My 2 year old daughter has congenital ... - British Liver Trust
My 2 year old daughter has congenital hepatic fibrosis - does anyone else have experience of this disease?
Dear Picklemum,
I am sorry to hear of your daughter’s condition.
Congenital hepatic fibrosis also known as CHF is a very rare condition, only affecting one in 20,000 people. The Trust has a website page explaining a little about the condition; diagnosis and treatment: britishlivertrust.org.uk/ho...
Alternatively, as symptoms mainly occur in new born babies and early childhood the Children’s Liver Disease Foundation may be able to help you further: childliverdisease.org/
Please post again or ring our free helpline for further information and support.
Kind regards
Sandy Forsyth
Health Information Manager
Thanks Sandy,
Isobel seems to have an unusual form of this disease in that she has the dominant form of polycystic kidney disease (which normally is associated with adults) rather than the recessive form which affects children. People with the dominant form don't get hepatic fibrosis, so she's a bit of an anomaly.
Isobel has also just had her first lot of banding for oesophageal varices at Kings, I know she'll have to have this done again in the future. Are there problems associated with repeated banding?
I have been in touch with CLDF, but haven't found anyone on their forum with the same diagnosis.
Picklemum
Hi,
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
Hi, I know this is old but I thought I should reply. I have CHF and didnt find out until I was 40. At 4 I had my spleen removed because it was so enlarged. At 12 I had stomach pain due to leasions, it was treated as an ulcer. At 17 I started having bleeding when the varicies in my esopagus would rupture. At 38 I needed my first transfusion. That led to the diagnosis at Scripps. I had a mesocavel shunt installed and since then I have been normal. No meds at all. Of course since this happened so long ago and such a rare condition it took a long time to diagnose. All in all it has been a normal life. The Bleeds only occured every 3 years or so. I wa told I wouldnt live past 40, proved that Dr. wrong. I am now 60. I believe the shunt is the best option for portal hyper tension. The banding is a temp fix. There are better shunts but I had to have a specific one because my spleen was removed. To date I have had no kidney issues so I may be a bit different since the two are usually tied together. In short CHF has not altered my life other than a greater relationship with Christ
