About MF progression

[Scaredy_cat]

I have progressed this year to MF from ET with grade 3 fibrosis. I'm reasonably well in myself but my platelets have slumped to 70. I started on 5mg ruxolitinib 6 weeks ago so I know it is too early to say if it's working, however I would like to know more about how it how it feels to have MF getting worse. I have done the DIPSS + score and that gives a median survival of about another 3 years. I had previously decided that a SCT was not for me but I'm thinking again. Can any of you with Level 2 + MF tell me what life could be like in the next few years? I don't want sugar coating; I prefer to know what life COULD be like if I don't have a transplant.

[Aneliv9]

How old are you? For how many years did you have ET? Why don't you want the transplant?

[Scaredy_cat in reply to Aneliv9]

I had ET for about 25 years with no bad symptoms and only aspirin. I'm now 68. I disregarded SCT when I thought I had a longer predicted life span because of the dangers . Now I want to find out what life is like with progressive MF so I have something to compare

[Aneliv9 in reply to Scaredy_cat]

You had ET or possible pre fibrotic MF? Aren't you sure that you want a longer life spam?

[Scaredy_cat in reply to Aneliv9]

I don't know.

[JediReject]

Hi Scaredy_cat , , afraid it's kinda the standard we're all different answer , I felt reasonably good overall at Intermediate 2 MF for a couple if years, was on Rux (but 15 or 20 mg a day - 5mg is very low ) which was controlling most of my symptoms but I still had that niggling feeling / nagging doubt that my underlying MF was ramping up. Small signs , a little more breathless on exertion, bit less stamina, tad more discomfort esp abdominal. Like you I might of had another 2 possibly 3 years at best.

However in my case there were extenuating circumstances, i was 53 , my local Doc was clueless, the Rux wasn't fully effective and I desparately wanted rid of my MF with a passion.

So I opted for SCT at the earliest opportunity. For that reason I can't honestly tell you how I might of felt but I guess my symptoms would of worsened especially the tiredness , I would of become reliant on transfusions and the over-riding possibility of transformation to AML which was high risk for me. This requires immediate treatment which reduces ones chances of a successful SCT for the Primary condition.

So theres my tale of woe. You did say no sugar coating ! .

Seriously you don't need anyone to tell you that life is short and incredibly precious , I get that from your having second thoughts about fighting to extend it. Just to let you know the oldest person I'm aware of successfully undergoing SCT is 76.

Regards - Chris

[EPguy in reply to JediReject]

I think you're one of few here that has some time on the SCT. Do you take rejection meds? Do you have any new info on your Covid antibodies?

Sorry for extra questions if you posted this info already.

Seems the SCT has worked well for you, that is wonderful.

[JediReject in reply to EPguy]

Hello ETguy, , ask away my friend I'm always happy to try and answer any reasonable questions whether it be once , twice or numerous times and with luck I won't contradict myself ! .

* no anti rejection drugs as such but still a small dose of immuno-suppressant (Ciclosporin ) which is part of the SCT regime mainly in relation to counter Graft Versus Host Disease which as you may know I had a severe dose of and remains active.

*no new info on my Covid antibody level but when I last did a test the line for antibodies appeared quickly which was encouraging. I don't see why I wouldn't have a decent response notwithstanding the immuno-suppressant. I would like to know for sure.

*my SCT was successful I guess because I'm still here however my Graft didnt take initially and it looked like i would need a top up of Stem Cells but my Ciclosporin which damps things down was stopped and the Graft kicked in however the level,of GVHD I then had was unpredicted and almost fatal. So I was very lucky all told and grateful for these extra years.

Chris

[EPguy in reply to JediReject]

Your resilience is a model for us all.

I noticed you hadn't posted lately hence the questions. Nice to hear you're fairly stable.

[JediReject in reply to EPguy]

It's kind of you to say that , I don't always feel resilient but I strive to be.

Just been a busy time , , agéd father to assist, recently bereaved Sis in law needing help with various things, Grandparent duties abound, time out with my good lady (a bit !) And a little me time. Just happy to be around and able to help my loved ones in their hour of need best I can. It makes me feel useful because I don't do sitting on my butt very well though I've had to accept its necessary at times.

Keep well !

[EPguy in reply to JediReject]

Keeping up normal stuff is what life's about. Having that option is a big deal.

I just posted an article from Dr. Harrison. Of possible note, I thought of your GVHD, did this come up?

For SCT <<there is preliminary data emerging that JAKi exposure before or during conditioning may in fact reduce the rates of graft-versus-host disease,>>

[Scaredy_cat]

Thanks do much for that. I don't want to curtail a good life nor do I want to prolong a rubbish one. It may be I'm unsuitable for a SCT anyway but I'm one of those people who likes to gather as much information as possible to help me make my decision.

[JediReject in reply to Scaredy_cat]

So do I , I prefer to be as well informed as possible , Ideally i need to get all my little ducks in a row before going ahead esp with something major. Having said that it soon became apparent that my opting for a SCT was a no brainer. Good luck to you going forward.

Chris

[Fika500]

Warning! is a factual & not sugar coated reply. I can understand your dilemma and you are wise to seek as much information as possible so you can make an informed decision. My advice is to seek as much information and speak to experts if you can. Maybe ask Maz fora buddy??

As has already been said everyone is different and the course of the disease is unpredictable. I can tell you how thjngs progressed with my husband but someone else may have a very different experience. I think it is the uncertainty with MPNs that is so challenging. The unpredictable course of the disease - acute episodes which could be fatal but then thankfully are often not if you get the right treatment promptly. Things can change suddenly for the worse equally the can stabilise. In the final two years we hoped gir the best but had to be prepared fir the worst but we didn’t allow worries about an uncertain future spoil the precious time we had. So this is just one person’s MPN experience, not sugar coated, as requested. My husband had PV for 28 years, from the age of 46 and lived well for all those years . He had six weekly venesections, suffered from itch but otherwise had a good quality of life. He progressed to Myelofibrosis aged 74 - his spleen was 23 cm on ultrasound. SCT was not an option due to his age. He was okay for a couple of years having an excellent quality of life but gradually becoming more fatigued which could nevertheless be managed. His prognosis was 3-5 years with a significant risk of progression to leukaemia. After three years he commenced Ruxolitinib , initially 5mg twice a day and it really helped - he had more energy. It was transformative. He gradually went up to 10 mg twice a day. His platelets increased from 70 to 149 after three months on Ruxolitinib, hus spleen reduced a little. He had a great first year on Ruxolitinib and was very active. However he did become anaemic but perhaps this didn’t affect him so badly as he’d had venesection induced anaemia for years so his body had adjusted . Apparently lowering of hgb is expected with Ruxolitinib but usually settles. However my husband’s Hgb continued to lower and his consultant thought it was caused by disease progression rather than the Ruxolitinib so he was happy to let him continue the treatment. He developed very painful gout which was controlled quite effectively with Allopurinol. If he had a flare up he used a short course of Diclofenac ( usually contraindicated due to low platelets but a short course was acceptable ) So his fourth year with Myelofibrosis was a very good year due to Ruxolitinib.

However the next year his blood counts deteriorated and he had a nasty chest infection , luckily effectively treated with intravenous antibiotics . The fatigue could be overwhelming at times but we managed it well. He rested a lot but was able to enjoy a good social life and walk about 5 miles. If you view your energy levels like a battery and try not to run it down too low and plan when to re- charge , the fatigue can be well managed. We loved live music but he couldn’t stand for long periods so we sometimes had to make special arrangements with a venue. We probably should’ve got a blue badge sooner rather than later.

Unfortunately , in his final two years symptoms became more difficult to control but overall he still had a good quality of life for most of his final two years. I was on high alert all the time - spotting acute infections , getting him in to hospital where intravenous antibiotics were effective etc His platelets & Hgb were going down, his white cells rising. He started Aranesp for anaemia and it helped a lot for about a year. He still had a good quality of life as we managed the fatigue well. However he became extremely ill after his Ruxolitinib was suddenly withdrawn due to bruising and infection which was treated with antibiotics. Within 48 hours of stopping Ruxolitinib he was in terrible pain , had multiple infractions of his spleen and his spleen enlarged to 32 cm. He had pneumonia , a massive PR bleed , was in hospital for six days , had several transfusions including platelets after the bleed. He had suffered terribly due to the sudden withdrawal of Ruxolitinib- it was recommenced after 5 days of withdrawal. It is dangerous to suddenly stop Ruxolitinib. After a few weeks he made a remarkable recovery - initially too weak to walk , after about three weeks he improved and could get back to normal activities with plenty of rest in between. . The Myelofibrosis was continuing to take its toll with energy levels but he still had a very active life due to us pacing things / managing fatigue. People who only saw him socially had no idea he was ill.

In his final year, six or more years after progression, he became transfusion dependent , his hgb & platelets going down & white cells going up . He was ok if hgb was over 100, white cells around 50, platelets fluctuating between 40-70. He bruised very easily, had a few bleeds in his eyes. Gradually over his final year he became weaker and things became harder to control - more infections, cold sores, bruising ,blood blisters in his mouth , fatigue. He was still fine in himself and in good spirits but I think thankfully lock down meant he didn’t realise the full impact of the disease progression, as we couldn’t go out / do things anyway. It may have been a bit more disheartening not being able to have his usually very active social life but due to COVID restrictions we had no choice anyway. He lived well until his last six weeks when he developed spleen pain ( in his shoulder & abdomen) , breathing problems - feeling he couldn’t get enough air in his lungs due to enlarged spleen pressing on his lungs. Although still enjoying food , he couldn’t eat as much due to the spleen pressing on his stomach . He gradually became very much weaker , slept more and passed away very peacefully in the hospice where he spent his final three weeks. Morphine, midazolam and Levomeprozamine helped keep him comfortable.

I hope this factual account of my wonderful , beloved husband’s experience is helpful . I wish you well as you ponder your decision.

[Scaredy_cat in reply to Fika500]

Thank you so much. When I first heard my prognosis it was 14 years : good. The next month due to falling platelets but was 4 years : oh bum! But I thought that probably meant 6 for me(probably deluded !) but how many good years?Then I read 33 months from diagnosis, in my case spring 21 . As I was beginning to get more symptoms I started to think I may not have many good years. Your post has helped. Your husband was able to progress to a higher rux dose which helped and he was doing good walks . My spleen was about 22 cm on ultrasound so similar to him. I love walking , taking my friend's dogs out in the Cotswolds where we live but only manage about an hour...they are only dachsies so have little legs. I now think that it is possible to have 4 good years after progression to MF so 3 or 4 more lovely springs. I will probably find out whether I'm suitable for SCT and then think again. Thanks again for the truthful account

[Cja1956 in reply to Fika500]

So sorry for the loss of your husband. He was very fortunate to have such a loving wife to care for him. Also, thanks for sharing your story. I was diagnosed with post Et Mf intermediate 1, in 2019, at age 63. I was just told last month my median life expectancy was 7 more years. I’m hopeful it’ll be longer and I’m making the best of each day, so I appreciate your detailed description of your husband’s experience, although it must have been very difficult to write. All the best.

[Fika500 in reply to Cja1956]

Thank you and I wish you well. Fedratinib had now been approved in England as a second line option in addition to the first line treatment of Ruxolitinib . There is a lot of promising research happening , so a lot of hope!

[Fika500]

You are welcome and I wish you all the best. Bear in mind that there are new treatments in the pipeline and Ruxolitinib can work very well for a number of years.The rate of progression in Myelofibrosis can vary greatly between patients I think / hope Fedratinib will be approved in the UK eventually. It is already being used effectively in the USA in patients for whom Ruxolitinib stops working . Hopefully you can have thorough discussions with a specialist MPN consultant who can look at your individual risk factors etc so you can find out all your options and weigh up the pros & cons of each.

[JediReject in reply to Fika500]

Hi Joe500, , I would also like to just say Thanks for eloquently sharing your husbands experience which is a no holds barred account because clearly you lived through every single moment with him which must of been incredibly difficult at times. Writing about it in detail cant have been easy and I actually find it a moving account of his last few years. I am truly very sorry your beloved husband isn't still with you. All you say about living and passing with MPN/MF is sadly true including the unpredictability of the disease.

And as you rightly point out hopefully new curative treatments will come along for our friend Scaredy_cat and others in the same boat because just managing the symptoms of MF ultimately isn't enough to stop its progression.

Hope you are well.

Chris

[Fika500 in reply to JediReject]

Thank you so much for your reply. It really helps to have people who understand. The support from this site is invaluable not only to patients but carers like me too. It means a lot to me and is a comfort as I learn to live without my soulmate - it is the painful cost of loving deeply and I am deeply thankful for our 31 wonderful years together.

[BlushNoisette]

Hi,

I was diagnosed with PV in 2006 and back then there was so little info about MPNs even on-line. Things have changed a lot since then. In 2015 I applied to participate in The Majic Study and as I was assigned to the Rux treatment group I was lucky enough to get to try what was a new medication back then. What I did not know at the time (before starting Rux) was that my PV had progressed to early stage MF. I was feeling pretty rough then with awful pruritus and fatigue, and periodic episodes of cellulitis and very sore joints.

I started Rux on a very low dose and then it was gradually increased to get my bloods into normal range. I can honestly say that Rux has been a life saver for me and I began to feel better during the first two weeks of treatment.

For what its worth, my advice would be to try not to think too far ahead. Just go one step at a time and give Rux time to work for you. I didn't think that I would make it to 70 but I celebrated my 70th last month and I think that I have quite a few more good years left in me.

I hope that you have the best result possible by taking Rux and that you have many more good years to come.

[Otterfield]

I also have MF with Grade 3 fibrosis and have decided to have an SCT (assuming that there is a donor and that all the preliminary tests they do are okay).After just over a year on Ruxolitinib, I feel incredibly well, with good energy levels and no itching or night sweats. My spleen has shrunk and I have a good appetite. The brutal truth, though, is that Ruxolitinib will stop working at some point.

My haemoglobin and platelets are both low on Ruxolitinib although I have the energy to walk over 3 miles a day.

In my experience quality of life can be be perfectly good with MF, BUT mine wouldn't be a long life. I am 60 and I want to reach 70 and beyond, which is why I am opting for the SCT.

I hope you make a decision you are happy with, it really is a tough position we are in.

Best wishes, Jennie

[Cja1956 in reply to Otterfield]

Hi, Jennie, I am surprised you still haven’t found a donor. I am praying that you find one soon, but happy that you are feeling so well at the moment. Take care,

Cindy

[Otterfield in reply to Cja1956]

Hi Cindy, that was a couple of months ago. It seems that I now have a donor but not a date yet. To be honest I would be happy not to get a date just yet! On the other hand, now the decision is made it would probably be best to just get on with it.Hope things are going well for you?

Best wishes, Jennie

[Cja1956 in reply to Otterfield]

So glad you found a donor but I completely understand your apprehension about having the transplant. It really is best to get it while you’re feeling well and don’t have other co-morbidities, I would think. I think you made a sound decision and sounds like you have a good medical team. Things are going pretty well for me right now. I’m off the Fedratinib for about 2 months now and only take hydrea and baby aspirin. My hemoglobin jumped from 10.3 to 11.8 in a month. So my energy levels and my brain fog have definitely improved. I was also told that a transplant would probably not increase my life span so at the moment, it’s on hold.

Keep me up to date and stay well.

[Mustang50]

Joe 500 best wishes incredibly brave and honest post much appreciated 🥰 the thing that worries me is how do we know if we are progressing MF or not when we are only getting hospital phone calls, tell them your getting itching and night sweats but seems to be ignored, would it show up in the blood tests?

[Otterfield in reply to Mustang50]

Itching can be a symptom of all three MPNs and I think night sweats are too. Increasing white cell count would be an indication and, for me at least, falling platelets and haemoglobin. Increasing fatigue and enlarged spleen would also be indicators. Signs from the blood test would (hopefully) prompt your haematologist to do a BMB.

[Mustang50 in reply to Otterfield]

Thank you! Many thanks for your reply great advice only been diagnosed about a year so all very new to me and still learning all the while with the help from this site, as lack of advice from hospital & GP.

[hollymae]

Joe500 thank you so much for your account of both of your journey it is helping me to get some idea of how things will go for me and my husband. It’s very hard for him being a carer. I try to help him but he doesn’t want to accept what we know is the outcome. We will get by and I am lucky to have his support god bless

[Fika500 in reply to hollymae]

I have just now seen your reply. I hope you are still doing well. It is very difficult when your loved one doesn’t want to accept that the condition is currently incurable. Our attitude was ‘hope for the best but be prepared for the worst’ ( that was the only way we could refer to it .. my husband didn’t want to go into details) and don’t let worries about the future spoil the precious time you have. .. sometimes easier said than done but I am glad we made the most of our time together and didn’t ket the diagnosis dominate our thoughts / life. My husband just wanted to take each day as it comes and wasn’t interested in ‘what ifs’ and it worked for him but was difficult for me at times but I respected his wishes no matter how hard I was for me. I think it is a good idea however to ask every now and again , ‘Do you want to talk / can we talk about what’s happening’