Biliary Atresia: Letter N - S
Click on a child's name below to show their story. Click again to collapse the panel, and select another to open.
by her mother, Becca
Natalie was born in September 2003. She was born five weeks early, because I developed preeclampsia. On day two of her life she was sent to the NICU for jaundice. She began her stay there under the UV lights, like any other jaundice case. After a few days, they did a full blood work-up. Her GGT level was around 1700 (normal is 5 - 55), letting the doctors know that something else was wrong. After three inconclusive ultrasounds, she had a HIDA scan, a cholangiogram and then a biopsy in October.
We were referred to a wonderful team of doctors at Children's Memorial in Chicago. The first biopsy was inconclusive; at first we were told it might be cystic fibrosis. Genetic tests were sent away, and those took 6 weeks to get back. The results came back negative, so we went to Children's for a follow up. During the exam Natalie happened to have a dirty diaper, the doctor took one look at it and suspected that Natalie had Biliary Atresia. She was admitted and had another biopsy, it was again inconclusive. She then had a cholangiogram followed by the Kasai operation 3 days later. She returned home on Christmas Eve. What a wonderful Christmas present.
Natalie had many of the complications of end stage liver disease including: jaundice, vitamin deficiency, portal vein hypertension (high blood pressure in the portal vein going to the liver), and ascites (fluid build up in the abdomen). In addition, in a year’s time period, she had three reoccurring infections of Cholangitis in her liver. In November of 2004, Natalie was added to the national transplant waiting list, UNOS. In February of 2005 she received a new (but slightly used) portion of her mommy's liver!!!
Following her transplant she had her share of complications...2 days following the transplant, she developed a clot in her hepatic artery and was relisted for a new liver - Status 1. Status 1 is given to kids with a life expectancy of 7 days. However, when they went in to operate, they were surprised to find that they could fix the problem.
Then a few days later she had a MASSIVE bleed. She was given 3 units of blood in an hour. A short while later her surgeon (Dr. Superina) met us in the waiting room. He was scratching his head. They could not figure out where the bleeding was coming from and as they tried to operate - the bleeding stopped.
Following this, Natalie had a few minor cases of rejection and some bile duct issues (caused by damage done to the liver when the hepatic artery clotted). She also developed Rotavirus while we were in the hospital. The road that we've traveled has been bumpy to say the least. But to look at her now - she is a miracle!!!
by his parents, Thomas and Krystal
Nathaniel was born in October 2003. All was well until October 31st when he became a little fussier than the norm. I thought it was colic, until the next day his breathing became shallow and he had a rash all over. By the time we got him to the hospital he spiked a 102.4 fever, which got the docs on their toes. For the next few weeks they ran a battery of tests on him, all coming back negative. On Nov 25, 2003 they did exploratory surgery and concluded our dear son had biliary atresia. They did the Kasai procedure.
In March 2004, Nathaniel wouldn’t respond to anything. He was put on a medic flight to a local hospital where they said because his liver didn’t produce the vitamin k that was needed to clot off his blood, he had a subdural hematoma (bleed in the head). He was in a coma for 2 days, in PICU for 2 weeks and in the hospital a total of 4 weeks. On July 1, he was placed on the liver transplant list, but began to get better by the day. In December 2004 Nathaniel was taken off the transplant list. HOORAY!
He is a tough little guy and in his short 19 months of life he has endured a lifetime of trauma. He is truly our miracle baby!!! The doctors didn’t expect him to pull through the bleed at all. They had to do surgery to remove the blood off of his brain but because of the brain being swollen due to the blood this added to the swelling from the surgery. The outcome shouldn’t have gone as wonderfully as it did. So EVERYONE with a child with biliary atresia, there is nothing you can do, or anyone can do. Just trust in the Lord, and he will get you through. Don’t blame yourself for any of it. This I’ve learned in the short 19 months he has been with us. It’s not my fault. Its God’s will to test our faith and our ability to endure the worst. He’s not doing it to punish us, but to strengthen us! God Bless!
by her mom, Christine and her grandmother, Kathy
Noel was born in December 1997. She weighed 6 pounds 2 ounces and all seemed well. But it's unsettling how your whole world can change in the blink of an eye. One of life's little ironies or one of life's great lessons?
Looking back now I can see the yellow jaundice in her skin against the red backdrop in the photos I took of her and her sister, Nicole, for Christmas. And she not only didn't gain weight, she started losing weight. Her jaundice became more apparent, especially in the corners of her eyes. Her pediatrician, Doctor Bob, realized that things were not as they should be when he felt her liver--it was hard and enlarged. He sent her to Children's Hospital for tests. At Children's, Noel was diagnosed with biliary atresia. We had never heard of that. Surely it couldn’t be too bad? When I got off the Internet, I was in tears. She might not make it.
Noel had the Kasai at 9 weeks old. For the first few weeks, it didn't seem to be helping. Her levels were all still up, but she was pretty much holding her own. We spent a lot of time at the hospital back then. She started getting better though and her levels were all heading in the right direction. She had other things, of course--bladder reflux, bladder infection and lots of itching. She had a PIC line for awhile so a visiting nurse could administer her medications at home. She was on antibiotics for about a year and the reflux went away. She was on Benadryl for even longer, but it helped the terrible itching.
Today, she's doing very well. She has her liver clinic checkups once a year. Her levels are all in the normal range. She does have portal hypertension, an enlarged spleen, and her liver is still enlarged. But none of that has gotten worse either. She is starting to grow out of her little "Buddha belly"--and her scar is fading. The best way I can describe her is in the words of my sister-in-law, who said after watching her play with her other cousins: "I hope you don't take this the wrong way, but that little girl doesn't look like she's been sick a day in her life!" I could never take that the wrong way.
I cry tears of joy and tears of sadness when I read about your children. I believe the children belong to all of us now and we are in this together. Their illness has bonded us for all time. I know, deep in my heart, that Noel may need a liver transplant at some point in her life. I know that things may not always go as well as they are now. But we have chosen to live each day more fully than I believe we would have without Noel and her biliary atresia. They are inextricably bound together. We don't define her by her disease, but it's taught us so much. You have all taught us so much--about courage, and love, and loyalty.
It's interesting how we never question some statements because they always seem so true and such a given or a constant in life. I was nearby when expectant parents were asked recently if they wanted a boy or a girl and they answered, "Oh, that doesn't matter, just as long as the baby's healthy." We always assume that. We assume that all babies will be born healthy. If only they knew what we know. They would love that baby anyway.
I choose to look at Noel's disease as one of life's great lessons and Noel as one of life's great miracles.
by her granny, Jane
This is Olivia's first school photograph which she had done a few months ago, just after her fourth birthday.
Olivia was diagnosed with biliary atresia when she was 8 weeks old and had a successful Kasai a few days later. Although there has been many problems along the way she is doing as well as hoped for at the moment. She is a beautiful little girl and has bought so much pleasure to me and her grand dad. My daughter Michelle does a brilliant job of bringing her up as she herself was only 18 when Olivia was born and is a single parent. I don't think anyone else could of done better than Michelle.
Olivia is doing well at school although she doesn't make it often through tiredness and illnesses (she also has asthma). She enjoys what she can when she can.
by her mum, Nina
Update: January 2006
This is Olivia Grace, born in March 2005. She was slightly jaundiced from birth and struggled to gain weight. At her 6 week check I voiced concern that she was still showing signs of being jaundiced and it was then that I was referred to our local hospital in London for a blood test. That was the first May bank holiday of this year - when she was 7 weeks old. It turned out to be the longest night of our lives and the beginning of many nights and days of worry. The night we first heard of Biliary Atresia and that Olivia probably had it!
Olivia was referred to Kings Hospital in London for a biopsy after the bank holiday and she was confirmed as having Biliary Atresia. She underwent the Kasai on May 11th, her 2nd month birthday. This is when my husband and I first realized how resilient children are and that we had given birth to a true fighter. Within 4 days, Olivia was smiling and almost back to how we remembered her before. We were discharged and back home within a week. Olivia started on a special formula called Caprilon and began to take more milk each day and finally started to gain a bit of weight.
At her first check at Kings a month later, Olivia showed signs that she was improving. Her bilirubin had come down to 117 (183 pre-Kasai) and her jaundice was definitely less. So we left that day feeling extremely positive and optimistic. In July, however, Olivia started to loose her appetite and she began to get a temperature. We gave her 2 days to see if she would improve, but she didn't so we took her to our local hospital and they began treating her for cholangitis. A couple of days later, she began to get ascites. She is suffering from this constantly now, in and out of hospital for albumin infusions.
Sadly the Kasai hasn't been successful for Olivia and at the beginning of September she will be going on the transplant list. We are very positive about the transplant, as this really is her only chance at having a normal life. We are just worried that she is so young and small. Unfortunately because of her size we cannot be living donors, but we are praying that a suitable donor will come up in time for her to get a 2nd chance at life.
Olivia is currently 5 1/2 months old, and despite not having had the easiest start to life, she is the happiest, full of smiles baby you could meet. She is a constant joy and inspiration to us and our families.
Olivia was luckily able to have a living related transplant on October 10th, donated by her Father Rob. Olivia was in hospital for just over 2 weeks following the operation and her father discharged just after one week. Olivia experienced slight rejection in November, but has since recovered and is just a completely different child, full of energy, a beautiful pink colour and is piling on the pounds!!. Her Dad is pretty much back to normal - running again and back at work.
We are so relieved and just overjoyed that Olivia has be given a 2nd chance at life and hope she continues to progress as well as she is.
Paul is an 11-year-old sports enthusiast. He loves to play a variety of sports and they have become a big part of his life.
One unique thing about Paul is that he's had three liver transplants. Paul's first transplant was performed at UCLA Medical Center. He was only six months old at the time and had biliary atresia.
Ten years later, problems arose and Paul required another transplant, this one being a living donor transplant using a section of his father's liver. There were many post operative complications and Paul received a third transplant five months later. Since then Paul has completely recovered and is back playing the sports he loves so well.
Like most soon-to-be-teenagers, Paul loves to hang out at the mall. Another hobby is collecting sports cards. Paul is in fifth grade and doing well in school. His family is understandably very proud of him.
by her mother, Shawn
Peyton was born in June 2001. She was jaundiced from the beginning. We brought her home from the hospital on a bili light. Two weeks later she was taken off the light and we thought everything was fine. Two weeks after that she was still yellow and my husband talked me into taking her to the pediatrician. Thank God I listened and our doctor was so thorough. Within days we found out Peyton had biliary atresia.
Peyton had her Kasai at 5 weeks. She spent 3 days in intensive care and 2 weeks in the hospital. Her bilirubin started to drop -- her Kasai was a success. Her bilirubin is now down to 0.7 and she's doing great. She's been in the hospital just once with a high fever and suspected cholangitis.
Peyton is now 2 years old and a very happy girl who smiles constantly. We feel that we are very fortunate that her Kasai is working so well and pray it continues throughout her life.
by her mother, Linanne
Rachel was born in September of 1998. She was a frail and delicate baby, but she seemed healthy. When she was eleven days old she developed rectal bleeding and it was determined to be caused by a severe milk-protein allergy. She was put on Neocate formula and seemed to improve. On a follow-up visit, her GI noted that now that the blood was gone, her stools were pale. That discovery set us on a whirlwind of tests leading us to biliary atresia.
The Kasai procedure was performed when she was 5 1/2 weeks old at the Children's Hospital of Philadelphia (a bowel malrotation was also corrected). Her Kasai works wonderfully--her bilirubin is 0.1!
In February of 1999 she developed a high fever and was diagnosed with cholangitis. We did IV antibiotics for 14 days, but when we stopped she immediately was sick again. We then began using IV antibiotics through a Broviac. She has been on quite the array of antibiotics in different combinations and has been on them ever since -- yes, 14 months! The first week of March we tried to take her off all antibiotics to confirm the presumed chronic cholangitis was still present, and within three days her liver enzymes had doubled.
Rachel has been through so many hospitalizations, ultrasounds, three biopsies, 3 Broviacs, ascites, RSV, the flu and a multitude of other liver and non-liver related issues, but she is so well in spite of it all. I have to believe that these trials will help her endure her transplant and she will bounce back well.
Rachel was listed for a liver last July as a status 3 and was upgraded last September to a 2B. We are prepared to do a living-related transplant from her dad, but we hope to not need to. Her condition seems to bounce around, sometimes she is very stable, and other times not too stable at all, but she is really great! She's small, but growing, she's bright and fresh and gets into everything! She and her 7 year-old sister, Elizabeth, are quite a team. My husband and I can hardly keep up!
I, like many other people, never thought one way or the other about organ donation. Needless to say, I am now as vocal about it as I can be. I have placed donor cards in doctor's offices, written newspapers, participated in awareness functions and am open to other suggestions. The area I live in isn't the region of my OPO, so even if Rachel doesn't get a liver as a result of our efforts, I can hope it will help save someone else!
Raul is known by his friends, family, and team mates as the "Big Slugger"! He started playing tee-ball at the age of four and now plays third base for the Rising Warriors of El Paso, Texas. Last season he played so well he was selected for the all-star game. Raul was diagnosed with biliary atresia at the age of three months. Although the Kasai procedure is rarely successful at such a late age, Raul has done very well. He is now seven years old, in first grade and so far, so good! He visits his GI doctor every six months and his liver function is excellent. His parents hope and pray his liver keeps on doing as well as it is now.
by his mom , Toni
Sam was born in December 2003 in Leicestershire, England, and although he was slightly jaundiced at birth, this soon faded and we were able to go home. However, just after Christmas the jaundice returned and from our six week check up we were sent to the local hospital for tests. From there we went by ambulance (with Daddy following in the car) to Birmingham Children’s Hospital for more tests and Sam was diagnosed with biliary atresia.
At seven weeks old Sam had his kasai and although it was a success, and his bilirubin levels dropped quickly, he was left with a very scarred liver and portal hypertension. The next 8 months seemed to be just one hospital stay after another – Sam had an operation to mend a double hernia, then he had suspected cholangitis shortly afterwards. That went, and then in August he went off his food for the first time ever, and we knew he was ill. Another ten days on IV antibiotics for cholangitis, and the consultants at Birmingham started to monitor him closely.
In November, Sam went for a transplant assessment, as his portal hypertension had got worse. But when we got to the ward we had our doubts. Seeing Sam amongst so many other children with liver disease made us realise it wasn’t his time just yet. The nurses couldn’t believe how much he ate, he was happy, playing, holding our hands to walk…and the consultants confirmed it – he just wasn’t ill enough to go on the list.
To begin with we weren’t sure what to think. We know it’s there around the corner – they said his liver is too badly damaged to go on forever – and we’d been built up and prepared so well that we almost wanted it done with… but that was 6 months ago, and (touch wood) he hasn’t been ill since. In fact, he’s continued to gain weight, learnt to walk, and is amazing everyone. Now we realise what a good decision they made, because every month sees him bigger and stronger, and more able to cope when the time for transplant does arrive.
Putting our feelings into words is impossible. We have the most incredible group of family and friends around us and we never feel like we are doing this alone. We have total faith in the people at BCH and will never be able to thank them enough. And Sam – well we’re just so proud of him. Our love and prayers go to all the children with liver disease – they’re an inspiring bunch of children.
Samantha was born with biliary atresia and received part of her mom’s liver in 1995. For her transplant, we traveled from our home in Boston to the University of California-San Francisco Medical Center. Samantha had a fantastic post-op recovery: She was in the ICU just 15 hours (a new hospital record) and out of the hospital in just 13 days (another record). There were no complications whatsoever and Samantha was back home in Boston just 29 days later.
Sam is now 8 and is doing great! She gets all A's and B's on her report card and makes us proud. She loves playing with her three sisters Alex, ToniAnn, and Jordan. She is a great big sister. She loves cooking with dad and going fishing. We now live in the great state of Maine where the air is clean. Sam has a big heart - she gave all the money she saved for two years to go to Disneyland, to the New York WTC kids. We were never more proud of our little girl than at that moment. We know she will grow up to be a great person no matter what she does.
by her mother, Sandra
Sarah was born in October 2001 with biliary atresia. She had a kasai when she was 10 weeks old. After having 3 episodes of infections, she needed a liver transplant. In September 2003, my husband, Tatsuya, was able to be a match for the liver. Sarah is doing great and had her first anniversary.
by her mother, Jennifer
Shelby was born in March of 2002. At 1 week she was hospitalized for jaundice. We left the hospital knowing that her direct bili level was rising, but we were told that it would be continued to be monitored.
At 3 weeks old she had an abdominal ultrasound which showed she had no gall bladder. When she was 6 weeks old her pediatric GI decided that a liver biopsy was necessary to rule out biliary atresia. It was found that her liver had some blockage, but not enough to indicate biliary atresia.
Weekly blood tests continued and her labs began to show improvement. Then, at 10 weeks, her numbers began to rise. A second biopsy was performed and the next day we met with doctors at Children's Memorial in Chicago. Shelby's symptoms were atypical so exploratory surgery was scheduled for the following week. During surgery they found that she did have biliary atresia and the Kasai was performed. She recovered well from surgery and left the hospital 6 days later. She's had 2 hospital stays since her Kasai. One stay was for a lab error (they though her platelets were dangerously low) and one for an unexplained fever, but they did her 3rd liver biopsy and determined it was not cholangitis.
Shelby is growing well and except for a few minor setbacks she's doing great.
by his mother, Shital
Shyam was born in January 2001, four weeks early. When he was put under "the lights," we were not at all alarmed. His older brother was 7 weeks early and was kept in the hospital for 6 days (for jaundice) before he was allowed to come home. One week passed and the neonatologist decided to do a full workup on his liver. He was then transferred to Texas Children's Hospital. His first car ride was in an ambulance.
The GI that was assigned to us ordered all the familiar tests and eventually told us that our little one had something called biliary atresia. He told us that Shyam was to undergo a procedure called "Kasai." As far as we knew, once this took place, Shyam would be fine. He was 2 weeks old and all of 5 pounds. We didn't learn until after the surgeon came out to talk to us that the Kasai was a temporary fix and in most cases, this disease led to a liver transplant. We were devastated. After a 5 hour surgery, we learned that this battle was not over. We were confused as to why someone did not tell us of this to begin with. Slowly his labs came down to normal by 2 years old. He has had many other minor problems not related to his liver.
Shyam has grown into a strong 2 1/2 year old and his labs continue to hold their own. He does everything a healthy child can do and then some. He is the light of his older brother's life and they both love each other so much. He is a complete rascal and cannot sit still for one minute!
We are so thrilled to have him with us today. I know that there is a reason that Shyam was sent to be with our family and I thank God every day that we were chosen to be his parents. He will only be stronger for what he has been through - and I look forward to the future and spending a lifetime of happiness with this most special child.
by her mom, Sally
Looking back it's funny because I often used to say to my husband Michael that it was all too perfect to be true. We'd been happily married for about two years and were living in our beautiful new home when we decided it was time to try for a baby. We started trying immediately and within weeks I was pregnant. From there I just sailed ahead, I continued to swim daily and remained very active. I loved every day of being pregnant.
At 39 weeks I went in to labor and after five hours I gave birth to a beautiful little girl named Sophie. I had even managed to do it without any drugs as I had hoped. Life was starting to look pretty rosy- ha ha! On day two the pediatrician commented that she was quite jaundiced but that it was really common and nothing to get alarmed about. All the same, he ran some blood tests just to be sure. A few days later I was bursting to go home but the doctors were still puzzled by Sophie. Even after all the usual tests and scans they were still unsure. In retrospect it was because Soph did have a gall bladder, was happily gaining weight and doing all the right things. One thing I remember quite clearly is them asking what color her stools were. Being a first time mum I just kept answering that they were 'normal baby color' as this is what they seemed to me.
At three weeks old they tried unsuccessfully to get a liver biopsy, which meant that the only way to confirm her diagnosis was to open her up completely. This is where we first heard the dreaded term 'Biliary Atresia' being thrown around. Even then though, the doctors were unconvinced and were hoping it may just be a cyst.
Finally, when Soph was six weeks old they opened her up and discovered the worst. Our little princess did indeed have Biliary Atresia. What followed were her Kasai operation that day and an eight day recovery in hospital. Unfortunately for Soph, she was too far gone and the Kasai never started working.
At five months old she was placed on the transplant waiting list and we were told she would need a liver before her first birthday. What began was a very rough six months as we watched our little princess deteriorate before our eyes. As all of you parents are aware, it is so hard to sit back and watch your little one suffering and know there is absolutely nothing you can do to help them.
Finally, just one month short of that dreaded first birthday, we received the call we feared may never come. The doctors had a healthy liver waiting for our Sophie.
What followed were for us some of the most exciting days of our lives. We were finally leaving limbo. They were going to take our deathly ill child and do their best to give her a fighting chance at life! It turned out, Sophie breezed through her surgery and recovery without a hitch, and after three weeks in hospital, she was declared fit enough for discharge.
The past two years have been spent catching up on much of the life Sophie missed out on while she was so sick. To this day she continues to astound us as she goes from strength to strength. We're just so proud of her! Except for taking her Prograf twice a day and having her blood tests every three months, she now lives the life of any other 'almost three' year old. We have been extremely fortunate to date, and so far she hasn't had any complications or rejection. For this we will be forever grateful to a family we have never met, who saw fit in their time of grief, to give our Sophie a second chance at life. Could there be a greater gift?