Biliary Atresia: Letter J - K
Click on a child's name below to show their story. Click again to collapse the panel, and select another to open.
by his mom and dad
Jack was born in June 2001. After a return trip to the local hospital for 5 days of bili lights, the local doctors referred us to Mott Children's Hospital at the University of Michigan. Jack was diagnosed with biliary atresia and a Kasai operation was performed when Jack was eight weeks old.
It is now May 2007 and Jack is doing well (see photo). The Kasai operation has really improved his liver function and his color is normal. His lab work has been excellent with most numbers in the "normal" range for the past two years. Right now, looking at him, you would never know that Jack has a liver disease. Still, the doctors think he will need a liver transplant "someday" and the transplant team is watching him just in case. For now we are enjoying his good health.
Jack is a wonderful boy who enjoys playing with his big brother (he laughs so hard!); swinging or playing outside; talking all the time; coloring and drawing Pokemon or dinosaurs; and going to school.
Considering all he has been through he has a very happy disposition.
A special thanks to all our families and friends who have been so supportive of us all. Special thanks to all of Jack's doctors and nurses who work so hard to help him. Thanks to CLASSKIDS for this great web site. God bless all the CLASS kids and their families.
by his mom, Michelle
Jack was my third child, born in an easy delivery. He was healthy, average weight and height. I noticed he was jaundiced at 5 weeks of age. He was diagnosed with Biliary Atresia at 7 weeks. Jack had the Kasai procedure at 8 weeks of age at Columbia University Hospital, NYC. His surgery went well, but his liver was significantly scarred. He remained jaundiced, and proceeded to show signs of liver failure.
Jack was listed for liver transplant in March 2003. Ultimately, Jack’s dad, Dominic became his living donor. April 8, 2003 was the day his dad gave him the greatest gift of life.
Jack had a tough couple of months after the transplant. Multiple hospital stays, which ultimately ended up in a surgery to repair a bile duct issue he was having. He did require physical therapy to help him strengthen his stomach muscles, but he walked at 17 ½ months!
Jack recently had his second birthday and is a feisty two year old. He is only on 1 medication and is doing well. We are so thankful for the prayers and love we have received from family, friends and the wonderful doctors who look after him.
by his mom, Jina
Jackson was born in September 2000. He was diagnosed with biliary atresia at 8 weeks and had the Kasai procedure at 9 weeks. His Kasai was quickly failing and Jackson was put on the list on January 9, 2001. We were admitted to the hospital on several different occasions for minor complications. At the end of January, Jackson was admitted and we were told that he would not be leaving until he received his transplant.
As the days passed Jackson was getting sicker and weaker. My husband, Brian, completed all the living donor tests and was found to be a good match. The transplant date was scheduled. Four days before the date a donor became available. Jackson received his life-saving transplant when he was 5 months old.
Today, Jackson is a happy, energetic 10-month-old. He loves to play, swim and watch his Baby Mozart video. He enjoys chasing his two dogs around the house in his walker and he is now learning to walk around the furniture. He especially loves for his great grandma to rock and sing to him.
We couldn't have made it without our family. They were there for us every step of the way. Friends and strangers have been so kind. A blood drive was started for Jackson and raised over 100 units of blood. University of Miami-Jackson Memorial Hospital performed Jackson's liver transplant. We can't thank them enough for their skill, knowledge, compassion, and for saving our baby's life.
Our donor and family will forever be in our hearts, thoughts, and prayers.
by her mother, Jennifer
Jacqueline was diagnosed with Biliary Atresia at 5 weeks old. Less than a week later she had her Kasai procedure. Her surgeon was concerned about the color of her liver, but other than that was very pleased with the way it went. He even called her a "fighter" because she was one of the few children that age who did not need a respirator after her surgery.
Since her diagnosis she has been hospitalized frequently with cholangitis. After many infections her liver Began to fail and she received her "new liver" from her mother in October 2002.
Currently, Jacqueline is awaiting her second liver transplant. She has to wait for a cadaver donor this time because in addition to Biliary Atresia, she also has Portal Vein Thrombosis. This condition is cased by a narrow portal vein, which is too narrow for her vein and liver to function properly. The doctors did a bypass as a temporary fix, but she still faces problems with escalated ammonia levels and will need a liver with a working portal vein. We were recently informed that she has moved up 12 more points on the waiting list. We are hopeful that the next CLASS update will include her and her new liver!
by her parents, Scott and Tammy
Janna was born in January of 2004. It was a normal pregnancy. Her bilirubin was checked the day she was discharged from the hospital. (I would love to have those results now to see just what was checked). I left the hospital with what I thought was an olive skinned baby. I asked about her fluorescent green stools in the hospital and at her two, four, and six weeks checkups. All I ever heard in response was that it was normal for the coloring to be different. (Hah! Not if you are breastfeeding)
Cold and snowy months followed in Kansas. Janna caught a cold when she was only two weeks old, so for the next six weeks we limited our outings to only brief visits at the doctor’s office. In early March, she was almost eight weeks old. It wasn’t until her first trip to Grandma’s, where I was taking pictures of her on a blanket next to her 10 day-older cousin, that I noticed, in contrast, her coloring was so very different. The next day I was at the pediatrician’s office for testing. A couple of hours later, the doctor called to say that Janna needed further testing because her bilirubin was abnormally high. The next day my husband and I took a three-hour drive to Kansas City to see a specialist. They ran some preliminary tests, but since it was a Friday, we had to come back the next week for further testing.
She was diagnosed with biliary atresia at about eight weeks old and had the Kasai at 64 days old. Eighteen days after the Kasai, she was back in the hospital with suspected cholangitis. After that episode in April, she seemed to be doing well and her bilirubin returned to the normal range. In July, she had another episode of cholangitis. In early October, her white blood count and platelets dropped and she was diagnosed with severe portal hypertension and esophageal varices. She had another bout of cholangitis in December. She had another scope in early February 2005 that showed no increase in the amount of varices, but the doctor was unable to “band” her because her petite mouth and throat physically precluded it—even though she is thriving at 26 pounds! So far, the varices seem to be holding. She will be scheduled for another try at banding when she gains another five pounds.
Janna has an older sister, Malina, who will be three in April. Janna is a happy and very active one year old. She loves making animal sounds and blowing kisses to everyone. She has brightened our days and has also taught us much about stress management. Our “Jannie Nannie” (as her sister says) is truly a joy. Though dealing with her condition is a challenge, we continually try to appreciate how fortunate we are to have a child with so few other serious complications right now.
by her mother, Andrea
Jayli was born in December 2000. Although she was 3 days past her due date, Jayli Grace was the best Christmas present we've ever received. I must admit that it's been a tough time with many ups and downs, but we wouldn't trade our precious gift for anything in the world.
Born with a rare liver disease, Jayli was jaundiced almost from birth. It took the doctors 11 weeks to find out that Jayli had biliary atresia. At that time, she underwent the Kasai operation. Unfortunately the operation was unsuccessful for her. In May 2001 Jayli was listed for a liver transplant at Childrens Memorial in Chicago.
Testing then began for a living donor. My husband, Tony was immediately ruled out because his blood type was incompatible with Jayli's. I, however, passed all of the tests and surgery was scheduled for July 11. It's so strange, but I had a gut feeling that Jayli's transplant wasn't going to happen on that day. I was right. When the doctors opened me up and found spots on my liver, the surgery was called off. My dad then stepped up and insisted on being tested. After weeks of testing, he was finally approved as a match and surgery was scheduled for August 22nd.
Everything went as planned and the surgeries went without any complications. Jayli was in the hospital only 9 days after her transplant. During that time she experienced rejection but it was treated successfully with high doses of steroids. The only other complication Jayli had was CMV. We were in Chicago for a total of 5 weeks and stayed at the transplant house because we live more than a couple hours from the hospital. What a great day it was when were finally able to take Jayli home!
In February 2002, Jayli needed to have some tests done because her liver numbers were elevated and she was having blood in her stools. After a bunch of tests, we found out that Jayli's bile ducts were blocked and her portal vein was clotted. Jayli had a couple drains put in to open up her bile ducts. She was in the hospital for two weeks. The first couple days were spent trying to get the drains positioned correctly. The rest of her stay was spent battling the RSV and C-diff that she got while in the hospital.
On April 3, Jayli had a bypass surgery for the clot in her portal vein. Unfortunately the portal vein was clotted too far up in the liver and the rex-shunt was unsuccessful. Jayli was then listed for a second transplant. She could not have a living donor transplant this time. Jayli was approved by UNOS to be a Status 1 and we waited for a new liver to become available.
Early in May, at 2:45 a.m., we got the call that a liver was available for Jayli. She went into surgery about 12:30 p.m. that afternoon and underwent a very long complicated surgery. We finally got to see her about 2:30 a.m. the next morning. What a long 24 hours!
Jayli had a very rough post-op period from her second transplant. She had many complications, including a major bleed that created a hematoma, multiple bacterial infections, multiple episodes of rejection, a bile leak, and bile duct strictures. During this time we bounced back and forth between the transplant house and the hospital. Jayli was finally released to go home on August 30, 2002.
A few days after being home, Jayli had a routine blood draw and we found out that her platelets were dangerously low. Her level was at 2,000. Normal platelet levels are 150,000 – 450,000. After a few days, we found out that Jayli had Immune Thrombocytopenic Purpura (I.T.P.). This is an autoimmune response to an illness. Antibodies that were fighting the illness somehow get attached to the platelets. Then the spleen recognizes these as something that needs to be destroyed and gobbles them up. We tried multiple things to help Jayli. She had infusions of IVIG and even had a chemotherapy treatment. Nothing helped, so Jayli underwent a splenectomy on the 15th of September. It worked and brought Jayli’s platelets up to 387,000! Jayli was released to go home on the 19th of September.
Jayli has a long way to go before she is fully recovered. But, we have faith in God that she will be healed and allowed to live a very long life. He has a beautiful plan for her! We are so thankful to my dad and Jayli’s donor family who gave Jayli her second and third chance at life! It was the most awesome gift that anyone could have ever given her....and to us. Jayli’s smile lights up our lives.
And thank you to everyone at CLASS for your thoughts and prayers during this last year!
by her mother, Cecilia
Jenna was born in December 1996. Biliary atresia was suspected within one week because of her severe jaundice. She had a Kasai at five weeks, but it was not successful.
When she was five months old, we flew from our home in Tarrytown, NY, to UCSF, in San Francisco, because we wanted Dr. Emond to see her. Once admitted, she developed ascites and was scheduled for a living-related transplant within a couple weeks. They wanted to beef-up her nutrition before the transplant. I began the screening to be her donor.
The day before the transplant Jenna spiked a fever and the transplant had to be postponed until the infection cleared up. She was hanging by a thread, needing the transplant, but besieged with infection. She became very lethargic and her eyes were dull. Needless to say, it was a very tenuous and stressful time.
The transplant, according to Dr. Emond, was particularly difficult. She lost six pints of blood because her ability to clot blood was severely compromised. He told us that perhaps one day he would tell us just how serious it was for Jenna, but not yet. I believe her operation lasted 8 hours and it was Dr. Emond's last transplant at UCSF before he left to start the program at Columbia, where we now go for Jenna's follow-up care.
One week after the transplant, surgeons opened her up as standard procedure for an open biopsy and in the process checked to see if all seemed okay. It is disturbing to know that they don't do open biopsies at all transplant centers but at least all went well.
One week after the open biopsy, they performed a needle biopsy that was a disaster. The needle went through her new liver and punctured a lymph node on the underside of the liver. The internal bleeding was discovered later in the day as her hematocrit dropped and she passed black, blood stained stool. She was rushed in for emergency surgery to cauterize the bleeding. In the middle of the night her hematocrit began dropping again. Again she was rushed in for emergency surgery, this time to stop bleeding of punctured vessels within the liver. So, the needle biopsy had not only pierced the lymph node, it had broken blood vessels on the way through the liver.
After having four operations within three weeks she took a while to recover. But recover she has and we have not had any trouble with her liver since.
A year and a half after her transplant, Jenna had to have surgery to correct urinary reflux. While it was miserable to go back into that "hospital world", which we had so gladly left, this surgery seemed minor compared to what she had been through. She sailed through it and has been perfectly healthy ever since.
Jenna is now three years old and is in nursery school and swimming classes. She is a very active, agile girl who climbs everything she can reach. She is very loving and so independent that she insists on doing everything by herself.
Jessica was born with biliary atresia and had the Kasai procedure at 7 weeks, but it was not successful. Her parents were in the process of working out details for a possible living donor transplant when a donated liver became available. Jessica received her new liver in January of 1999, at Hermann Hospital in Houston, Texas. She was 11 months old at the time. Her transplant operation was picture perfect and she was in the hospital for just 9 days. Her labs couldn't be more perfect. Her doctors continue to be amazed at how well she is doing. She just started walking and already loves to chase her big sister Rhonda. She is doing so many new things everyday.
March 2004 Update
Jessica is now 5 years post transplant and in school! She just turned six and loves school. I was so worried about how she would do in school, but all the worrying was for nothing. We are still doing monthly labs and Jessica is only taking Prograf.
by his mother, Stacy
Joey is eight years old. He was diagnosed with biliary atresia and underwent the Kasai procedure at two months. The operation was a big success. The bile began to flow properly and his jaundice went away within a week.
He suffered from one bout of cholangitis about two months later. He was treated with I.V antibiotics for 10 days and released. He has since done fairly well with the disease. They did find that he has polysplenia, (multiple spleens), and his main spleen is enlarged. Therefore his activities are somewhat limited, but he copes well. He knows he can't play football, soccer, or go on the monkey bars at school. He is on Actigall and vitamin K.
Joey loves to read and is doing exceptional in school, considering all the time he spent in the hospital as a baby. He also has other medical problems not related to the liver disease. We continue to see the GI doctors every 6 months for an exam and blood work. He has recently developed what they think is a mild form of portal hypertension, so we will watch that closely now. He is not on the waiting list yet, but if and when the day comes I know he will come through with flying colors -- he always has.
Joey would also like me to mention that he has a baby sister, Emily Ann. He is a wonderful big brother and a big help to Mommy!
by his mother, Mary
Play Ball!! - OR - Gentlemen start your engines!! Besides school that’s how John spends most of his days, like any other nine-year-old boy. It wasn’t always this carefree and easy for him.
John was born in March 1992 and appeared healthy at birth. When he was nine weeks old he was diagnosed with biliary atresia. John had the Kasai procedure immediately after diagnosis. The Kasai established bile flow (he was never jaundiced again); however, his liver was severely damaged. John spent much time at Long Island Jewish Medical Center (Schneider’s Children’s Hospital) and was nicknamed “The Mayor.” He developed ascites immediately after the Kasai and esophageal varices within a year.
John was listed for transplant in the Spring of 1993 and by summer his health was failing. Sclerotherapy was a monthly procedure for bleeding varices; blood transfusions and abdominal drains were the norm. It is so painful as a parent to watch your child suffer so much.
In November 1993 John received the gift of life and had a successful transplant at The University of Minnesota (currently Fairview University). He had a few complications and a bout of rejection but he was on the rebound. It was wonderful to see him come into his own. As he grows up he asks questions about his scar and the transplant and seems to understand all the answers. John went from taking six medicines several times a day to only one twice a day.
The future is bright for John who is in the third grade and a star student. He excels in reading and physically he is very active. He loves to play baseball and is a lefty pitcher on his little league team and enjoys watching The New York Mets. John also enjoys skiing, bowling, basketball and camping with his family and friends. Recently John has become a huge NASCAR fan and is hoping his favorite driver, Jeremy Mayfield, makes it to Victory Lane this season. Hopefully at the Pocono 500 when our family will attend our first NASCAR race in June 2001.
Through our long journey discovering and always learning about biliary atresia, transplantation and the liver, we have met many wonderful people and some incredible kids! Their help and guidance has helped tremendously. Perhaps the greatest mention goes to John’s donor and his or her family. It was their strength, courage and unselfish decision on a dark day that helped John and our family see light at the end of the tunnel and emerge into the sunshine.
by her dad, David
Josephine was born in November 1999. At 8 days old she was diagnosed with biliary atresia. She was placed on the active waiting list at one month old but then was removed. When Josephine turned 4 years old she was placed back on the active waiting list and we have been waiting for 14 months.
Written by, Bill, Josh’s father. At Josh’s third birthday, the Josh’s family decided to hold a fundraiser by asking their guests to bring a donation instead of gifts. They raised almost $3,400 for CLASS and over $9,100 for Lucile Packard Foundation to benefit the Liver Transplant Parent Mentor Program.
I was recently asked what was the scariest thing I've ever done? I immediately thought of some of my outdoor hobbies: backcountry ski touring, kayaking and especially rock climbing. But then I realized these were easily topped by having my 14-month-old get a liver transplant. People have asked me what this whole thing felt like, and I finally thought of a description that comes close to capturing it. You know when your child starts to choke on a piece of food, and for a few agonizing seconds he's not breathing, and every cell in your body is flooded with adrenaline, waiting, straining desperately for a sign of that next breath? . . . Well, that's what it felt like, every minute, for two months.
Josh's was a complicated pregnancy and delivery, but we finally brought home a beautiful little baby boy, healthy aside from a bit of jaundice, which is not unusual. So we enjoyed short sunning sessions. But one day we mentioned to his pediatrician that his feces were white and a small alarm bell went off in her mind. She had some tests done and referred us to a gastroenterologist at Lucile Packard Children’s Hospital. After a week of testing and a liver biopsy, Dr. Bass informed us that Josh had biliary atresia and that most, but not all kids with this disease, require a liver transplant before the age of two. Obviously, we were pulling for Josh to be one of the rare ones. We asked lots of dumb questions like “Is it a bad idea to take him on a multi-day boat trip in Alaska?” Dr. Bass recommended that we go on trips one at a time.
At 2-1/2 months old, Josh had the Kasai surgery to attach his intestine directly to his liver, bypassing the clogged bile duct; his non-functioning gall bladder was also removed. The Kasai put him at higher risk for cholangitis (liver infection), but it was hoped that it would also allow his liver to function normally. It didn’t. It soon became clear to the doctors that he would eventually need a liver transplant. Josh was put on the list to receive a liver at age 5 months. He had several bouts of cholangitis that landed him at the ER, and finally he was on antibiotic regimens: sometimes with a catheter (PICC) line and other times oral antibiotics.
His situation was not critical enough to actually receive a liver yet though, and we tried various things to keep him growing, since bigger is better for transplant surgery. He had to take tons of vitamins, which he really hated. And eventually he needed to be fed during the night directly into his belly (nasal-gastric feeds). Writing this now, it’s hard to believe how many medical machines and drug delivery methods we’re familiar with.
In late June 2002, we got a call to come in, that they had a liver for Josh through the system. We sat anxiously in the waiting room for a really long time in the middle of the night. But we were sent home because the lobe that would’ve gone to Josh was too big, and cutting it to fit would produce unwanted scar tissues.
Eventually, we started looking at the option of living donor liver transplantation, where adults can donate a piece of a liver to a child. The lobe of my liver that could be used was too big for Josh, and my wife had various issues that disqualified her. We started to let people know of Josh’s dire situation. Some friends and relatives came forward, and one was picked to do the donation. A date was scheduled when Josh’s medical situation worsened; his ascites (fluid in the abdomen) had gotten worse. In the meantime, Josh’s PELD score increased to 29 and he was moved up on the list. As the date neared, Josh got a cold and the anesthesiologist wanted to wait two weeks to decrease the chances of respiratory problems during and after surgery. A new date was scheduled for the living donor liver transplantation.
A week before the scheduled date, we got another call that Josh got a liver from the transplant list. This one was from a baby Josh’s size. We went to the hospital and they took Josh in. And we waited and waited and waited and waited. We met with his surgeon some 10 hours later who told us that everything went well. We took 8-hour shifts to stay with Josh full time during recovery; shifts were divided among my wife and I and his devoted nanny who took the night shifts. Josh had an astounding number of tubes and connections to equipment. He was on a serious cocktail of drugs. He had a collapsed lobe in his right lung and pneumonia, and was being watched very carefully. And the staff had to be watched too. For example, I once watched one of the respiratory therapists drop a syringe of medicine onto the floor, pick it up and start to put the medicine into Josh’s inhaler. I was furious, and I told her that she needed to get new meds for him. Another time, the PICC (central catheter) line nurses left a long needle and various choking hazards on his bed. On two occasions, the nurses did not secure Josh’s hands to his bed well enough: first time, he extubated himself; second time, he pulled his central catheter out. Unfortunately, the list goes on, which made our stay at the pediatric ICU very stressful.
During the hospital stay, I would read to him and sing. It was hard to know if he was even there. Then one day, I was doing some finger walking along his crib rail and the two finger people bumped into each other and fell down. And Josh smiled. This was the best smile I have had ever, and probably will ever see. During this time we spent a lot of time kicking around the hospital and using the sleeping rooms and talking to other parents. One of them said to us that a week in the hospital was nothing. They had been there since their baby was born 6 months ago with a heart defect. I spent one of those evenings writing down all the words that Josh could say and came up with 63, which is pretty good for a 15-month-old.
His liver was doing fine, but his right lobe remained deflated, despite the bronchoscopies and positive pressure respiratory treatments. At one point it was even recommended to us that he have a tracheotomy. We insisted on waiting. At the end of 3 weeks, Josh was extubated and then transferred from PICU to a regular ward. Yay!! At the end of 4 weeks, his strength returned and he was ok, except for the deflated lobe. Josh was released with some more machinery and complicated drug regimen (12 different medicines 9 times a day). We were glad go home. As Josh became stronger and became more active, his right lobe returned to normal. The 2 months of nebulizer treatments helped too.
Now he’s a bright little three-year-old, who loves music and dancing. He negotiates every decision. He can jump off a diving board and tread water for hours. He loves hugging people. Perhaps the world is a better place with him in it. Ours is anyway.
We are extremely grateful for the donated liver (as was Josh’s potential live donor!). We encourage everyone to be an organ donor and to let their families know of their decision.
by this mother, Sheryl
Justice was born in December 2001. He is an only child and has been spoiled accordingly. Justice was a little jaundiced from the beginning. Our pediatrician attributed most of his coloring to my being half Chinese. It wasn’t until his two-month well baby check that Justice had his first bilirubin levels drawn. It was immediately obvious that we had a problem. The next week brought introductions to the term 'biliary atresia' and the impending possibility that Justice would need a liver transplant to make it to his first birthday.
Justice’s Kasai happened when he was 9½ weeks old, just six days after that pediatrician’s appointment. Two weeks later, we were told that we had two options, redo the Kasai (with little hope for success given the first failure) or prepare for transplantation. We met the Stanford GI team the following month and began researching like mad. We also began to frantically prepare for our two month relocation from Oregon to California, which included a lot of fundraising!
Justice continued to grow slowly and was a fussy little mama’s boy. On April 26 he was listed for his liver transplant. We had a series of miscommunications and setbacks but fortunately Justice never suffered for them. He did not experience many of the complications and problems that most BA babies do. He never had cholangitis and although we were told after his liver transplant that he had significant varices, he never had an active bleed.
Justice received his gift of life from a very special person. My husband was not the same blood type as Justice and I am overweight making me a less than ideal living donor candidate. Regardless, my best friend from nursing school, Charity, insisted on being the first person tested as a donor candidate. The magical day was June 3, 2002. It was an anxious day full of tears, heartache, and hope. Although we have had a few bumps in the road, Justice has recovered beautifully. We spent a total of four weeks in the hospital following transplant. Justice then continued at “day hospital” for another week to receive amphotericin to help clear his yeast infection while we did vancomycin for his staph infection at "home." He also had three blood transfusions as an outpatient for GI bleeding that they never found a source for. We had a short readmission to the hospital for it as well, but my tough little boy seems to have resolved the problem on his own. We flew back to Oregon on Justice’s two month transplant anniversary. We continue to worry about his health but have come to accept that Justice’s life is in God’s hands and thank Him daily for the gift He has given us.
Justice’s great joys in life are chewing on blankets; he prefers the soft cotton hospital ones (don’t tell them but we snagged a few), splashing in any kind of water he can get into, watching Monster’s Clubhouse on Sesame Street, and cuddling with mom and dad. He’s such a little man now, we love to just watch him discover new things and grow and are so thankful that we have the opportunity to do so now.
Ka'apeha is 2 years old and lives in Hawaii. He was born with biliary atresia and received his first transplant, a split liver, at the age of 8 months. This liver quickly failed and he lived for 88 hours without a liver before a second organ was found. He has done very well ever since. His family says he is truly their miracle baby.
by her mother, Angie
Kaitlin was born in December 2001. She was jaundiced from birth and at one week of age she was being treated at home with a bili light blanket. At two weeks of age she was referred to Children's Mercy Hospital in Kansas City and it was mentioned that she might have something called biliary atresia. When she was 5 1/2 weeks old she was diagnosed with biliary atresia and had the Kasai.
Unfortunately the Kasai was not successful and on May 21, 2002 she listed for a new liver with a PELD score of 15 at Children's Mercy. During the time she waited for her new liver she was hospitalized twice and was treated for ascending cholangitis. At the end of May her grandmother was tested at the University of Nebraska Medical Center in Omaha to see if she would be a suitable donor. Kaitlin was also placed on the waiting list at Omaha. Her grandmother was thought to be a suitable donor but the day before surgery it was canceled due to her artery structure in her liver. Her mother was then tested and surgery was scheduled for the end of July.
In July at 2:30 a.m. Kaitlin's family received a call from Nebraska that there was an organ available for her and to be at the hospital by 10:00 a.m. Kaitlin went into surgery at 2:00 pm and was in the PICU by 8:00 p.m. She was out of the hospital after 2 1/2 weeks and went home after staying 2 weeks in the Omaha area. Since transplant Kaitlin has been hospitalized twice and diagnosed with PTLD.
There has been such a change in Kaitlin since transplant. The difference is amazing. Kaitlin has so much energy and she looks so healthy. Her family will always be thankful to the donor family for the choice that they made.
by her mother, Mary
Kaitlyn, aka Katie, begged me to send in this picture of her in her graduation cap and gown. Yes, my little angel is graduating kindergarten!
Katie was born in July 1995, just 5 minutes after her twin brother, Trevor. It wasn't until she was 8 weeks old that we realized something wasn't quite right with her. Within one week Katie was diagnosed with biliary atresia and the Kasai was performed. For the first few years, Katie was the picture of good health, with the exception of a couple bouts of cholangitis. Things were so good that we could almost for a minute forget about the whole BA thing.
For the past 2+ years Katie has been dealing with portal hypertension and bleeding varices. Most of her bleeds were minor, however she had a major bleed last December that nearly took her life. Her GI and transplant center felt that Kaitlyn was a perfect candidate for a distal splenorenal shunt. Since her LFTs are good, they felt it would be foolish to transplant her when she still has a good functioning liver. The shunt would redirect the blood flow thus avoiding the bleeding varices. But when they scoped her, so they would have more reasons to support the shunt, Kaitlyn was picture perfect. Not a varices in sight. Katie tells me she was praying to God that it would get better so she wouldn't need the operation. She must have a direct line to heaven!
Anyhow, for now the shunt is on hold. The varices are not gone forever, but at least they are not as bad as we thought. In the meantime Kaitlyn is focusing her energy on her birthday and her graduation. In her free time, Katie loves to play house, Barbies, and Doctor's Office. I do worry that she gets too much joy from pretending her stuffed animals are getting blood work done.
Katie Lady is the sunshine of our lives. She really does make us happy when skies are gray. Her faith and strength are what gets me through all this! We are so thankful to have her in our lives and pray that her guardian angel will always be looking over her shoulder.
by her mother, Kristen
Kathryn was born in August 2000, and we took her home from the hospital 2 days later believing that nothing was amiss. For the following few days, she was lethargic and had no desire to eat whatsoever. When we took her to the emergency room, we expected to have her on some IV fluids for a day or two and then go home. She ended up in the hospital for almost 6 weeks. She was found to have a portal vein thrombosis. Once they had cleared that though, her liver functions did not improve. To confirm the doctors' suspicions, she had exploratory surgery at 4 weeks old and was diagnosed with biliary atresia. By that time, her liver was so damaged, the surgeon had considered not even doing the Kasai and sending her straight for transplant evaluation. He did however do the Kasai, but it had very little effect. Two weeks later she was transferred to University of Nebraska Medical Center in Omaha for transplant evaluation. Her daddy was evaluated for living related and was a match, but Katie had to be at least 17 pounds to be able to accept a lobe of his liver, and she was only around 12 pounds and not growing. We went home to wait for our "call".
One day in December, the doctor called us and asked us to come back to Omaha to start on TPN as Katie was failing to thrive. A few hours after she was admitted, in mid-December 2000, the doctor came in and said that Katie was actually going to have her transplant. At 11 p.m. that night she went into surgery and came out the next morning with her new liver. Later that morning, she was rushed back to surgery as she had developed another portal vein thrombosis. They were able to clear it and she began on her road to recovery. For the first week, she fought fevers and was not doing
well at all. Finally they took her back to surgery where they had
discovered a perforated intestine. Once that was repaired, she pretty much breezed through her recovery. We were able to go home in the beginning of February where life has been relatively normal since.
Katie has had to deal twice now with biliary strictures, once
immediately after transplant and once 4 years after transplant. She is now being followed by the doctors at Children's Hospital of Pittsburgh as we have since relocated. Katie takes medications every day and we know we have to watch her labs for signs of the strictures coming back, but otherwise, she is pretty much living a normal life, with preschool, soccer, swimming and the regular mischief of a little girl!
We always keep Katie's donor family in our prayers as they gave the most amazing gift from their son, Matt.
by her mom, Amber
Let me start out by thanking everyone that participates in C.L.A.S.S. When I first found out that Kayleigh had biliary atresia I tried to find out as much as I could on it. We live in a fairly small town and I never had heard of anything like it. I went on the internet and found your website and started reading all the children’s stories. I couldn’t bring myself to read the memory ones until after Kayleigh’s transplant. Anyway, I wanted to let you know how much the success stories helped my family and me through the hard times.
Kayleigh Lynn was born June 2001 and was a healthy baby with what we thought was normal jaundice. When I took her for her PKU, I was called later that day and was told to bring Kayleigh back to the hospital to be admitted for abnormally high jaundice count (bili was 19). She was in the hospital for 4 days under the bili light and her jaundice level had only gone down to10. Three weeks later we were headed to Albuquerque for a HIDA-scan, and 2 weeks later we had to go back to have the Kasai procedure done. After a week in the hospital, we were sent back home to Carlsbad to play the waiting game. Unfortunately, the Kasai was not successful.
In February of 2002, Kayleigh was hospitalized with the rotavirus. I could tell that she was just going downhill. Her stomach was protruding so much and she was the color of a sunflower. Her PCP made some calls and got us a consultation at LPCH at Stanford Hospital. In March her name was added to the transplant donor waiting list.
On a morning in March our 9-month-old Kayleigh was on an airplane headed back to California to receive her gift of life. The Yates Petroleum Corp. of Artesia donated the use of their private jet and all expenses to get Kayleigh back to California within the six hours allotted. “After the call came, I packed and Kayleigh unpacked,” Kayleigh’s grandmother, Beverly, reported. At 10 a.m. the pilot had the plane ready in hopes of making the 3 p.m. schedule hospital arrival.
The pilot had called ahead for a taxi to be waiting on the runway. A strong head wind resulted in an unscheduled refueling stop, but they arrived at the hospital at 4 p.m.
Once at the hospital, Kayleigh underwent a battery of blood tests and surgical preparations. At 7 p.m. they took her to surgery.
Kayleigh’s grandmother said that everything happened so fast that doubts about whether they were doing the right thing for Kayleigh filled their minds. But one thing for sure was that they made it to California on time thanks to Yates Petroleum, she added.
Kayleigh was out of surgery by 4:30 a.m. It wasn’t until June 28 that Kayleigh was released from the medical care in California. During the surgery and after her release from the hospital, home was motels and the Ronald McDonald house.
Although she was taking at least a dozen different medications after surgery, she’s only taking three medications now. She had blood lab work done each week to check her liver enzyme levels through a Bard implant port-a-catheter in her chest to eliminate being stuck with needles.
Kayleigh was taken to Albuquerque twice in the month of July for minor bouts of rejection, but “she’s a good picture of health right now,” her grandmother reports.
“It’s been a difficult time to get her liver numbers to level out and stabilize,” her mother, Amber, said. “Minor rejections are common, and she’ll be on rejection drugs all her life.”
Since her transplant she had had some problems finding the right medication for her. She was put on Prograf right after transplant and her liver numbers never went down to the normal range. Then in July 2003, we made another trip to LPCH and had her medication changed. The doctors changed her anti-rejection drug from Prograf to Neoral. Her liver numbers started to go down almost immediately and have been great ever since. Come to find out, she was having a drug reaction to the Prograf.
Kayleigh turned 3 in June. She is a healthy, happy, normal 3 year old. She loves to paint, play with chalk and play outside. She has a wonderful personality and she makes sure she lives every day to the fullest. She had to fight to stay alive, and she’s not waiting a day. She loves life and could really teach other people how to live it.
It’s hard to think of the loss suffered by the donor family and the possibility of a meeting in the future. Meanwhile, life has changed for Kayleigh’s entire family. Going out to eat in public or going shopping is not on their extracurricular list of activities. The family can’t take a chance of Kayleigh being exposed to any disease or illness because she is highly susceptible to catching anything. “We love to take her out and show her off, but we have to think about it and then think about it again,” her grandmother said. “We have to play out every scenario in our mind to what could happen.”
The Yates Petroleum Corp has transported two other liver transplant patients, both to the Mayo Clinic in Scottsdale. An employee of the company opened the airplane roster to show Kayleigh’s grandmother something miraculous. Kayleigh's transplant day was the only day that month that the plane was in town!
“Kayleigh went to hell and back several times,” her grandmother added. “We’re thankful to Yates Petroleum and all the doctors and nurses in an unbelievable hospital.
Kelsey was born with biliary atresia and was just 10 weeks old when listed for a liver transplant. While her family waited and prayed they worked to promote organ donation in their community. One of the first things they did was to have T-shirts with the message "Give the Gift of Life" made up for their family and friends. Their goal was to get people thinking and asking questions about organ donation. Their prayers were answered when Kelsey received her new liver just a few month later. Kelsey was the first pediatric liver recipient at University Medical Center in Tucson, Arizona. In the years since her transplant Kelsey has grown into a beautiful, spirited little girl. She loves to dance, both tap and ballet. She also loves whales and her all-time favorite movie is Free Willy.
by his mother, Shawn
Kyle was born in September, 1999, and was diagnosed with biliary atresia at 8 weeks. He had the Kasai surgery on November 29, 1999. He had significant portal hypertension even before his Kasai surgery, which enlarged his spleen and caused internal bleeding. After the Kasai he had many complications, including pneumonia with a collapsed left lung. He was hospitalized for his first Thanksgiving, Christmas, and New Years.
Although he stabilized and began to grow, the damage had been done and his liver disease progressed to cirrhosis. He was listed for transplant in January 2000 and we immediately started living donor testing. I was refused as a living donor even before he was officially listed. I have polycystic kidney disease and couldn't donate. One by one, other family members were also ruled out.
We had a pager and waited for the call, but months went by with no call. In November 2000, Kyle's Aunt Lyn was tested to see if she would be a match. Lyn is my brother's wife and is not a blood relative, but when my brother was ruled out as a donor, she volunteered to be tested. She was a match and finally in December 2000, Kyle got his new liver -- a gift of love from his aunt.
During the 5 months before transplant, Kyle had almost constant infections and IV poles were a regular sight in our home. He had MANY antibiotics and other meds. He had internal bleeding for 9 months before transplant. Five days after transplant, Kyle was walking around the ICU with no IVs and no internal bleeding.
Kyle is now in school, and is a very smart and funny child. He has not been in the hospital overnight in over 3 years. His big concern now is that his favorite doctors will "forget" his name! To newcomers he is a normal little boy who doesn't look like he's ever been sick. All his liver numbers are in the normal range, and he's completely caught up to other children his age in height and weight. We now have a happy, healthy little boy and a tremendous network of new friends because of his illness. I am glad to have the chance to tell his story!
by his mother, Kathy
Kyle received his living donor liver transplant from his dad, Craig, on January 31, 1994 (at 18 months). Dr. Emond at UCSF performed the surgery. Although we live in New York, he is still followed closely by his transplant coordinator at UCSF.
Kyle is 8 now and has grown into a warm and wonderful, sensitive boy. His parents could not be prouder of him! He is doing very well at school, both academically and socially, and is the best big brother to his sister, Courtney.
Kyle is currently on just one medication, cyclosporine, where his dose is so low it is considered non-therapeutic. It is our hope that Kyle can be weaned from this drug and no longer be immunosuppressed!
by her mother, Renee
We were blessed with this beautiful little girl in June of 2003. She weighted in at 5lbs.-11oz. and seemed to be a healthy baby. At about four weeks old we noticed her skin and eyes were yellow and took her to her pediatrician. We had some lab work and her numbers cam back elevated. From there we had to take her in to do several different tests and finally they told us she needed to have surgery. Kyrie's intestines were malrotated and while they were fixing that they would look at her liver to determine if she had biliary atresia or Alagille syndrome.
In August at 6 weeks old, Kyrie had her operation. After about an hour they determined that she had biliary atresia and they needed to proceed to do the Kasai. She was in surgery for about 6 hours. That was the hardest and longest 6 hours of my life. Finally she was out of surgery and on the way to recovery. She was in the hospital for 2 weeks and came home with a PICC line and had a home nurse for almost 2 months. Soon after, everything went back to normal. Today her labs are great. We have weight gain issues, but she's gaining slowly and steadily.
Kyrie is a strong, happy, and curious little girl. You wouldn't know that she went through all of this. We are truly grateful to our Heavenly Father for blessing us with Kyrie. We're grateful everything went as well as it did and she's doing so well. We're grateful to her doctors at Primary Children's Medical Center and Cottonwood Hospital in Salt Lake City, Utah, for all of their hard work and help during this time. And lastly we're grateful to our family and friends who gave us the support and strength we needed during this time. Thank-you.