My Story - ITP and All That

Firstly here, what does ITP stand for?

a) Information Technology Panic

b) Immune thrombocytopenia, aka Idiopathic thrombocytopenic purpura 

c) Innovative Tidal Power - a consortium proposing to build a barrage across the Severn estuary to generate electricity, or

d) A high speed train, like they have in Japan?

Until the summer of 2012, I was none the wiser.

ITP - Immune thrombocytopenia, also known as immune or idiopathic thrombocytopenic purpura - is a rare bleeding disorder characterized by a low amount of platelets in the blood. Platelets are needed for clotting of the blood. In patients with ITP, a person's own immune system creates antibodies that mark healthy platelets as "foreign substances" and then mistakenly attack and destroy them. As an autoimmune disease that results in the destruction of platelets, patients with ITP have a tendency to bleed or bruise.

The platelet counts here are in units of one billion cells per litre of blood. The normal range is 150 - 400. 100-150 is still safe for most normal activities. Below about 50-60, anything invasive (eg surgery) or physically impacting (eg hockey) is unwise. Below 10-20 is getting dangerous.

Crossing the Severn, mixed media (2012), Copyright Joan Lee

Thanks to the tacrolimus, the liver problems I’d had during the 1990s stabilised and then disappeared. Unfortunately, despite tacrolimus probably being the most effective anti-rejection drug there is and still being on a low (maintenance) dose of steroids (prednisolone), I went on to develop other conditions in which in an overactive immune system played a part . In 2003, I was diagnosed with Crohn's disease, an inflammatory bowel disease (IBD). This is believed to be more to do with gut bacteria and genes than autoantibodies, but there's probably an immune response involved. I’d felt unwell and became anaemic with this during the run up to this diagnosis and had to have some more unpleasant tests. Thankfully, this responded almost immediately to a course of increased prednisolone and ongoing mesalazine. This continues to be monitored by a Gastroenterologist. Thankfully, the disease stayed remission after the steroids came down and, compared to many people with IBD, I got off lightly.

Then, for nine years, between my mid-30s and mid-40s, I enjoyed a period of stability, so good that I'd almost forgotten I'd had a transplant or had ever had any health problems. In 2011, I reached the milestone of living longer with my transplant than my own liver. During the first half of 2012, everything was going well: at my then annual visit to the Kings liver clinic, I had a good report, with nothing in my blood tests indicating any problems. My artwork was being accepted for open exhibitions, I even had a few sales. Come the early summer, I still felt well in myself.  I was painting hard, fired up after trips to Scotland and The Gower, walking along coasts, lochs, rivers and climbing mountains. What happened next, came as a great shock…

The painting above was done shortly before my ITP diagnosis.

An ominous sky sketched in mid-July 2012 - Copyright Joan Lee

I didn’t feel or look unwell until the latter half of July 2012. Then, a day or two after taking my regular walk on the South Downs, I developed a rash of purple spots all over my body and big bruises on my legs and hands which I couldn't explain. I had nosebleeds and my dentist (thankfully only a check-up) noticed my tongue was bleeding. Still at the dentist, I saw that the rash had spread to my face. I called the surgery and got an appointment with the nurse. She took one look at my skin and called in my GP. He took one look at me and said I had low platelets. The next night, after a blood test, I was taken to my local hospital by ambulance.

I'd had short stays in hospital for tests such as liver biopsies, but this was the first time, post-transplant I'd been admitted to hospital because I was “ill”. This time, it was ten days, rather ten weeks, with everything generally happening on a faster time scale. I was in very good hands and received high quality care from the doctors and nursing staff who had a high workload and were invariably working under pressure. Nevertheless, it was a difficult, frightening time which still haunts me over a year on – the mental bruising taking far longer to heal than all the physical bruising from the low platelets. There were parallels with my pre-transplant illness: the six o’clock phone call from my GP with bad news about the blood test, followed by even grimmer news on the ward. Once again, my blood wasn’t clotting properly; and, initially, at least I did not respond to treatment. I found the noise, light, heat and the generally heavy, bad vibes on the ward very difficult.

Newly arrived on the main admissions ward, the house officer said my platelet count was only 3. This wasn't the first time my platelets had been low. I recall, just after my transplant, my count was in the 20s “Still only 23 – the spleen may have to come out!”, I heard my consultant say on the ward round on about day 6 post-op (the count recovered over the next few days). That had been due to my spleen still being enlarge, not yet settled after the liver cirrhosis.  What I heard now was frightening. I didn’t know they could go that low, and lower. All the more so when the count didn't improve after an overnight transfusion of platelets. I was then put on the Haematologists' books and had a bone marrow biopsy. Because my platelet count was so low, I had a lot of bleeding around the wound afterwards, but from this, they were able to make a diagnosis fairly quickly. I was making platelets but they were being destroyed as soon as they got into my blood stream, presumably by auto antibodies.  They said I had ITP – or was it IPT? It sounded like a technophobic condition - Information Technology Panic which afflicts my nervous mother, causing her to put up a panic wall whenever anything new comes up on the computer such as unrelenting software update nags.  

Anyway, they started me on steroids - 60mg / day. This was about the same as my starting dose of prednisolone post-transplant. I was moved to the Haematology and Oncology ward. They said 85% of ITPees respond to steroids and then mentioned a list of other options for the other 15% which I didn't take in at the time. Within a couple of days, it was clear I was among the awkward 15%.  Over the next few days my platelet count was 5, then 3 again, 7, then down to 2.

After a dismal weekend on the ward wondering what the future would hold for me, I was given a two day infusion of intravenous immunoglobulin (IVIG). This made me very headachy and nauseous, but it worked: the platelet count increased from 2 to 67 and I came home at the beginning of August.  After a week taking it easy, glad to be out of hospital, I went out for short walks along favourite rivers and well-loved bits of the local coast, visited various art exhibitions and had my first outpatient appointment with the consultant who was to handle my case from then on.

This all took my mind off what might happen in the longer term, though I knew I couldn't bury my head in the sand. The IVIG was only a temporary fix, lasting up to 4 weeks, and any drug changes / new treatment had to be compatible with my existing post-transplant medication and liver.  When the 4 weeks were up, I was shocked to find the platelet count falling again (19-10-6 over three days). I’d been warned but I was shocked because I hadn't noticed anything indicating a drop - no new bruises or any bleeding, until I got to the clinic and the consultant said I was bleeding on my tongue. From there I was readmitted me for another round of IVIG. This time, I was in a sideroom, making my hospital stay much easier. The liver team at Kings were now involved, too.

There then followed several months of drug changes and complications before everyone established that my platelet problem was a separate autoimmune condition and not a rare side effect or reaction to my pre-existing post-transplant medications.  I was being seen by two different specialist teams, at two different hospitals. Everyone was doing their best, with all the available resources, but I was now a more complex case than I'd been if I'd "just" had a liver transplant or ITP alone. During this time, my platelets rollercoastered. I had a pleasant surprise in the early autumn when the count shot up to normal, just after an additional immunosuppressant was introduced (mycophenolate), only to crash from 167 to 12 in a fortnight a few weeks later. I think the Haematogist was even more stunned than I was.  I now suspect this crash was a delayed reaction to stopping the tacrolimus which I had now been taking for 15+ years. I wondered, too, if my flu' jab I'd had ten days earlier had an effect, but no one was sure. By then, too, my liver function tests had deteriorated - I didn't have jaundice, but all the blood tests showed elevated liver enzymes. This meant more trips Kings and a liver biopsy.

The liver biopsy a few weeks later, seemed to confirm what I'd suspected: the chronic rejection affecting my bile ducts during the late 1990s had recurred after stopping the tacrolimus. When the Kings team re-introduced tacrolimus over a few weeks, in combination with the mycophenolate, the liver tests improved. Likewise my platelet count. By early 2013, my platelets were comfortably normal again.  I enjoyed a peaceful Christmas and New Year.

During 2013, a fair chunk of my time continued to be taken up by blood tests, appointments and investigations, on all three fronts (liver, ITP and IBD). The resurfacing of my liver problems  prompted two further scans at Kings. I've also been trying to grapple with some "side effects" which can be very disruptive: migraine-like headaches with strong sensitivity to light (side effects of tacrolimus) and sensitive / itchy irritated skin - still unexplained). Having to too and fro’ from / keep on top of three different clinics, felt a bit overwhelming, though I know I was by no means unique here. I try to be an expert patient, something my doctors have complemented me on repeatedly.  Psychologically, I'm still trying to manage anxiety - about the fore mentioned side effects as much anything else - and not let unhappy memories of the bad times intrude, all the more with the first anniversary of the ITP diagnosis in July.

I know, however, that I'm in good hands at Kings, together with my vigilant GP and consultants closer to home. I've been very impressed with all the doctors and nurses, all the more as they are working under pressure, with high case loads and limited financial resources. I've glad, too, to have access to a wealth of information and support over the Internet, something unavailable at the time of my transplant. On and offline, I've been grateful for the support from organisations such as the British Liver Trust and the ITP Support Association.

It is still early days with my ITP. My platelet count has fallen and is more wobbly than it was at the beginning of the year, though all the counts up to the time of writing (August 2013) have been in three figures. For now, my ITP seems to be benefiting from the combination of anti-rejection drugs commonly taken after a liver transplant, particularly mycophenolate.

Having been wary of planning too far ahead / booking holidays during the latter half of 2012, my other half and me have enjoyed good trips away during 2013, to Shropshire, Scotland and southwest Wales. Artwise, I've been focused on printmaking and have been sketching outside during the summer.

Fingers crossed and paint on...