Thanks for posting Steven and David. I too have doubts about the 'happy' bit and find it difficult to join in a 'celebration'.
Although I cannot say 'Happy World CML Day' because I have never been happy about CML and never will really come to terms with it, I do understand the initiative- which in fact was started 4 or so years ago by the CML Society of Canada who came up with the obvious date 22nd of the 9th month in order to raise awareness.
They used the date to lobby their government about access to therapy etc. and this is where I think it can be valuable to have such a day.
My own story started in December (4th) 1998- not a day that I -or my family- will ever forget nor be happy about and I always remember it in very sharp focus, and with a lot of sadness, because it changed our lives so profoundly.
But that is a common experience for all those who are diagnosed with life threatening diseases.
At that time, I was not aware of the phase 1 trial of STI571 (imatinib) that had started in the US. I was desperate to find a therapy that would not be such a threat to my life as that posed by an SCT- which was what my doctors were telling me was the only way for me to go- if I wanted to live!
I must say here that I was diagnosed quite late in chronic phase, even though my white count was only 17 and I had no particular symptoms other than general tiredness etc.
However I did have a massively enlarged spleen which I later learned was because all my Ph+ cells (plus others) were gathered in there rather than in my peripheral blood.
"Good job you don't indulge in a contact sport" was the remark Prof. John Goldman made (he lead the team at Hammersmith in those days) when he was trying to persuade me to submit to a splenectomy before the planned SCT.
I think I thought that if I agreed to 'lose' a major part of my anatomy I would somehow be on a slippery slope that I would never get back from.
As it turned out, I agreed to the removal of my spleen, and after some weeks of recovery I did feel better.
During that interim period, post splenectomy and while I was getting my head around the need for a transplant (my younger brother was a good match for me) I/we did a lot of research.
This was a very hard time my partner, David. He was in the front line of our research- facing everything, mostly bad news, that came up on the internet searches and somehow managing to filter the worst news out before he told me about possible options.
He was recently asked to write something about his experience as a 'carer' and found it so difficult to relive that period that he eventually had to admit he could't do it. His strength and determination got me/us through those first 6 months but I think the emotional effects on him have not, even yet, healed 13 years on!
After some months of struggling with the fear of transplantation and 'watching' my test results show a gradual progression of my disease, a nervous time for both my docotrs and family, I agreed to start the work-up to a reduced intensity transplant.
During those rather scary weeks I decided I had nothing to lose by joining the CML list on ACOR.org. That was the best decision I could have made. Within 24 hours of my introducing myself, a member of that forum contacted me with information that would turn out to be life-saving for me. His name is Peter and I will always be grateful for his help during that first year.
His wife was enrolled in the phase 1 trial of STI571 (imatinib) which was showing unprecedented success in the small group of patients taking part. I think there were only 35 or so of those early pioneers and some of them did not live long enough go on to see the impact that imatinib later had, including Peter's wife. That is because phase 1 trial protocol is basically a way of finding the dose range that a drugs is most effective. So some are inevitably given what turns out to be a sub-optimal dose- and with CML that can mean that the Ph+ cell is given an advantage and becomes resistant.
Through the information I was given by this kind and generous man, I was able to arrange enrolment in the Phase 2 of the same trial and David and our 13 year old daughter Nina travelled to Portland Oregon to live for 2 months to see if I would respond to STI571.
Portland is a beautiful city on the north west coast of the US, and it was no hardship for us to live there. However, it was a risk as my disease was starting to enter early accelerated phase, but I felt strongly that I had to give it a try. We did not have to pay for the medical costs at it was an industry sponsored trial, but we were fortunate to have enough to pay for the airfares and living costs for 2 months.
I did respond well and returned to the UK, where I went on responding achieving an MR of 0.57...% BCR-ABL in a little over 18 months. I held that for another 6-9 months or so, but..... subsequent PCR tests showed several consistent, if small, rises in BCR-ABL transcripts and it was found that I had developed a P-loop mutation (Y253H) which means I was becoming resistant to imatinib.
At that point, BMS had not set up international trials for dasatinib, and the rules for international patients enrolling in US trials seemed to make it more difficult to enrol.
UK/European trials were some months away and it was decided that I had best 'bite the bullet' so I enrolled in a new study at HH for reduced intensity SCT + imatinib + DLI.
To cut this long story a little - I responded well and got through the SCT part without too much distress- although again David, Nina and my family suffered terribly with their own fears of my not surviving the SCT. There was (is) a lack of awareness and support for family, if only because understandably everything is centred on getting the patient through the process. I think the scars of that time are still there and even now it remains difficult for us to revisit how we all felt during that intense period and the months afterwards. I have some photos of my time in the transplant ward- and whilst I am OK with looking at my own image, I cannot look at David and the obvious distress he displays. I have put those photo's away, I am not sure I will every be able to look at them.
Nevertheless, I responded to the protocol and in 2006 (it is a slow process due to the need for extended time lines between giving DLI) my PCR result were negative.
Since then Q-PCR has become even more sensitive and over the last 12 months or so my results (and a lot of other post SCT patients have had the same experience) do show a very small amount of residual disease.
My latest test is unchanged and seem to be stable at around 0.001% BCR-ABL. Prof.Jane Apperly (now the head of Haematology at HH) is not at all phased by this and is confident that somehow my 'new' immune system is successfully coping and keeping a lid on the small amount of disease that is obviously 'lurking'.
I cannot say that I can 'celebrate' my experience with CML, but it did happened to me and I accept that it is part of my own, and my families, life story. We are thankful that, so far, we have not had to deal with anything else, just the normal stuff ;o)
I would never have made it through without the love and dedication of my partner David, and our daughter has come through all this too. She is a sensitive and compassionate person who is willing and able to help and support others. That does make me happy ;o)
I dedicate this website and forum to the memory of all those who did not survive the early days of TKI therapy and hope this forum- and others like it- provide the same level of help and support that I had when I was newly diagnosed. Without this kind of patient to patient support over the last decade or so, the story of CML would have been very different.
Very best wishes to all of you, keep taking the tablets ;o)
Sandy
