Congenital hepatic fibrosis

Hi

We have had numerous tests and I guess I need to accept he has this disease. I was trying to ask the constant questions like ,

1 will this be terminal for my son and 2 can they have a transplant in the future. He's fairly well at the moment. He's tired got muscle aches and head aches. Does any one else know anything about this. ? Gutted for my 12 year old son.

Elaine. X

19 Replies

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  • It must be awful for you. I think Children's Liver Disease Foundaiton have a leaflet on congenital hepatioc fibrosis. Might be worth starting there?

  • Hello Elaine,

    My 2 year old daughter has congenital hepatic fibrosis, her liver function is fine but her problems are associated with the portal hypertension - enlarged spleen, varices. In September she had to have her first banding to treat oesophageal varices. We've really struggled with not knowing what to expect in the future - no one seems able to tell us how it will progress. We know that the worse case scenario is that she may need a liver transplant one day, but we don't know how likely that is. We've just had to try and accept that we'll just have to deal with any problems as they arise. Isobel is coping really well with hospital visits and taking her medications - her 4 year old sister is struggling more and has become quite anxious since our last stay in hospital.

    I think CHF is quite rare - you're the first person I've come across with a child with the same diagnosis.

    Good to speak to you

    Lexi

  • Hi lexi. Conor was completely well till may this year. I think I will definitely get the leaflet. I feel exactly the same way as you. It's so hard being in hospital and being a parent to other children. I am happy to support you and share any information I get. It can't be that rare if there's a leaflet lol.

    Best wishes to all your family.

    York mum. Elaine. X

  • Lexi.

    What medications does your daughter take. ?

  • Sorry Elaine - only just seen your question, Isobel takes Urso, ranitidine and propranol. Is Conor taking anything?

    Lexi

  • Hi I live in Scotland and have this illness and so does my sister there are only 3 ppl in scotland that have it .I am 34 and my sister is 33 I ve grew up with this from age 11 and she 21 . We both have different things wrong she has Portail Hypertention and I dont. I left a comment on pickle mum if you want to read this but you can contact me if you d liked to here anymore.

  • It's great news to hear you are so well. I hope Conor stays so well. I have a couple of questions.

    1. Is there anything you can do to help this condition. Ie homeopathic remedies Etc.

    2.did they say you could have a normal life expectancy. ?

    Many thanks.

    Elaine.

  • Hi, my daughter has suspected CHF. This was discovered during a biopsy performed while removing a Choledochal cyst. She is now almost 7 months old (almost 8 weeks post op) and is in good health.

    She had her first scan and nothing showed up.

    Can I ask how old you were when they discovered CHF with you and you sister?

    We meet with doctors next week up from London but don't know what to ask other than the obvious (long term effects).

    Any help or comments would be appreciated.

    I'm from Aberdeen.

  • Well the consultant told me that people with this condition lead relatively normal lives. Except they may have to have varisis banded. X

  • That's good to hear. I hope that is the case. We will hopefully get more info on Tuesday.

  • Hi Elaine, My daughter was diagnosed with congenital hepatic fibrosis, cystic kidneys, portal hypertension and varices on the gullet and stomach back in 1981 when she was only 18 months old. She is now 32 years of age, so if there is anything you would like to know, I hope I am able to answer some of your questions.

    Johnboy.....

  • Hi Johnboy, my daughter is 3 and has exactly the same problems as your daughter - would you be able to tell me a bit more about how it has affected her life and what sort of treatment/follow up she has now? Is she able to have children?

    Its reassuring to hear from people who've been through the same thing.

    Lexi

  • How is your daughter doing? This board is the 1st I've found other people with CHF. My 3 year old daughter also has Congenital Hepatic Fibrosis of the Liver. She has an enlarged liver and spleen, frequent nosebleeds and is very small and skinny for her age.

    How is your child's condition and what symptoms does she have?

    Her scarring is getting worse and we aren't sure what to think about her condition and future. There is no treatment or cure from what we've been told.

  • Hi, generally my daughter is fine, she's 6 now. She's growing well, doing well at school. We go to King's every 6 months for outpatient appointment and scan, and have admissions usually yearly to treat varices. She hadn't had any bleeds due to varices but she's on a preventative treatment programme. They think her portal hypertension is getting worse as her spleen is increasing in size so increased her dose of propanolol so we will see how effective that is. When first diagnosed isobels liver was quite hard but that has improved, i think since they started her on urso. Isobel gets a little pain and isn't as active as her older sister, but that may be due to personality. Is your daughter on medication? Which specialist hospital are you under? It was very hard in the beginning, not knowing what the future would hold, I think we're learning to live with it and not worry too much in between appointments. All the doctors we've seen have different opinions, we just go on how Isobel is. We were told in the worst case scenario that transplant would be an option, but so far Isobels liver function is fine so that's been a relief.

  • It's so reassuring to know there are people doing so well. I am hoping Conor's condition doesn't get any worse. Is there anything you can think of which helps the condition.? Conor is currently well and has started playing outside again.

    Thanks for getting in touch , I appreciate it.

    Elaine.

  • I know this topic is several months old but I thought I should reply as I have CHF and I am 33 years old.

    Not really sure what to say to be honest as my experience of the disease is pretty good, (I am sure my parents would disagree) nor am I sure where to start so I'll just type whatever comes into my head!

    I remember it vividly even though I was only 5 years old. My parents were out shopping and had me with them, and I was caught short and needed a poo! My dad took to the loo and whilst I was there he noticed that my stools were thick black and tarry. As he was registered nurse (albeit mental health), he immediately new something was amiss and took me straight to the GPs. I was then admitted to the Children's Ward in Newcastle's RVI hospital and here it gets a bit blurry... I remember getting a blood transfusion and being very bored.

    Fast forward 12 years and I am 17 years old (despite having CHF it had never bothered me, it did not stop me from playing with the other kids or doing what they do and I had a perfectly normal childhood) and this is when I had my first major running with oesophageal varices and the danger I present. It was a normal day at college, only I didn't feel quite right, very tired and out of sorts all day. Zero appetite and severe lethargy. I eventually got home and into bed, then around about 7pm in the evening I had an urgent need to be sick - which I was - and I vomited congealed blood, loads of it. It was horrific, pint upon pint of the stuff. It must have been very frightening for my parents (when it's yourself and you're 17 you just sort of brush it off!) and they called an ambulance, I was taken into hospital. More blood transfusions, more banding and then home.

    Unfortunately I was only home a night when one of the bands became loose and I started to bleed again. Another ambulance and this time I was taken to hospital in a helicopter. I was patched up again and sent home.

    Then when I was in my 20s I noticed I just could not keep up with my friends as much, I was always the first to complain about being tired, the one with the worst hangover, the one who could simply not be bothered. This went on and on until I had enough, and to cut a long story short, my then girlfriend forced me to go and see the doctor. I requested to be seen by a consultant because I knew something was a miss, don't ask me how, I just knew.

    So I was sent to the Freeman Hospital in Newcastle upon Tyne, they did an ultrasound. The technician said she'd be back in a minute and she came back with he consultant. Straight away I knew something was up... HCC, liver cancer. I am the only person that they are aware of with CHF that had developed HCC as a result of the CHF.

    By the time I was 31 I had developed cirrhosis, cancer, had a blocked portal vein, and maybe two months at the most to live. I was stuck on a transplant waiting list and then the urgent list, a week later I had my donor, but I was so, so, so close to death I was ready for it.

    Anyway... over all I'd say your daughter will live a normal life, she may get tired easier, she'll have some *REALLY* bad hangovers but if like me, she was born with it, she'll not know anything different and continue to live her life to it's full potential. Because you do - you don't know what it is like to be "normal" as it were :)

    If you or anyone else has any questions about CHF and my experience of it please just ask!

    Dale

  • I know this topic is several months old but I thought I should reply as I have CHF and I am 33 years old.

    Not really sure what to say to be honest as my experience of the disease is pretty good, (I am sure my parents would disagree) nor am I sure where to start so I'll just type whatever comes into my head!

    I remember it vividly even though I was only 5 years old. My parents were out shopping and had me with them, and I was caught short and needed a poo! My dad took to the loo and whilst I was there he noticed that my stools were thick black and tarry. As he was registered nurse (albeit mental health), he immediately new something was amiss and took me straight to the GPs. I was then admitted to the Children's Ward in Newcastle's RVI hospital and here it gets a bit blurry... I remember getting a blood transfusion and being very bored.

    Fast forward 12 years and I am 17 years old (despite having CHF it had never bothered me, it did not stop me from playing with the other kids or doing what they do and I had a perfectly normal childhood) and this is when I had my first major running with oesophageal varices and the danger I present. It was a normal day at college, only I didn't feel quite right, very tired and out of sorts all day. Zero appetite and severe lethargy. I eventually got home and into bed, then around about 7pm in the evening I had an urgent need to be sick - which I was - and I vomited congealed blood, loads of it. It was horrific, pint upon pint of the stuff. It must have been very frightening for my parents (when it's yourself and you're 17 you just sort of brush it off!) and they called an ambulance, I was taken into hospital. More blood transfusions, more banding and then home.

    Unfortunately I was only home a night when one of the bands became loose and I started to bleed again. Another ambulance and this time I was taken to hospital in a helicopter. I was patched up again and sent home.

    Then when I was in my 20s I noticed I just could not keep up with my friends as much, I was always the first to complain about being tired, the one with the worst hangover, the one who could simply not be bothered. This went on and on until I had enough, and to cut a long story short, my then girlfriend forced me to go and see the doctor. I requested to be seen by a consultant because I knew something was a miss, don't ask me how, I just knew.

    So I was sent to the Freeman Hospital in Newcastle upon Tyne, they did an ultrasound. The technician said she'd be back in a minute and she came back with he consultant. Straight away I knew something was up... HCC, liver cancer. I am the only person that they are aware of with CHF that had developed HCC as a result of the CHF.

    By the time I was 31 I had developed cirrhosis, cancer, had a blocked portal vein, and maybe two months at the most to live. I was stuck on a transplant waiting list and then the urgent list, a week later I had my donor, but I was so, so, so close to death I was ready for it.

    Anyway... over all I'd say your daughter will live a normal life, she may get tired easier, she'll have some *REALLY* bad hangovers but if like me, she was born with it, she'll not know anything different and continue to live her life to it's full potential. Because you do - you don't know what it is like to be "normal" as it were :)

    If you or anyone else has any questions about CHF and my experience of it please just ask!

    Dale

  • It turns out he didn't have CHF and is now awaiting a transplant. Still don't know what it is. X

  • I'm sorry to hear that, I hope you don't have to wait too long and that he makes a speedy recovery. Thinking of you x

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