I was diagnosed aged 44 with essential thrombocythaemia (ET), 20 years ago. In those “dinosaur days” the internet information was just for the privileged few who had access to medical sites and there wasn’t very much even then. My GP had to go and look up the condition and told me that at that time it wasn’t even in the average medical textbook. I was diagnosed because of a blood test for severe headaches and referred to a haematologist. I was put on hydroxycarbamide straight away as the parameters for treatment were different then ;target platelet count under 600 and treat if above. My platelets were over 1,000 and I didn’t question treatment as I would probably have taken arsenic if someone told me it would cure the headaches! Hydroxycarbamide did not help so after a while I was changed to busulphan , a drug not often used nowadays and the next haematologist was not prepared to carry on with it so I went back to hydroxycarbamide.
Over the years I tried as much as possible to put my ET very much on the back burner as I had four energetic young boys to bring up and didn’t have time to dwell on it. I just keep the appointments, keep taking the tablets and got on with life! My platelet count was never really stabilised and the dose of hydroxycarbamide kept going up. I tried switching to anagrelide at one point but my heart said no thanks to that!
A couple of years ago I moved again and the new haematologist took a look at my blood results over some time and said that it was possible that my ET was progressing to MF and I had my first bone marrow biopsy (BMB), which proved the progression. I have been fortunate enough to obtain Jakavi on compassionate grounds and that has gone some way to relieving some of the symptoms of itching and sweating and enable better control of my blood counts, but with little or no spleen shrinkage and higher blood pressure, it has not yet been quite the miracle drug for me that others have found it to be. I am considered to be at an age when bone marrow transplant would be more risky to my health than the MF so I have put that idea to bed.
You would think that somebody with a blood disorder would at least have good access to a supply of blood ? No such luck! I have always had “difficult” veins and have now opted for a Portacath for easier access following a failed attempt at transfusion, as they couldn’t access a vein and an internal bleed that needed emergency transfusion through a central line. Life is taken at a slower pace nowadays out of necessity due to the ever present fatigue and I no longer work.
My boys, now all grown up, ask questions to which I am always honest and they worry about me now instead of the other way round (actually I still worry about them too!). We live lots of miles apart and they have their own lives but they are in regular contact and send me jokes when I am bored in hospital having blood transfusions. I am not transfusion dependent yet, fortunately as their jokes are sometimes bad!
Post ET, MF is still part of who I am and how I have to live my life but it in no way defines me and my glass is always half full. I am waiting for the next drug to come along and praying that gene-specific medicine is not too far ahead for me to take advantage. There is always hope out there , look at how far we’ve come in 20 years ! Now plenty of information, much research and very importantly, the chance to support each other along our MPN journeys. Thank you MPN Voice for being there for us all.
