Biliary Atresia: Letter T - Z
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by his parents, Carol and David
It was a cold winter in Pennsylvania in 1994 when I was pregnant with our second son. My husband lost his job after being a coal miner for nineteen years due to the mine closing. His union told him to not take another job until our baby was born so that we would be sure and have health coverage. I remember the day our son was born thinking that our life could now return too normal. Little did I know that life, as I had known it would never be the same again.
Our son came into the world in April 1994 weighing 81bs. 13oz. We named him after my side of the family giving him the name Thomas Jerald. He would go by the name of TJ being my brother's name was Tom. Everything seemed perfect at birth but looking back now I can see some things from the very early days that were just not right.
When TJ was ten days old we noticed some blood in his diaper so baby and diaper went straight to the doctors. He thought it might be from an allergy to the formula and changed that but then we asked if he thought TJ might be a little jaundice. He was not real sure but sent us for some blood work to check things out. The test came back with a slightly higher than normal bilirubin count but nothing was overly alarming at that time and we were to return in a few days for another blood test. After many more days and many more blood tests TJ was admitted to our local hospital. Two days later our doctor knew TJ's problems were beyond what he and this local hospital could deal with so he transferred us to Children's Hospital of Pittsburgh.
Over the next few months TJ was in and out of the hospital with many more tests being done only to never be able to come up with any answers. TJ was becoming more and more jaundice and not maturing, as a baby should. By the fall of that year it was decided that we should go see the transplant surgeons and have TJ placed on the transplant list. We spent that Christmas and New Year in the hospital with TJ being very sick and in need of a transplant soon. It was very hard waiting and praying for a liver knowing that someone else would have to loose something they loved if you were going to be able to hold onto something you loved so much.
When the call came on the January night that there might be a possible match for TJ, I felt a loss for someone I never knew. Your emotions are all over the place from happiness to sadness with a lot of fear in between. Someone who you don't know and will probably never meet has just given you the most wonderful gift you will ever receive.
After TJ's transplant he did quite well. They had said he was a strong fighter and he proved them right from the start. Seeing him the first night in ICU after the transplant I was amazed at how much better his coloring was already and how good he looked even with all the tubes and wires. Each day TJ got stronger and we were soon home from the hospital.
After the transplant when they did a study of TJ's liver they found that he had Biliary Atresia. That was something they had definitely ruled out early in their search for what was wrong. They found no outlets in the bile ducts but somehow bile was escaping his liver. When we asked how this could be the doctor pointed towards the heavens and said, "There is a power greater than I."
TJ has done remarkably well sense his transplant. It has been 9 years and he has had only a few minor setbacks with rejection and a case of chicken pox. He enjoys growing plants and taking care of animals. People who meet him do not even know he has had a transplant. I always love when sometimes they find out and really can't believe it. I, too, have to stop and remember because everything in our life seems normal for us again. The daily medicine has become routine to us only causing a problem last year when on vacation I left it on the roof of the car to get all wet and ruined. Thanks to a very nice pharmacy that gave us what we needed until we got home the vacation went on as planned.
No, it is not what I ordered when I decided to have a baby but it has changed my life in ways that are truly wonderful. I have learned to let the little things slide and cherish every silly moment that comes along. I used to say "Why me God?" but in some strange way I have learned to thank God that it was me who he decided to give this special gift to.
by his mother, Nicole
Taij finally arrived in November 2002, 9 days late. He was born in Adelaide, South Australia at the Women’s and Children’s Hospital (W&CH). We left the hospital on day 2 to head home to Ceduna (which is a small country town 800 km from the nearest capital city) in South Australia where we live. Taij had developed very mild jaundice but we were told that it would go away. At 3 weeks of age, we saw the local Child and Youth Health Nurse who told us, that due to Taij being yellow, that we should visit our local doctor for further testing. This is where our big adventure began. Fast forward to 5 weeks of age, we were back in Adelaide at the W&CH and we spent the week of Christmas 2002 in hospital having thousands of tests & scans. On the 29th of December we were told that Taij had biliary atresia and had to undergo a Kasai procedure. Taij had his Kasai at 5 1/2 weeks of age and spent New Years Eve in PICU. It was strange watching the fireworks through the windows to celebrate the beginning of a New Year and here we were not sure what to expect. People were getting on with their lives and ours was at a standstill.
We were finally allowed to leave the hospital after 3 weeks. We spent the weekend at my sister’s house. Taij was very unsettled and we eventually were readmitted to the W&CH with weight loss and poor appetite. He was admitted for 4 more long days. We finally went home to begin our new life with our baby; a time for his older sister, Kyla, 10 and brothers, Brayden, 8 and Jye, 2 to get to know their little brother better. We were home for 4 weeks when we attended clinic for Taij's monthly check up. He was admitted again for failure to thrive. I was breastfeeding and this was not enough for him, so he was put onto formula which was hard. I had breastfed my other 3 children for over 12 months, so I felt like a failure, but with a bit of persistence I continued to feed Taij breast milk and formula until he was 10 months old.
We lead a fairly "normal" life for the next 8 months with only having monthly checkups in Adelaide (which meant leaving my family at home overnight to fly to the city), seeing our pediatrician monthly at home and weekly bloods test which eventually extended to fortnightly then monthly. Then came the 22nd of October 2002, the wheel began to spin. We were transported to the W&CH due to cholangitis where he was given IV antibiotics for the week. We returned home after 7 days and we were home for a week then back in the W&CH with cholangitis again. Taij was sent to Sydney to be listed and we spent a week there before we were sent home because Taij was too well to be there. We were home for 6 days from Sydney when Taij had another bout of cholangitis. This went on every second week, with me calling Taij’s doctor and attending our local hospital then being transferred to Adelaide either by Air Ambulance or regional flights.
Christmas Day was a new chapter to our book. After having a lovely day with friends, Taij had bleed which meant being air lifted out again. Another Christmas not knowing what was going to happen and being far away from my family. Luckily, I have my mum and sister in Adelaide, so I have a great support circle around me. Some people are not so lucky.
We were home for 6 days again and he had another black stool, so back to the Big Smoke again. We were home in time for the 2003 New Years but once again we had another celebratory night at home welcoming another new year uncertain to our son’s future. Through all of this, we were also packing up to make the move to Sydney, due to Taij having so many bouts of cholangitis. We were to relocate so he could be listed for transplantation. We arrived in Sydney in January 2004 and we were there for 8 days. Then Taij was admitted again for cholangitis. We requested that a central line be inserted as it was becoming quite hard to obtain blood when they were needed and it would also be easier to give Taij antibiotics. Funny enough, the central line has not had to be used for anything other than blood test up until August, when Taij had another bout of the dreaded cholangitis. Taij is so resilient; he bounces back so quickly like nothing is wrong.
So here we are, 8 months living in Sydney, having relocated halfway across Australia. Kyla and Brayden have settled into their new school really well. They play sports on the weekends like they did at home and they have made heaps of friends. Jye is in childcare one day a week so that he can have that interaction with kids his own age also. We have now been on the active waiting list since February 2004 and Taij is doing extremely well. He does most things a normal 22-month-old does; he is very cheeky, full of mischief, and stands up to his older siblings. He is mildly jaundiced but is hard to see as he is quite olive skinned. He loves to give kisses and cuddles and he is showing more of his personality every day. He doesn't say a lot, but he is very loud and we always know what he wants. I think that comes with having older siblings.
We take every day as it comes and we are very positive. We have a wonderful group of people surrounding us with their love and support, and we have excellent people taking care of our beautiful baby boy; among them are Michael, Vicki, Diana and Robin. Thank you from the bottom of our hearts for taking good care of him. Also to Cherian and Colleen, back in Ceduna, for all the care you gave us at home well beyond your duty. To Suzy, Casey and Renee at W&CH thanks for being there for me. And finally to our families, thank you for being there. We love you all so much.
by his mom, Stacy
Tanner and his twin sister, Morgan, entered this world at just 27 weeks gestation in April 2004. Each child weighed just under 1 kg at birth. Due to their extreme prematurity, we knew our babies would have an extended stay in the NICU. However, both kids did extremely well by NICU standards - neither one had to be on a ventilator due to their prematurity.
At 5 days of life, a stopcock on Tanner's IV leaked enough that he required a blood transfusion. Shortly after transfusion, his liver functions spiked. The doctors started closely monitoring his labs while in the NICU. We were able to watch his labs rise and fall over the next few weeks. In late May, he began to have acholic (pale) stools. At 5 weeks old, Tanner had a HIDA scan. The scan showed nothing going through his liver. However, the hospital we were in did not routinely test infants. Tanner was transferred to the University of Kansas hospital for further tests. Tanner underwent two other HIDA scans and multiple ultrasounds with no diagnosis. In mid-June, Tanner underwent a cholangiogram and liver biopsy. During the procedure, the surgeon saw a bile duct structure and a very small gall bladder. He said he was 90% certain Tanner did not have biliary atresia. Tanner was sent home after 72 days in the NICU.
Fearing a choledocal cyst, Tanner was re-admitted to the NICU and went back into surgery just 10 days after being discharged. This time, the gall bladder was gone and the bile structure completely withered up. The Kasai procedure was performed and a diagnosis of Biliary Atresia was made. He returned home 9 days later.
Tanner's Kasai has had moderate success. About 8 weeks post-kasai, his bilirubin levels were within normal ranges (his liver enzymes were not) and he was gaining weight. In early October, things took a turn. Tanner started having pale stools and appeared jaundiced again. Our doctors thought it might be a case of cholangitis so he did a 23 day course of IV antibiotics. That did not help, and in November 2004, we were told that transplant would be inevitable.
In January 2005, we transferred to St. Louis Children’s Hospital for transplant evaluation. After being told by our Kansas City medical team that Tanner did not need to be listed yet, we were astonished to learn that St. Louis felt like Tanner should be transplanted in 4 - 6 weeks. On February 8, 2005, Tanner was added to the UNOS waiting list with a PELD of 25.
In late February, we returned to St. Louis to have Tanner’s grandma evaluated as a potential living donor. We found out on a Friday that she was a match, and the following Monday, Tanner received 24% of his paternal grandma’s liver. Tanner was 10 months old (7 months adjusted age) and weighed only 12 pounds. He spent 10 days in-patient and then was released to our apartment in St. Louis. Twenty-six days post-transplant we were released to return home to Kansas City.
Tanner has continued to do well in the last three months. He has packed on 3.5 pounds since transplant, and is catching up developmentally. He and Morgan recently celebrated their first birthday at home.
her mom, Jodi
Taylor was born in June 1991 and is a healthy, active 10-year-old. She was diagnosed with biliary atresia and had the Kasai procedure when she was 3 months old. Taylor has been growing very well since diagnosed and continues to take Actigall and ADEK vitamins daily.
The week after September 11, 2001, we received a call from Taylor's doctor saying that her blood tests were such that she thought it was time for us to make a decision on a transplant center and go to meet the transplant team. On November 26, 2001, we made a trip to Cincinnati Children's Hospital to meet the team. The first day consisted of meeting all the doctors, surgeons, support nurses, finance people, social workers, etc. It was a very long day, but very informative. The second day consisted of Taylor having some blood tests and an MRI. It was pretty stressful, as it has always been very difficult to find a vein in Taylor's arm to draw blood. She is a very brave little girl and always has a smile for the nurses and technicians working with her.
We were so pleased to find out that the team thought she was too healthy to be put on the transplant list at this time. We will continue to alternate between Cincinnati and Columbus every 3 months for blood tests so they can keep a close watch on her. We are glad that we have the 2-day pre-transplant evaluation behind us now. We were very impressed with Cincinnati Children's Hospital and the transplant team. We're glad that they know Taylor now, and that she will get to know them a little better too, before time for her transplant.
Every day we are thankful that Taylor has been so very healthy throughout her life. She now plays volleyball, softball and basketball and she wears a spleen guard to protect her enlarged spleen. She's a very active 4th grader and we thank God every day for her.
by her mother, Crystal
Tigerlily is a happy 6-month-old girl with a protective 3-year-old brother, Zedekiah, and loving parents, Donovan and Crystal. She was diagnosed with biliary atresia in August, 2000, and underwent the Kasai at Maine Medical Center in Portland, Maine. Since then she has gone up and down, finally ending up in Children's Hospital in Boston with bi-directional portal vein flow and ascites. The team there determined it was time to list her for transplant and we are now reviewing with Mount Sinai Medical Center in New York for the actual transplant. Thank you for this site to bring these families together to deal with such difficult issues surrounding our children. My prayers go out to all of the children.
Six-year-old Trent was born with biliary atresia and had two unsuccessful Kasai procedures as an infant. Five years ago on Christmas Eve, Trent was near death and his doctors didn't think he would make it through the night. Then, for Trent and his family a miracle took place and he received the gift of a liver after being on the transplant list for less than 24 hours. What a beautiful Christmas gift!
Today, Trent is a healthy, happy little boy with a sweet smile. He loves animals of all kinds, great and small. He also loves going to kindergarten and playing soccer.
by his parents, Jeff and Colleen
Our son Trevor was born in July 2002. There were no complications with his mother’s pregnancy or delivery. The doctors told us that Trevor was a healthy baby boy and discharged us after the standard 48-hour hospital stay. Prior to our discharge, we inquired about his “yellowish” appearance, but were told that it was just a mild case of jaundice and that we had nothing to worry about.
However, during our first few pediatric visits, Trevor’s pediatrician had become increasingly more concerned since Trevor was losing, not gaining weight. Trevor was breast feeding at the time and we had decided to supplement his feedings with formula to see if it would improve his weight gain. Two days after introducing formula, Trevor broke out in a head-to-toe rash that covered 90% of his tiny body. We rushed him to the hospital for tests and soon found out that he had more problems that just gaining weight…
The rash turned out to be nothing more than a protein allergy, however it proved to be a lifesaving event. A blood test confirmed that his bilirubin was critically high (hence the yellowish color) and that he was to be admitted to hospital immediately. Dozens of blood tests, HIDA scans, ultrasounds and a liver biopsy later, we were told the shocking news that Trevor had a rare disease called biliary atresia.
Unfortunately, we already knew all too well how serious the condition was even before the GI doctor began to explain it. By that time, we had already discovered the C.L.A.S.S. web site through the 24-hour research we had been doing on the laptop from the hospital room and were very educated on the topic. After two weeks in the hospital in Tampa, we were transferred to Jackson Memorial Hospital in Miami where Trevor was to undergo immediate surgery (i.e. “Kasai procedure”).
Upon arriving in Miami, Trevor endured more blood tests, HIDA scans and ultrasounds. He underwent surgery during his 6th week of life and recovered amazingly well. His liver began working 3 days after the operation and a week later (5 weeks after his admission to the hospital) we were allowed to go home.
We have treasured every second with him and are so grateful to the advances in medicine which have kept him alive. His doctors have all been skilled and compassionate for which we are forever in their debt. If the day comes where a transplant is needed, we know that we will be in good hands and that we will make it through just fine.
by his mother, Brenda 0000.jpg
Tyler just had his third liver transplant in January 2000 and is doing very well. He is such a fighter and we are so proud of him!
Tyler had his first transplant (biliary atresia) at the age of 6 months, and the second, a month later. He had a lot of complications with bleeding over the past 4 years. Finally, it got so bad that he needed a third transplant. So, Tyler actually got a new liver along with all the plumbing.
After our last transplant stay of 3½ months, we were thankful this stay was only 32 days. We were so glad to be home! Tyler played with all this toys like they were brand new. He is very excited to get back in school. You can sure tell that he is feeling better. He's now running around and acting like a normal 4½ year old.
We are so blessed to have this little boy in our lives and we cannot thank the donor family enough.
While pregnant in 1999 with our 3rd son, Tyler, we had no reason to think that we wouldn’t be having anything other than a healthy child. Our two older boys, Kevin and Joshua, were always healthy and we assumed that Tyler would be also. Yet a few hours after he was born, all that seemed to change. Tyler wouldn’t nurse or go to the bathroom and the tests began. After being checked for everything imaginable, at two days old, he was diagnosed with colon atresia and underwent his first surgery the next day. We were finally able to bring him home at 10 days old! At a follow-up appointment, his surgeon noticed the yellow colored skin and the fact that some other things weren’t adding up, so she ordered more tests. At about four weeks old we were told that Tyler had biliary atresia and he was scheduled for a liver biopsy. At six weeks old, our world turned upside down. Tyler had the biopsy and the surgeon attempted a Kasai, but we were informed that night that it was unsuccessful and Tyler would need a transplant!
When Tyler was not quite four months old we moved from Lansdale, Pennsylvania to Pleasant Hill, Missouri. We started his transplant evaluation in February 2000, at the University of Nebraska Medical Center in Omaha and in April 2000, Tyler was placed on a feeding tube in an attempt to put some weight on him pending the transplant. In June 2000, while at our oldest son’s baseball game, we received the call that would give our baby a second chance at life. After 6 weeks in Omaha, and two minor surgeries after transplant (portal vein block and a nick in an artery in his stomach while trying to get rid of some fluid), we were able to bring him home.
Tyler’s had several setbacks and some developmental delays since the transplant, but recently celebrated his five-year anniversary! He underwent surgery to have his teeth capped and the top four removed due to the medications he was on, as well as surgery for hypospadius and an undescended testicle. He also has several food allergies that make life interesting. He just started kindergarten and is looking forward to meeting some new friends.
Tyler competed at the U.S. Transplant Games in Minneapolis, MN in 2004 with Team MO-KAN and brought home a bronze medal in bowling. At the World Transplant Games in London, Ontario, Canada in 2005, Tyler made friends around the world and brought home 5 gold medals; one each in swimming, bowling, long jump, cricket ball throw and 25M run!
We are extremely grateful to the numerous doctors and nurses who have been involved in his care the last 6 years! Most of all we are forever thankful to his liver donor’s family, whom we had the chance to meet last summer; and to organ donors everywhere!!! THEY TRULY ARE HEROES AND ANGELS!!!
MAKE A MIRACLE – BE AN ORGAN DONOR!!!
by his mom, Nicole
Tyler was born in November 1997. When he was 3 days old I started to notice he was jaundiced. His pediatrician referred us to Children’s Mercy Hospital in Kansas City, Missouri. After seeing the specialist, he was scheduled for a liver biopsy and before the results could return he was scheduled for a kasai, which wasn't a success. Tyler was diagnosed with biliary atresia, but we later found out with genetic tests it was Alagille Syndrome.
He was put on the transplant list at 6 weeks old. While waiting, he slowly stopped eating and at 2 1/2 months old he got his first Hickman, one of 8 he would receive. This is when he began TPN/Lipids. At 4 months old he had to have a feeding tube. He was hospitalized often and we were rarely home more than 5 days before he had to return to CMH for a 2-3+ weeks stay due to different infections from cholangitis to multiple staph infections in his blood to dehydration and failure to thrive. Tyler began to get calcium deposits all over his body.
We finally got the call in March 2000. Surgery took 8 hours. Tyler received a split -liver transplant. Tyler had some minor complications with leaking bile ducts, but after 2 months they were healed up. On top of the liver failure he has pulmonary stenosis and a cyst on his left kidney, both which aren’t causing any serious problems at this time. Other than reoccurring ear infections, Tyler is now a healthy and active 7 year old.
by her mother, Martha
Victoria Rose was born in June of 1999. She was diagnosed with biliary atresia on October 15, 1999. Victoria was in-patient for 16 days and during that time she was listed for a liver. She received her liver transplant at Children's Hospital of Pittsburgh in November 1999 at the age of four months.
Victoria has been very healthy since her transplant. We are nearing the two year anniversary and she has only had two short in-patient stays since then. One for pneumonia in December of 1999, and this past May for rotavirus.
She has not had any significant rejection and she is currently taking only her Prograf and Bactrim. Victoria is truly our miracle baby.
We are thankful for her donor everyday. Each time I hug her I silently pray for her little angel who gave her the greatest gift of all.
by his mom, Dot
Wyatt was born in April 2002. We considered his 37 week arrival as a most auspicious date, as his mom is a liberal tree-hugger type!
Wyatt was a crabby newborn who cried incessantly and pooped constantly. His big sister, Maia, called it "scrambled egg poop" because that's what it looked like! Wyatt was slightly jaundiced, but we attributed this to his Italian heritage and nursing. It wasn't until his 2 month well-baby check-up that his pediatrician suspected a liver problem.
On June 24 Wyatt was diagnosed with biliary atresia and within one week had a Kasai procedure at Children's Hospital in Denver. The Pediatric Liver Group at Children's Hospital has been fantastic. They have provided not just physical healing, but emotional support during a most difficult time for our family.
Today Wyatt is almost 8 months old and almost 21 pounds!
by his mother, Jodi
Zachary was born in January of 2000. At six weeks of age he was diagnosed with biliary atresia, dextrocardia (heart on the opposite side and flipped), situs inversus (insides are reversed), and polysplenia (multiple spleens). Three days after his diagnosis he had the Kasai operation. His numbers never really improved and after a few bouts of cholangitis he was referred to the transplant department.
Zachary was listed in September 2000 at Children's Hospital in Pittsburgh. He had multiple episodes of cholangitis and needed to have a NG tube placed to help with feedings. Zachary, our little Houdini as we like to call him, pulled his NG tube out four times. He was also on TPN. He received his gift of life in August of 2001. He did well after transplant and had emergency surgery on September 10, 2001 to repair one of his spleens. He came home a couple weeks later. After Halloween 2001, Zachary was readmitted for PTLD. He had another emergency surgery after Thanksgiving for lesions in his intestinal tract. He came home after the new year. After one more hospital visit for rejection in March he has been home and running wild. He is such a "terrible" two year old.
Zachary enjoys Blue's Clues and Dora the Explorer, playing with his brothers and sister, and getting into trouble. He is definitely a joy in our life and we treasure him daily.
by his mother, Debbi
Zachary was born in August 2000 and was diagnosed with unknown liver disorder at the age of 8 weeks. He was put on various medications and his liver functions improved – but only temporarily. Two days after his first Christmas, on his four-month birthday, the lab results were worse than ever. By the age of five months, Zachary was evaluated at the Children’s Hospital Medical Center in Cincinnati, Ohio, and diagnosed with biliary atresia. At that time, however, we were told that Zachary’s health was exceptional for a BA baby and that it would be at least six months to a year before his health would start to deteriorate. His symptoms at that time were jaundice, ascites, portal hypertension, frequent reflux and vomiting, a hernia in his navel, and a rapidly waning appetite.
Just three short weeks later, however, Zachary suffered a massive, life-threatening hemorrhage caused by esophageal varices and was rushed to the hospital by ambulance. On Zachary’s fourth day in the PICU, our insurance company finally and reluctantly gave the go ahead that enabled him to get on the UNOS transplant waiting list.
In the weeks that followed, Zachary’s health began to deteriorate very quickly. I underwent testing and found that I would be a compatible donor for my son. Finally, an end was in sight and our surgery was set for April 10, 2001. Unfortunately, at the last minute our transplant center decided that maybe I wasn’t such a good donor after all and canceled our surgery.
We knew Zachary didn’t have much time to wait and chose not to take the chance that a donated liver would become available in time or that another living donor could be worked up quickly enough. On the advice of a new friend (another transplant mom!), we made arrangements to travel 600 miles to New York City to the meet Dr. Jean Emond and his team at the Columbia/Presbyterian Medical Center. We arrived on April 14 and Zachary was transplanted five days later, on April 19, 2001, with me as his donor.
Thanks to God and to the amazing expertise of the surgeons, doctors and nurses who took such incredible care of our son, Zachary’s recovery was nothing short of miraculous! He was discharged from the hospital in less than two weeks and allowed to return home to Kentucky after only a month. Two weeks later, though, Zachary had a mild rejection which required another five day hospitalization in NYC. He has not spent another night in the hospital since June 2001.
Zachary is now almost 22 months old (14 months post-transplant) and never stops! He has an endearing disposition and is quite the flirt and comedian – when he’s not too busy staring at cars and trucks, that is. He takes only one transplant medication now (Prograf) and has had nothing more serious than a mild ear infection in the past year.
My family and I are so grateful to everyone at C.L.A.S.S. for creating this place where we can come to share our experiences – to laugh, cry, pray, rejoice and learn together. God bless all the kids!