My ET is now progressing to Post ET Myelofibrisis. My hemo has recommended I start on Ruxolitinib & also to be considered for a stem cell transplant. I know some of you have been in the same situation.
Am I correct in thinking MF is life limiting? Also how have any of you coped on Rux? I feel very anxious about the whole situation.
I have had ET since 2009 & I am MPL +, I see my Hemo in about 4 weeks, but feel I need a bit of info from people who have been through this.
Thank you,
Shirley
My ET progressed to PV, so I am not in the same situation as you. Your concern is certainly understandable. The good news is that treatment options for MF have improved considerably. RUX is effective for many but not the only option. Fedratinib, Pacritinib Momelotinib, and other options are available or soon will be. There is research underway for even more options.
Stem cell transplant is the one and only curative option to treat MF. Others who have been through it can speak to their experience. I have one friend who went through it several years ago. He is doing quite well.
Here is one presentation you may find of interest. There is more out there.
thepatientstory.com/mpn-spe...
More than anything, know that there is hope. All the best to you on this journey.
Thank you Hunter, I will certainly have a look.
You are not alone. A number of us have progressed and of course it's a frightening experience. I was diagnosed in 2020 and was put Ruxolitinib which gave me my life back - more energy, shrank my spleen and stopped the itching. Someone on this forum advised me take time to process the news then "get my boxing gloves on." That was perfect advice.
I felt really well as time went on, then was gently nudged by Prof Harrison into considering a stem cell transplant. I am going into hospital for this next week.
Many people live well for many years with MF - others in this group will confirm that.
Please let us all know how you get on.
Best wishes, Jennie
Hi Jennie, Thanks for your reply, it’s good to hear that you did well on Rux, makes me feel a bit better. Good advice to take time to process the information, there is a lot to take in. There is a lot of good advice on here, but you sometimes forget what’s been said if it doesn’t apply to you.
I read on here that you were in the process of going through a stem cell transplant, I hope it all goes well for you.
Good luck,
Shirley
Very Best to you Jennie for next week.You will be in my thoughts.xxx
It seems you have a good caring Dr. There have been recent posts on when or whether to consider SCT. One report suggests the best time for SCT is when your Rux or other treatment is working fine for the MF, rather than waiting till or if it becomes less effective. It seems your Dr is considering this approach. You can ask Dr for details on Dr's thinking
As Hunter notes, there are several new treatments available or likely to be soon, so Rux is no longer the only option.
I was diagnosed with ET- jak2 in 2008 and progressed to MF in 2015 and changed from hydroxy idea to ruxilitinib at that time. It was a real life changer for me. I did so much better on ruxilitinib! I had some life again. Now I am struggling more with fatigue again! A transplant is not an option for me. I am 75. All the best as you continue on your journey and how it plays out for you.
Thank you, I am 67 & my haematologist said if I had a sct it would be best done before I was 70.
I am waiting to hear whether my ET is transforming to something else. I had a bone marrow biopsy over 3 weeks ago but haven’t heard anything back yet. I’m hoping no news is good news. I have a telephone consultation on 7th September. So I am very interested in what options you are given. I hope all goes well for you.
Dear Shirley
I had MF diagnosed six years ago now and it had probably transformed from ET well before diagnosis. I’ve been on ruxolitinib almost since diagnosis which, apart from causing skin cancers on my face, has definitely helped with the blood picture. However, I now feel very fatigued and am becoming transfusion dependent. Stem cell transplant wasn’t an option for me, I never really was clear why, but I suspect I wouldn’t have taken it even had it been offered, as it needs a level personal support that I didn’t have.
There are new drugs coming along, as Hunter has said, that can deal with the MF symptoms without the more unpleasant effects of rux.
But you need to be guided by your haematologist as to which drug is the most suitable for you at this stage in your disease.
Hope this helps. Take care.
Yes it does thank you.
I've been on Rux for my PV since 2019, reduced my spleen by a couple of cm and stopped the itching. Brought all but white counts into range. No side effects.Carol.
Hi Shirley. I was diagnosed with ET in1994 and post ET MF in 2012. The progression came as a shock as I had just moved and changed haematologist. She looked at my past results and said straight away that it looked to her like fibrosis. A BMB confirmed that. I guess I was pretty scared at first as by now there was a wealth of info on the internet. I was referred to Prof Harrison as by her own admission my haematologist wasn’t an MPN specialist. She has supported me annually ever since then. She is a lovely person with an abundance of knowledge delivered in a caring way
When I first met her I was living in Scotland and they had not yet approved Ruxolitinib so she found a specialist who could prescribe it initially on compassionate grounds
I saw the transplant team twice around that time but for many reasons and a lot of family support I decided not to go for it
Now in 2022 I have transfusions every 3 weeks which has caused iron overload being treated with Exjade. My blood counts are now all low and this year has seen many temperature spikes which have to be treated as neutropenic sepsis so need hospital admission and IV antibiotics. It is becoming apparent that the temperature spikes might have been caused by an acquired infection of Hepatitis B from contaminated blood transfusion ( this is extremely rare nowadays and since my infection another test has been added to the screening process)
I think the phrase ‘keep calm and carry on’ is pretty much the way I deal with my health issues. Since progressing to MF I have welcomed 3 grandchildren and plan to be around for more arrivals in the future!!
I eat the best diet I can - mostly fruit and vegetables with some lean protein. I am almost 73 so being retired I can rest when I need to. I have given in to the need for a cleaner and gardener but do try to do at least a short walk every day.
I hope my story gives you hope for the future. I am eagerly anticipating the arrival of disease modifying medications and I feel blessed with the local haematology team I have who are magnificent and the regular input from Claire Harrison.
The decision to go for transplant or not is always a difficult one and has to be a personal one with health and social circumstances taken into consideration. My haematologist at the time told me it can be brilliant or it can kill you but with many grades of success between the two. I hope you get a lot of support in making that decision
I hope you keep well and wish you all the very best. Jan
ShirleyRux is easy to tolerate, most have no side effects, in fact most report feeling much better on it, I have been on it for 5 years for PV and have no side effects apart from slight tendancy for weight increase which is controllable. Good luck with the journey ahead, I know of many MF patients who function very well and have busy lives, it’s harder than ET but you can still have a good life. It’s important to have a good Haem and ideally to get a second opinion or two from a MPN expert or two if poss.
I hope that helps. 😀
Hi I was diagnosed firstly with ET in 2010. Progressed to PV and was diagnosed with MF in February. My main symptom was and still is spleen discomfort. I have been on Rux since April and it has worked well except that it caused anaemia for which I am now on weekly EPO injections. There have brought my red count up again. I feel quite well if maybe not as much energy but I am 71 so probably should expect that. My only difficulty is the ongoing bouts of spleen pain which haven’t gone away I asked about a transplant but unfortunately the transplant team did not think I would do well so have abandoned the idea
I would have no hesitation about going on Rux again and hope that something new will be on the horizon soon.
Good luck with whatever decision you come to
Margo
My situation is similar to other posters. I've been on ruxolitinib for 4 years for Post ET MF. I progressed from ET 10 years ago and was diagnosed with ET in 1985 so have a long relationship with MPNs. I wasn't surprised by the progression as the then consultant had been hinting at it for about 5 years.
Rux has reduced my spleen, platelet levels and generally stopped night sweats. Anaemia has been a problem and I give myself EPO injections for that. On the other hand I haven't put on weight, a common side effect. I think rux has been a life saver and there are other drugs hopefully in the pipeline if it fails. An SCT, initially considered, is not an option because of damage done to my immune system by another drug.
With regard to MF being life limiting as your consultant will tell you there are 4 levels of MF and even then the figures given are only median ones. There are many of us on here on rux or other drugs, albeit perhaps with fatigue or other symptoms, but getting on with life.
I hope your consultant is helpful and can alleviate your anxiety - quite understandable in the circumstances.
Diana
Hi, Shirley, I was diagnosed with Et jak 2 positive in 2008 at age 52, and then MF in 2019. I was on an emotional roller coaster for months, so I understand how you feel. I have tried several different medications over the years, including hydroxyurea, anagrelide, Ruxo, and Fedratinib. For a while, I felt really good on Ruxo, but after a while, I became anemic and had to go off it.
I was also told in 2019, that I’d probably need a SCT within 3-5 years. But I’m 66 now and I’ve been advised that at this point, it wouldn’t make a difference in my life span. Also, I have developed other health issues and don’t think I’d be a good candidate. But if you are relatively healthy and the doctor thinks you should have one, then I would consider it.
All the best,
Cindy
Hi Cindy, I understand what you’re saying about it being an emotional roller coaster.
It has been really helpful reading everyone’s comments.
Thanks,
Shirley
I definitely agree. I am thankful to have everyone’s stories, concerns, and comments to read and follow. I am dealing with ET “in the setting of MF”. I don’t quite understand that last part so I’ll have to remember to ask my MPN specialist. I am on Jakafi and Hydrea. The anemia and the fatigue it brings along with muscle pain in my legs are my main difficulties. I also have multiple myeloma and take 2 different chemo drugs for that. I am not a candidate for SCT although I had hoped to be. I am 72. My doctors have told me that I have a complicated medical situation, treating 2 blood cancers at the same time, I’m just so tired all the time. I’m interested in finding out about EPO injections for anemia. Thank you for sharing your experience.
But why they told you that the SCT will not make any difference in your life span? And what is your life span according to them? I am confused
I was told last year my median life span was 9 years from the year I got diagnosed with MF which was in 2019. To me, it’s a little vague and disturbing because median is not the same as average. I think she said that based on my age. I was 63 when I got diagnosed.
So are they sure that you are not a candidate for SCT?
That median value can only be based on retrospective data. Better drugs and better understanding of the disease will keep you with us much longer than that I expect. Jennie
Hi. I was diagnosed with MF in 2014. My symptoms have stayed pretty good. My only MPN related problems are some fatigue (both physical & mental) & heat intolerance. As Jan said, eating a low inflammatory diet is important. I’m bad & don’t follow this advice. For meds I’m on hydroxyurea & blood thinners. I believe you can be stable with MF for a long time. I believe however that it increases your risk for developing a bad type of leukemia which is fatal. But it doesn’t happen to everyone. Good luck with your decision making. Btw, I’ve never considered SCT or had it recommended but I’m also 71 & have comorbidities. Katie
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