Has anyone else got PFIC and bad itching

[SGJ2013]

[Gemob]

Hi my little girl also has pfic and yes the itching is unbearable. We are now out of all the meds for it and are going in for pebd surgery. It is a really debilitating symptom of pfic and it seems they struggle to help with it. Do you mind if i ask what medications have been tried. Practical things youcan do are keeping the skin cool and well moisturised... Ask your gp for an emmolient cream. Also wearing cotton clothes helps too. I really feel for you as it is horrible to deal with. X

[SGJ2013 in reply to Gemob]

Hi, thanks for the reply. My son is now 15months and was diagnosed with PFIC in December but had been on the meds and stuff since he was only 5 months. he has tried all meds apart from one we have yet to try - I am unsure of its name but its used for people who have taken OD of morphene. we had app with Leeds consultant A few weeks back and she gave us the option of the PEBD. We use a cream from the GP called dermacool which has a menthol in it but not a lot seems to help us. When is your daughter going for her surgery?

[SGJ2013 in reply to Gemob]

Hi there, how is your daughter doing? Did she have the pebd surgery x

[Gemob]

Hi, We are not sure when she is having surgery yet. The medication you are talking aout is Naltrexone. This one only gives relief for a short while then they have to come off it. I have weighed up all our options and decided surgery is the est option long term. I really feel for you as I know the itching is difficult for us mummys to deal with too. The cream we have for our little girl doesnt stop the itch but helps keep her skin well moisturised helping when she breaks the skin. What other symptoms does your little boy have?

[SGJ2013 in reply to Gemob]

Yes that is what it is called, I know it's rubbish that's it's only short term then come off it wish we could just find a solution that helps. Jacks weight gain isn't great, he hasn't put on any weight from about ten months till just last month when he gained 200gram. He doesn't have a great apatite and has broken sleep. What age is your daughter? When did you find out she had liver disease.

[Gemob]

Hi, Sorry for my late reply. We knew Sofia had something wrong from day 1 and she has been on liver meds since she was a few months old but it took us until she was 18mnts to get a diagnosis. She suffers with severe diahrrhea as part of her PFIC so the docs were mislead into thinking she had bowel problems. She doesnt have a very good appetite at all, I think thats part of the PFIC. She also has a rubbish nights sleep she is usually awake about 3-4 times per night usually with itching and diarrhea. She was 2 years old in January. She is also very small for her age. How much does Jack weigh? xx

[SGJ2013 in reply to Gemob]

Yes that's the same as jack with the wakening and the symptoms not good!! Just now at 16 months he is 8.8kg with nappy and vest on. What about Sofia? Have yous been told that transplant will be nessecary at some point?x

[Gemob]

Yeah sofia is 9.7 kg at 2 1/2 yrs old good things come in small packages. Our dr hasnt said anything about s transplant we r taking each day as it comes. Sofias vit levels are rubbish at the mo.... How about jack? Have they mentioned transplant to u? How are his vit levels? Dors he suffer with diarrhea too? Xx

[SGJ2013 in reply to Gemob]

Jack takes the peadia sure plus drinks to try help his weight because he hasn't put on any weight from 10 months till just last months so I asked the doctor if there was something I could get to help. When consultant gave us the option of PEBD she had said jack would need to go for another liver biopsy first to check the scarring on his liver wasnt to bad (because if there is significant scarring they wouldn't recommend it as it prob wouldn't help) so at this point I asked her what would happen then if we went for liver biopsy and it showed that there was a lot more scarring? She said it could lead to liver transplant at some point so that's why I have decided not to go for the PEBD as it would mean jack for another biopsy (and doctor can feel his spleen more now than before so that kinda indicates that there is more scarring) jacks levels aren't great, we go for IM vit injections one a month as well as him taking all the vits daily and he gets his bloods done at same time. Yes jack had trouble with toilet, he screams the place down Everytime even tho it's so soft.x

[Jamiemack81 in reply to SGJ2013]

This is very similar to my sons symptoms. He is 14 months and weighs on 8.4kg. Only puting on 0.3kg since 10 months. Can you advise how Jack and Sofia are now and how their condition progressed. Any help on this would be greatly appreciated

[Hannahailu in reply to SGJ2013]

My daughter has PFIC2 and is not gaining much weight either. She was taking pregitmil formula and now that she is a year old, she takes peptide pediasure as the doctors said it’s easier to digest and hopefully be able to absorb some of the fat. Of course she is on so many vitamins because they couldn’t detect them in her blood work.

[MumtoEleanor]

Hi, my little girl has pfic 2 and the itching was the most difficult aspect of her condition. Sleepless nights, blood everywhere etc. There were times when I just felt we couldn't go on. However, she did respond to rifampicin. We religiously gave it half an hour before tea. When we thought she had become immune to it, BCH increased the dose just to try it before trying something new and that worked. I'm pleased to say the itching has improved no end. She still takes rifampicin as I'm frightened to change anything but our consultant said that some children seem to'grow out of the itching' at around 4 years old. I think it will probably return but hopefully not until adolescence. I do understand how terrible it is but there may be hope! Claire

[Jamiemack81 in reply to MumtoEleanor]

Claire,

How is your daughter getting on now?

Did your daughters itching get better?

Regards

Jamie