I was diagnosed with essential thrombocytosis at age 24. I had a small child and thought I might be pregnant again, so I had a blood test at my local GP surgery. I was contacted that night by my doctor (never a good sign when your Dr calls in the evening), and he advised that my pregnancy test was negative but I had a very high platelet count (1200), and he wished to repeat it just to make sure that it was correct. After repeating the test, and eliminating any possible cause of infection, I was referred to a haematologist for further investigations. The haematologist suspected leukaemia and arranged a bone marrow biopsy to clarify the diagnosis. I endured a two week wait for results, at the end of which I was very relieved to find out that I did not have leukaemia, but instead had ET.
I was told that it was a disease more commonly found in older people and that it was very rare to find it in a person of my age. Available treatment at that time consisted mainly of busulphan, a drug which was not very effective and was considered too harsh for someone in my age group. I was warned I had a higher than normal risk of clotting and haemorrhaging and that I also had a 5% chance of developing leukaemia. I had never heard of this blood disorder before and lacked means to read up about it (there was no internet in the 1980’s), so after a few months of feeling anxious and afraid, I tried to forget about it and got on with my life. I was otherwise in good health, so it was agreed that I would be monitored yearly to see how I progressed. In retrospect, I realise that I had many symptoms that are now associated with ET, however at that time they were not recognised as being part of this disease.
Fast forward to 2006, my three children were all independent and I was planning a backpacking trip around Europe. I had been increasingly tired and feeling run down but put this down to being burnt out in my job. I was looking forward to increasing my fitness while away. I had a wonderful time in Europe but found that I was no fitter on my return than I had been when I left, despite walking miles every day and climbing hundreds of stairs to view ‘just one more’ Cathedral.
On my return, I visited my GP. Blood tests were arranged and it was ascertained that I was anaemic, along with persisting thrombocytosis. Screening tests for common anaemias were all normal so my GP immediately referred me back to the hospital for further investigations.
I was diagnosed with MF (which I had never heard of), after a very difficult and painful bone marrow biopsy.
I was advised that the prognosis for MF was not good, however, all going well they expected that I could live for another 10 years. Stem cell transplantation was mentioned as an option but would only be offered as a last resort. I had read up about SCT – it sounded very daunting, and I was determined that it was not going to be in my future. Over the ensuing years I spent many hours researching MF. I joined International forums and learned about how patients were being treated overseas. I arranged a consultation with Prof Claire Harrison when visiting London in 2008 and she urged me to consider SCT, eventually, as a viable option for cure.
In 2010, I attended a ten-day retreat at the Gawler Centre in Melbourne and this was very life-affirming for me. It enabled me to take control of the situation that I was in and taught me to make informed decisions that reflected my own perspective on life. I was very proactive about improving and maintaining my general health and utilised many different complementary therapies along the way. These therapies did not alter the path of my disease, however, I believe they placed me in the best position possible to withstand the rigors of SCT.
In 2011, we experienced massive earthquakes in Christchurch. Many lives were lost and the city was plunged into chaos. At that time, I was working part-time in Social Services, despite being significantly anaemic (haemoglobin in the mid-70’s). I had steadfastly resisted blood transfusions as I had read that this was an adverse risk factor if I needed to have a transplant. I left my employment soon after that as I found the increased workload brought about by the earthquakes too taxing. My health was deteriorating rapidly. My spleen was becoming very enlarged and was affecting my appetite and I was losing weight and condition. I had read about the Jak2 inhibitors on one of the forums and I sought a second opinion in Melbourne at the Peter MacCallum Cancer Centre. I was admitted into a drug trial for one of the new Jak-2 inhibitors (which never made it to market). This involved an initial 6-week stay in Melbourne followed by monthly visits for the duration of the trial. I responded well to the drug and my spleen shrunk almost overnight. My symptom load reduced, and my quality of life was much better, however, because the drug initially reduced my Hgb, I became transfusion dependent from that point. It was explained to me by the team at Peter Mac that an important goal of the trial was to improve my health to a point that I would be ready for transplant – without a transplant, I only had two years to live, at most.
Despite the improvement of some symptoms, my life became increasingly narrowed. I lived from transfusion to transfusion and I was no longer capable of enjoying anything resembling a normal life. I made the difficult decision to move forward with SCT. I know for some, SCT is an obvious choice, but for me, it was not – it was a process that took two years of research and angst before I finally came to terms with and committed to having a transplant. I researched SCT very thoroughly and made sure that I knew every possible nuance of the procedure, including complications. This was really important for me in the decision-making process because I wanted to fully own the decision to opt for SCT and have no regrets, regardless of the outcome.
I had no donor available in my immediate family, however, I was very lucky to find that I had a compatible match with a young German male. In mid-2013 I discussed possible dates for SCT with the specialist team at Auckland Hospital. It was decided that optimal time would be January 2014 however due to a large number of acute admissions it was deferred until April. I received the life-saving cells from my donor on April 25th, after six days of intensive conditioning. I was discharged 21 days later, but remained in very close contact with the hospital for a further five months. The transplant process is never easy – I did experience a number of complications.
This year I passed the four-year post-SCT milestone. All of my blood counts are now well within the normal range, there is a normal amount of fibrosis in my bone marrow (compared to normal people of my age) and my spleen is no longer enlarged. Despite being transfusion dependent for two years prior to transplant I received only two red-cell transfusions after the procedure. I have had no significant problems with graft versus host disease, just some minor mucosal issues early on which have now fully resolved. I feel completely well in a way that I never dreamed would be possible. For me, SCT has been hugely successful. I am disease free, I lead a full and physically active life, and I am grateful every day for having been given the chance to have a normal life.
I am often asked why I think my transplant was so successful and the truth is I don’t know. Going into transplant I was by no means a perfect candidate. Unfortunately, there is no exact formula to ensure a successful outcome. However, there is a lot that you can do to increase your fitness and wellbeing before and after a successful transplant. Below are a few of the things that I believe helped me to cope mentally and physically with the procedure and recovery:
1. Choose your medical team with care.
Find a transplant team that you trust completely. Clinical decisions they make during this complex treatment (especially when complications or setbacks arise) may be critical. When you’re very unwell you need to be confident enough to leave all of the decision making to them, and concentrate only on yourself and your recovery.
2. Mindset helps.
Being fully informed about the process and expected outcomes of SCT is essential before committing to the process. Once you have made the decision, commit fully. We all know that the chemotherapy is toxic, but so is our disease. You are going into battle for your life, and chemo is your weapon of choice. Own it!
3. Expect the unexpected.
Even with an ultimately successful transplant, there can be serious setbacks during and after the procedure. I had an unexpected seizure during my initial hospitalization and was readmitted twice with unidentified viral infections. On one occasion my counts crashed dramatically for no identifiable reason, and we thought I had lost my graft. These events were terrifying but were all part of the transplant process.
4. It’s OK to be selfish.
The recovery process goes on long after you leave the hospital, and sometimes friends and family do not understand the enormity of what you have been through or the lengthy period of low energy and psychological adjustment that follows. Learn to say no to anything that does not serve your recovery and don’t feel guilty about it.
5. Set small daily goals.
Even if it’s just making your own tea or coffee, if you can achieve something different every day you will feel like you are making progress.