Biliary Atresia: Letter D - H

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Daniel

by his mom Kathyann

My "Book of Daniel" is, in part, a story about an amazingly strong little boy whose tenacity and will to survive have inspired me more in the past 2 years than anything previously in my life. It is also, in part, a story about what life has been and done to a family confronted with a terrible illness and that particular family’s struggle to not only survive but to truly be a family.

Our story began when, knowing we were expecting, we received frightening news that a blood test came back positive for neural tube defects and Down syndrome. We immediately went in for an ultrasound only to discover the cause of the test result wasn’t that the baby was in trouble, but because there were TWO babies. It was a feeling of great relief and complete amazement all at once.

Twins do not run in either of our families and we’d taken no fertility medications. It was not something we had even considered a possibility. We were thrilled and freaked out simultaneously. We had a 2-year-old at home and now we would go from a cozy family of three to a big (by today’s standards) family of five.

The pregnancy progressed with no other surprises. An emotional low point came when we lost our beloved cat. I cried so much that I became dehydrated and had a few concerning contractions. The result was bedrest for the remainder of the pregnancy (about 10 weeks). I followed doctor’s orders, working from home on a laptop, and anxiously – and I do mean anxiously – awaited the big day.

Now, looking back on that pregnancy, I realize those boys (yes – 2 boys!) had distinct personalities even in the womb. Antonio was head down and happy to remain in the launch position pretty much the last 2 months of the pregnancy. Daniel was busy even then. Every week he had a new position; breach, head down, transverse breach and back again. We approached the day realizing a C-section was a probability.

At 37 weeks, the doctor gave me the nod to get out of bed. Freedom for me was hitting the yard sale across the street, walking to the park with José and, of course, shopping! Three days later, the babies arrived – in grand fashion.

We barely made it to the hospital. There was no time for an epidural (I nearly cried) and en route to the operating room (30 minutes after we got to the hospital) Antonio was born – on the gurney. Daniel, who was in the transverse breach position on this particular day, was attempting to enter the world butt and feet first. He took more work but was still just 3 minutes behind his brother. Two beautiful little boys.

We named them almost immediately. Antonio for his great great uncle and Daniel for a dear friend we lost some years back. Both boys were healthy.

It wasn’t until December 9th (a date etched in our brains) that that all came to a crashing halt. We hadn’t the slightest inkling of the frightening road we’d just turned down.

Daniel had a temperature. We thought it was an ear infection so took him in. The doctor noted he looked jaundiced. We hadn’t noticed and weren’t really looking for it. All our boys had newborn jaundice. José was hospitalized for 5 days with it. But once passed, it didn’t occur to us that it would be of any concern. Daniel is Mexican, African American and White. He is darker than Antonio who is quite fair. We assumed his coloring was natural. It wasn’t.

Blood tests revealed his bilirubin was higher than normal. Additional tests showed no infection (i.e. Hepatitis. C). I went on-line to investigate. There’s nothing – I mean nothing – that you would ‘hope’ a baby that had jaundice at 3+ months would have. The most frightening of the options was biliary atresia. We’d never even heard of it.

Louie called his dad – a family practitioner in San Diego. Always a pragmatic, calming voice when we (I should say “I”) have been worried about health issues in the past. This time, there was concern in that comforting baritone on the other end of the line. “That’s not good,” he said. Later, Louie’s mom told us he sat up half the night researching it.

More tests and a scan that shows the movement of radioactive dye through the liver. The idea is the liver takes it and passes it on. They administer the dye and then do the scan every 20-30 minutes until they see it move on. The test was to take 2-3 hours. The doctor that first caught the jaundice met us at the hospital. He’d never had a baby with jaundice not be diagnosed with something before now. His presence was heartwarming and alarming.

The test took 8 hours. The liver never passed on the dye. The technician, who had been friendly to us throughout, suddenly became excessively so. My heart was pounding. Louie looked tired but Daniel cooed and played; happy as a little pig in mud that he was eating.

The doctor told us she feared biliary atresia. It might not be – he might be quirky and would eventually outgrow it. The only way to be certain was exploratory surgery… in 2 days. “We’d like to admit him tonight,” she’d said. I was frantic, frightened for my child, trying to figure out how we would do this. He was still nursing, I had another one nursing at home and a 3-year-old who needed his parents and his little brother at home. I clutched Daniel and tried, badly, to explain. Louie paced like a caged tiger. The doctor suggested we do the prep as outpatients instead. Thank God.

The next day we took Daniel to the pediatrician for his pre-op physical. The doctor who made the original diagnosis wasn’t there but his usual doctor was. He looked worried. Daniel checked out fine and the doctor gave us a plate of Christmas cookies his wife had made. My sense was he didn’t think we’d have much of a Christmas.

December 23, 2001, Daniel underwent his first operation. I remember lying with him the night before stroking that perfect little tummy and feeling that things weren’t supposed to be like this. We should have been exhausted from 2 a.m. feedings for two babies not endless doctor’s appointments; second-guessing ourselves for not getting that 2nd Diaper Genie – not for whether or not there was something I could have done when I was pregnant or something we should have noticed weeks ago. We were supposed to be putting the final touches on the babies’ first Christmas tree, baking cookies with José and taking pictures with Santa. Instead we were on the eve of the biggest fight of our lives.

As a note of reference, biliary atresia (BA) is a disease in which the bile ducts between the liver and small intestine never form or are clogged by illness. Only 1 in 15,000-20,000 babies are born with it. It’s so rare, they still aren’t sure what causes it. There is no cure or preventative measure for it. The body is unable to digest food and the liver slowly destroys itself from the bile it’s unable to excrete. Babies with BA generally show symptoms at 4-6 weeks (jaundice, failure to thrive, and clay colored stools). Daniel didn’t or at least not so pronounced that either his parents or doctors noticed.

While most BA babies will need a liver transplant at some point, a procedure called the Kasai is often done initially to buy time for babies to get bigger and stronger. The surgeon rewires the small intestine to connect directly to the liver. It is not a cure but the sooner the diagnosis (prefer under 8 weeks) the more likelihood the baby will manage with the Kasai for a lot longer. Daniel was 4 days shy of 4 months.

The first part of the surgery is to confirm or rule out biliary atresia. If it’s not BA, they sew him up and he’s out of there in 2-3 days. If it is, they perform the Kasai.

One hour into the operation, the Operating Room called. “It’s biliary atresia,” said the doctor. “We’re beginning the Kasai.” I still don’t think we “got it” even then but – there we were – waiting and wondering what this meant to our family; all alone on a Sunday, 2 days before the babies’ first Christmas. It’s important to note there is NOTHING open in hospitals on major holidays. No food, no lounge… nothing. Everything runs on a ‘reduced schedule.' Even getting out of the parking ramp was an ordeal.

January 1, 2002. They thought he would be out of the hospital in 10 days… a late Christmas but that would be okay. We had celebrated on a smaller level for José’s benefit. Antonio and Daniel were just 4 months old so the meaning of the day was lost on them. It wasn’t lost on us, though, and a hospital is a lonely place to be during the holidays.

The new year dawned with Daniel in bad shape. The surgery had failed and he had developed peritonitis. He was borderline septic and at risk for heart failure. En route to the Pediatric Intensive Care Unit (PICU), his veins collapsed and they couldn’t reestablish an IV.

Louie and I stood in PICU holding hands so tightly as if by doing so, we could hold on to our little boy. The doctor couldn’t get an arterial line established. The nurse brought us a chair. The anesthesiologist came in. “He’s a sick little guy,” he said repeatedly as he gave us what we’ve come to refer to as the “doom and gloom” anesthesia speech the lawyers make them tell frightened parents about the potential “albeit unlikely” risks of anesthesia and surgery.

Finally they took him – again – to surgery. The first time I’d handed my baby to these kind strangers was one of the hardest things I think I’ve ever done. This time was a blur of fear. New Years Day we sat, alone, in the waiting room, wondering, praying and playing Trivial Pursuit as if our lives depended on it.

Daniel didn’t leave PICU for the next 3 weeks. One week after this surgery, the peritonitis returned. On January 8, they operated again. Five days after that, they found an abscess that later tested positive for E-Coli. There seemed no end to this nightmare.

All this while Daniel was on a respirator. I was at the hospital most of the time although sleeping at home to try and nurse Antonio and be at least some presence for José and Louie. Louie was trying to make things normal and okay for both José and Antonio. Antonio was rolling over – sitting up and smiling. He was such a happy baby and I missed him terribly. I felt so blessed that he still needed me to nurse him. It let me feel like I was still a Mommy. José was busy with his preschool (we were so grateful for Miss Bernie’s). But not having Mommy and Daddy around was hard for him and he always seemed a little lost when I’d walk out the door in the morning.

Meanwhile, our “divide and conquer” approach to this situation was causing friction between us. There was never any time to talk or to share our fears. When we would try to connect, we were so busy dealing with our own demons and all that had to be done (work, take care of the house and the kids and the dogs and be there for rounds and for Daniel) there just wasn’t any time for us. I wanted Louie to make it all okay and there just wasn’t any one person that could do that. I felt like I was always afraid. Did I ask the right questions? Was there a question I’d missed? How did the doctor’s know from shift to shift what was going on if I wasn’t there to tell them?

It was the dead of winter – cold and bleak as Minnesota is in January.

January 29, 2002, over a month after when he went in, Daniel came home. But he was so sick and suffering from withdrawal having been weaned off the morphine and methadone too quickly. It was painful and disruptive and wonderful at the same time.

Two and a half weeks later, only a couple of days after he was evaluated for a liver transplant and listed, he was readmitted to the hospital – now Fairview University – where he would receive his new liver. This time, it was spontaneous bacterial peritonitis. The surgeon explained that he was #1 on the transplant list for pediatric patients in our region because of his condition. He remained hospitalized for 2 weeks.

Managing the home front, Louie had tried to set aside time that was just José’s. We sat down with him to talk about Daniel and ask if he understood why he was back in the hospital. “What did I do?” he asked, big brown eyes full of concern. This nearly killed us. Of course he would think this was his fault. I think of the times that I’d checked his brotherly enthusiasm when Daniel was home. “Be careful around your brother,” I would caution. We promised to keep him better informed and assured him it was not his – not anybody’s – fault.

Louie and I learned to administer IV medications, TPN (IV ‘meal in a bag’), oral medication management and a whole new organizational system for cannulas, syringes, sharps, dressing change kits, swabs, etc. The left-hand produce drawer in the fridge became a medicine drawer. The living room looked like a really messy hospital room. But we did it. José would check us on our technique. “Daddy, make sure you get all the bubbles out of the line.”

Two days after José’s 3rd birthday the surgeon called, “we have a liver for Daniel.” “But, he is too little!” I thought in panic. The doctors had told us (and we had read) that the chances for surviving the surgery dropped dramatically for infants under 20 pounds. Daniel was only 12 lb. He had just turned 7 months old and we were hoping to get him to 2 years! It seemed like our efforts were working… he was doing okay. It was the last thing we were expecting to hear so soon.

But Daniel’s blood type wasn’t that common and we had no living donor back up. I was the wrong type. Louie was too big. My mother was too old. My aunt’s cholesterol was too high. And, he wasn’t doing that okay. The decision was made. We would proceed with the surgery. We would, once again, put our baby in God’s hands and pray we were making the right decision.

My parents came over – again – and we bundled him up and went to the hospital. The liver was coming from an 18-month-old baby in Chicago who had been killed in a ‘tragic accident.’ That’s all we knew. It was enough.

We waited… and waited. We paced. We slept. It was an interminable wait and yet it went too quickly.

At 3 a.m., they came to get our baby. The liver was still en route to the hospital but it would take 2-3 hours just to set all the lines and drains they would need. The nurse told us not to make a fuss over saying goodbye – giving us pause to consider making a fuss over her chiding us NOT to make a fuss over saying goodbye. We kissed our son and stole just one last stroke on his head. He looked at us with more strength than I knew I had in me at that moment. Not a tear from his precious eyes as they carried him away from us.

Louie and I walked in fear and wonder at the moment to the very dark – very lonely – waiting room. We held hands. Neither spoke of our shared fear; would this be the last time we would see our son alive? The hole in my heart seemed cavernous.

Amazingly, the sun rose. People came and went. At 2 p.m., the doctor came to tell us the surgery went well. Daniel, our special angel, had come through like the fighter we had come to know him to be. The surgeon seemed pleased and we were euphoric. But - of course, it was Daniel and nothing could ever go smoothly or according to someone’s plan.

Daniel’s stay in PICU was expected to be 4-10 days. Complications with infection kept him there a month on chest tubes and intubated too long. When he did get transferred to the transplant wing, he improved quickly. We brought him home the end of April 2002.

That summer was relatively calm. A couple of infections that landed him back for a couple of days but not too bad. Better were the days we, as a family, went to the zoo. I remembered the endless hours watching his tiny frail body buried beneath a freeway of tubes and drains; his own blood staining his small hands, wondering if I would ever be able to take him home; if he would ever get to play with his brothers or go to the zoo. The day we stuck his little feet into the warm sand at the Meerkat exhibit and saw the look of wonder on his face, I knew we’d be okay.

A few months later, just after the babies turned 1, Daniel started having problems. He was in pain – couldn’t sleep – couldn’t eat. We took him to the doctor on call (these things ALWAYS happen on weekends). The doctor suspected an ear infection - proceeding to give me a 10-minute lecture on how I could prevent ear wax build up in all my children by simply irrigating them nightly after bath. We’d seen ear infections and this wasn’t one of them. Antonio, having been blessed with good health up until this year, had had two perforated eardrums over the course of July/August along with several less severe infections. He had gone in for ear tubes just a couple of weeks before this situation with Daniel.

We called the transplant folks. In he went. It was Clostridium Difficile (a gastrointestinal bug). Routine labs came back a few days later indicating it was also Epstein Barr Virus (EBV), commonly known as ‘mono’. For immune suppressed children, it’s not good.

Days turned into weeks. The EBV was all over the map and the concern was that it would result in proliferating lymph nodes that could develop into lymphoma. Tests were inconclusive and troublesome. Another test in the 3rd or 4th week revealed that the lymph nodes were abnormal but also beyond the reach of a scope. An open lap biopsy (which of course later became infected) found a complete hole in the intestine.

The next day, as I sat rocking Daniel, we were visited by doctors from Hematology and Oncology. “You must have the wrong room,” I argued. “Daniel doesn’t have cancer!” But the doctors were concerned. Their concern manifested itself in a spinal tap and bone marrow biopsy. More waiting – the longest ever. Ultimately, it was determined that he did, indeed have the initial stages of Post-Transplant Lymphoproliferative Disease (PTLD).

In a way it was a relief that we could finally call it something. He began a series of treatments the doctors insisted was “NOT CHEMO” and the nurses referred to as “CHEMO” at the CANCER center. So, we call it “chemo-lite”! Whatever its proper designation, it worked and Daniel came home the week before Thanksgiving. We were so very very thankful.

Of course the story cannot end without the little twists of irony. That came shortly after Thanksgiving as we prepared for what would truly be the babies’ first Christmas and would mark the end of this frightening road. The year had taken a hard toll on Louie. Having had to be a single parent to José and Antonio, worrying about Daniel and alternately worrying about and fighting with me, there wasn’t much in reserve. He had dropped 40 lb. in 6 months leading into the winter. His back had gone out twice. So, we limped into the holiday eager to bask in the hope for a happier New Year.

The doctors, concerned now about the threat of chicken pox, ordered him to get vaccinated as he didn’t remember ever having had them. I was safe and José and Antonio were vaccinated but Daniel cannot be because it’s a live virus vaccine. With the PTLD still a vivid memory, a case of chicken pox would be very bad. So Louie got vaccinated and I’ll be damned if 3 weeks later – right after Thanksgiving, he didn’t come down with the Chicken Pox. The doctors felt it would be safer to keep him away from Daniel even though it was unlikely that he was contagious. Because of his weight loss, they feared he was, himself, immune compromised and the pox were a hybrid of the wild variety. So, we shipped him off to San Diego; banished until he scabbed over, which he did – the day before Christmas. The family was finally reunited Christmas day.

In Retrospect and Thanks:

Daniel missed his first Christmas, New Years, Valentine’s Day, Easter, Mother’s Day and Halloween…and we missed him. Our family has stretched more than we would have thought possible. People from all over the world have prayed for Daniel and been there for all of us – and we truly believe it has been those prayers and acts of kindness that brought us through.

The nurses and doctors at Children’s Hospital and Fairview University Hospital in Minneapolis have been a source of great strength. They advocated for Daniel when we couldn’t be there. They patiently listened to and answered our endless questions. Children’s Hospital put Daniel in the Karuna program for children with an ‘uncertain’ future before the transplant and he remains in their care to this day. They have come to our house in the middle of the night to check on him. They have cared for and about him since he first came home that bitter cold January. They also provided us with a volunteer who gives us 3 hours off each week. That’s given us back the gift of conversation. Now we have that time that we can count on to reconnect and share our fears.

The doctors and nurses at Metropolitan Pediatrics in Edina were, and continue to be, an invaluable resource when one (or all) comes down with something. Dr. Sercumbe first diagnosed Daniel and helped us through the initial tests and Dr. Kane is constantly there (even rearranging his schedule) for Daniel, Antonio and José (although in his world, they are “Danny”, “Tones”, and “José-can-you-see”). They are a comforting crew every time we make a run for the doctor’s office.

Daniel’s namesake was an orthopedic surgeon named Dan Gaither. On a particularly difficult day in recovery, we noticed a slip of paper on the table. There was only one word – GAITHER. No one knew of any Gaither in recovery. We believe Dan is Daniel’s guardian angel. He has been guiding us and holding our little bear’s hand from the beginning.

Our families have been there for us every step of the way - praying, baby-sitting, cleaning the house, grocery shopping and listening. We have been an extraordinarily ‘needy’ family these past couple of years and yet they never tire from offering to give us a hand or a place to get away for a few days.

My coworkers and bosses supported a completely fluid work schedule for months. They were supportive and caring friends throughout.

We are so blessed that the family of the baby that died that night sought to give life to another child they didn’t even know. And Daniel lives life fully. He is the mischief-maker with a smile that lights up a room. He and his twin brother have reconnected and they adore their big brother who wants only to be where his “babies” are.

Now, one year later, we look back at these events with new eyes and a fresh awareness and appreciation for things that, I won’t say we took for granted but we would not have looked at in the same way. Silly things like giggles and bubble baths and brotherly spats, cotton candy, ladybugs, earthworms, kitty cats, Tarzan yells and baby feet. All are funnier, sweeter and more precious now that we feel we have reached the other side of the abyss. We know there are no guarantees but no one really knows what lies ahead. We live each day, grateful for it and so very alive.

Now we just have to figure out how the heck you raise 3 rambunctious boys. It’s a wonderful life!

Daniel G

Daniel G

by his Parents

We are thankful for all of the doctors and nurses who cared for Daniel. Even when they had bad news to tell us, their sensitivity and compassion somehow made it a little less painful.

Two days after Daniel was born he was becoming jaundiced in appearance. Other than the yellowish coloring, he was a beautiful and healthy baby boy. When he was three days old, we took him to the on-call pediatrician who was concerned about the jaundice and felt there was a need for some tests to determine its cause. Daniel's direct bilirubin was checked and found to be quite high around 6 or 7. (Normal is less than 1) The pediatrician thought it could be one of two things: neonatal hepatitis or biliary atresia. He leaned towards biliary atresia.

 

What is Biliary Atresia?

"What is biliary atresia?", we asked the doctor. Biliary Atresia is a blockage of the biliary duct which leads from the liver to the large intestine. This duct serves as a conduit to remove bile from the liver to where it can be excreted from the body. Daniel's jaundice was probably a result of a blockage to the common bile duct. Besides the blood tests, Daniel had an ultrasound and a MRI to determine any cause for the blockage of bile. All signs pointed to biliary atresia. The pediatrician had referred us to a neurologist at the local children's hospital. He agreed with the pediatrician and told us he would need surgery as soon as possible. Daniel's case was unusual because they had never diagnosed biliary atresia so early. Other times it had been diagnosed between 4 weeks and 2 months. By that time the back up of unsecreted bile could damage the liver. In fact almost all patients diagnosed with biliary atresia would at some time in the future require a liver transplant. After getting a second opinion, we scheduled the surgery. Two days before his surgery, his stools began to turn grayish, losing pigmentation; this is another sign of the disease.

The Kasai Procedure

The surgery Daniel required was called the Kasai Portoenterostomy named after Dr. Morio Kasai, a japanese doctor who invented the procedure. The procedure Daniel would undergo would be in two parts. During the surgery

and before the Kasai procedure, the surgeon would inject a dye into Daniels's gall bladder. Sometimes this dye would flush out whatever was blocking the bile duct causing the biliary atresia. If that didn't work, the Kasai would be done. The Kasai is an operation using a loop of bowel to form a duct to allow bile to drain from the liver. If unsuccessful, a liver transplant would be inevitable.

A call to prayer.

We got the word out quickly, "Pray for Baby Daniel!". Thanks to e-mail technology, people around the world were praying for Baby Daniel. We needed prayer too. It was a very emotional time for my wife and I, our baby was so young, we were just getting to know him. Fears and worries of all of the "what ifs" were constantly bombarding our minds. The prayers and support of our family and friends would keep us from falling

apart.

The Word from God

It was the day before the surgery when I received a word from God that would give us peace, encouragement and faith to face the words of the surgeon following Daniel's surgery. It may sound strange to you, but God spoke to me. Not audibly, I was reading the Bible when the words "jumped off of the page" and entered my heart. I knew Daniel was going to be ok. I was not afraid of the surgery or the outcome. What was the word, and how did make a difference? I'm glad you asked. Around the time that Daniel was born I had been reading in the Bible the Passover story, the slavery in Egypt, the plagues and such. The day before Daniel's surgery I read about the parting of the Red Sea in Exodus chapter 14. A great story but what I read in Exodus 14 verse 13 became part of Daniel's story! Just as he breathed life into Adam at creation, God breathed life into that verse, speaking into this situation exactly what he would do. Exodus 14:13 reads, And Moses said to the people, "Do not be afraid. Stand still, and see the salvation of the Lord, which He will accomplish for you today. For the Egyptians whom you see today, you shall see again

no more forever." NKJV

"I don't think it went well. I'm not very optimistic."

"I don't think it went well. I'm not very optimistic." These were the words of the surgeon when she briefed us on Daniel's surgery which she had just completed. Daniel had been born only three weeks before, two days after Hanukkah and two days before Christmas. The "word" from God was being tested. The surgeon went on to tell us what she saw during the surgery: Daniel was born without a gall bladder. The common bile duct thought to be blocked was nothing but a fibrous strand that had never developed. When attaching the loop of bowel to his liver she could not see any miniature ducts from which the bile would flow into the new surgically-made common bile duct. Believing that Daniel would most certainly need a liver transplant, the surgeon gave us the name of a surgeon at another children's hospital who did pediatric liver transplant surgery and she told us that she would put Daniel on the list for a new liver as soon as possible.

Signs of Success

"When will we know if the surgery has been successful?", we asked, almost ignoring the surgeon's negative report. The signs of success would be: pigmented stools, lower bilirubin count and the jaundice fading away. Now we had to wait for the signs to become evident. Three days after his surgery, while I was at work he had his first bowel movements. The contents of his little diapers were examined by the doctors of the GI team. The contents were as green as grass. " Is this a good sign?", my wife asked. Their answer was exactly what my wife needed to hear; "Yes, it's a good sign." for the next week in the hospital, his blood was taken and his stools were checked. It was so hard to see him with tubes coming out all over the place, but we were excited by what we were hearing of the results. His bilirubin count kept dropping and by all accounts Daniel was flourishing. Several months later his quarterly check-up with the gastroenterologist and surgeon showed that his progress was remarkable. The gastroenterologist said that he had never seen a patient's bilirubin level decrease so dramatically as his and he put Daniel in the 95th percentile of patients he had diagnosed with biliary atresia.

Miracles

Miracles? Yes I believe in miracles. Life is a miracle. Without a doubt, the fact that my son is alive and well is by the grace of God. The fact that the biliary atresia was diagnosed so soon giving him a better chance at survival without a liver transplant. The fact that although no mini ducts were visible, the bile continues to flow. The fact that Daniel's liver appears as healthly as if he hadn't been born with biliary atresia. Daniel is now 6 years old. Twice in 2001 Daniel was admitted to the hospital with a diagnosis of cholangitis based on the combination of high fevers and elevated liver enzyme counts (AST and ALT). After receiving antibiotic treatments for a week without the expected drop in the enzyme counts and with the

appearance of other viral related symptoms (rash and congestion) the diagnosis of cholangitis was questionable.

God continues to bless Daniel with excellent health and no adverse changes in the function of his liver. He was hospitalized twice for cholangitis last year (2003) and was sent home with IV medications administered by mom and dad. The presence of the infection testifies to the success of the Kasai procedure he had when he was three weeks old. I am confident that G-d has not changed His mind about Daniel; the day will come when Daniel will be completely healed. Currently his AST's and ALT's are normal (as if he didn't have biliary atresia).

Do YOU need a miracle? Please feel free to e-mail me. Our family would be honored to bring your needs before God as we pray. The same God who healed Daniel will heal you or your loved one.

Medications

Part of Daniel's continued success over biliary atresia is due to the wisdom of Daniel's gastroenterologist's post surgical prescriptions: 5cc's of Bactrim twice daily, an antibiotic used to fight off cholangitis in which the bile duct would suffer infection. 1ml of Tri-Vi-Sol plus iron daily (now a multi-vitamin with Iron), a vitamin supplement by Mead Johnson. 15ml's of Liqui-E by Twin Labs, a liquid form of vitamin E that is easier for Daniel's to digest (Now we are using the water solubilized 400 IU capsules squeezed into Bactrim and juice). Lastly, 2.5mg's Mephyton taken 3 times a week, a tablet form of vitamin K.

The Necessity of Early Referral

In researching about biliary atresia I have found that successful treatment of the disease is more likely when: it is caught early as in Daniel's case or when treatment comes from those specialists familiar with biliary atresia. If you know of a newborn experiencing the symptoms of biliary atresia, please have the patient referred to a competent specialist immediately to rule out biliary atresia or confirm it. It could save their life.

Darrius

Darrius

by his mother Susan

Darrius was born in June 2000. He was 8 lbs., 12 oz. and looked very healthy. Two weeks went by and I realized that something was wrong. All he did was cry and didn't eat. When he was 8 weeks old, we found out he had biliary atresia. My life felt like it was up side down.

Darrius was in and out of the hospital numerous times while waiting for his liver transplant. It was a rocky time of touch and go. He was called four times to the hospital for the transplant and each of those times something had happened and he was unable to get one. The fifth time, in May 2001, he got his Gift of Life. He had waited almost a year. I am so sorry and so thankful to the parents of his donor child.

Darrius stayed in ICU for some time after his transplant and then returned home. Ever since then we have been in and out of the hospital so much it is still our 2nd home. He continues to have medical issues but God is good and he will see a way for us to make it through this time. With all he has been through and how often he is sick, he laughs so much and talks better than my 3-year-old. He loves to show everyone his port and tells them, "Look I have a port but you can not touch it, it hurts me." Then he will laugh and say, "Okay me done now."

Thank you all so much for the many thoughts and prayers. This whole process has been very hard on my children and me, but I never lose sight of what He has blessed me with -- my three beautiful children, Walter, Yasmin & Darrius. All of us ask you to remember the Angels that watch over Darrius: his donor child, and Emily & Chloe. They will forever be in our memories.

Dawna

Dawna

Dawna was born in November 1997 and diagnosed with biliary atresia at 8 weeks. A Kasai procedure was done, which failed. In September of 1998 she was placed on the waiting list for a transplant. Thankfully, in December she received her new liver. Dawna has blossomed into a beautiful, healthy little girl since her transplant and her family is grateful for her newfound health. They hope other parents who are anxiously waiting for an organ for their child will find some comfort in Dawna's success story.

Dean

Dean

by his mother Susan

Dean was born in July 2003. He was full term and healthy at birth. He seemed a bit jaundiced from the beginning but our pediatrician assured us it was probably newborn jaundice so we didn't worry. When he was 6 weeks old he had a minor surgery for an inguinal hernia. Luckily his surgeon saw his jaundice and decided to test his blood. That started some more tests, an ultrasound which showed no gallbladder and then a HIDA scan that showed no bile flow from his liver. He underwent exploratory surgery which confirmed his biliary atresia and then had his Kasai procedure during the same surgery at 6 1/2 weeks. We started giving him many medications by mouth, three times a day. It was very difficult at first but we grew accustomed to the new routine.

Initially, his bilirubin levels dropped and the Kasai appeared to be successful. At 3 months old, he had another hernia on the other side and had another surgery. When he was 3 1/2 months old, the bilirubin levels in his blood started to climb again. The doctors thought he might have cholangitis and so he had another operation to place a central IV line in his chest so we could administer antibiotics at home. We had a visiting nurse once a week to change the dressing and we gave him medications twice a day through his IV in addition to the other oral ones. Through all this, Dean was growing and smiling and happy every day.

The IV medications did not change the declining trend in his blood so he was taken off. He is now 5 months old and we know he will need a liver transplant soon. His bilirubin levels continue to climb and we are starting to look into transplant centers. We are hoping that my husband or myself can be a match so we can do a living donor transplant. All three of us are the same blood type so we are optimistic.

Dean is a strong and happy baby. He likes having stories read to him and going for walks. We are doing our best to help him grow and give him all our love so he will be ready for his transplant. We are confident that he will come out of this okay and that God will look after him.

Drew

Drew

by her mother, Mika

Our daughter Drew Catherine was born in November 1999. She was diagnosed with biliary atresia and had her Kasai procedure at 63 days old. At first they thought the Kasai didn't work because her stools turned pale again shortly after her surgery. We waited and were in the process of scheduling exploratory surgery when her stools started coming out green again. I never thought I would cry over green poop!

Drew had a Broviac catheter in for about three months after the Kasai. Her bilirubin level came down to 1 at six-months old and her other liver numbers settled down to almost normal. The first two years after her diagnosis and surgery were uneventful with no hospitalizations. Drew never had cholangitis and remained stable and seemingly healthy. We truly believed that Drew might be one of the handful of kids with biliary atresia that would never need to have a transplant.

Unfortunately for Drew, that was not the case. We did not realize that despite her stable liver numbers, she had developed severe portal hypertension. In June of 2002, Drew had a major, life-threatening bleed that required a 911 ambulance ride to the hospital. She received 2 units of blood and it took 5 days in the PICU for her to recover. They did an endoscopy that revealed grade 4 varices in her esophagus and we were told to schedule an appointment at UCLA for a liver transplant evaluation.

After several tests and discussion with the transplant team, Drew was listed and remains so at UCLA Medical Center in California. Drew¹s case is somewhat unique in that her liver function and her bilirubin levels are normal or close to normal. Her biggest issue is the complication of portal hypertension and the risk of having another bleed. She has been treated with beta-blockers and endoscopic procedures in an effort to reduce the size of her varices and decrease the risk of another bleed.

Looking at Drew you would never know that there was anything wrong with her. She is as feisty as ever and would stay up all night running, singing and jumping if we let her. We have learned to take it one day at a time and look to God, our families and friends for support. The people here at C.L.A.S.S. are like a second family to us and we appreciate all of the encouragement and knowledge shared here.

Dylan

Dylan

by his father, Noah

Dylan was born 06/05. While giving birth my wife developed an infection and as a preventative method they put Dylan in the Neonatal Intensive Care Unit on antibiotics. We never were told but at two days old they were concerned with some jaundice and did a direct bilirubin test (normally they due a complete bilirubin test, which does not differentiate between direct and indirect bilirubin) and if we had known the results to this crucial test, we might not even being telling this story. His direct bilirubin at 2 days old was over 4.

Not knowing the above information we went home as two newly proud parents…But…

-At 3 weeks of age we took Dylan to the Dr. because he seemed unhappy and irritable, he also had uncontrollable crying. The Dr. said he was most likely a little colic.

-At 4 weeks of age we took Dylan to the Dr. because Dylan had a bad rash on his face, the Dr. said it was most likely do to prickly heat.

-At 5 weeks old we went back to the Dr. because the rash had spread and went all over his body and had white pimples to it, the Dr. once again said it’s due to the hot weather here in California.

-At 6 weeks old my wife noticed that there were lumps on each side of his neck, we of course went to the Dr. and he immediately ran blood work, which came back with high liver counts and showed that Dylan was indeed sick, the question was from what.

-At 7 weeks, we were referred to a Hematologist/Oncologist to rule out cancer. After seeing the Hematologist/Oncologist he said that he couldn’t be certain it wasn’t cancer but felt we didn’t need to worry too much about it at this time. Our Pediatrician ordered some more follow up labs and as he called them “random ones he would never normally order” were done. The Dr. received the results back and ruled out Hepatitis A-D, a series of viruses like CMV, a bunch of Genetic tests. The main ones that stuck out was his liver function and that his bilirubin was very high.

-By week 8, we were referred to a Gastroenterologist, who in turn did some more specific blood tests and ordered an Ultrasound, a HIDA Scan, and informed us that he believes Dylan will need a liver biopsy and thinks that Dylan might have what is called Biliary Atresia. We had a meeting with the Pediatric Surgeon within 3 days and 4 days later at 9 weeks and 1 day old, Dylan underwent a laparoscopic liver biopsy and cholangiogram. Two hours into the procedure we received word that this is Biliary Atresia and they would be performing the Kasai Procedure.

Dylan today is 2 weeks post-Kasai and is doing okay. His direct bilirubin has gone from 4 (pre-op) to 8 (post-op) and now as of last bilirubin test has gone down to 5. The Dr’s are hopeful to get these numbers lower but at the same time they have also informed us that Dylan will most likely not be in the 30% of kids who get the Kasai and never need anything again. Knowing/fearing this, I have already asked to donate a portion of my liver when needed and they said that shouldn’t be a problem, as we have the same blood type.

All-in-all this is a very stressful and frustrating time but you have to make the best of it. After all you have to be strong for your baby…they deserve at least that.

In our eyes the future is good. We have medical insurance and fantastic dedicated Dr’s who are committed to Dylan and his well being.

We would like to thank the individuals involved in saving our baby’s life:

Dr. Holly Williams, MD
Chief of Pediatric Surgery (Oakland)

Dr. Edward Rich, MD
Pediatric Gastroenterology

Dr. Matthew Gracianette, MD
Pediatrician

Emma Anne

Emma Anne

by her mom, Sheri

Emma Anne, was born in February 2000. She has been diagnosed with biliary atresia. She had her Kasai done at 6 weeks old. It was thought at first that her surgery only partially worked because her bilirubin level had stabilized at 2.8. The first 5 months were rough and we spent a lot of time at Children’s Hospital, Wauwatosa, Wisconsin. But it is now believed that cholangitis was the culprit, keeping her bilirubin level up, because after her last treatment of antibiotics, at 5 months old, her level has gone down to 0.6 and we are thrilled!

Emma is such a happy baby despite all that she has been through. She has a winning smile that seems to melt the hearts of all the nurses and doctors she has met.

With biliary atresia we take it day by day, treasuring each day Emma is healthy. My husband and I have found out that learning everything we can along the way and having a lot of faith has helped tremendously.

We also feel very fortunate to have the C.L.A.S.S. site for all of the information and support from other parents. I hope that more and more parents of kids with liver disease find their way to C.L.A.S.S.

Emma Marie

Emma Marie

by her mom, Angela

Our daughter, Emma Marie, was born in August 2003. She was a beautiful baby with a full head of black hair (much to our surprise...we both are fair). She weighed 6 lbs. 10 oz. and she appeared to be very healthy. The second night in the hospital, Emma's blood sugar dropped dangerously low. It is unknown why this happened, but we thank the Lord for the nurse who was on call that night for noticing Emma's lethargy and mottled skin. She was given formula supplement and sugar water, and was back to normal within 12 hours. It never happened again.

At 3 weeks of age, Emma began to look jaundiced. We remember thinking, "Oh no, we're going to have to go to the hospital and spend the weekend under the bili lights. We have so much to do...there is no way that we can take time out of our schedule to spend a weekend in the hospital!" If only we knew of the road that our precious angel would lead us on.

At 6 weeks of age, we were sent to Denver Children's Hospital. After many tests and a liver biopsy, Emma was diagnosed with biliary atresia. Her Kasai was done at 7 1/2 weeks. The surgery appeared to be very successful, at first! She had bile flow in the hospital, and we were so happy. However, after the first two days, the bile flow decreased. We were finally able to go home after 5 1/2 weeks in Denver.

In December, we went back to Denver for Emma to be evaluated for liver transplant. During the evaluation, she was scheduled to see the cardiologist (a valve in Emma's heart did not close when she was born). They said this happens in about 50% of babies, however it usually closes within the first 3 months. We were told it would be fairly routine, and more than likely the valve would be closed and we would be cleared by cardiology. I remember the echocardiogram taking a very long time! Then, they put us in a room to wait. When the door opened, the Chief Attending Cardiologist and the Attending came in to tell us that Emma had pulmonary hypertension. It was causing the valve to enlarge, and it was putting pressure on the lungs. They would need to do surgery to see if the pressure could be controlled. If the pressure could not be controlled, then she would not be a candidate for a transplant. Our world was spinning! We were thinking, "There is no way this is happening to our baby." We are both healthy people. "How much can this little girl go through?" Emma went into surgery on December 10. She had a cardiac catheterization, and they were administering different meds to see what would control the pressure. We were told the procedure would take 5-6 hours. After being in surgery for 2 hours they called to tell us she was going to recovery. The surgery was complete. The first med that they tried, oxygen, worked. We were so relieved and overjoyed. Emma pulled through like a champ! She was sent home on oxygen.

Emma was listed for transplant on January 21, 2004. She has also graduated from oxygen. She is a tough little girl, and her strength and smiles are what get us through. We are so blessed to have Emma in our lives! It has been a journey full of hills, valleys, and wonderful moments with our daughter. She knows no strangers, and we learn things from her everyday about the wonderful innocence that children bring to our world.

Our daughter, Emma Marie, was born in August 2003. She was a beautiful baby with a full head of black hair (much to our surprise...we both are fair). She weighed 6 lbs. 10 oz. and she appeared to be very healthy. The second night in the hospital, Emma's blood sugar dropped dangerously low. It is unknown why this happened, but we thank the Lord for the nurse who was on call that night for noticing Emma's lethargy and mottled skin. She was given formula supplement and sugar water, and was back to normal within 12 hours. It never happened again.

At 3 weeks of age, Emma began to look jaundiced. We remember thinking, "Oh no, we're going to have to go to the hospital and spend the weekend under the bili lights. We have so much to do...there is no way that we can take time out of our schedule to spend a weekend in the hospital!" If only we knew of the road that our precious angel would lead us on.

At 6 weeks of age, we were sent to Denver Children's Hospital. After many tests and a liver biopsy, Emma was diagnosed with biliary atresia. Her Kasai was done at 7 1/2 weeks. The surgery appeared to be very successful, at first! She had bile flow in the hospital, and we were so happy. However, after the first two days, the bile flow decreased. We were finally able to go home after 5 1/2 weeks in Denver.

In December, we went back to Denver for Emma to be evaluated for liver transplant. During the evaluation, she was scheduled to see the cardiologist (a valve in Emma's heart did not close when she was born). They said this happens in about 50% of babies, however it usually closes within the first 3 months. We were told it would be fairly routine, and more than likely the valve would be closed and we would be cleared by cardiology. I remember the echocardiogram taking a very long time! Then, they put us in a room to wait. When the door opened, the Chief Attending Cardiologist and the Attending came in to tell us that Emma had pulmonary hypertension. It was causing the valve to enlarge, and it was putting pressure on the lungs. They would need to do surgery to see if the pressure could be controlled. If the pressure could not be controlled, then she would not be a candidate for a transplant. Our world was spinning! We were thinking, "There is no way this is happening to our baby." We are both healthy people. "How much can this little girl go through?" Emma went into surgery on December 10. She had a cardiac catheterization, and they were administering different meds to see what would control the pressure. We were told the procedure would take 5-6 hours. After being in surgery for 2 hours they called to tell us she was going to recovery. The surgery was complete. The first med that they tried, oxygen, worked. We were so relieved and overjoyed. Emma pulled through like a champ! She was sent home on oxygen.

Emma was listed for transplant on January 21, 2004. She has also graduated from oxygen. She is a tough little girl, and her strength and smiles are what get us through. We are so blessed to have Emma in our lives! It has been a journey full of hills, valleys, and wonderful moments with our daughter. She knows no strangers, and we learn things from her everyday about the wonderful innocence that children bring to our world.

Our daughter, Emma Marie, was born in August 2003. She was a beautiful baby with a full head of black hair (much to our surprise...we both are fair). She weighed 6 lbs. 10 oz. and she appeared to be very healthy. The second night in the hospital, Emma's blood sugar dropped dangerously low. It is unknown why this happened, but we thank the Lord for the nurse who was on call that night for noticing Emma's lethargy and mottled skin. She was given formula supplement and sugar water, and was back to normal within 12 hours. It never happened again.

At 3 weeks of age, Emma began to look jaundiced. We remember thinking, "Oh no, we're going to have to go to the hospital and spend the weekend under the bili lights. We have so much to do...there is no way that we can take time out of our schedule to spend a weekend in the hospital!" If only we knew of the road that our precious angel would lead us on.

At 6 weeks of age, we were sent to Denver Children's Hospital. After many tests and a liver biopsy, Emma was diagnosed with biliary atresia. Her Kasai was done at 7 1/2 weeks. The surgery appeared to be very successful, at first! She had bile flow in the hospital, and we were so happy. However, after the first two days, the bile flow decreased. We were finally able to go home after 5 1/2 weeks in Denver.

In December, we went back to Denver for Emma to be evaluated for liver transplant. During the evaluation, she was scheduled to see the cardiologist (a valve in Emma's heart did not close when she was born). They said this happens in about 50% of babies, however it usually closes within the first 3 months. We were told it would be fairly routine, and more than likely the valve would be closed and we would be cleared by cardiology. I remember the echocardiogram taking a very long time! Then, they put us in a room to wait. When the door opened, the Chief Attending Cardiologist and the Attending came in to tell us that Emma had pulmonary hypertension. It was causing the valve to enlarge, and it was putting pressure on the lungs. They would need to do surgery to see if the pressure could be controlled. If the pressure could not be controlled, then she would not be a candidate for a transplant. Our world was spinning! We were thinking, "There is no way this is happening to our baby." We are both healthy people. "How much can this little girl go through?" Emma went into surgery on December 10. She had a cardiac catheterization, and they were administering different meds to see what would control the pressure. We were told the procedure would take 5-6 hours. After being in surgery for 2 hours they called to tell us she was going to recovery. The surgery was complete. The first med that they tried, oxygen, worked. We were so relieved and overjoyed. Emma pulled through like a champ! She was sent home on oxygen.

Emma was listed for transplant on January 21, 2004. She has also graduated from oxygen. She is a tough little girl, and her strength and smiles are what get us through. We are so blessed to have Emma in our lives! It has been a journey full of hills, valleys, and wonderful moments with our daughter. She knows no strangers, and we learn things from her everyday about the wonderful innocence that children bring to our world.

Emma H

Emma H

by her mom, Robyn

Our daughter was born in July 2002 -- one of the greatest moments of our lives. While we were still in the hospital I noticed she was jaundiced. My regular pediatrician ordered lab work and 1 hour before we were supposed to go home, we were told that the "wrong" bilirubin (the direct) was high.

We then had countless follow ups and tests and new doctors and emotional ups and downs. The day of her ultrasound at 2 weeks, when the tech couldn't find her gallbladder, I decided to do some research on the Internet. It was there I learned about biliary atresia. One journal article I read stated "some children even make it to their 30's"! Of course I panicked and called my pediatrician. He told me to wait and talk to the GI and he was right. The GI and the surgeon both gave us some hope that Emma might have a long life -- not an easy one for sure, especially at first.

Her Kasai was on August 28, when she was 6 weeks old. Her total bilirubin had gone down from 6.9 in the hospital after surgery to 3.9. A setback on October 12 (probably cholangitis) resulted in a week's stay at the hospital. Her bili went from 5.4 to 4.6 during that time. The surgeon has said he usually gives 3 months post Kasai before he says whether the procedure has worked or not.

Emma is the most wonderful baby. She has an easy smile for everyone especially her worshipful, 4-year-old big brother, Jake. She hardly fusses, hardly seems sick, plays and coos and acts like any baby should. She's got big, bright eyes that seem to take in everything. We are the luckiest family in the world to have this baby.

Eric

Eric

by his mother, Desiree

Eric was born in January 2001. He was 2 weeks overdue just like his sister and brother before him. He adores his 3 sisters, Audra, 16, Emily, 13, Hannah, 7, and a brother Adrien, 3. Eric was born just as beautiful and healthy looking as ever but was sent home with jaundice. His pediatrician was concerned. At 4 weeks old she ordered liver function tests and referred him to a GI at the children's hospital in a neighboring city. After 2 weeks of tests, prayers, tears, and more tests, we were informed that Eric had biliary atresia. His gall bladder had shriveled to a minute scar tissue. I just kept waiting to hear that the tests showed some bile drainage, but with every test, his condition was verified. It was overwhelming, but I had to act strong for my other children. I released my pain with a lot of secret late-night crying.

At 6 weeks old Eric was scheduled for a Kasai procedure. He was dedicated in our church the night before, and the priest came to our home the morning of surgery to baptize him. At this point I had a lot of faith that everything was going to be all right for my little man of God.

Eric came out of surgery without a blood transfusion and recuperated with flying colors. After just 5 days we were home and I thought Eric was going to be fine for the rest of his life. We did some “plumbing” and now he was “fixed”…Right?

At Eric’s follow up visit the surgeon apologized that Eric’s Kasai did not work and his GI would refer us to the liver transplant center in Cincinnati, Ohio. However, after giving it 2 more weeks Eric's bilirubin levels had gone down! They continue to do so.

After 6 months of doing great, Eric started having unexplained fevers and emergency room visits. Every month he has been spending about a week in the hospital and going home on IV antibiotics. He has a MediPort now. He likes to comfort himself by sucking on 2 fingers and gently patting his MediPort with his other hand.

Eric spent his first Christmas in the hospital, but with the love and support of good friends and family, it was an unforgettable holiday that brought us all very close. I don’t know what the future holds for Eric’s condition. I now realize that we have a long haul ahead of us. It’s a new way of life, but with God's grace, dedicated doctors and nurses, the love and prayers of good friends and family, and the support of all you good people here, I just know we’re going to get stronger. Eric is so very blessed, as is true with all the other precious children here.

These children are miracles and what we are going through is a miracle in disguise.

Erin

Erin

Erin is 10 years old, in fifth grade, and recently celebrated her first transplant anniversary. Congratulations Erin! On April 21, 1998, she received part of her father’s liver at Children’s Memorial Hospital Medical Center in Cincinnati. Erin was born with biliary atresia and later developed hepatopulmonary syndrome. Erin loves animals and wants to be a marine biologist or veterinarian when she grows up. Her favorite subjects in school are history, reading, and science. She is her mom's best friend and an inspiration to everyone who knows her.

Germana

Germana

Hi there!

My name is Germana -- Geri for short. I was born with a liver disorder called biliary atresia. I am on medications, but I did not need a transplant. I go to the University of Minnesota for my check-ups.

I am 14 years old and in 10th grade at CHHS. I like my school. I have lots of nice friends, but I would like to be in touch with kids who have liver problems like me.

Hannah H

Hannah H

Hannah, 2, was born with biliary atresia. Her Kasai was unsuccessful and she needed a transplant by the time she was just a few months old. She is now 1½ years post-transplant and doing well. Her mom, Kim, says "Yes, there really is life after a transplant!" Hannah and her family just took their first vacation since Hannah was born and had a wonderful time.

Hannah S

Hannah S

by her father, Terry

UPDATE!

In March, 2000, our precious first child, Hannah Rae, was born at Shannon Medical Center in San Angelo, Texas. Hannah started out jaundiced, but we were told this was not out of the ordinary and to just try to put her in the sunlight. At Hannah's two-month checkup questions arose as to why she was still jaundiced. A few days later these questions were answered at Dallas Children's Hospital in Dallas, Texas.

Upon arrival in Dallas, a week-long series of tests eventually resulted in a tentative diagnosis of biliary atresia. On May 15, 2000, at 9½ weeks of age, Hannah's Kasai procedure was performed. The operation lasted approximately 4 hours. The first part of the surgery was used to perform an additional test that would 100 percent confirm Hannah's diagnosis.

After the Kasai, my wife and I were in no way prepared to see our daughter in the critical state that she began her recovery. We began to realize the battle that lay ahead. Later that evening, after Hannah was released from the recovery room, she began having complications from the surgery and was placed in ICU under critical condition. There was a period of uncertainty for a few hours because no one seemed to know exactly what the problem was, other than there was unusual swelling and her body temperature was low. The doctors immediately started a wide spectrum antibiotic because of their fears of infection or some kind of virus. A respirator was also put into place because the swelling was so great that Hannah was having shallow breathing. Three days later Hannah awoke and began to become more alert. On May 21, 2000, Hannah was released from Intensive Care and placed on the GI floor.

Hannah gradually recovered and was released from the hospital three weeks after she left the ICU with the unfortunate news that her Kasai was not successful. Hannah has since been hospitalized twice with bouts of cholangitis and in August of 2000 she was placed on the liver transplant waiting list at status 2B.

Since Hannah's Kasai, our main goal has been to keep her as healthy as possible so that she will continue to be a good candidate for a liver transplant. This has been challenging at times with the upkeep of a central line, feeding tube, and all the medications she must take daily. I'm pleased to note that at Hannah's last checkup her doctor proclaimed that Hannah was healthier than he had ever seen her and that she is still an excellent candidate for a liver transplant.

In the meantime, we would like to thank the Lord for watching over our little girl and listening to our prayers. In addition, I'm also very thankful for all of those who have supported and sent prayers throughout this ordeal, including our families, friends, and the professionals at Dallas Children's Hospital. The enormous support that we have received continues to keep us positive and looking toward the future.

UPDATE: We are pleased to announce that in February 2001, Hannah received her new liver. The surgery lasted approximately nine hours and she spent four days in intensive care. Thus far, Hannah has only been hospitalized once with a bout of rejection which was treated with a round of steroids. According to Hannah's transplant team she is having an excellent post transplant follow-up. At home, Hannah is much more active and has recently begun to crawl, proving that life really can be normal after a liver transplant. We are very thankful for all of our supporters and their prayers. We would also like to thank the Lord for this special gift.

Havalah

Havalah

by her mother, Kristen

Havalah was born in June 2003. I had gestational diabetes so we were surprised when she was only 5lbs 6oz. At the hospital they told us her bili was a little bit high but not enough that she needed treatment. Right from the start we had a lot of doctor appointments. The day after we left the hospital we had an appointment at Children's for an ultrasound because her kidneys were shown to be echogenic while she was in utero. Also we had an appointment with the pediatrician who wanted to keep an eye on her because of her low birth weight. The first couple weeks they kept monitoring her bilirubin. The total was going down quickly but the conjugated was barely going down at all. They kept running tests and nothing really was turning up. They were also concerned because her weight gain was slow. She seemed so healthy and responsive that I wasn't worried and was more concerned about putting her through so many tests. Right from the start her stools were very light in color but being a new mom I didn't realize. All the books said yellow... and hers were, just very light yellow. After our pediatrician saw one by chance she referred us to a GI doctor.

We first saw him at about 4.5 weeks and had a HIDA scan and more blood work a few days later. A couple days after that they called me and told me either biliary atresia or Alagille syndrome. I flipped when I looked them up online and found out how serious they were. Next came a biopsy. Then on July 29th she had the Kasai at just under 6 weeks. After surgery her bilirubin went up from 3.5 total/1.9 conjugated to approximately 6 total/3.2 conjugated and her other liver numbers went up a bit as well. She recovered from the surgery fairly quickly and had a few dark colored stools, but then they turned back to light or varied. We almost went home after 10 days but her WBC went up and they put her on triple IV antibiotics and more steroids. We finally went home about 10 days after that.

Since then her numbers have all stayed stable but haven't gone down so they didn't call the Kasai a success and started talking to us about transplants back in October. Otherwise life went back to normal. Doctor visits got further apart and Havalah has continued to grow slowly and seems normal developmentally. She hasn't had any fevers or obvious health problems but continues to be jaundiced. In early January her lab numbers all went up, her bili went to 8 and then 12 and her pt went from 12/13 to 18 but has since went down with vitamin K. Her other liver numbers are creeping up as well and she was added to the liver waiting list on January 23, 2004. There is no one in our region waiting ahead of her and we anticipate the call any time now. I'm very nervous/scared about the surgery but optimistic about her future in general.

Havalah is a beautiful smiley baby who seems to charm everyone she comes in contact with. Other than her jaundice you wouldn't suspect anything is wrong with her. Despite her disease she is the best thing that's ever happened to me and I love being a mom more than I ever imagined I would.

We are located in Milwaukee Wisconsin and are interested in meeting other families in similar situations or communicating with people online.

Hope

Hope

by her dad, John

Hope was born in January, 1991, five weeks premature. She was very jaundiced and had a enlarged liver. Just seven weeks old, Hope required a Kasai. She was transported to the IWK Children's Hospital in Halifax, Nova Scotia. After 7 1/2 hours the procedure was complete then she spent 12 hours in intensive care. The Kasai was very successful. By the next day she lost the yellowish completion. We took Hope home a few days after the surgery. Hope suffered several infections in the bile tracks until she was about one year old. At first she required to see her doctor every six months, then every year. But in July, 2000, her doctor didn't even ask for liver function tests and told her to come back in two years.

Hope has no brothers or sisters. She is going into grade 5, she is very active in dance, skating, biking, and swimming. She loves playing with her dog Peanuts and going to movies. Hope would like to thank the doctors & nurses who performed her Kasai. She would also like to say hi and wish the best to all the CLASS kids.

end faq