Biliary Agenesis

[reuben]

My son Reuben was born 20th March 2012 and was diagnosed with biliary agenesis at 5 weeks old and will need a transplant.he is a content cheeky fella always smiling.

[Victoria2]

Hello, I'm so sorry your new born baby has Biliary Agenesis , it must be a huge shock to adjust to and the news of a future transplant. I had to go away and find out about your sons condition as I had only ever heard of biliary atresia. I had my first op at 3 weeks old due to a combination of biliary atresia/choledochal cyst which is also very,very rare. I have never had a transplant (instead I have had 7 surgeries with more to come), although there have been times when they thought I might need it but I have met people who have had transplants and are considerably healthier than I am, so I think once your baby has got through the surgery there is a lot of hope for a good life. One lady I met had hers 16 years ago and had not been ill since which I think is amazing--I also think it is wise not to consider a transplant as a cure all though as there are a range of stories after transplant but the over riding theme is one of hope. When have they said he might need the transplant?

best wishes

Vickie

[reuben]

Hi thanks for replying.When we went in hospital with our son reuben at the start they thought he had biliary atresia and took him to theatre to perform the kasai procedure but wasnt sucessful as he has no bile ducts at all in his liver,we had no idea what that meant but the surgeon explained he will need a transplant he said 6-12mnths that was in may.we go back to the hospital every 4 week for check up and blood tests but at the minute his liver functions working fine and still soft which is a good sign.Our main priority is to build him up hes still small weighing only 8 lb 6 at 6 months old and is quite jaundice which draws alot of attention from passers by.The consultant also mentioned alagille syndrome to us all to do with his liver and slow growth.Its just a waiting game until he can go on the waiting list its heartbreaking we just want it done and over with.

How are u doing now hope everything goes ok.

Hope to hear from you soon

Beth

[Victoria2]

Hi Beth,

Sorry I didn't see that you wrote a reply on my emails. I can imagine the waiting is difficult and explaining to people who have never heard of babies/children having liver disease is diffcult too--for adults you always get the alcohol or catchy hepatitis prejudices. I've even had a nasty boss mock me when I wanted to leave saying I would end up an alcoholic in the gutter if I left. That was in England when I was 21.

I wrote a blog on my condition the other day under choledochal cyst/biliary atresia. Most patients even with biliary atresia or choledocahl cyst don't go through 7 surgeries , mine has just been unfortunate. But I am still hopeful that the next planned surgery will stop my pain and I can go to work and start enjoying life. I do try and get pleasure from watching the bird life --I feed them and my little dogs and reading and writing and art. I used to try and go out despite the pain but found people than didn't believe I was ill and I was trying to get out in immense pain and not say anything , I won't do it anymore. Watching a movie can take your mnd off it and you can teach your mind to tune out to a certain extent but keeping that up is a big ask of the pain clinic over here (Nz)who have never dealt with the levels of pain I have in their own bodies , I would cautiously say.

If it helps I had slow growth as a baby and my hair would not grow for years but I am a normal weight as an adult and as a woman have long hair so it did improve! I understand he would need to be a good weight for the transplant. Although it's awful waiting and I totally understand the wish to get it over and done with I would hope the bigger he is the better that will be for his recovery--I used to wish my liver would just pack up and I would get a transplant and it would solve the problem but in the end now I have a diagnose for all the pain of the last 15 years I know it was the loop of bowel that acts as my main bile duct that is the problem so my wish was on the wrong track. I'm actually amazed my liver has lasted so long with the flack it has--infections of cholangitis etc, amazing really.

Please let us know when your little boy has his surgery.Or if you want to talk at anytime.

best wishes Vickie

[reuben]

Thank you i will keep you updated,we go back to clinic on Monday so we will see what they say about his weight gain again.

Beth

[Victoria2 in reply to reuben]

Hi Beth,

Just wondering how you are all doing as it has been a few weeks? How is Reuben?