Choledochal cyst/biliary atresia

[Victoria2]

Hello, My name is Vickie. I was born in New Zealand but have lived in both England and New Zealand over my life time. Your very lucky in Britain to have an excellant health service for rare diseases like childhood liver disease and even adult liver disease. Although we have had a liver transplant centre in Auckland for more than ten years there are medications you can not get in NZ due to the strict critieria of the government agency that recommends funding for products. My care has gone down hill with the introduction of community based health care ten years ago which is about to be brought in to your country. I have even been told that NZ can not afford chronic health care.

I was born with a choledochal cyst/biliary atresia 53 years ago and was lucky enough to have a general surgeon who had had to think on his feet during World War Two otherwise I woud not be here. 20 years later at the Royal Free Hospital in London they said he was 20 years ahead of his time and babies still die born like me. There are five types of choledochal cyst and all of them still have a join to the duodenum but mine didn't hence the biliary atresia. The doctor fashioned a tube from a piece he cut off the gallbladder when I was 3 weeks old. Unfortuately, I had bouts of cholangitis through out my preschool years and was in and out of hospital. Finally, I had a second op age 4 which I remember.due to the first tube narrowing. I have had doctors argue that I didn't need the 2nd op and others argue about my insistance that I had a combination of the choledochal cyst/biliary atresia--even some top docs have never heard of it, though they should do now, they all thought I just had a choledochal cyst as my baby notes were thrown out by the hospital but I have since found there is such a condition. I plan to write to the Royal Free Hospital and see if they still have my notes and can confirm it as they would not have said 'babies still die born like this' you don't die of choledochal cyst as a baby that I know of, you don't usually get the amount of cholangitis I get either. We still have a baby book with the drawing that my surgeon did when I was born which also proves the diagnoses. I was well for 9 years until age 13 when cholangitis infection started again.

I had a 3rd op at the Royal free as I have said as there was no one in Nz able to do so at the time. I had complications of ascities, gangrene in my scar, pancreatitis and was sick for some months afterwards with a salt free diet and bed rest for the ascities. I had 2nd primary biliary cirrhosis. I have had a number of miracles happen in my life and the first was the birth of my daughter when I was 24. I was the 23rd woman in the world to survive with 2nd degree cirrhosis, my blood clotting time was 13 mins normal is 3mins. I did haemorrage but obviously not as bad as they expected.My daughter was lying on the cord though and there was nothing they could do to help her , so we're lucky she is ok.The 2nd miracle was at age 29 when I had my 4th op which was roux-en y surgery so 6 hours , all signs of cirrhosis was gone. Doctors will still try and say I may have been misdiagnosed but it doesn't account for the 13 mins clotting problem when my daughter was born if that was the case. There have been a few other cases of patients with 2nd cirrhosis reversing.

I was well for 4 years and than started with cholangitis and bouts of severe pain without temperatures which within two years became pain every single day 24 hours a day. For 15 years there had been no diagnose and very poor treatment due to the community health objective in NZ. I was constantly told I could not go to hospital in severe pain ,I would have at least two days a week like that and sometimes bouts as long as two months where I could hardly walk. I had very few tests, a PTC test was put off for two years and I had to write to Auckland Transplant centre to see if they would help. A year later I had the test and two hepatic ducts had narrowed so I quickly got an op. I was still in pain everyday and food and movement made it worse. The pain clinic in Nz advocated pushing through the pain to live normally which meant increased pain for me and a huge strain on me--I will not do that now. The pain is just like a cholangitis infection. I went back to the Uk as my daughter was there 4 years ago and they did two ops, one was the wrong one as I made a mistake and told them it might be chronic pancreatitis --the pancreatitic duct was slightly narrowed but when they went to do the whipple ( a huge op) it was clear that was not needed, so they shortened the loop of bowel that replaces my main bile duct. I was in hospital 5 weeks as my bowel stopped, I was on TPN.I had a bad time with pain and one very bad situation with a nurse who would not get the anethetist up to put in extra medication as the block kept slipping. She was banned from the ward after leaving me in pain twice for 8 hours each time. Incrediably cruel. She also treated others on the ward poorly though not as bad as that. I had a second op a year later as they thought maybe the food was going up in to the liver , so lengthened the loop to half a metre. (In the end this was the right op for what was wrong with me but perhaps too late after 15 years of unrestricted bacteria growth.)

During this time I was in contact the Auckland Transplant Centre letting them know what my results were and what was happening, the doc there came up with several ideas but has finally worked out I have bacterial overgrowth of the roux en y loop which is rare however it should never have taken 15 years of severe pain to diagnose this and I think my treatment has been a disgrace. I am very grateful to both my doc in Auckland and Kings for at least trying to work it out and at least Kings let me go in to hospital. I am now allowed to go in to hospital for cholangitis back in NZ (I had to go back as my Father died), because I need the second to last antibiotic available as the others no longer work. I am on 4 times the normal dose of one oral antibiotic and 3 x the other otherwise I get an increase in pain. I can't eat meals anymore it is just too painful for me to keep coping with that as morphine at 30mg no longer works. I have anti-inflammatories to help with pain but tablets don't work so its susposteries. So I live on liquid food and try not to do too much, mainly sit on the couch and try to write stories and paint. I find it hard to lie down at night on the mattress, can't wear a bra and have problems with bucket seats in cars due to pain. I hope I will get my op next year and that it works. They will join the liver straight to the duodenum which I can't find on the internet so I am told by other docs that its specfic to my situation. I will be glad when I can work again as I'm worried about having enough savings for retirement in the future and there are lots of things I want to do.

Vickie

[majidshehzad]

Hi Vickie, its me Majid, read your story, hope things go the right way and my prayers are with you for your speedy endless recovery following operation. Personally I have noticed following me being the liver donor and my daughter being the recipient that so far chronic disease management is very good in England.

[Victoria2]

Hi Majid,

yes it is very good, a good place to find out about the changes to the UK medical treatment is the Kings Fund site, as they advice government on policy.The world has a big problem with an aging population so all first world countries are taking on community based health care an idea that comes from the USA to keep their Medicare costs down. In New Zealand as it probably is in the rest of the world ,children take priority so chronic care is only cut back for adults. However, as parents it's important that people take note of what is happening to the NHS as obviously children grow up. I know that NHS hospital doctors have protested and asked for safety nets--not the right words but can't quite think today lol. We have already been told by the cancer society in NZ that not everyone will get cancer treatment in the future ie 20 years time when the biggest group of baby boomers retire. Some ideas in community care work well ie people who won't take their medication for say heart conditions get followed up by G.P nurses. G.P's have control here over patient care and they expect to do everything, at one time they were telling people to drive down to the practise if they had heart pain and than they would call the ambulance for hospital but that has changed since I have come back as I expect some people have died with that type of over control. On the phone now is a message to call an ambulance if there is an emergency.

I have a friend who's husband has very bad diabetes and he was always in and out of hospital but the community care programme have come in and stablised his lows so that he doesn't use the hospital as often. These things work fine for common chronic conditions but not in situations like mine which is rare. My hospital doctors weren't even looking for what the cause was of my condition and were just waiting for something to happen for years because I didn't fit the average case. I wrote to the Health Minister about my poor care and have been advised to put in a complaint with the Health commission which I am still thinking of doing but I don't want the doctors blamed as I think it is the policy that is at fault. Maybe I should for other people who have something unusal that is being ignored. My current hospital doctor has double the normal amount of patients ie 2500 instead of 1250, so I can't get in for 6 months and I am very ill and should be seen every three months. In the Uk I would be seen 10 days after a hospital visit, in NZ your to go to your G.P who would not be able to get you an early appointment with your specialist unless you are dying.

That al sounds very depressing I know, I'm hoping the UK won't get as bad as here as they have more doctors etc and a better service, more money but it's dependant on the Government and what they want to do and how much money they have.

Anyway, thanks for the kind thoughts.

best wishes Vickie