Acute Vanishing Bile Duct Syndrome (VBDS)

In September 2015, I became very ill with a sore side beneath my right side rib,
Itchy skin, dizziness and the starting of sores on my skin. First, I thought I had the flu and that I thought I would get over it within a few hours. I didn’t go into work first thing in the morning and later on in the afternoon I started to feel better so I went into work. I was working out of town at the time and by 4pm I called it a day. I still felt ill and ended up throwing, diarrhea and for the next 2 days in the hotel room. I returned on the Friday from the week away to hopefully rest up and be better for the next, however this was not the case.

I decided that I should go back to work even though it was out of town I thought this would have passed. When I returned home on that Friday, my wife took me to the emergency room as by this time I had yellowing of the eyes and skin. Once there the doctor on call took blood samples and urine samples. When the results came back the doctor thought I had gallstones or possibly a viral hepatitis. They asked the normal question due to the jaundice and in the end decided to admit me to keep on eye on my blood work because they still didn’t know the reasoning for the high white cell count and the high bilirubin count. The next morning they ordered a CT scan and an ultrasound on my gallbladder and liver. Later that day a new doctor came in and said that I had a viral hepatitis and that it needed to run its course, so they sent me home with orders to get more blood work done the next morning.

The next morning I went in for the blood work and went home. That afternoon I received a call and was told to come back into the hospital. Once there a new doctor examined my blood work and ordered more. When the results came back he then admitted me to keep running blood work through the night to see if there was any changes. In the morning hepatitis was ruled out and so were the gallstones. The doctor basically said that all signs pointed in the direction of a blocked bile duct with in or around the liver and that I was a mystery. He referred to me a local Nurse Practitioner as I did not have a family doctor and needed one to do the referral to a specialist.

After about a month’s waiting to get in to see the specialist, I received my appointment. Once there the specialist ordered an MRI, chest x-ray and more bloodwork. Within 2 weeks of the test it showed no blockages or abnormalities with in the liver, bile ducts, pancreas and gallbladder. I was then ordered a liver biopsy.

The results came back about 2 weeks after. I was then told that I had lost over 75% of the bile ducts in my liver and that I had what is called “Acute Vanishing Bile Duct Syndrome” a very rare disease.

Throughout my conversations with the specialist and my Nurse Practitioner, my liver will never recover the loss of the bile ducts, once they are gone they are gone and that body will have to adjust to having what is left working. At some point the reality will be that I will have to have a liver transplant, but no one knows exactly when that could occur. What I have been told is that the body could function as is for a while but that would determine the amount of damage that is continuously done over time. There is no known cause of the “Acute Vanishing Bile Duct Syndrome” or any known cure. Once assumption that is being discussed is that it could be a combination of over the counter medications and prescribed ones as well. I have a whole list or medication and over the counter medicines I will never be able to take.

Throughout this nine month ordeal it has taken a mental and physical toll. I guess the unknown of not knowing where this disease is going concerns me and my family, and that no one seems to know anything about this disease or how to cure it.

I thought I would just tell my story do to fact that this is not a well-known disease and it is very rare. If this can benefit someone now or in the future I am happy that I could post this here.
Jason, Fort Frances, Ontario