After 21 years, my time of low risk primary myelofibrosis has come to an end. In the middle of last year my haemoglobin level was 104g/L and now it is 88, moving me from the low risk category to the intermediate risk category. The drop in haemoglobin level has been quite noticeable. Previously I had not really noticed changes. Still in very good health.
My haematologist referred me for splenectomy, however I have declined for now. I still consider my spleen to be my friend. My spleen was extended 6cm past the costal margin at the time of diagnosis (20 cm by ultrasound) and is now 16cm past the costal margin (so guess that would be a length of 30cm).
He also raised the prospect of SCT and discussed my changes with his collegues. The group feeling is that it is too early for SCT. I have mixed feelings, however today I am quite happy to defer the SCT decision to another day.
The revised care plan is to increase the frequency of my visits to 3 monthly and continue with the daily 1g HU and aspirin. The next trigger for review is becoming transfusion dependant.
All the best on your MPN journey, cheers Simon
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Simon96
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Hi Jane, sorry to read you have high risk MF. Great that rux has helped with your spleen. Rux is not funded here so not discussed as an option. Thanks for raising it ...cheers Simon
rux may not help anyway with falling counts as someone else has mentioned: pre rux I always had stable Hb and low platelets, now the other way round. I was offered SCT but decided not on the basis that I have a fairly reasonable quality of life and SCT seems rather a hit-and-miss option - am 60 - would have found the decision more difficult had I still had enormous spleen as that can bring complications. Would greatly appreciate your keeping us up to date with how things go.
Thanks for sharing your journey. Slipping towards MF is something we are all aware of and fear. It's ironic isn't it that we spend years keeping counts down by various means and then we reach a situation where they go too low and we need to get them up again. I have read that some people on MF benefit from Ruxolutinib as has been mentioned below. I also hear there is a separate ACOR blog solely devoted to SCT which may help when you wish to consider it.
Your optimistic and positive outlook is admirable. And your calm way of dealing with spleen etc is so helpful. Best of luck in your choices and keeping informed of all options.
Hi Mairead, thanks for your lovely reply. I have been very fortunate to have kept good health for a very long time, and still do. Thanks also for pointing out the ACOR blog ...cheers, Simon
Can you please clarify how you were initially dx as ‘low risk’? I assume you did not have any genetic testing?
Is it worth trying again for a Ruxo prescription? This drug appears to be very effective for spleen reduction although not sure whether you can now take it if falling blood counts.
Re SCT, there appears to have been a major breakthrough last December re inhibiting Graft Host rejection. This seems to be the greatest risk (for someone ‘young’ and healthy) and the greatest cause of horrible side effects such as one’s lungs, eyes etc partly reject the doner cells.
Thanks for the link. Timing is a difficult decision. Go when you are healthy and better able to fight or wait for advances in technology. Certainly a very interesting era technology wise ...cheers Simon
Hi Paul, back in 1996 when I was diagnosed the Human Genome Project was still in it's infancy and it was the great hope of the future. Back then everyone was lumped in the same bucket. At my diagnosis visit I was told that the median survival was 5 years and then, towards the end of the visit, that considering my age and good health, for me 10 years. It was a different era back then. My current haematologist uses the IPSS model for risk stratification, plus transfusion dependence. I am JAK2+. I am not aware of having had other genetic testing ...cheers, Simon
Thanks Simon. I know some MF patients have certain mutations which cause much more aggressive progression. Thankfully you appear to not have this genetic complication.
Re splenectomy, I assume your spleen is now producing RBC, hence removing it would make you transfusion dependent. Have you recently asked about Ruxo?
Or even Ruxo combined with low dose Pegasys?
Have you had a recent BMB to check out state of fibrosis and whether your marrow still producing sufficient RBC/WBC? That you are still in good health is a good sign.
If you are able to now access Ruxo and if your counts/fibrosis permit its use, this could ‘buy’ you quite a few years while SCTs further refined.
Hi Paul, a spleen can be a two edged sword. At the moment, I still consider my spleen to be my friend, so not concerned about it at the moment. Will keep your comments in mind. It has been a rather long time since my last BMB. Not in a hurry to have more. With MPNs it is a long game so first up I will take simply take some time to adjust to the change ...cheers Simon
So impressed with your spirit and good humour. I wish you as long a time as you wish for yourself in your new elevated risk status. What you tell us flies in the face of the outlooks we are all given for long term survival - first you were told 5 years, then 10, but here you are moving to higher risk status after 21 years! And you are by no means the first. Sometimes the medical profession seems to me to be in a terrible hurry to write us off... Good luck to you, and my very best wishes.
Thanks Robin. It was median of 5 and 10 for me. A pretty reasonably guess at the time. The stats were a bit different back then and there is such a wide variation. Best wishes to you also ...cheers Simon
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