PFIC 1

I have just joined this group in an effort to help others with children diagnosed with PFIC 1. My daughter is going on 3 years post liver transplant and we still have ups and downs. Should anyone have questions or has a child that has PFIC 1. I would greatly love to hear from you. Thank you.

[Trayee]

Hi

My son has undergone liver transplant for pfic1.Post transplant he is having fatty liver which is very disturbing.His AST level are around 87.

Rgds,

Hari

[Aly123]

Hiya, my daughter has pfic type 1 and had a transplant at 14 months old, it's now been 5 1/2 years since her transplant and she is doing fine apart from her diahroea which is a side effect of having a transplant with pfic1. Obviously you do have good days and bad days but you learn to live with it. All I can say with experience is that things do start to get better

[Trayee]

Oh right,its good to hear that.how is the weight gain after transplant.my son has just put on 2 kgs after 6 months.doctors say we need to wait

[Aly123]

Hi trayee,

My daughter has an ng tube through which she still has an overnight feed using a feeding pump, she has been on this since she was 8 months old! She is getting her extra 500 calories per day with this! If she didn't have this, I think she wouldn't really put on much weight! Which hospital are you under? You need to have a word with the dietician! How old is your son and how much does he weigh??

[Trayee in reply to Aly123]

What's an Ng tube?my son ,Veda vaishvik,is 2 & half years old now and weighs 8kgs.v R in India,& getting treatment in global hospital.whats ur daughter name and how much does she weigh now?how is her food intake?

[Aly123]

Hiya, my daughter had transplant in uk and that's where she is having treatment, ng is a tube which goes through her nose and into her stomach and her milk and medicines go directly through that! Her food intake is just average maybe like another child of same age but she has diarrhoea problem so it tends to pass through her system very quickly!! She is nearly 7 years old and is about 19kg.

[Trayee]

Hi

Sorry fr so many questions but eager 2knw how R things with other kids of same problem. Hw abt d schooling of ur kid?

isn't it difficult 2manage Ng tube. Doesn't she resist it. My kid ws diagnosed with steostasis(fat in liver cells) aftr transplant. Doctors say its common wth kids of pfic1 . Is tht so. Hv U gt any other problem than diahroea!Hw R her ast levels.

[Aly123]

Well, she is doing fine in school, she has never had a problem with her ng tube, since she was a baby she hasn't touched it or pulled it out! It's only a problem when I have to change it!! I personally have not really heard of anyone here with fat in liver cells after transplant but I can't say for sure, this is certainly never mentioned in appointments! Other than the diarrhoea there are no major problems! Just the usual when the immune system is weakened. I suppose every country has there own ways to deal with each illness but the main thing is that if you have a good doctors team then you've nothing to worry about!!

[Trayee]

Appreciate ur time for replying to our questions, I think v had a good inter active chat.hope to have a continuous rapport going forward & share our experience.BTW we have put him to play school last week!!!

[Hidden]

Chloe also has a fatty liver now due to steroids but we are in the process of correcting it now...

[Trayee]

Hi even my son has undergone a liver transplant (July 2013) & he was diagnosed with fatty liver within a month after transplant. Doctors said there is no medication for this. V hv 2 wait n c hw things progress. V R really worried. Hs stool color is also pale or grey in color. Do let us knw whts d treatment U R giving fr fatty liver. wld b eagerly waiting for ur response

[Aly123]

Hiya pficcure,

Just been reading about your little girl, which hospital are you with at the moment? How did they diagnose colitis? Is it just because of the diarrhoea problem? Sorry I'm asking you so many questions, it's just that the hospital should have told you before the transplant that diarrhoea would be a problem, this is due to the fact that this PFIC doesn't only affect the liver but it can also persist in the gut, which is what is causing the diarrhoea. Does your daughter take anything for diarrhoea?? I hope things start looking more positive for you and I am sure they will!! Xx

[Aly123]

Hiya, my daughter is on Imodium and also codiene, for the diahroea! I know the difficulties of this especially when you are out somewhere and your child needs the toilet, you have to basically drop everything and run for the nearest loo!!! Even nappies don't work because it is soooo watery!!

[Trayee]

Hi

Really upset 2 know that there is no cure for these little kids. Depressing 2 c d kids suffer even after d major surgery d transplant. Is d girl in d snap Chloe?? Is she d only kid!!

I wld like 2 know how d siblings would b. Is there any treatment b4 planning for a second kid.

[Aly123]

I think every situation is what you make of it!!!....only when you go into hospital you see other children who are a lot worse than your own!!.......we are lucky to even receive a new liver as here in uk there is a shortage of donors! Even though there is no cure for this disease, the quality of life after transplant is a lot better than quality of life before transplant and yes there are many hurdles along the way but you need to deal with each one as It comes and then carry on!..from my experience things do get better with time!

[Aly123]

Hi, they have never checked her colon, maybe it's because this is what they are expecting, at first it was about 8-9 times a day but slowly and gradually as they get older I think they learn how to control themselves! Now it averages out at about 4-5 times a day and each and every time it's like water gushing out!!

[Aly123]

Aaawwww!!!! So sorry to hear this but don't be disheartened, I bet she's little fighter and you will all get through this!! Which hospital are you at? It's gonna take a little while for things to sink in properly but hey, like you say, things will be a lot better afterwards, you just need to stay positive through all this! Did they give Chloe any cholestyramine? It's a powder you mix with water and its supposed to reduce the bile salts! How do you give Chloe her boluses, does she have an ng tube??

[Trayee]

Oh god!!! sorry 2 hear that. But s b positive as U R. Do nt loose ur confidence. Sure she wld b all right. Is it d ascending or descending or d whole colon.?What would they replace it with? i would say chloe is lucky 2 have u as parents!! Aly123 R U using cholestyramine for ur kid? My kid was asked 2 take that but he never had it. Its dificult 2 force him. Is it worth of forcing d kid n gv it. If so i would make a try.

[Aly123]

Trayee, with cholestyramine it's a powder and ur supposed to mix it with water but I think u can mix it with other food as well for example soups. It comes in orange flavour as well but see if u can mix it with other things and then give it!! Don't force it as he will be sick! Try it in sum juice!

[Trayee in reply to Aly123]

Hi aly123

v do have cholestyramine with us(the orange flavoured 1) v tried 2 give it in different forms but in vain. My kid identifies even a small change in taste n refuse 2 take it. Also doctor have advised 2 give it with atleast 1hour gap b/w feed n other medicines. Do U also follow d same!! What's your kid studying now?

[Aly123]

Hiya all!

From what I can gather, I think every different hospital/country deals with things differently! My daughter has had ng tube for 6 years now, I did ask about the g tube which goes through the side of her belly but the consultant said they don't give them to liver patients as they can cause more problems!

Maybe it's just the way consultants are taught how to deal with issues! Pfic cure, which hospital are you at by the way??

[Trayee]

Very sorry to hear that. My throat choked after reading your message. I can understand what agony you R going through. But if doctors say she would have a quality life after that then go ahead with d same positive approach. Wish this would b an end to chloe struggle and she would have colorful life ahead. How would you b managing d bag. Should she require gtube even after surgery. Would there b any restrictions in her diet?

By d way chloe is very very cute!!

[Trayee]

Hi hwz Chloe dng nw?

[Pfic1_mum]

Hi my son has pfic 1 already has a internal biliary diversion, suffers server scratching, does not sleep, he struggles with weight gain he is 2 and weighs 10kg he has a feeding peg to help give more calories since having a tube he does not eat, I am winding if it's time for a transplant? As he is in so much pain, doctors suggested. A transplant months ago for better qaulity of life I'm so worried if it's left his scaring will worsen and he will end up with liver cancer, please help I am in australia and my some is currently the 3 with pfic1 little information here

[Hidden in reply to Pfic1_mum]

Pfic_mum, how is your son doing? I see you posted a month ago? You can hardly find any information about PFIC 1. Did you decide on transplant?

[Mamabear2 in reply to Pfic1_mum]

Hi, hope your little one is doing okay. Did you get the transplant? My son is 2 as well he is always itchy, no medicine helps.😢 he recently had a biliary diversion surgery. Which helped his labs but not the itching, And barley sleeps.. He hasn't been formally dignosed with pfic but they did find a single gene for pfic 1 during a live biopsy and are treating him as such. Was your son helped by any medicine? Also if you did have the transplant has it helped? It's so hard seeing him so uncomfortable all the time

[Pfic1_mum]

Hi he is okay now. Had a really bad month, head cold, oral herpes, and no weight gain. Itch is frustrating! I'm seeing our specialist next week to talk about a transplant, Jaxon liver function goes up and down, they think Jaxons problem could be due to his body still having way to much bile even after his diversion, bile salts level use to be high 577 to 800s then it goes down to 28, and back up. I think they want to give him a gj tube instead of just the peg in the tummy so he can be fed aswell as have bile drained hopefully it helps, he does not sleep at all! Maybe 2-3 hours a night. I honestly don't know what to do! As he seems okay now full of life apart from itch and not sleeping but then when his down it breaks my heart to see him struggling to make it through the day. What is your thought? Yes

I know I only know one other mum here and she has a daughter with pfic2 thank you for your reply

[Hidden]

How old is he now? Goodness, I remember those days, nights..they all ran together. Chloe was the same with the itch even after having done the internal and external bile diversion. Itching never ceased till transplant and that has its ups and downs itself but the child has no quality of life due to the agonizing attempt to relieve themselves of the itching. Have you been to the specialist yet?

[Hidden]

Still waiting to hear from you Pfic_mum. Worried about you and Jaxon. Please give me an update.

[Hidden]

pfic1_mum you can Email us at anytime and let us know how you are.

childliverdisease.org/

Regards

Tom