A significant worsening of the symptoms was noticed about ten years ago (while working in the USA), although they had been present before, but were never so bad, nor did they last so long. On visiting my GP, the familiar lines of 'liquids only for 24hours, etc' were uttered. However, as the symptoms persisted, another visit resulted in some blood tests. A phone call from my GP revealed that my Liver Function Tests (LFT's) were abnormally high.
The GP instructed me to immediately stop taking all vitamins and medication and informed me that the tests should be repeated in a few weeks. LFT's at that time were lower and steadily decreased over time. Unfortunately, the same problems arose at a later date, and this time, as my GP scratched his head, he decided to send me for a barrage of tests, which included more blood work, X-Rays, Ultrasound (sonogram) and MRI. The latter was carried out with a contrast enhancing injected fluid.
The only real result from these tests was that I had 'mild dilatation of the common hepatic duct'. At this stage I was referred to a gastroenterologist, and after the usual prodding of the abdomen and a 'finger' test, I was informed that I could have colon cancer (with the 'hepatic dilatation' totally ignored). I was informed that 'the most direct approach would be a colonoscopy'. This was duly carried out showing a clear result, and my symptoms subsided.
During the following four years, symptoms came and went, but by mid 1999 they became severe again. I was now living in mid-Wales and was referred to the gastroenterology department of the local hospital and seen by a number of different consultants. I started to make my own record of the LFT results, as I had noticed that doctors seem to just look at the last results versus the current ones, rather than look at long term trends.
More prodding resulted, then a sigmoidoscopy, more blood tests and an ultrasound. Again a dilated bile duct noted, and I was sent to another hospital for an ERCP - the 'gold standard' test for ducts of the biliary system. Unfortunately, the report showed that the procedure could not be completed - for whatever reason. Next came an MRI, but there was no feedback from this test!
The local consultant was brave enough to eventually inform me that 'he did not know what was going on' and could I wait for a young doctor who was joining the team in a few months time. This was the best thing that could have happened as the 'new' man mentioned PSC from the initial contact. I restarted all the tests through blood, ultrasound, another MRI and onward to a liver biopsy. Another year passes.
All the results pointed to PSC and at this stage I was eventually referred to the Liver Unit in Birmingham. Another MRI, as I was reluctant to have an ERCP, given previous experience.
I was now in 'self help' mode, thanks to the Internet, the PSC support group and the excellent information available from that source, I could ask more relevant questions about PSC in general, and the tests in particular. In addition, having a running record of blood results for four years or more, meant that a long-term picture was in my own hands.
My local consultant expertly carried out another ERCP with the results confirming PSC. However, my diarrhoea continued and got to the stage where I was losing a lot of weight. A colonoscopy confirmed Ulcerative Colitis covering the whole length of the colon. Medication provided quick results and my weight recovered slowly.
Currently, there is basically only one medication that may help PSC symptoms, and that is Ursodeoxycholic Acid (URSO), but even during my early Birmingham visits, there seemed to be reluctance in prescribing the medication. On seeing a different consultant a few months later, URSO was recommended. My daily diet now includes URSO at 20mg/kg of body weight, and my LFTs have improved significantly.
Back to the beginning...as you will note this has been an uphill and lengthy struggle to actually obtain a real diagnosis of PSC. I was very lucky to, finally, have the right man in the right place -not everyone is so lucky. More information, support and help needs to be made available to patients, GPs, Hospitals and Liver Units.
PSC is a rare disease which primarily affects males, at an estimated rate of about 20 in 100,000 population. Also, between 75 and 85% of PSC patients are Ulcerative Colitis (UC) sufferers.
Footnote:
William Williams recently ran 10 kilometres in Aberwyswith, Wales which raised £3,035 for PSC Support. If you would like to raise money for the British Liver Trust please call the fundraising team on 0800 652 7330 or email fundraising@britishlivertrust.org.uk