How I got here; the story so far.

Flashback to 2006. After lots of  prodding, poking, scanning and stabbing I was finally given an answer as to why I was extremely tired all the time! I was diagnosed with Primary Sclerosing Cholangitis, an uncommon, chronic liver disease.

As a seventeen year old who was in her first year at college studying dance, a diagnosis of liver disease was a bit of an inconvenience really. A year before my diagnosis my Mum dragged me to my GP to get some answers as to why her sixteen year old daughter was constantly tired. After several tests it became apparent that something was up with my liver. A few scans, a referral to a Gastroenterologist and a liver biopsy later and…BAM! We had our answer. The answer we had been waiting for was written in a letter, addressed to me that I opened one morning before college. I was at home on my own and completely unaware of what was inside of  this envelope. To say the news hit me like a ton of bricks would be an understatement. ‘Primary Scle…who?’ I had no idea what the hell it was, what it meant for me or what on earth I was supposed to do! So I did the worst thing I could do…I GOOGLED! I read pages and pages of information about PSC, all telling me roughly the same thing; that it is very rare, that most patients are men in their 40s (excuse me??) and that within ten years of diagnosis I should expect to die. I was seventeen, I had plans and ambition but suddenly my whole world felt like it was collapsing in on me. I had no clue what was going to happen to me. I was terrified.

Like I said, googling was probably the worst thing I could have done. Once I was diagnosed, I of course had an appointment with my Gastro doctor who explained what PSC is and  kind of what it meant for me. Yes it was rare and quite unusual for a girl my age to have it but he told me that the information I had been reading was outdated and  inaccurate and that I shouldn’t take notice of things I read online unless they are from ‘reliable sources’. I came away from the appointment feeling much less panicked about my diagnosis but still unsure and curious to find out more. I found online support groups for PSC patients. The information from these websites and forums was much more accurate and people shared and discussed their own personal experiences with PSC. I no longer felt like an oddball with some mysterious disease!

Here is a brief, yet thorough description of PSC taken from one of these websites, a great charity called PSC Support.

PSC is short for Primary Sclerosing Cholangitis, a chronic liver disease whereby the bile ducts, which are the passages that carry bile from the liver to the intestines, become narrowed from inflammation and fibrosis. Over time, the scar tissue builds up and can block parts of the bile ducts completely. The blockages can occur in any part of the biliary tree: inside the liver itself (intrahepatic) or outside the liver (extrahepatic).

The  narrowed or blocked bile ducts interrupt the flow of bile out of the liver, causing a variety of symptoms; ultimately in some patients leading to biliary cirrhosis, portal hypertension, and liver failure.

So After talking to my doctor and hearing from other PSC patients, I slowly began to understand more and more about my diagnosis but I knew that I probably wouldn’t ever fully understand PSC! What I did know was that everyone who has PSC experiences it differently. Symptoms, prognosis, treatments and complications are different for every PSC patient. Some will die of  PSC and some will die with it. Some will spend a lot of their lives fighting it upfront and for some people it will stay in the background barely making an appearance. It is unpredictable and things can change very quickly. There is no cure for it but at the end stage of the disease a liver transplant is the final treatment and the only hope.
At this point I kind of understood. I would have to take several tablets every day for the rest of my life and go for regular check-ups at the hospital – no big deal! For now, my PSC was under control and manageable. The only symptom I really suffered with was constant tiredness but I could deal with that. I still didn’t know how it was going to affect the rest of my life, not really. There is no way of ever really knowing, not with anything. I just knew that I had this disease and it could drastically change my life at any time it decided it wanted to. So this was the point in my life where I decided to always take each day as it comes and to not let this thing define me.

The next few years were all about my life at university, working hard, trying to figure out what I wanted to do and having the most fun whilst doing it! As a dance student it was sometimes harder for me to push aside the fatigue that PSC so kindly provided me with but apart from that and the intermittent spells of my PSC wanting to be a bitch, I was generally well. I even went to study in Canada for 6 months, of course still under the watchful eye of my doctors and parents! Then in 2010 I graduated!
Life was good! My PSC had progressed slightly more rapidly than the doctors thought it would but apart from a few new symptoms (itching being the most annoying one) it still wasn’t stopping me in any way and it took a back seat the majority of the time. Although I knew that one day the time would come where it was probably going to be in the driver’s seat, I didn’t allow myself to focus on that. I just knew I would deal with it when that day came. What I didn’t know was just how soon that day would come and just how much of an impact it would have on my life.

Fast Forward to March 1st 2013. 1.30am (ish) I find myself being wheeled into the back of an ambulance after waking up feeling very peculiar, aching from head to toe and then violently vomiting considerable amounts of blood. My whole body was freezing, I was shaking from head to toe but my stomach was red hot. Possibly the scariest thing I have ever experienced, yet I still found the thought of being taken to A&E in an ambulance completely ridiculous and quite funny. Turns out, there was nothing funny about it at all. One of the problems that can occur with PSC is portal hypertension (high blood pressure in the portal vein) which can cause varices to form in the oesophagus. Basically these are little veins that become enlarged due to the high pressure in the portal vein. These veins are at risk of bleeding which is very dangerous and can be life threatening. Well, this is what happened to me that night. The varices in my oesophagus had burst and were bleeding. By the time I got to A&E I had lost a lot of blood and continued to lose more, I turned a really nice shade of white/yellow/grey and the doctors became increasingly worried about my condition. So they gave me a blood transfusion and took me to theatre to see what was going on and to try to stop the bleeding. I was then on a high dependency unit for 24 hours and had to stay in hospital for a further 10 days – NIGHTMARE!

To cut a long story short, 3 weeks after this happened the same thing happened again! This time I was transferred to the Liver Unit at Derriford Hospital in Plymouth, I have been under their care since 2011. The doctors there performed a TIPSS procedure on me, basically putting a metal stent in my liver to reduce the pressure in the portal vein and hopefully taking away the risk of these bleeds happening again.
With all of this, it was clear to see that my disease had progressed quite significantly. My fatigue was now so bad that it was hard for me to get out of bed or move from the sofa most days, I was constantly itchy all over my body to the point where I’d want to scratch all of my skin off, I had frequent pain and my once milky white skin was now getting closer to the shade of a Simpson’s character. My doctors decided it was time to talk about the ‘T’ word and start thinking about it as something that was now going to happen sooner rather than later (I’m talking about transplant by the way). My doctors at Plymouth decided it was time for me to go through the transplant assessment – a series of medical tests to see if I’d be fit and healthy enough to survive the surgery and to look at the stage of my disease to see if I was in need of being on the transplant waiting list now or if my liver is working well enough to carry on as I am for now.
I spent three days in Derriford hospital undergoing the assessment and at the end of it they told me they would present my case to the doctors at King’s College Hospital in London to get their opinion.  The transplant team at Derriford work closely with King’s and this is where I’d go to have the transplant. The doctors at King’s College decided they wanted to see me and make the decision after assessing me themselves. So I was given a date in July where I would go up to London for 3 days for more tests, to meet the transplant team and to learn pretty much everything there is to know about liver transplantation.
An overwhelming few months to say the least. I was starting to realise that my life was definitely changing. My PSC certainly wasn’t taking a back seat any more, it was suddenly stopping me from doing things I loved and living my ‘normal life’. I felt like my life was on hold and the plans I had and the things I was working towards were now going to have to take the back seat. I just had to carry on the best I could and wait and see if the doctors at King’s thought it was the right time for me to be put on the transplant waiting list. At least if I was put on the list I knew I’d be able to see a way out of this and have hope of getting my life back.

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5 thoughts on “How I got here; the story so far.

  1. Thank you for sharing your story. As a parent of a 9yo daughter who has AIH/PSC, it is the fear of the unknown that I find hard to handle. The not knowing which hand PSC is going to deal her!! I will read your other entries tomorrow when I’m less tired (it’s 2am here in Australia).
    Thank you once again for being willing to share you story with others.

    1. Not knowing is definitely one of the hardest parts of it all. But that is why it’s so important to take each day as it comes and to not worry about what might happen in the future (easier said than done, I know). No one knows what the future will bring and we can never be fully prepared for what might happen but knowing people that have been through the worst of it and come out the other side on top is so encouraging and keeps me full of hope.
      Are you part of any PSC groups or in touch with any other PSC mums?

  2. Thank you for sharing your story. I was dxd at 20, 17 years ago, and feeling like the only one going through this makes it so much harder. I hope you get your liver soon and feel so much better.

    1. You’re definitely not on your own, it’s so good to be in contact with others who are in the same boat as you. I hope you’re well, feel free to contact me any time you want a chat x

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