myelofibrosis

[glyndale]

hello anyone with mf,I'm not sure if I should start treatment a lot of people on here are on treatment. my platelets are normal, hb slightly low ,slightly enlarged spleen ,fatigue is main symptom. I'm on watch and wait but should I be on something to stop the mf progressing.

[Ekkles]

Hi Glyndale, I was diagnosed with secondary MF 18 months ago. I have no symptoms, just whacky blood counts. Like you, I was uneasy to be on watch and wait, especially with a horribly high JAK2 allele burden. Interferon worked really well for me when I had PV (for 10 years) reducing JAK2 from 57% to 7% at the last test. I stopped taking it due to side effects. As my blood counts have gone even more whacky, (all in range, bar the enthusiastic whites and odd sized red blood cells) I've just started injecting a low dose (45mcg) of Interferon, to be done every 3 weeks. The consultant I see is not sure it will make much difference. Further bone marrow test and myeloid panel soon. Not wanting to start RUX until necessary as it can stop working.

It is a worry being on watch and wait and I too have been anxious about it. My solution ..... I might as well enjoy life as much as poss, especially as I feel physically well, and to try to avoid stress as much as possible.

Thinking of you, and hope this helps.

[glyndale in reply to Ekkles]

Thank you for your reply, much appreciated.I only take aspirin, so I am going to ask my consultant about any treatment I should be on.Thanks again and take care.

[MCW22 in reply to Ekkles]

Hi Ekkles, can I ask what your white blood cell count is? I was diagnosed with PV just over 5 years ago. I have been on ruxolitinib about 3 years and like you all but my white counts are in range. I also have teardrop shaped reds. I do worry that these are signs of progression to MF. My whites are usually around 30 but at my last test were 43. What signs did you have when progressing from PV

Carol

[Ekkles in reply to MCW22]

Hi Carol, my latest white cell count was 34, the highest it's been is 36. I seem to remember teardrop shaped reds being mentioned too, but I have no documentation of that. I have a high critical RDW count as well. I didn't have any symptoms and still don't. I just noticed a gradual increase in white cells which hadn't happened before. The whites had behaved impeccably when I had PV! My LDH level was above 1000, but had dropped somewhat at the last test. Hope that's been helpful.

I just feel at the moment as though I have looked into a crystal ball and seen something I would rather not have.

[Flyingsteamer]

Hello Glyndale, I guess it all depends on the severity of your mf as to what the correct process should be. I assume you are seeing a MPN specialist, if not then I suggest you ask for a referral as many others have said on here before. I was diagnosed with primary MF in September, quite well advanced at intermediate 2. Symptoms were severe fatigue and breathlessness, associated with Anemia. My Hb level was 53 when first admitted to hospital and even with weekly/fortnightly RBC transfusions has only been maintained between 65 and 90 . I was immediately invited to join the FEDORA clinical trial which entails me taking 400mg of Fedratinib daily and Ropeginterferon Alpha-2b injection fortnightly, started at 100mcg now at 150 and will increase each 4 weeks to 250mcg as long as my body doesn't object. I hope you get sorted with a plan that meets your own needs.

[glyndale in reply to Flyingsteamer]

thank you for your reply and advice think i need a referral to an mpn specalist, good luck in the trial and I wish you all the best.

[Itch]

Hello Glendale, I’ve had primary MF for seven years ( tho’ undiagnosed for about thirteen according to historic blood results). I’m also on “Watch & Wait”, taking prescribed antihistamines only, to ease unbearable itching. Apart from Stem Cell Transplant I don’t think there is currently any treatment known to stop disease progression. Definitely speak with MPN specialist; depending on your specific gene mutation you may be reassured to find that you to can also live a long time with this diagnosis. I wish you well; I hope you find the support & guidance you can get from this forum also helpful. Susie

[glyndale in reply to Itch]

Thank you, yes I need a referall to a specalist, and this forum is very informative. Thanks again and best wishes.

[lucieboo in reply to Itch]

I had horrible aquagenic pruritis, mad itching after a shower, when I was on Hydrea. This stopped when my specialist prescribed Jakavi for my progression to myelofibrosis. It has not recurred. Mine is myelofibrosis secondary to PCV, but I think the treatment options are the same. Jakavi slows disease progression and helps with side effects, but is very expensive. I have it prescribed free of charge here in France.

[monarch5000]

Some specialists think early primary MF patients should consider taking interferon to try and slow or stop disease progression: youtu.be/JHyYQOVy1ec(Not working as expected?) youtu.be/XGEAlov50mY(Not working as expected?)

[glyndale in reply to monarch5000]

Thank you very interesting.

[tracey13]

My husband started off with PV he's now got primary MF he was suffering exhaustion he's on ruxolitnib and is doing loads better. All his bloods are in normal range . The fatigue has settled .His spleen has reduced in size so all is good for him .

He takes 20mg twice a day of ruxolitnib/jakafi

He was 40 when diagnosed he's just turned 50 . He's been on ruxolitnib 4 years next month .

Tracey

[glyndale in reply to tracey13]

Thank you ,I need to discuss my case with a specalist.

[Grizly]

hi Glyndale I have primary mf Cal-r positive. I’ve my blood cells slowly going south every time I see my Haematologist I always ask the question is there any trials or treatments available to me. Of course the answer is a big no. The only change for me is that I have been put on Epo injections to try and raise my red blood cells. This is to delay the time when I need transfusions. The Watch and Wait or the wait and see is not the greatest especially when your blood counts are getting a bit serious but sadly it is what it is. This has not stopped me from living the dream we like to Travel since I was diagnosed I have travelled to China, Japan the Caribbean and seen the northern lights and booked a river cruise. Life is for living. What will be will be. God I sound like Marg Proops lol what I’m trying to say don’t let it get you down.

[glyndale in reply to Grizly]

Ah thank you I try to stay positive and this forum helps a lot, Best wishes.

[tracey13 in reply to glyndale]

I found this forum to be so comforting when my husband was diagnosed.I was googling and it kept saying about life expectancy it was so worrying. Then you speak to people on here who have diagnosis and there's people had MPNs for over 20 years .

Now we don't look into things too much now . My husband seems well enough to enjoy life so we do as much as we can. So in all nothing has changed.

He had three monthly blood tests done and two consultations a year with his consultant. So In all things are going well.

Tracey

[glyndale in reply to tracey13]

Thanks for your reply,yes I have stopped googling this condition just use this forum.all the best to your husband.

[Scaredy_cat]

I progressed to mf from et about 2 years ago and am now about to have a stem cell transplant. If you are in the UK the nhs has various criteria before prescribing various drugs. For me this meant my platelets falling below a certain level and then I was started on ruxolitinib which was good. However it then lowered my platelets even further so I was swapped to fedratinib. These drugs work on symptoms but there is a juggling act with not letting you blood counts go too low. Some new drugs are on the horizon which work better at stopping your red blood cells fall to low.

[glyndale in reply to Scaredy_cat]

Thank you and good luck with your transplant, best wishes.

[Leighcox85]

all treatments only last for so long so I know if your bloods are ok they will do watch and wait till treatment is needed so they must feel you are ok at the minute when I was diagnosed I started on watch and wait then when I had my second bone marrow biopsy it was decided I had to start treatment so as long as your team are keeping a good eye on your bloods it’s ok we progress no matter what even with treatment unfortunately it’s more about management and symptom control hope this helps a little

[glyndale in reply to Leighcox85]

yes that helps thank you ,best wishes

[Aderyncoch66]

Hi Glendale

I discovered that my PV had progressed to MF last week. In my brain fog I think the Consultant said I was at level 2 (I probably should find out what that means)

I’ve been put on Ruxilitineb (only on day 4 so watch this space). My red cells are very low, white cells bottom of normal but platelet’s are ok.

I had been on Hydroxy for the PV which worked well until the symptoms went mad, but bloods appeared normal.

My main issue is managing symptoms, which might be why I’ve been put on the meds.

Good luck with watch and wait and try not to stress.

[glyndale in reply to Aderyncoch66]

Thanks for your repl,y best wishes.

[merlisa]

I propose you start with Rux etc. inhibitors, because you have been facing anemia, Interferon can't play a better role at this stage.

[glyndale in reply to merlisa]

Thank you

[katiewalsh]

HI. ONLY AN MPN SPECIALIST (or hematologist until you have a specialist) should be advising you on your medical treatment. Not one of us. Others have already recommended you get an MPN specialist & I can’t say it strongly enough that you do so as soon as you can. So many patients on here, treated only by hematologists, have had their treatment approach changed once under the care of an MPN specialist. Everyone’s situation is different and only a doctor familiar with your case & your body can advise you on the best treatment if any. Many of us see a specialist twice a year and our hematologists treat us the other months. Best of luck to you. Katie

[glyndale in reply to katiewalsh]

ok thanks I will speak to my haematologist.