Billirubin levels in patients with alagille syndrome

[Dina1986]

Hi everyone,

We've been to doctors yesterday, my 8 months old daughter has been diagnosed with alagille syndrome. Her bili levels are still high, about 50, she started with 180 at 2,5 months old. Liver team wants to keep eye on her and not really happy with her levels, told us that she will probably need liver transplant in early childhood, 30% chance she will need it. I am just wondering if anyone have experienced situation and is it possible that levels can go back to normal after some time ? Thank you, just don't know what to think, apparently 25-30% kids woth alagille need liver transplant in infancy or childhood , which is a bit hopeless...

[Ktleesond]

Hi I don't have any experience with alagille syndrome but I understand your worries so I didn't want to read your post and not comment. My son has Biliary Atresia and is now 6 months old. The statistics are quite similar- I think the vast majority need a transplant at some point in their life. The only thing I can say is no one's future is certain and transplants from what I've read are now quite successful if that's needed. There is hope. We just have to keep fighting for our little ones.

[Dina1986 in reply to Ktleesond]

Thank you🙏 we hope everything will be fine and she could enjoy life just like other kids amd adults do .

[Emma-676]

Hi, sorry for the delay with my reply I had been trying to find my sons last blood results. His total Bilrubin is still 174 and direct is 124 so a lot higher than your daughters. He also had Alagille syndrome which was diagnosed at 8 weeks old. His levels were going down but have picked up again in the last month or 2 even tho his weight gain is better. Out of interest what is your daughters weight and how is she getting on developmentally? Is she crawling or making clear sounds yet? My son is also 8 months and it will be quite interesting to know others who have babies at the same age.

[Dina1986 in reply to Emma-676]

Hi Emma, thank you for your reply.

Doctors said to us that levels can go up and down that's why they want keep an eye on her, it's normal for alagilles, especially first year of life. My daughter is very little, just over 6 kg, sometimes I have feeling she doesn't grow at all, but finally can see that she is growing out of her cloths. She is developing ok, so far so good, crawling and standing up, she also make some noises, but not saying words yet. Quite active and happy bub, just tiny, people keep commenting all the time about her size.

How much is your son's weight?

[Emma-676 in reply to Dina1986]

Ok that sounds great development wise as I know they can be behind, my son is commando crawling, not yet doing it properly and can only stand if I pick him up and lean him against something. He is 6.5kg and almost 9 months now. I think it's quite funny that people think he is super advanced for his age moving around because he looks so small. We r in size 3-6 month clothes now which is nice as we seemed to be in 0-3 month clothes for aages!

[Dina1986 in reply to Emma-676]

Yes, the same people say about my little one, and i know people who have healthy kids the same age 8-9 months who don't crawl yet, so its very good our kids do all this amazing things, great achievement 🏆

When is your son born? My daughter b-day is 26th of February and mine is 17 th, so he should be somewhere close to these dates too 😀

What kind of formula you use? Does he like it? My little one isnt great eater, doesn't put much weight on, has skinny legs, some people even asked me if she is feeding well))) I hate all this sort of comments, if they only could know things we went through and still going...

[Emma-676 in reply to Dina1986]

He is on infatrini peptisorb from the hospital with a feed thickener (carobel) and extra calories (duocal) in it. We have had a nightmare with his weight and been in hospital several times because of it. His Birthday is on the 9th. He also has an NG tube to give him his milk. He used to take some by mouth but now doesn't take any, I'm not sure if he has become wise to the taste as it doesn't smell nice. he will have a very small amount of fruit puree. Is your daughter taking solids?

[Dina1986 in reply to Emma-676]

The formula she is on is disgusting, its called Alfaire, doesn't have any taste, no wonder she is not into it, I will ask dietician about milk your son has, maybe it could be addition to what we are eating. She is partially on solids since 4,5 months old, she eats porridge or baby rice with blended fruits or vegetable puree in the morning and then in the afternoon some protein like chicken or meat puree and vegetables I try mostly make myself, started giving her eggs too, she is very interested in food we eat, so sometimes I give her different things to try. But mostly she is on formula, coz its full of protein and good for her weight. So we try different foods and see how it goes, experimenting is a key as alagilles are very fussy eaters .

Forgot to write that I add some olive or hazelnut oils in to her foods, it makes it easier to digest as they don't have enough bile ...i read somewhere that walnut or almond oils are great for digestion too. Haven't tryed yet

[Emma-676 in reply to Dina1986]

O right that's interesting about the oils, I hadn't heard that one. My son is always interested in what we are eating but gags and chokes on his food. Not sure if it's because of the NG tube in the way. Does your daughter also have a heart condition at all?

[Dina1986 in reply to Emma-676]

Yes, she has pulmonary stenosis, the most common in alagilles . Does your son has one ?

[Dina1986 in reply to Emma-676]

And she also has thing which is called superior vena cava, I almost forgot about it))) it's little different heart formation, they picked it up when I was pregnant, but we didn't know of course that it could indicate to alagille syndrome ...

[Emma-676 in reply to Dina1986]

He has pulmonary stenosis as well and a small hole inbetween 2 of his chambers which hasn't closed up.

[Dina1986 in reply to Emma-676]

My friends daughter had one and it closed itself by the age of 1 . She doesn't have any other desease, but was born with heart defect, which is now gone away . I hope your sons one also will just resolve woth age

X

[Dina1986 in reply to Emma-676]

Have you tryed pumkin and sweet potatoes yet? Babies love it and it has lots of good in it , then you can just mix it with spinach or broccoli as I do and it still tastes great👍

[Declansmum]

Hi Dina, I hope your child is well. I've just received word that my baby has alagille's and it's a bitter pill to swallow. I stare at him all day and my heart breaks. I'm terrified of losing him. He was born 2.13kgs and was small in utero. I fed him EBM for 5 weeks before his diagnosis. He's now on peptijunior and thickened formula. I wonder if alagille babies suffer severe reflux. I read someone's post their child is on thickened too.

There's not much in the way of support here in Australia which is rather sad. I wish both our children get better.

[Dina1986 in reply to Declansmum]

Hi, I am sorry to hear that your child is diagnosed with Alagille syndrome, however early diagnosis and treatment leads to positive dynamics in growth and development of your baby. My daughter is fine at the moment, first year was extremely hard for us, but it does get a bit easier as she grows. Her liver enzymes are still higher than normal and change all the time if she gets sick, also she is very petite and doesn’t eat much, still likes her bottles but in general she is a normal little girl, enjoying life and happy. Itching worsened a little but still manageable. It’s very emotional journey and sometimes you can get very upset but don’t be scared, each case is different and many kids improve with age, try to think positive, I was so upset first year that I don’t remember important moments of my daughter’s life, when she started walking or talking ) just enjoy every moment , it’s going to be ok!

[Declansmum in reply to Dina1986]

Thank you Dina for your positive response. It's comforting to know it does get better. I am hopeful that all our kids grow up healthy.

[Dina1986 in reply to Declansmum]

You are always welcome, I am happy to answer all your questions and share our journey and experience with you !

[Babycj2018]

Hello I’m going through the same but my sons levels are 5.2 to 6.2 which are total and direct bilirubin I too have the same question because he’s my first and he seems to might having Alagille Syndrome we’re just waiting for genetics but all he’s going through indicate he has it

[Dina1986 in reply to Babycj2018]

Hi, sorry to hear that, how old is your son?

[Cesarjr2018 in reply to Dina1986]

Hi, my son is three months and 2 days old!

[Dina1986 in reply to Cesarjr2018]

Bilirubin and other levels can settle down in first year of life. My daughter is two now she doesn’t have jaundice anymore, but she gets very itchy, especially in summer, her sleep is not really good at the moment but she was itch free first year of life. But her feeding improved a bit with time too, she was terrible eater.

[Cesarjr2018]

Thank you very much they just confirmed this morning that he does have alagille but it’s all so scar he’s only three months I’m just praying to god he won’t get worst

[Dina1986 in reply to Cesarjr2018]

🙏🏻🙏🏻🙏🏻 I know, it’s hard, but try to think positive.

[Cesarjr2018]

I do try my best, but it can get hard at times! Was your baby diagnosed with alagille as well?

[Dina1986 in reply to Cesarjr2018]

Yes, when she was 6 months old. It’s very variable, everyone’s is so different. She also has pulmonary stenosis and liver disease.

[Dina1986 in reply to Cesarjr2018]

It’s hard everyday but gets a little bit easier as they get older. Believe me there are much worse disorders than Alagille syndrome.

[Cesarjr2018]

Awwww yes my son does two but they did a liver biopsy when he was two weeks old so he was treated very young my poor baby! And how’s it been for you guys?

[Cesarjr2018]

This has given me lots of hope and motivation thank you very much!