when evaluating someone for a new liver.
7. Cholangitis is an infection of the bile ducts that is typically caused by bacteria. In the case of children who have undergone a Kasai procedure, cholangitis is much more common because the small intestine is connected directly to the liver allowing for bacteria to flow more easily into the bile ducts within the liver. Escherichia coli is the most common bacteria that causes cholangitis and is present in all human intestines.
8. Lesion is a general term used for an abnormal finding on an image test. In the case of children with BA the lesions may represent fluid, an abscess, a benign growth or a malignant tumor. In one study, 8% of children who underwent Kasai procedure only experienced a lesion. 75% of them where benign (focal nodular hyperplasia) and 25% turned out be more malignant (hepatocellular carcinoma)
2. Jaundice or a yellowing of the skin is caused when bilirubin is not cleared from the body appropriately and eventually deposited in the skin. This results in the yellow color. As discussed earlier, many newborns can have an elevated bilirubin level and thus be somewhat jaundiced. If levels do not come down after 2-3 weeks than further tests may need to be done to establish the cause. One such common cause may be seen in women who exclusively breastfeed their child. It is thought that breast milk may have an inhibitory effect on enzymes that are responsible for the breakdown bilirubin. It is harmless to the child but levels should be monitored. Bilirubin levels will usually decrease in 3-12 weeks and the process may be aided by discontinuing breast milk for 24 hrs.
3. Trancutaneous bilirubin checks are done as an alternative to sticking your child with a needle to draw blood. It is a good way to non-invasively monitor levels, however, there is a larger margin for error than blood tests. If results are consistently elevated it is a good idea to have then rechecked at a lab.
4. An intraoperative cholangiogram is a procedure that is done by placing a catheter into the gallbladder or the duct that drains it (cystic duct) and injecting a radiopaque dye. Imaging (x-ray) can than be taking to see if there is any narrowing or obstructions of the bile duct system.
5. The Kasai procedure also known as the biliary roux-en-y or a Heptoportoenterostomy is a surgical procedure done to relieve the effects of cholestasis (blockage of bile). It is performed by removing the gallbladder and all of the obstructed/damaged ductwork. The surgeon will then dissect into the hilum of the liver (this is the area where the bile ducts exit) in an attempt to establish good bile flow. Once this is done the small intestine will be cut and connected to the liver.
6. PELD score is a method used to measure the likelihood of liver failure in children. This measurement can than be used to place a child on a transplant list pertaining to their current need. It is calculated using age, bilirubin, albumin, INR and history of growth failure. It is not an absolute measurement and there are other things a physician can take into account when evaluating someone for a new liver.
7. Cholangitis is an infection of the bile ducts that is typically caused by bacteria. In the case of children who have undergone a Kasai procedure, cholangitis is much more common because the small intestine is connected directly to the liver allowing for bacteria to flow more easily into the bile ducts within the liver. Escherichia coli is the most common bacteria that causes cholangitis and is present in all human intestines.
8. Lesion is a general term used for an abnormal finding on an image test. In the case of children with BA the lesions may represent fluid, an abscess, a benign growth or a malignant tumor. In one study, 8% of children who underwent Kasai procedure only experienced a lesion. 75% of them where benign (focal nodular hyperplasia) and 25% turned out be more malignant (hepatocellular carcinoma)