PFIC 2 / PEBD

Hi All,

My name is Ashley. This is my first time here. My 15 months old daughter has PFIC 2 and is recommended to have a partial external biliary diversion drain (PEBD). I am looking to get in touch with any parents with children who have already undergone this Op. Or anyone who has a child with the same condition to share some info... Hope all your little ones are well! Thank you in advance.

Ash xx

8 Replies

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  • Hi. My daughter too has pfic. She had a pebd 2 years ago and other than once when she had a few complications we have literally never looked back. It was the beat decision we made. Feel free to message me if you have any questions xx

  • Hi Gemob, glad that ypur daughter's doing well now with the itch and that the pebd decision was the right one. Also many thanks for your prompt reply and for being open to sharing. Really appreciate it very very much. :) May I know how old your daughter was when she had the PBED? Also which type of PFIC does she have? How bad was her itching? And after the op, how was her itching in comparison? Did she have to undergo another op, due to complications or what had to be done? And was the decision to go ahead with the op based on? Her liver readings or doctor's recommendations or because the itching was that bad? Thanks.

  • She was 3 when she had the surgery. They recommended it because her itch was so bad and nothing worked to ease it any. They think she has pfic 1 but we are not 100% sure. Our docs don't like to label it. She had her second surgery because where the section of bowel was attached to her hall bladder healed up and her itching returned. Her itching was so horrendous before she never played ate it anything before now her itch is none existent. She does have other problems as well as her liver due to her genetic mutation but from a liver/itching point if view we have literally never looked back xx

  • Hi Ashley, I don't know if you are aware of Children's Liver Disease Foundation. We support families who have a child with a liver condition, to find out more go to: childliverdisease.org/ - with best wishes - Jacquie

  • Hi. My son has PFIC2. He is 8 and had his partial biliary diversion almost 4 years ago now. He does actually hate it now he is older due to the problems it sometimes causes but it has allowed his itch to be more manageable over the last few years. Just been listed for transplant as his liver is not very coping well anymore. Let me know if you have any questions xx

  • Hi Ebony123, many thanks for your sharing. Sorry to hear that your son's liver isn't coping well anymore, but glad that your son has done well so far.

    You've mentioned that the pebd sometimes causes problems. May I know what are these so-called problems? Did your son have any post-surgery problems or complications? How bad was your son's itching before the pebd op? And how much has it improved after the op? Has the itching remained constant throughout the past 4 years or has it deteriorated over time? Does your son need to take any medication for the itching after pebd?

    Did your son have any weight or thriving issues? My daughter didn't thrive and stopped eating or drinking altogether since she was 9 months old. She has a naso gastro tube.

    Thanks! xx

  • Hi. Before the op, Adam had just started reception in school and was finding it hard to concentrate on anything because of the itching and also falling asleep in school as not sleeping well at night. We tried all the itching meds and he has always remained on itching meds and still wakes up in the night with it. We put a rotating fan on the wall in his room to keep him cooler and tried everything with his skin but as they get older they can get out of any sleep suit themselves but at least he cuts and files his own nails now! :) The months post op is when we had the most problems as got some infections in the wound which took a while to heal and also Adam started to lose too much bile to the point of dehydration. I believe he was the first this happened to and so is not usual. Taking care of the wound and it leaking at night (when he itches the dressings away etc) or at school are the worst things. However, once we got used to it all it really improved his life for a good few years. I think the itching goes completely with some children but it has always been still there with Adam, just not to the same awful extent. The diversion is getting harder to cope with now as it still stops him staying overnight somewhere else (still wakes up itching and we often had to do stuff with his dressing or bile draining overnight), it makes him feel different to his frineds and he really hates it when it leaks in school. I think the diversion is having less of an effect now in terms of itching but I don't think we are back to the level he was at before the op.

    Adam has never had a gastro tube and is an extremely fussy eater but eats bread rolls and drinks milk (which we mix fortini into) like it is going out of fashion! He is short and small (except for his abdomen) for his age but not unusually so when compared to his class at school. Am hoping for a post transplant growth spurt!

    I hope that covers all your questions! The main thing I felt post op was that I wasn't really prepared for how to care for it all when we got home and thought we didn't have very good advice from the ward before discharge, but sounds like you are getting prepared already, which is a much better idea!

    xx

  • Hello

    My son is two month old , and he's diagnosed biliary Artesia , He's a premature baby and weak by health. By birth he has destro cardia and situs inverses aswell.

    We are very much worried as parents, as we think that surgery for such weak baby will be a great risk , but doctors saying that surgery is the only option. Because if we not going to take this step ,may be this disease cause any other problems like harming kidney , or any other organ , so it's good to take this step .

    Believe in God and he will be with you . Save your child from any other complications

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