Other Liver Diseases

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Aiden H.

Aiden H.

Aiden’s story begins in August 2004. He came to us a tiny 4 lbs 8 oz and as sweet as a button. Aiden was born with Gastroschisis, an abdominal wall defect that causes the intestines to remain outside of the body. We knew about the defect from our 17th week ultrasound, but what we did not know was that this was not going to be Aiden’s only battle to stay alive.

As soon as he was stable we were transported to the Children’s Hospital next door. He had his corrective surgery six days later. The surgery went well and his bowels seemed to be in pretty good shape, all we had to do was wait for them to regain function. He was put on total parenteral nutrition (TPN) in the meantime to keep him alive. We were warned about the effects of TPN on the liver but no one thought that he would be on it that long.

He became jaundiced after a few days but it was over looked because of the TPN. Aiden showed us that he was a fighter right from the beginning, enduring endless infections from his lines, a nasty systemic yeast infection and even a small bout of Necrotizing Enterocolitis (NEC). Our next nightmare began shortly after Aiden began to eat (at 3 months old). Aiden finally began to pass stool, but all everyone was talking about was how pale and stinky they were. We had a meeting with our doctors and it was decided that Aiden must have sludge in his bile ducts from the TPN. So, off he went to his second surgery on December 4th to try to flush his gallbladder and take a liver biopsy. The attempt failed because his gallbladder was collapsed so they did the biopsy and closed. His liver was green and the biopsy came back showing some scarring and changes consistent with TPN cholestasis. A few days after the operation, Aiden began to vomit bile. Although this put him back on TPN and off food, everyone seemed to be happy to see that his bile was flowing (even if it was the wrong way). They decided that he had a blockage and that it must have cleared itself.

Two weeks later, we were given the “OK” to begin eating again and after that first poop came (still as pale as ever), the testing began again. We ruled out Cystic Fibrosis, Biliary Atresia, Progressive Familial Intrahepatic Cholestasis (PFIC), and Alagille Syndrome(AGS). We had our first meeting with the gastroenterologist and she decided that it was a choledochal cyst. We had surgery two weeks later to remove the cyst but like the rest of our journey, it did not go as planned. The surgeon came to us after 3 hours in the operating room and sat us down to talk. I will never forget his words; “Sorry, it doesn’t seem to be what we thought, in fact, I’ve never seen anything quite like this.” So, Aiden was closed up and sent for two more tests; a cholangiogram, and an MRI.

Both tests revealed dilated but grossly normal bile ducts except for the common bile duct, which seemed to narrow and almost disappear right beneath his gallbladder. We went home for a few weeks after that while they decided what to do with our new situation. They decided that a Kasai would be our best option, but they could not tell us what would happen with the liver disease after that. Would it get worse and require a transplant? Or would it stabilize?

Aiden had his Kasai on March 5, 2004, and recovered well. He is now 7 months old and weight 10 pounds. We are home now, and watching him closely for signs of any problems. Our biggest worry now is getting a bowel obstruction due to adhesions from his four previous abdominal surgeries. We see our doctors weekly for now and are hoping to see his LFT’s fall soon.

I fell in love with all the kids here and we are so happy to be welcomed as a part of the family. If ever things get rough, I know that we can come here to find support.

Here’s hoping for a hospital-free 2004!!

Aiden’s story begins in August 2004. He came to us a tiny 4 lbs 8 oz and as sweet as a button. Aiden was born with Gastroschisis, an abdominal wall defect that causes the intestines to remain outside of the body. We knew about the defect from our 17th week ultrasound, but what we did not know was that this was not going to be Aiden’s only battle to stay alive.

As soon as he was stable we were transported to the Children’s Hospital next door. He had his corrective surgery six days later. The surgery went well and his bowels seemed to be in pretty good shape, all we had to do was wait for them to regain function. He was put on total parenteral nutrition (TPN) in the meantime to keep him alive. We were warned about the effects of TPN on the liver but no one thought that he would be on it that long.

He became jaundiced after a few days but it was over looked because of the TPN. Aiden showed us that he was a fighter right from the beginning, enduring endless infections from his lines, a nasty systemic yeast infection and even a small bout of Necrotizing Enterocolitis (NEC). Our next nightmare began shortly after Aiden began to eat (at 3 months old). Aiden finally began to pass stool, but all everyone was talking about was how pale and stinky they were. We had a meeting with our doctors and it was decided that Aiden must have sludge in his bile ducts from the TPN. So, off he went to his second surgery on December 4th to try to flush his gallbladder and take a liver biopsy. The attempt failed because his gallbladder was collapsed so they did the biopsy and closed. His liver was green and the biopsy came back showing some scarring and changes consistent with TPN cholestasis. A few days after the operation, Aiden began to vomit bile. Although this put him back on TPN and off food, everyone seemed to be happy to see that his bile was flowing (even if it was the wrong way). They decided that he had a blockage and that it must have cleared itself.

Two weeks later, we were given the “OK” to begin eating again and after that first poop came (still as pale as ever), the testing began again. We ruled out Cystic Fibrosis, Biliary Atresia, Progressive Familial Intrahepatic Cholestasis (PFIC), and Alagille Syndrome(AGS). We had our first meeting with the gastroenterologist and she decided that it was a choledochal cyst. We had surgery two weeks later to remove the cyst but like the rest of our journey, it did not go as planned. The surgeon came to us after 3 hours in the operating room and sat us down to talk. I will never forget his words; “Sorry, it doesn’t seem to be what we thought, in fact, I’ve never seen anything quite like this.” So, Aiden was closed up and sent for two more tests; a cholangiogram, and an MRI.

Both tests revealed dilated but grossly normal bile ducts except for the common bile duct, which seemed to narrow and almost disappear right beneath his gallbladder. We went home for a few weeks after that while they decided what to do with our new situation. They decided that a Kasai would be our best option, but they could not tell us what would happen with the liver disease after that. Would it get worse and require a transplant? Or would it stabilize?

Aiden had his Kasai on March 5, 2004, and recovered well. He is now 7 months old and weight 10 pounds. We are home now, and watching him closely for signs of any problems. Our biggest worry now is getting a bowel obstruction due to adhesions from his four previous abdominal surgeries. We see our doctors weekly for now and are hoping to see his LFT’s fall soon.

I fell in love with all the kids here and we are so happy to be welcomed as a part of the family. If ever things get rough, I know that we can come here to find support.

Amy

Amy

By her mother, Pam

One young girl, four transplants: Amy’s Amazing Story

Think back to the days when you were just about to start school, with kindergarten or perhaps first grade, right around the corner. Remember the anxious, butterflies-in-the stomach feelings you had, worrying and wondering if you’d like your new school, your teacher and your classmates. Now imagine, on top of that, you are just about to start school, you are only five years old, and you’ve just found out that you need a liver transplant to survive.

That’s exactly when Amy of Lenoir found out she needed a liver transplant. She had been given medication to treat a disease, which caused her liver failure. Because she needed a transplant desperately, she was flown to the University of Chicago, where she was placed on a liver transplant waiting list. Her family was told that she had just days to live. Thankfully, Amy and her family found support from other kids and their families at the hospital and Ronald McDonald House who were all facing the same thing – waiting for transplant to save their children’s lives. After staying in the hospital for three months, Amy received a life-saving liver transplant in March 1990.

Because she was so sick, she does not remember much of the transplant or how she felt afterwards. She does, however, have great memories of her sixth birthday party, celebrated at home a few weeks late because on her real birthday she was still in Chicago recuperating from her liver transplant surgery. Back home in Lenoir, she and a dozen playmates played hard, opened gifts and blew out candles in front of her thankful parents and other relatives. “She had been fussing in the hospital because she hadn’t had her birthday party yet,” said Amy’s mother, Pam. “That’s when we knew she was getting back to normal.”

Her daughter’s liver, Pam learned, had come from a 10-year-old boy who had been killed in a car accident. A few years after her transplant, Amy wrote the donor family a letter, thanking them and telling them about her seven-year-old’s life.

The liver transplant kept Amy going strong for almost ten years. She was able to attend most of elementary school, where Amy was very up front with her classmates, insisting that they not treat her any differently because of what she went through, "I don’t want that at all,” said Amy, who is fiercely independent.

There were only a few things Amy couldn’t do, like play certain types of sports, for fear of damaging her new liver. But, for the most part, Amy’s childhood was normal, although she had quite a few more doctors’ appointments and daily medications than most kids have growing up.

When Amy turned 15, her health took a turn for the worse, and Amy found herself in liver failure once more. “My biggest fear always was having to go through the surgery again, and whether my life will ever be the same afterwards,” said Amy.

Amy received her second liver transplant in February, but it failed almost right away. She was quickly re-listed, needing another liver transplant to save her life. “After the second transplant failed, I was so tired, so sick and so frustrated,” she said. “I knew I needed another transplant, but I didn’t know if one would come for me.”

Amy was very lucky. Just one month later, she got her third liver transplant. Amy’s remarkable transplant story doesn’t end after her third transplant. When she was 17 years old, her kidneys began to fail, due to the medications she had been taking since her first liver transplant 12 years before. She had to go on dialysis for four hours a day, three days each week.

“At first, I couldn’t believe I needed to go on dialysis. I just kept putting it off and off until one day I just couldn’t put it off any more. My feet and hands would swell each day, and I cried every day because I wanted to do things with my friends, but instead, I was hooked up to the dialysis machine,” she said. She continued dialysis for six months, waiting for a kidney transplant. Her kidney transplant came in April when she was in the 12th grade, and she made it home from the hospital on the day of her high school prom. The most moving moment, though, came for Amy when she was able to walk with her high school graduating class to receive her diploma and her admiring classmates, their friends and families gave her a standing ovation.

When asked how she made it through the roller coaster of waiting and praying for, and then receiving four different life-saving organ transplants, Amy attributes her survival to her stubbornness and determination. Her mother echoes that sentiment, but still worries a lot “especially because she is a teenager, but her friends have been so good, so supportive, understanding when she is too tired to go out, but always making sure that she stays a part of their lives,” said Pam, her mother.

This past summer, Amy volunteered with the National Kidney Foundation of North Carolina’s Kidney Kamp, a week-long camp for kids ages 8-17 who are either on dialysis or have received transplants. While there, she found that she really enjoys working with people who are faced with needing a transplant, an experience she knows all too well. “I encourage them not to feel afraid or alone,” said Amy, who has plenty of experience and wisdom to share.

For Amy, who has been through more than most people twice or three times her age, the future looks bright. Her next goal is to get a college degree and work as an elementary school teacher. “At Kidney Kamp, I realized my calling is to work with people, especially children,” she said. After four transplant miracles, Amy certainly knows that anything is possible, and that nothing can stop her from pursuing her dreams.

Anna Marie

Anna Marie

By her mother, Robin

Anna was born May 2002. She was healthy and showed no signs of liver disease. By five months of age she started developing symptoms of itching, yellow eyes, light colored stools and unexplained fevers. We went to a GI Specialist at Cooks Children's Hospital in Ft Worth. Many tests were run and while it was clear the liver was having problems nothing was able to be diagnosed. We were referred to a second specialist at Dallas Childrens Hospital. He told us he thought it was PFIC II but that they could not confirm it.

After much internet research I found that there was a new test that could diagnose her, available only at Kings College Hospital in London. After some discussion with our doctors, in May 2003 my husband flew Anna's biopsy slides to London to test for PFIC II. In about a week it was confirmed that she had PFIC II. Now diagnosed, the decision was made to go to Mt Sinai Hospital in NY and try a new procedure. This surgery would internally re-route part of the small intestine to try get some of the bile acid off the liver. Our hope is that it would slow down or stop the disease and relieve the itching/puritus. The surgery was done in July 2003 .

That surgery was one of the first to try to treat her disease. PFIC II is very rare and other than transplant not a lot has been tried in the way of treatment. The surgery was not as successful as we hoped and by September it was clear we were going to need a liver transplant. The puritus was extremely disruptive and quality of life was now becoming an issue. In September 2003, we went to Texas Children's Hospital in Houston and got on the waiting list for a liver transplant. In December our prayers were answered and in December 2003 at 17 months old, Anna received her liver transplant. Five days later Anna was released from the hospital. She no longer itched, and her scratch marks were all healing. She was up dancing and running around. It was incredible! We stayed in Houston another month to adjust medication doses and do blood work. The first of February, we returned home.

In December 2004 we celebrated Anna's one year transplant anniversary with a party and by giving out 250 organ donor cards and green ribbon awareness pins. I am thankful for all the people we met along the way and for the encouragement and support they provided. PFIC II is a genetic disease that neither of our family's had a history of. It took a lot of of time and a lot help to get through the maze of insurance, medical research, and doctors. We could not have done it without all the people who went out of their way to help us. Anna is now turning three years old. She still goes in for regular lab work but, overall she is doing extremely well. Our only problem now is trying to keep up with her. She is now a healthy, active, three year old.

Ashley O.

Ashley O.

By her mother, Dina

My daughter is Ashley and she is 5 years old. Ashley was born with ASA (argininosuccinic aciduria). We found out 7 days into our new world with her. That morning she wouldn’t have any formula. She had the hiccups and kept crying. Her body temperature was low so we went to her doctor who in turn sent us to our local hospital. They were trying everything they could do. When they knew what the problem was they told us that they had to send her up to Boston Children because our local hospital didn’t, at the time, do kidney transfusions. She was in the hospital for 14 days in Boston and they were able to figure out that she had ASA Ashley inherited it from my husband and me. Our missing genes matched, causing this rare condition.

Then as Ashley was just a little over 1 year old, she had reflux and she would no longer drink her milk or hold it down in her stomach. So at the time, she had a Mickey Button put in for feeding. She had some really funky tasting formulas she didn’t like the taste of them.

When she went in for her monthly check, they noticed her liver was very large so we went and met with the specialist and found out about transplants. So, in 2003, she was put on the transplant list and she received a split liver transplant in June 2003. Ashley is mentally behind and physically behind. She is graded at a 27 to 30 month old child when she is 5 ½ years old.

Since her transplant she has had pneumonia and one rejection, all within a month after her transplant.

Ashley made a wish to go to Disney in June 2004.

Chantelle

Chantelle

By her parents

Chantelle was born on July 15th 2000. She seemed perfectly healthy at birth, with no complications or jaundice. For the first year of Channy’s life, she suffered through constant fevers, double ear infections and she scratched herself all the time. When she was a year, she had ear tubes put in and we hoped this would solve all of her issues.

At 15 months of age, after having numerous nosebleeds random seizures and a spell of “mini strokes”, her pediatrician sent her for extensive blood-work and a follow up with a gastroenterologist at Phoenix Childrens. At this point, we found that her bile acid levels were extremely high and that her liver, gallbladder and spleen were enlarged. She was admitted to Childrens for her first open biopsy and they determined that she had a massive amount of cirrhosis on her liver. She was very ill at this point in time and had been refusing to eat, we were informed that her doctors had not seen her type of illness before and that she might not live to see her second birthday.

After a week in the hospital, she had a feeding tube put in, she was put on a 3x’s daily dose of Actigall and was sent home. Well, she hated the ng tube enough that she slowly began eating at home, and gradually began to regain strength. She did have another open biopsy at Cincinnati Childrens 3 months later, and they also concluded that her disease was very rare, and could not give us a diagnosis.

Chantelle has had her ups and downs over the past seven years, she has exhibited symptoms of MSUD with any respiratory illness (which has her doctors baffled, as MSUD is diagnosed in infancy or if not leads to severe mental retardation), she has had some vision issues which they believe is a factor in the disease, but aside from a few downs a year we have mostly ups! Chantelle is a Girl Scout, she loves to ride her horse friend Captain, and she is a number one fan of anything Hannah Montana. Channy loves life, and can be found any given day of the week surrounded by friends while she practices the monkey bars! Chantelle has been a blessing to all those in her family and we pray that one day we will have either a diagnosis for her, or the good news that she is disease free.

Nathan and Heidi Goding (proud parents)

Alec and Braeden Goding (best brothers ever)

Christine

Christine

I am currently 18 years old and just finished my freshman year in college. I am studying nursing. So far I have only attended a junior college but I'm hoping to transfer to a University somewhere in the states (Hawaii would be nice). My ultimate dream is to work in the Pediatric Transplant ward at LSPCH with my old doctors (they need someone to put them in their place and I nominate myself!).

I was born with hemangioendotheliomas (I think that's how it's spelled - now there's a National Spelling Bee word for you!) and had my transplant when I was 15 years old. I've been problem-free for almost 4 years (health-wise anyway, I was born to make trouble).

I enjoy doing all sorts of different things like driving around, river rafting, swimming, fishing, dancing, going out, just doing the normal teenage things; breaking curfew, getting into trouble, and doing things the docs tell me not to (my tattoo is the PERFECT example). I also enjoy reading (a lot), and listening to different, but fun kinds of music (although not a lot is allowed on radio stations, especially my favorite song). I am also ALWAYS open to new fun and exciting opportunities, which makes for a fun time all the time.

Connor

Connor

Connor, age 3, was born premature and needed surgery to correct reflux at 5 weeks old. He developed scar tissue 2 months later that caused an obstructed bowel and then needed much of his small bowel removed. He experienced many complications after this second surgery and soon developed TPN induced liver damage that was irreversible. At 10 months of age Connor received a liver transplant. His mom writes that he may not have lived if he'd had to wait for a donated liver. His parents' blood types were not compatible with his, so his maternal aunt became his liver donor. He has done very well since his transplant. Fortunately, he has had no signs of rejection, although he has had difficulty with the short gut syndrome. He loves being with other children and is a very happy child.

Devin

Devin

By his mother, Vicky

My son, Devin, had a liver transplant at UCLA in 1992. He was 11 months old. He had what they called "fulminate liver failure," which basically meant its onslaught was sudden and severe and its origin unknown. It all happened so fast that I really had no idea what was going on. From one moment to the next, my son had gone from being healthy to being in ICU having just undergone a life-saving liver transplant. He came home two months later and I was left with many unanswered questions and a need to give back something to someone. I tried as best as I knew how to get the word out there about the unbelievable shortage of organs and the success of transplants. I began speaking for the American Liver Foundation and for the San Diego Transplant Association. As much as I hated being in the spotlight and speaking in front of a lot of people, I did it for the cause and it wasn't so awful after the first 10 times.

Then one day, my sister (who is a high school teacher) suggested I speak to the health classes at her school. I agreed. Then she added the Drivers Ed classes and before she or I knew it, several teachers wanted to set up an assembly to hear me speak. I contacted the San Diego Transplant Association who provided more speakers and plenty of handouts. This became a yearly thing for us to do. We did it for 4 years until the San Diego Transplant Association fizzled out of existence. By then I felt I had accomplished what I had set out to do: have one person donate their organs because I had informed them of that choice.

A 15-year-old boy had been to the assembly on one of those days and later told his parents that he had heard an "emotional discussion about organ donation" and should something happen to him, he wished to donate all of his organs. Unbeknownst to any of us, he was declared brain dead three months later after a skate boarding accident. He donated all of his organs. I know that his liver was split between a woman in her 20s and an 18-month-old baby. I think it was on that day that I finally had closure.

I may have had personal closure on that day, but my need to "give back" has never left me. In every possible social setting I try to educate people without embarrassing Devin. I've spoken to many different classrooms and schools. You don't need a relative who is a teacher to get the ball rolling. My suggestion is that if you are able to speak publicly, write to your local high schools or junior high schools and attach your personal story and see if you can get an assembly set up. Your kids all go to school, don't they? That is your "in." Talk to their teachers and the principal. Bring your child (if her or she will allow it) or bring pictures. It always amazes people that these transplant kids look so normal. You would be surprised at how willing most of these youngsters are. They haven't heard all the stories out there and are very open minded. If you need handouts your local organ procurement organization will be more than happy to supply what you need.

The heroes in this story are my sister, Vivian, Lance (donor), Devin's donor family and anyone who promotes organ donation! Go out there and get it done! I know you can and you never know when you'll need to call upon your karma again!

Erin B.

Erin B.

by her mother, Irene

Erin was born in June 2001. When she was 5 weeks old we were told she was in kidney failure and was going to die. We prayed to God and every Saint for anything but death.

A couple of days later God sent us our miracle worker, Dr. Seth Schulman. Dr. Schulman explained Erin had an extremely rare genetic disease called Primary Hyperoxaluria Type 1 (Type 1 being the worse). The last time he saw this disease was in the 1970's and it had a catastrophic ending. He explained Erin would need both a new liver and a new kidney. Until she was transplanted she would need dialysis 5-7 days a week, 10-12 hours a day. Waiting for two organs could take years and in all likelihood may not happen in time for her. It was our "anything but" we had asked God for. We could do this!

In the months that followed, Erin's kidney function miraculously improved. It became apparent that as long as a liver came quickly she would not need a kidney transplant.

Thanks to the wonderful transplant team at CHOP, Erin got her liver in April 2002. She was 10 months old. Most importantly, thanks must go to Erin's donor family. They have changed our lives and touched us in a way that nothing can compare to.

Erin has been doing fantastic ever since her transplant. She loves to play with her older brother, Matthew, and older sister, Megan. They absolutely adore her as does everyone who meets her. Her smile and happiness are infectious. I think she is going to be a clown -- she loves to make everyone laugh with her crazy antics.

Gilbert

Gilbert

By his mom, Susan

This is my son Gilbert. He had a liver transplant in June of 2000 due to a very rare disease called Progressive Familial Intrahepatic Cholestasis (PFIC). It is a disease that causes retention of the bile salts resulting in cirrhosis. We went through a lot to get to this diagnosis, but in the end it all worked out.

Gilbert was born in September 1999, and weighed an amazing 9 pounds, 5 ounces. They warned us about the possibility of neonatal jaundice. The day after being discharged we were back at the hospital for photo-therapy. Although his bili level went down, they were concerned that his direct was still slightly elevated. We thought "no problem." We monitored it every week for about a month. It continued going down until month's end, then it doubled its original value. At that point, we were referred to a pediatric gastroenterologist.

Then we began a series of tests, but other than an elevated bili level, all his tests came back negative. His bili level kept increasing, so at 10 weeks they did a liver biopsy. His slides were read at Joe DiMaggio's Children's Hospital, University of Miami, and by a specialist in Chicago. The biopsy showed some fibrosis and some giant cell hepatitis, but our GI doctor told us not to worry. 'It is neonatal hepatitis, but it will eventually resolve itself." I believed him. After all, I was nurse and I trusted him as a colleague.

Gilbert's levels did not get any better. His liver was became firmer and his jaundice was getting more pronounced. We decided to get a second opinion. We went to Miami Children's Hospital, where the doctors were close to a diagnosis. Luckily I had connections at Massachusetts General in Boston and the Chief of pedi GI was gracious enough to review his file. Just by reviewing the file, he knew it was serious. We flew to Boston and in 2 days we had our diagnosis of PFIC. They told us that he would need a liver transplant. At that time Gilbert was 7 months old.

We flew back home saddened with our news. He was listed in April and in 2 months to the day, we had a liver. We were in the hospital for 20 days and had a wonderful recovery.

Now at 9 months post transplant, Gilbert is a happy and energetic 18-month-old. He loves to go to the park and to Gymboree classes. He also loves Blue's Clues and Barney. We are thankful to God and especially to our donor family. We pray every day that the Lord will watch over him always.

Jack

Jack

By his parents, Matt and Nicole

In October 2004, a beautiful baby boy named Jack was born in Ayr, Ontario. Shortly after Jack's birth, a nurse noticed that he had jaundice. A bilirubin test showed that his liver enzymes were quite high. Days turned into weeks, weeks turned into months and the situation had not improved.

Doctors said that one possibility was that Jack had biliary atresia. However, after exploratory surgery, we were told that Jack did not have biliary atresia, he had idiopathic neonatal hepatitis, there is no known cause.

By May 2005, with no cause or solution, we were told that Jack would require a liver transplant in order to survive. He was put on a transplant waiting list. Needless to say this was an emotional roller coaster for all of us.

Jack received is gift of life in June 2005 thanks to his angel donor!

Jacob

Jacob

By his mother, Ginger

Jacob was a healthy, normal child until he was 15 months old when he suddenly became jaundiced. He underwent many blood tests as his doctors tried to figure out what was wrong, but everything came back negative. When his condition continued to worsen, we were sent to University of Alabama Hospital at Birmingham. Five days after arriving, he was listed for a liver transplant.

Jacob was in liver failure due to an unknown hepatitis virus. He slipped into a coma and was moved to Surgical Intensive Care. He waited for a week at the top of the list for the region. We were losing him, but finally, a liver was found. He received a cut down liver from a 30-year-old male donor. Within four days he was out of the intensive care unit. Within two weeks he was released from the hospital.

Today, Jacob is four years old and doing great. His liver numbers have been perfect since the day he was transplanted and he has never had any rejection. There have been a few problems due to his being immunosuppressed, but overall, he is doing very well. Every day we realize how blessed we are by his presence here and we pray for his continued health.

Jacob R.

Jacob R.

By his mother, Alisha

My son Jacob is on TPN and is listed for a small bowel transplant. He has liver damage and has portal hypertension and elevated LFTs constantly. At this time it is not known if he will need a liver transplant or not.

Jacob lost his bowel at 1 month of age and has been on TPN since. He is a Millenium baby (January 2000). We tried to have him on January 1st but didn't work out that way. Jacob spent the first 5 months of his life in the hospital and has spent a total of over 12 months of his life in the hospital for staph or Klebsiella and yeast infections.

Jacob is a very happy little boy and enjoys spending time with his brothers Nicholas, 12, and Zachary, 4. He carries his TPN around on his back and does real well with it.

Jacob V.

Jacob V.

Jacob, age 2, was born with familial cholestasis syndrome. He’s had a rough start in life, but he is currently stable. He takes rifampin and Actigall and they seem to be working well for him. His itching is reduced, his weight has increased and he is the happiest his family has seen him in a long time. Jacob is a very bright little boy and a quick learner. He loves anything to do with Blue's Clues.

Jason

Jason

By his grandmother, Janet

Looking back at my grandson's newborn picture, I think he knew before we did that something was wrong. His head is tilted to one side with his hand on his head.

Jason was born in May 1998. He was a healthy baby boy weighing 7 lb. 12 oz. In three days all that changed. His mom (my daughter Julie) was breast feeding him and he was beginning to settle into a routine. He'd only been home a day and a half when he became fussy and didn't want to eat. I called the doctor and was told not to worry, newborns do that, and to just keep on trying to feed him.

By 5 o'clock that evening, a voice in my head was screaming to get Jason to the hospital. When we got there, they immediately started tests and for some reason a doctor on duty said to check his ammonia count. Jason's ammonia count was at 200, normal is 0-35. He laid there for a day until they decided to transfer him to another hospital. They took him to the University of California in San Francisco (UCSF) by ambulance. When he arrived at UCSF his ammonia level was at 500 (we later found out that a level of 1000 was lethal). By the time they got him on dialysis he had hit 1400.

When my daughter met with Dr. Philip Rosenthal and his staff she learned that Jason had what is called Ornithine Transcarbamylase Deficiency, or OTC. This is a disorder of the urea cycle characterized by the accumulation of ammonia resulting in an altered level of consciousness and/or persistent vomiting. We had never heard of this before. Why us? Why anybody?

A neurologist named Dr. Bluestone examined Jason and said that he would never hold up his head, he would never see, and he would never be able to hear anything. After being in the hospital for a month, Jason came home with a feeding tube that went into his nose down to his stomach. He had lost his instinct to suck on a bottle. His mother learned how to replace the feeding tube every week. She also learned how to mix and administer his medicines. This was the only way she could feed him and monitor his intake. Naturally we decided to treat him like any other baby. We just loved and cared for him. We also prayed for him, as did many other people. Just pray, trust, and have faith in the Lord was all I could think of to do. Jason was taken off the liver transplant list because the doctors decided he wasn't a good candidate.

Dr. Amos checked Jason's progress weekly. He was growing like any other baby. His hearing was checked and he could hear. He was following things with his eyes and reaching for his mother.

Dr. Bluestone wanted to see him again by the time December rolled around. He couldn't believe this was the same baby. Jason was 23 pounds and about 25 inches long. He could hear, see, and hold his head up. He was cooing and saying Mama -- not bad for a baby that wasn't supposed to do anything. Dr. Rosenthal and his staff also saw his progress. They put him back on the list for liver transplant.

One night in December, 1998, at 2 AM my daughter called, crying. They had a liver for Jason. I told her to calm down and that it was going to be okay. We knew it could go one way or another.

The surgery went fine. A few days afterwards, the doctors went back in to check the progress of the liver and found a blood clot. This meant that Jason would need to have another liver transplant. Surgeon John Roberts was willing to give it another try. In the meantime, Jason was to stay in the hospital until another liver was donated.

One New Years Eve another liver was found and Jason was in surgery again. God saw the doctors, Jason, and us through the operation safely. This time everything was successful.

Jason came home in January of 1999, four days before my birthday. It was the best present I ever got! We know there are going to be ups and downs, but with God on our side we will get through it.

We want to thank the donors and their families for sharing so others may live. We want to thank Dr. Rosenthal and his staff, and the genetic team at Santa Teresa Kaiser for all their support. They call him their miracle baby! God bless!

Josiah

Josiah

By his mom

Josiah was born in September 2002, he was a healthy baby until he was 8 months old. We noticed red spots in his scalp something like ant bites. We took him to his Pediatrician and even to him the red spots didn’t look familiar so he referred Josiah to a Dermatologist, he took a biopsy of the red spots in his head. I took Josiah back two weeks later for the results and he diagnosed him with Congenital Self Healing Histiocytosis, a disease that he wasn’t even familiar with at all. The doctor told me to keep and eye on the dots because all he knew was that they would get big like a size of a quarter.

At the beginning of July we noticed Josiah not been a normal 9 month old, his skin and eye color started changing, didn’t have appetite, the red dots were spreading all over his body, started having infections, fever and loosing a lot of weight. In October, his Pediatrician send him in for labs and his white blood cell count & platelets had came back elevated. The doctor wanted to repeat the labs again in November. Unfortunately the results were the same. Josiah's doctor was very concerned about his labs and also the continuous infections and fevers.

In December 2003, he referred me to a Hematology/Oncology doctor. Josiah's appointment with this doctor wasn't until the end of January 2004. On December. 29th Josiah had more fevers, we took him to the ER. There they ran a lot of blood test and gave him antibiotics. His fever went down and apparently everything was fine. He was discharged from the ER and went home As soon as we got home, my sister-in-law told us that their was a mistake in Josiah’s labs and that we had to go back to the hospital, something with his Liver functions, they were kind of elevated. We took him back and he was admitted.

Dr. Thomas saw Josiah the following day and order more test and biopsies, first he had a bone marrow biopsy. According to the Dr’s he was having all the symptoms of Leukemia, that test came back negative. Then he had a liver biopsy and that one came back with Langerhan Cell Histiocytosis, apparently that was the cause of Josiah’s color change, fevers and infections. We were told that it was a rare disorder in the blood and that 1 out of 200,000 kids are diagnosed with it. The only treatment was Chemotherapy for a year, along with other steroids and medicines required of cancer patients.

We were devastated and worried that our son wasn’t going to make it thru the treatment. After a few months of treatment his labs wouldn’t get any better so he was referred to the Gastroenterology doctor and that’s when Josiah was diagnosed him with Primary Biliary Cirrhosis, Josiah started having severe itching and color changing drastically, he was going to need and liver transplant. On November 13, 2004, he was officially placed on the waiting list for a Liver. On November 27 about 2:30 a.m. Josiah woke up vomiting blood, we rushed to the ER and two varices busted and had to be intubated to try to close them. That gave Josiah more points in the list.

At the end of November, we were notified of a liver for Josiah, we were so happy and scared at the same time. He was transferred to the University Hospital and we waited for the liver. Since his transplant Josiah has been fine. We thank God for Dr. Mejia (transplant surgeon), Dr. Neigut and their staff for the wonderful care they have given Josiah.

Justin Lars

Justin Lars

By his parents, Joe and Paula

Justin Lars was born in March 1999 in Glenwood Springs, Colorado. He was born on a Tuesday. By the following Friday night he was refusing to feed. The following morning we couldn't get his attention. He was very dopey and still would not feed. By Saturday night his temperature had dropped to 95 degrees. We were ordered back to the hospital where he fell into a coma. All day Sunday they ran every test in the book to try to determine why precious little Justin was dying. I remember his birth announcement was printed in the newspaper that day and thinking how devastatingly ironic it would be if he died that day.

Children's Hospital in Denver suggested that the Glenwood neonatologist run an ammonia level. Four months earlier they had encountered a pair from a set of triplets who had been found to have a very rare condition called a "urea cycle disorder. "The ammonia test came back and it was a dangerously 12 times normal. He was airlifted to Denver. They had us sign release forms to use "experimental" drugs to lower his ammonia.

By Monday morning Justin awoke from his coma. That Monday afternoon a group of doctors came into my son's newborn intensive care cubicle. "Your son has 'argininosuccinic aciduria.' They continued, "You will need to feed him three special medical formulas, give him arginine every eight hours and he won't be able to eat protein."

Now, fast forward past a handful of ER visits and hospitalizations to when Justin turned 8 months old. It was early November and Justin's ammonia had risen to 270. He was put in the hospital and was running a high fever. That's when Justin developed a seizure disorder. He was again airlifted to Denver. He spent the next month at Children's Hospital. He underwent tests to see if he had gastrointestinal reflux disorder, neurological problems, and other maladies. He also underwent his second spinal tap in his fledgling life. He had surgery to receive a g-tube (a port for a feeding tube straight to his stomach) and they took a liver biopsy at the same time. The liver biopsy showed that his enlarged liver had a lot of fibrosis.

Justin was hospitalized for a week at a time in January, February, April, and May, August and September of 2000. Each hospitalization included high fevers and seizures and the May hospitalization included a very rare case of cholera. To our disappointment he had a 200 hyperammonemia in January, a 150 hyperammonemia in February and a 259 hyperammonemia in August (we had hoped the g-tube would prevent hyperammonemia episodes since we were now able to meet his formula quota with his g-tube).

In August I started hearing and reading medical opinion that the high plasma level of argininosuccinic acid kids with ASA have was toxic and was resulting in poor mental development and liver problems.

In February 2000 we met with the liver transplant team at The Children's Hospital of Denver and Justin was placed the liver transplant list. In October 2000 he was elevated to status one because of his recurrent hyperammonemias. Justin received the gift of life in January, 2001, at 22 months old.

When the hospital pathologist examined his old liver she found it to be in "a very poor state of affairs" and on the path to failure.

Justin spent most of the three months following his transplant in the hospital. He had three rejection episodes (including a third "Flight for Life" trip to Denver), a bout of dangerously high potassium (which later in the year became too low) and he had to have a second major surgery to open a bile duct that had become obstructed because he had prematurely passed the stent holding it open.

In May we stopped his phenobarbital (which some believed was contributing to the rejection episodes because it was making him metabolize his immunosuppression too fast and making his cyclosporine and then Prograf levels erratic). And, we added Rapamune to his regimen (the powerful yet different mechanism of this newer immunosuppression agent is just what he needed).

He has been rejection free ever since and with his metabolic problems alleviated.

He was hospital-free from May to September.

In September, he came down with a virus and spent a week in the hospital but his liver numbers stayed good.

Between September and December he got sick a few times but his numbers stayed great.

Then on December 23, 2001, he became dehydrated and was hospitalized. He was in the hospital December 23, 24, and Christmas Day! However, his numbers stayed great (other than a temporary spike in his Alk Phos possibly related to Bactrim).

His EBV PCR is still zero (boy am I jinxing that. . . I know he can't hide from EBV forever - especially since we're told his donor was EBV+).

In February 2002 he spent a day and a half in the hospital because he came down with influenza A. Regrettably, the flu vaccination was very late coming to our town this year and somehow we let getting him vaccinated slip through the cracks. With the fever he got from the flu, his seizure disorder returned. This time since we are convinced more than ever that his seizures only come around when he has a rapid spike in temperature so we are not going to treat them long term. Instead we are going to keep emergency seizure medicine in his travel bag and resolve to reduce any of his fevers ASAP via Tylenol and, if possible, a bath.

Justin has come a long way from when he was 5 days old laying in a coma in the hospital dying from a mysterious illness.

Now he is a happy, active, nearly 3-year-old toddler, whose mind has awakened and is re-networking to overcome the damage his disease caused. He loves preschool and is starting to regularly use and understand speech (e.g., "go get your coat, throw that away and shut that door")

Kayla

Kayla

By her mother, Joetta

This is a story of a 6-year-old girl who's had more than her share of adversity and discomfort. It all began just after Kayla turned 2 and started spitting out her food. That turned into vomiting several times a day, for days on end and into weeks. It seemed like we were always taking her to the pediatrician and GI doctor, and even occasional ER visits. The first, and most common, diagnosis was "it must be a virus," or possibly reflux. Four months passed and she learned to avoid almost all meat and was throwing up on a daily basis and during the night. Somehow she managed to maintain a normal, healthy appearance, but I knew there was nothing normal about her at this point. I insisted that any and all tests be done to find out what was wrong with her because it was obvious to me that this was not going to pass. Eventually, gastroparesis (paralysis of the stomach) was diagnosed and this led to her first upper endoscopy.

The morning of that first endoscopy Kayla was admitted as an outpatient and put under with a light sedative. As the doctor looked through the scope he found absolutely no mobility of the stomach or intestines. After the procedure she was expected to regain consciousness within 20 minutes, but she didn't. We couldn't wake her up. This, of course, was cause for great concern and she was admitted inpatient for an MRI. Later that evening she began to come around, but she was very trembly and not at all herself. She was vomiting profusely, incoherent, and very disoriented. It was like she was falling apart before our eyes. She didn't appear well at all.

Blood tests of every kind imaginable were drawn and a spinal tap was done, but nothing stood out that would cause what we were seeing until her ammonia level came back on the 3rd day. It was repeated because the level was so high the doctors were certain it was a botched test, but the second test came back even higher. It amazed the doctors that she was as alert as she was (which wasn't very) based on what her blood work was telling them. Another spinal tap was done to check the ammonia level in her central nervous system. She was taken to surgery for an emergency liver biopsy to confirm the diagnosis of a urea cycle disorder so we could begin treatment to lower her ammonia before it became fatal. After the biopsy, they were certain it was a urea cycle disorders (there are several different types) and began treating it as ornithine transcarbamylase deficiency (OTC deficiency). OTC is the main enzyme responsible for clearing ammonia out of the blood. This is an inherited condition that runs in families and females are the carriers in most all instances, but to our knowledge Kayla is the first in our family to have this disorder. After several DNA tests, she was identified as a "new mutation carrier" with the faulty gene on one of her X chromosomes.

Over the next couple weeks inpatient we were taught how to give her medicines to help her body rid itself of the ammonia load her liver didn't have the ability to break down itself. We were taught the importance of a very low protein diet to avoid the buildup of ammonia. With OTC deficiency, any stress to the body, be it a viral illness, injury, or protein overload, can cause ammonia to build to a toxic level, which can result in brain injury, coma, or even death. When Kayla was released from the hospital we were told to return for frequent blood tests or if she began to show symptoms of fatigue or illness.

Fast forward several months and we lived in the hospital more than we did at home. Further testing showed Kayla to be at the severe end of the spectrum of this disorder and we found it almost impossible to keep her ammonia level in check. She had a stomach tube placed for feeding due to her loss of appetite and need for finely balanced nutrition. The tube also made it easier to give her the horrible tasting medications she needed. She had more tests, including several liver biopsies and skin and muscle biopsies, to confirm the diagnosis of OTC deficiency and rule out any mitochondrial diseases.

Because of her constantly elevated ammonia levels, a slowdown in her growth, and evidence of possible brain damage, the option of a liver transplant was discussed. We met the team of doctors and nurses and we were given all the standard information on the risks and benefits of liver transplantation. We came to the conclusion that this was the path to take. Our once energetic and bright-eyed little girl was tired, sick, and appeared to be slipping backwards in her development. We knew we had to proceed with a transplant if it could cure her of all this. She was worked up for transplant while in the hospital for yet another ammonia crisis.

Within a month of our first discussion about liver transplants we received the call to come to the hospital so Kayla could receive her gift. The liver was a small portion of an adult liver that had been released for use in a pediatric patient, who was lucky enough to be Kayla. We had been told it could take up to 18 - 24 months to get the call, but here it was not even 2 weeks after she was listed. We were not at all mentally prepared, but we packed our bags and took off. (I don't think we would have ever been prepared for this call anyway.) We left our three other children at home with a friend until my mom came in on the first available flight to take care of them. That had to be one of the longest drives ever made. As we drove, we looked at our little girl, who had just said good-bye to her brothers, and wondered "what if...?" I walked into that hospital with feet made of lead, wanting to run in, but wanting time to stand still all the same. But then it hit me -- for this to happen so quickly meant that it had to be the RIGHT one for Kayla.

Upon admission Kayla was examined and found to be in good health for surgery, but to our surprise her ammonia level was much higher than any of us would have guessed. She would have undoubtedly been in the hospital before the day's end, but we hadn't seen any symptoms that it was rising. For me, that was the final proof that she was going to be just fine. Someone was looking out for her because this may have been the one time her level went unchecked and allowed to rise dangerously higher than before.

Kayla went to surgery and 7 hours later she came out, breathing on her own and looking great. She was taken to PICU, but 6 hours later she started to show signs of trouble and required another trip to surgery. She was found to have an arterial thrombosis and it was repaired, but not before there was significant damage to the small portion of liver she had just received. She remained listed for another transplant and in critical condition for a week with significant encephalitis and in a coma. She didn't wake up or even open her eyes until 7 days after the transplant. Kayla faced uncertainty at many points and we were prepared for the worst until plasma paresis helped clean her blood of all the toxins. She slowly showed signs of improvement and eventually came out of the coma, she had regressed in her oral abilities and even had memory loss. She required oral therapy to learn how to eat again. Two weeks after the transplant, she finally looked at her father and I with certainty that she knew us. After 26 days in the hospital we came home with our little girl. She had a second chance at a quality life, cured of her previous disease, but now with new issues to work on. We learned all the medications given orally and through IVs and we also learned what the word "tired" really means.

Life since transplant has not been without trouble. She was in and out of the hospital often that first year for viruses and infections of every kind, it seemed. We are now 3 years post-transplant and for the past 19 months she has not been hospitalized. By all appearances she is a normal, healthy 6-year-old girl without a worry in the world. She is beginning Kindergarten this fall, which prior to transplant we could not have imagined in her future. Her biggest issues these days are with her cognitive and speech abilities. This seems to be the area of the brain where most of the damage took place, but she is making progress. The future belongs to Kayla to become all she can be.

Kelly

Kelly

Kelly had a liver transplant when she was 2 years old because of neonatal hepatitis. Today, Kelly is a 10-year-old dynamo of energy and enthusiasm. She loves soccer and enjoys tennis, singing, jumping rope, bike riding, playing with her babies, games, and watching movies. “Kelly is always ready to try something new,” says her mom, Cathy. “She definitely lives life to the fullest!”

Kelly

Kelly

By her mother, Leslie

Our daughter Kelly came to us at the age of 3 years old as a Foster child. She was in need of a Small Bowel Transplant. During our wait we tried to see if they could get her gut to work, but it never happened.

Ten months after she was placed with us I noticed her eyes were getting yellow and brought it up to her nurse. They had noticed it also and were setting up a liver biopsy. What we were afraid of came back; she was in early stage of liver failure due to TPN use. One month later she had her first bleed. It was the most frightening thing I had ever had to deal with! She came home about a month later and right away had another bleed. She remained in the hospital from April until August. She had multiple bleeding episodes and one of her worst ones we almost lost her! Finally they were able to find the problem; an artery near her g-tube sight was where all the bleeding was coming from. They cauterized it and a few days later she came home. Needless to say I was very nervous.

During her time in the hospital her parents, who were very young, decided it would be best if she lived with us and we were able to start the process of adopting our precious little girl! Three weeks later in September 2000, we received the call we were praying for--there were organs available! Kelly was in surgery for close to 17 hours. When the doctor came to see us we were told the organs came just in time. She wouldn't have made it more than a couple more weeks! Kelly had some complications. She had an infection that caused her incision not to close and her intestine would pop out through it. They finally put a piece of mesh and let her granulate up. Well that did the trick! We were able to take her home a couple of weeks later!

Then on December 17, 2000, we made her our forever daughter! She has done fantastic since and has had very few issues!! She has made us and her doctors at Pittsburgh Children's Hospital proud! She now attends 3rd grade and is doing very well. She is a bit delayed but is moving forward quickly. We are so proud of what a wonderful young lady she is turning into and are looking forward to many beautiful memories with her!

Kristy

Kristy

By her mother, Sharon

KRISTY... A BEAUTIFUL GIFT... AND A LIFE IN PROGRESS

UPDATE June 2003

“Bye Mom, bye Dad. Love you” Kristy called, heading out the front door on her way to the mall with friends. I sat at the kitchen table thinking about what a great kid she is. Life wasn’t always this simple though.

Kristy was born almost two months ahead of schedule in April 1985. After a month in the neonatal intensive care unit, she appeared to be healthy and ready to take on the world.

Soon after, we decided to take a trip to Winnipeg to see some relatives. Only minutes after our arrival, Kristy’s pediatrician called to tell us that our baby had some sort of immunity disorder and advised us to return home immediately. With his help, we opted on seeing a doctor in Winnipeg (as we had just driven 14 hours).

Following days of tests, we were told that Kristy had Hereditary Tyrosinemia Type 1. We had to face the fact that our daughter would not likely see her first birthday. The only possible hope was a liver transplant, but even that was no guarantee. Furthermore, nowhere in Canada were pediatric liver transplants being done.

The next year was a whirlwind of information and tangled emotions. HT1, as it is called, is a rare genetic disorder that eventually causes severe, and often fatal, liver disease in infancy. We were overwhelmed and devastated.

My husband, Peter, and I took Kristy to see doctors in Montreal and Calgary. Eventually we took her to Minnesota, where she was finally accepted as a patient and placed on the transplant waiting list. She was such a tiny baby, barely 14 pounds. She had to follow a specialized diet, take a variety of medications, and have blood work done on a daily basis.

Kristy spent most of the winter and early spring in the Red Deer, Calgary, or Minnesota hospitals. By March of 1986 she was so ill the doctors at home were not able to help her. At this point, we had no choice but to leave our 7-year-old son with our best friends, who lived down the street. As we hugged Cory goodbye and set off without him we wondered if we would bring his sister back to him. He had already lost one sister two years earlier (from bacterial meningitis). He was just too young to go through that again. We told him we would call him every day and for him to know we were bringing Kristy back with a new liver.

Once again, we flew an emergency run by Lear jet to the USA. My sister, an emergency nurse, accompanied us to help us understand what was happening and to offer what comfort she could.

Kristy’s life at this point seemed to be nearing the end. We were so fearful of losing her and our emotions were constantly in turmoil. She was taken into surgery to have catheters inserted into her chest, taken for x rays with techniques that blew our minds, and given highly specialized drugs. We hardly ate or slept. We just sat by her side watching the doctors poke and prod and giving her the blood exchanges that were keeping her alive. She was so swollen; she could barely open her eyes and was almost unrecognizable to us.

The doctors told us she was now on the priority list for the next available child’s liver. However, they warned us that we should not expect more than another 48 hours with her if a liver was not found. Her year would be up the following day. Kristy needed a birthday present that could not be wrapped in pretty paper and bows.

My brother, Dave, who had flown in from New York, was preparing to go home when the phone in the hotel rang. I must have screamed loudly as Peter and Dave just stared at me. “They found Kristy a liver,” I cried with tears running down my face.

The three of us ran across the street to the hospital. I sat with Kristy during her last blood exchange, wondering if I would ever see her grow up. One of the toughest things about losing a child is the loss of the hopes and dreams go with them. I thought about all the good things in the world she still had not seen. I thought about her brother having to bury another sister, and my heart wrenched at thoughts of the family who no longer had their baby, and……

”It’s time. The liver is on its' way.” The doctor came in and looked at me. As Kristy was being prepared for surgery the nurses came in saying, “Let’s just take a picture of mom and baby.” I didn’t need an explanation.

A few hours later the doctors came to tell us that the weather was bad and the helicopter would have to wait a while before landing. We were getting scared, knowing the organ only had so much time to get to our daughter. After pacing for what seemed like forever, they finally came to take our baby away, saying it was time.

Fourteen hours after Kristy disappeared from my sight through those double doors I saw the chief surgeon and another doctor walking straight towards me. “Oh no,” I grabbed my brother’s arm, “What happened?” There had been a problem, an accident that caused Kristy’s heart to stop beating, but perseverance and quick thinking helped revive her. The neurosurgeon would be down to see her when she awoke. I won’t tell you what was going through my mind at that moment, but my baby was alive. She made it past her first birthday just like I knew she would. She received the best gift of all…life. I cried tears of joy for us and sadness for the family of the lost child.

Over the next few days Kristy amazed us all. Showing no signs of neurological problems, nor rejection, she was out of the intensive care unit on the second day, sitting up and eating an orange Popsicle. By the third day she was ready to try food and grabbed a bag of chips, a familiar food. Over the course of the next two weeks, Kristy became known as the “Chip Queen” as she wowed everyone with her antics and thrilled everyone with her progress.

Kristy was discharged from the hospital three weeks after the surgery. Without getting into the hilarious story of her grandma and grandpa getting a flat tire on the way to the airport and having to hitch a ride to be reunited with their granddaughter, we arrived home on May 8, 1986.

Kristy is now 18 and her brother is her best friend. At seven years of age she started competitive figure skating and dancing. She enjoys playing soccer in the summer, skis and snowboards in the winter, runs for the track team, plays badminton with the school team and has played in the school band for 5 years. She has maintained academic honors since the fifth grade, proving again and again that there were no adverse neurological problems resulting from the surgery.

Update June 2003
After 17 wonderful, amazing post transplant years, Kristy is getting ready to attend Red Deer College in the fall to pursue a career in the field of criminology. Now, of course, this may change over the course of the year, but that is okay. We are always reminded of the pain of the donor family on this day as Kristy received her new liver the day before her first birthday. Without them, we would not be celebrating anything. So, we say thanks a million times over for this special time in our lives.

Kyle B.

Kyle B.

By his mother, Lisa

My baby, Kyle, was born in August 1999. He had jaundice on the first day, but the nurses and doctors said it was normal.

In September he was rushed to the hospital in Albany, and the next day he was flown to Boston. They did tests but could not find out the cause of his liver failure.

By the end of the 3rd week he started to deteriorate and was given only one week to live. He was in end stage liver failure and was described by doctors as in a pre-coma state. He was then flown to New York City to have a living donor transplant. We were told this would be his only chance for survival. After being in NYC for 5 days, Kyle received a donated liver.

He is 7 months old now and doing wonderful. We thank God for answering our prayers.

Lindsey

Lindsey

Lindsey, age 13, received her liver transplant five years ago at Children's Hospital of Pittsburgh due to cryptogenic cirrhosis. Her surgery, recovery and post-transplant life have been wonderfully uncomplicated. She has been hospitalized just once since her liver transplant. Her mother Melinda knows how scary it is to face a liver transplant and hopes Lindsey's positive experience will encourage others.

Matthew

Matthew

By his parents, Kathy and Jerry

Matthew was born six weeks early in August 1995. He was in the NICU of Fairview Riverside Hospital in Minneapolis. He seemed pretty good for three days, and then on the third day our world collapsed. Matthew quit breathing and went into a coma. His ammonia level was over 2500, as you know it should be close to zero. Lucky for us, we were at the NICU so they could respond fast. Matthew was quickly diagnosed with Ornithine Transcarbamylase Deficiency, OTC. He had hemodialysis immediately (twice) to clear his blood of the ammonia. I found out I am a carrier for OTC.

We went home after three weeks and were put into home isolation to protect Matthew from anything that could make him sick, which in turn would cause an ammonia rise. With OTC, it is where your liver can not break down protein; Matthew was on a special formula that had to be specially made. I also asked if we could mix in breast milk and they agreed. So Matthew was the first one that had ever had breast milk and now they use that as protocol if possible because they think that is why he had such a good outcome.

At six months, March 1996, Matthew had a liver transplant. We thought all was well and we went home in week. A few days later again our world fell apart. There had been a small leak after transplant, they thought it would scar over, but it did not. On April 12, 1996 Matthew almost died again. That duct that was leaking "blew apart" leaking bile all over and forming a biloma. They took Matthew to surgery and he was very septic. This surgery took longer than the transplant, over ten hours. They put bile duct drains in. They told us it was in God's hands. Matthew is fighter, he pulled through.

For three years he had to go into the OR every four weeks to have his bile ducts dilated and new stents put in. He had repeated episodes of sepsis and cholangitis. He had stents until he was four. When he was two, almost three, Matthew was really sick and they didn't know why. He was losing weight, throwing up all the time, just really sick for months. It was finally diagnosed as EBV, Lymphoproliferative disease. He received a year of CytoGam and Ganciclovir. The list goes on and on for Matthew. It always seems whatever can go wrong for Matthew does. He also has hypertension and reflux. He has had a rough fall. He has been having headaches and "chest pains.” They finally did CT's and PET scans and found an area in his chest that we are doing a repeat CT on in a few weeks.

Matthew is an incredible kid. He is in the third grade. He does really well in school. He plays baseball, basketball and soccer. He is an inspiration to us all. He is awesome.

We would very much appreciate all your prayers for Matthew. Lymphoproliferative disease is scary. We would love to hear from any family that has similar situation as ours. Especially complicated post transplant history which included:

  • Lymphoproliferative disease
  • Prednisone and Neoral problems
  • High blood pressure
  • Rejection (ours is without LFT's even raising)
  • OTC

Thank you for your time. If we can help any families out there, we would love to.

Patrick

Patrick

By his mom, Joanna

The summer Patrick turned 10, we noticed he had started limping a little. Thinking it was bad shoes, we went through several pairs with no resolution. By late September, he was walking down the stairs sideways and ached every morning. He had stopped gaining weight, always a worry for a small kid. (He weighed about 75 lbs – 30th percentile stuff) Doctors said not to worry, it was some virus slow to resolve.

In November, I remember the morning I saw his hands silhouetted against the window – knuckles swollen past his fingertips. The senior doc at our peds office looked at him for 2 minutes, and said ‘He’s got arthritis, and he’s got it badly” By now he was down to about 60 pounds and the worst was coming.

We never got to arthritis meds. By Thanksgiving, he started having chronic diarrhea, and we took him out of school. By December, our rheumy pulled in every favor she had and got us a endoscopy– “help us” I pleaded “he’s starving to death”. Our doctor and the rheumy suspected celiac disease. The scope confirmed it and we went gluten-free, glad to have it over. He doesn’t have jra – the joint problems were untreated celiac disease. He was started on a high dose of steroids to speed the healing and control his joint pain.

But after 3 weeks of a gf diet, Patrick was still a victim of “unremitting” diarrhea, and was down to 52 pounds. We drove 5 hours through the worst snowstorm of the year to Children’s Hospital of Pittsburgh, where we stayed for 7 weeks. Peds GI there felt the dx was wrong – Patrick has celiac AND autoimmune enteropathy. His body is rejecting his GI system wholesale. His weight continued to fall, down to its lower limit of 47 pounds. He was put on TPN, Vacomycin, etc……. and then the Prograf, which saved his life. He gained 20 pounds in the hospital. He returned to a joyous reunion at his school in late May.

No one knows how long he’ll be on Prograf. He is one of a handful of child-onset patients with AE. There are babies with AE who never come off TPN in their relatively short life; there are adults with AE who stay on Prograf, and there are AE patients who abruptly go into complete remission. Doctors at Johns Hopkins said that quite frankly, nobody knows for sure if Patrick has celiac and AE (he’d be the first), or just AE, or just celiac; they don’t know what the course will look like or how long it will last. But he weighs 75 pounds again, and today is a good day with my baby.

Ricky

Ricky

Ricky was diagnosed at four months with neonatal hepatitis. When he was two years old, his doctors were thinking about listing him for transplant, but then his condition improved and it was decided to wait. Eleven years later, he continues to have elevated labs, but he is doing very well and hasn't needed a transplant. Ricky plays soccer, basketball, and karate. He enjoys his dog, Mia, a flat-coated retriever and his pygmy goat, Tubby. Ricky has been in three television commercials and is with an agency. He has two older sisters, Amanda and Melissa. Ricky and his family are very active in their church. He loves life and is very grateful for his continued good health.

Riley

Riley

Riley is a 4 ½ year old boy who on June 21, 2005 was taken to Children’s Hospital in Columbus because of extreme Jaundice and exhaustion as the main concerns.

Children’s in Columbus immediately admitted Riley and continued testing his blood to try to find a cause for what was happening to him. By Thursday morning, Columbus Children’s transported us to Cincinnati Children’s because liver transplants are not done at Columbus Children’s. This was such a shock to us as we had no idea he was that ill. A week prior to this, Riley had just started getting sick, he was healthy before that point.

Upon arriving at Cincinnati Children’s Hospital Medical Center, again the tests continued, but Riley was not getting better. His liver had failed to respond to the tests. Finding a cause was no longer the priority. They moved him to ICU on Friday morning, and by Friday night, June 24, Riley was listed as needing a new liver and as a status 1, which means they did not believe he would survive 7 days without a liver transplant.

Lots of prayers went up at this point. I had a peace about it and in my heart something told me that Riley would be ok. I was still terrified and cried with my husband for a long time. They told us to expect a 3-4 day wait on the liver as they had to “weed out all the ones that wouldn’t work first”. This was the worst thing, as we could already see how Riley’s liver not working was affecting his mental state. Prayer was all we had. We had to get a liver.

On Saturday morning, at 4:30 am, we received the call that they had a liver. God heard our prayer! Not just a liver, but the very first one that came through. It was from a young adult and it would have to be cut to size, but it was a match.

By 5 pm that night, June 25, Riley was in surgery receiving his new liver that would give him a second chance on life. To this date, they still do not know the cause of his Fulminant Liver Failure.

Riley’s spirits are great. He is so awesome. He loves stickers, watching the cranes outside build more buildings, and playing. He has not lost any of his charm. We are so thankful to God for giving us a second chance with our son. We are thankful to the donor family for making that decision. The road ahead still looks bumpy, but with God on our side, he will cushion those bumps for us and hold our hand through every one! Praise God!!!

 

Robin

Robin

By her mom, Faith

Update: August 2005

Robin was a never-ever baby--I was told I would never ever have anymore children. Well Robin fooled them. Robin was born at 36 weeks in October 2001 by an emergency c-section because she was transverse. Robin wasn't breathing when she was born and her apgars were 1 and 4. Finally, Robin started breathing. What a relief! Robin was pink when we were released from the hospital but her urine was brown. We asked about that and were told that was normal. We took her home but when we took her to the doctors for her first bilirubin test for newborn jaundice hers was elevated, so she went on the bili lights for 7 days. The home health nurse came out 3 times to take her bili, and finally she was taken off the lights because we were told she was fine. When I took her to all her shot visits, no one saw anything wrong until she was 4 months old. The health nurse noticed her whites of her eyes were real yellow and I said I noticed that also, but no one seemed to think anything was wrong. The nurse told me to take her to our doctor and see if he thinks this is normal. So I did and that’s when our lives started to change for the unknown.

Robin went to Children’s in Denver, Colorado and was seen there by the GI team. They did a lot of blood tests, genetic testing, autoimmune testing, liver ultrasound, liver biopsy and x-rays. They came in and informed us that they did not really know what was wrong with Robin except her liver was failing and she would need a liver transplant by time she was 1 year old. Talk about the world coming to a stop! Here we lived 700 miles from family and they are telling us our little angel, Robin, needs a life saving liver transplant. WOW! So we went through all the testing and meetings, etc. to be listed and the wait began. Robin was constantly vomiting, at least 4 times a day, everyday. She wasn't gaining weight; she ate but she didn't gain. They had her on vitamin K, E, D, A, plus Zantac and she was placed on 24 hour NG feeds of Alimentum and TPN. Still she had no weight gain or growth. It was so hard to watch our angel get so sick and there was nothing I could do.

We waited a long 9 1/2 months for her first liver transplant that happened in Denver in December 2002. Robin spent the first 17 days in PICU, almost dying twice and then needing FFP and blood several times. The doctors didn't know what was going on. We kept hearing this is normal. Well, after we were finally moved to the floor and Robin was still not getting better, I took it upon myself to contact another liver transplant center for advice and to possibly moving Robin there. Robin was still on 24 hour NG feeds, TPN, IV's etc. still with no weight gain or improvement. The famous words of the doctors were, "WE JUST DON'T KNOW WHAT IS GOING ON!" She had ascites that they were removing from her and it was measuring 2 liters at a time. This was happening weekly for at total of 23 weeks. Well enough was enough and I spoke with Robin's main doctor about transferring her to Kansas City Children’s Hospital. He agreed and after she was released from the Ronald McDonald House and from Children’s we changed to Kansas City in Feb 2003. We were back and forth to Denver from Colorado Springs, where we lived, 4 times a week until we moved to Kansas City, to be close to family and to give another center a shot at saving Robin's life.

We moved back in March of 2003 to Kansas City, Missouri, and the wonderful doctors here have done everything they could and everything that is normally done for post liver transplant patients but nothing worked here either. Robin continued to have ascites and it was still measured 2 liters. She had a bad bout of EBV and she still wasn't growing. After 16 months of 1000's of blood test and 29 liver biopsies, a full body scan, 15 liver ultrasounds, stomach biopsy, 14 weeks of peritonitis, continuous steroids, 5 weeks of IVIG, Simulect, Sirolimus, Prograf, CellCept, Zantac, Vit D, Vit K, Actigall, Spirolactone, Lasix, Valacyclovir, Nystatin, Bactrim, Benadryl (for itching), Potassium Phosphate, we all decided that enough was enough. After 16 months of weekly and sometime biweekly trips to the hospital and 7 stays in the hospital, 3 port-a-cath insertion, 2 Broviac insertions, NG tube still for feeding, she only gained 5 lb. and grew only 3 inches. Robin was replaced on the liver transplant list again, this time for chronic rejection which has lead into end stage liver disease. You know you have to go through the ups and downs one time, but twice, I thought it would be easier but, to be honest, it is much harder. We just wanted Robin to be toddler but she couldn’t and that isn't fair to her. We love her so much that to have another transplant was ripping us to pieces. Then we worried she will not have any better luck with this transplant.

In September 2004, Robin received her second life saving liver transplant. After a 12 hour surgery, Robin was doing remarkably well. She had the breathing tube removed at 12 hours post transplant, was awake just 4 hours after transplant, was walking 24 hours after transplant and we were moved to the floor 36 hour after! Robin only was in the hospital a total of 10 days and we came home on her 3rd birthday. For the first time in her life all her liver numbers are NORMAL. She is finally getting to be real toddler, except for the fracture in her right leg from all the steroids she has had since birth. But that too will heal.

We are so very thankful for the wonderful doctors and super nursing staff at the hospital. We will forever be in debt to the family that decided to donate their daughter’s organs so that our daughter would live. They will always be in our prayers and hearts. Their angel lives on in our angel Robin. Thank You. Robin now only takes 4 meds a day. What a difference and no steroids anymore.

Update 2005

Robin is now 11 months post transplant and all is well. She had a wish granted through the Make-A-Wish Foundation which was going to Disney World and meeting Snow White. She loved it. We stayed at Give Kids the World Village, which is a wonderful place; it's all about the kids. Robin has grown over 6 inches and has gained more than 6 pounds in the last several months since transplant. To finally see her running and dancing and just having a great time is truly a miracle. It is odd to be only going to Mercy Hospital for once a month labs after having gone two to three times a week for so long. She is only on three medications. We go to the hospital every three months to have her infusions for her bones. It is a miracle that she received her second liver in time and that all is finally going her way. We never thought we would see Robin so white again.

Scotty

Scotty

By his parents, Chris and Holly
Scotty was our beautiful blonde haired, blue eyed son, our first born child. He was born in January 2000. We did not have a care in the world and enjoyed every milestone that Scotty had. When Scotty's sister Sophie was born in 2002, we decided that Scotty needed playtime with other children his age and so we sent him to preschool. Little did we know what was to come. Exactly 8 months later tragedy struck our family.

Scotty was playing at a birthday party in March 2003 and 3 days later was in the emergency room for jaundice. The doctors said it was just Hepatitis A and sent us home. I laid in bed for the next 4 nights with my hand on Scotty's 105 degree head waiting for his fever to break. I finally took him back to the doctor and they decided to do further tests since Scotty was unable to even get up and walk around. The ultrasound showed an enlarged bile duct, possibly a blockage and no liver cancer. We were so relieved, little did we know...

When the CAT scan did not reveal why Scotty's liver was so enlarged his pediatrician called the head GI doctor at Children's Hospital in Los Angeles for advice (this is where Scotty's pediatrician happened to do his residency). Dr. Thomas told our pediatrician to send Scotty down right away. We avoided the ambulance and drove him the 3 hours ourselves, longest trip of our lives. On the way I began to sort through Scotty's medical charts and I read, "This type of liver abnormality is likely to be fatal without a transplant." My stomach was sick and I could barely breathe the rest of the trip.

The moment we arrived at Children's, we were told that Scotty would be Status 1 on the transplant list and that he would have a biopsy the next morning. The biopsy revealed Scotty's liver was dying and he had less than a week to live. We were vigilant the entire week, watching liver function numbers, PT and ammonia levels. I was tested to be the donor and had a liver that was unable to donate. By this time we had networked with over 1,000 employees in our county and had over 30 people on a list able to donate to Scotty. The doctors went to the first person on the list, Carolynn.

We did not know this person that worked with my husband and my husband could barely even describe her. She saw our e-mail asking for help for Scotty and figured, why not. Carolynn went to USC for her work up and was a perfect match. In April 2003, 17% of Carolynn's liver was removed and transplanted to Scotty who laid in a semi-coma only 5 miles away. The doctors said that Carolynn was a better match for Scotty than a child his own age. It was a success and Carolynn was amazed at how mobile Scotty was compared to her post-transplant!

Carolynn began to recover and returned home and we waited at Children's for the next 4 weeks to go to the Ronald McDonald house. My husband, Chris and I were shifting our daughter, Sophie back and forth between us and my parents, all the while never leaving Scotty. Immediately after transplant, the doctors informed us that Scotty had a bone marrow biopsy and had hypoplastic anemia which quickly became aplastic anemia. That was the worst for us. Scotty's platelets left then the red blood cells and then eventually his white blood cells. This was scarier for us then the liver. Everybody was masked and Scotty could not hardly move. For the next four weeks Scotty received numerous platelet infusions, blood transfusions and Neupogen shots. We flew friends in from San Luis Obispo, Las Vegas and San Diego to make sure that we had a sufficient supply of platelets for Scotty. Scotty's liver began to leak and they took him back into surgery to repair it. On Mother's Day the hematologist came to see me and said, "Happy Mother's Day, Scotty is making his own neutrophils!" That was never a gift I thought my 3 year old would give me for Mother's Day, but it was the best! Two days later we went to the Ronald McDonald House and 2 weeks later we went home!

Scotty is 18 months post transplant, he has had no rejection episodes, is down to 3 meds per day and has fully recovered from aplastic anemia. He is attending preschool 3 days a week and has had very few viruses. He is happy, healthy and looking forward to kindergarten. He has blood draws every 2 months and clinic every 6 months. We do not take for granted the seriousness of his illness and the reality that he was almost taken from us, but we try to live everyday for what it is and thank God that we still have our sweet and brave boy! As for his donor, she is doing great and is fully recovered. We see her all of the time and she still wonders where Scotty gets all of his energy from!


Zoe

Zoe

By her mother, Maria

Zashayrah Zoe born in July of 1997 in Norfolk, Virginia. She was diagnosed with Byler's disease, a very rare liver disease. For the first four years of her life she was in and out of the hospital due diarrhea and failure to thrive. In March 2001 she had a surgery called "partial external biliary diversion" and she has an ostomy bag where her bile fluids drain. Since her surgery Zoe has been another child. Last year her G-tube was taken out (she had it since 2 months of age).

Zoe is a blessing for our family of five (she has two sisters). She is our sunshine. Currently she is in first grade. She is still very little for her age weighing only 29 pounds and 3 feet tall .....but she is doing fantastic. She is not on the liver transplant list since her liver is doing so much better after her surgery.

Zoe loves going to school and playing with her sisters......oh!!! She loves Danimals yogurt.

end faq