Anyone with Chronic Idiopathic Myelofibrosis?

[Mrbeasley]

Hello I am new here. Polycythemia vera Jak 2 positive, have been taking hydroxyurea for 2yrs now. Have terrible leg pain in bed not all the time anyone else with this .

[hunter5582]

Also have PV. Also had leg cramps/pain. Resolved with Magnesium supplements. I was magnesium deficient, One can also get paresthesia and microvascular symptoms with PV. Interventions for these is different, I did also get some microvascular pain in my feet/toes. This resolved with aspirin.

Suggest you review your symptoms with your MPN Specialist. If the pain you are experiencing feels like cramps, definitely get your magnesium levels checks.

Hope you get answers soon.

[Mrbeasley in reply to hunter5582]

Hello Hunter. Thank you for replying. Yes my oncologist did encourage me to take calcium and vitamin D. I also have COPD and take meds for that sometimes find it hard juggling with the vitamins not to mix with meds. Sounds like you have been through the wars with other problems! What is Besremi. I do take the daily aspirin but still have pain in my feet and toes. I guess being 80 doesnt help things.

[hunter5582 in reply to Mrbeasley]

Ir the pain in your feet/toes is a burning/swelling kind of pain, that is a classic PV microvascular symptom. Hydroxyurea and aspirin will usually help with these symptoms. Sometimes more than 1 low-dose aspirin is needed when microvascular issues are a problem.

While aging certainly causes its own issues, it is important to know that the JAK2 mutation does more than cause your body to make too many blood cells. It also causes the body to make too many inflammatory cytokines. At the core, MPNs like PV are inflammatory disorders. This systemic inflammation is thought to be responsible for many of the secondary symptoms we experience.

Besremi (ropeginterferon alpha 2b) is the newly FDA approved medication for PV (November 2021). It is one of only two FDA approved medication for PV. Jakafi is the other FDA approved med for PV. While in common use, hydroxyurea is technically off-label for PV. The other common off-label medication for PV is Pegasys (PEGylated interferon alpha 2a).

You hematologist should have already reviewed the choice between hydroxyurea and the interferons. Both are first-line treatment options for PV. The doc should also have mentioned Jakafi as a second-line choice. Jakafi is a JAK-inhibitor. It can be much more effective for certain symptoms associated with PV. It is also approved for use with Myelofibrosis.

Hope that answers your question.

[Annula in reply to hunter5582]

That's a very comprehensive reply from Hunter.... Hope Mrbeasley has taken some comfort from this.

[Mrbeasley in reply to Annula]

Thank you .Yes he seems better informed than my doctor! Hope you are doing well😍

[Mrbeasley in reply to hunter5582]

Thank you Hunter you seem very informed on blood disease. I have had one plebotomy ( exuse my spelling ) that brought down my hematocrit . Hydroxyurea brought down my platelets was on it 1000mg twice daily to begin with then reduced to 500 twice daily . My white blood cells crashed so came of it for a while. I now after 2yrs only take it Monday to friday. For many years I get a very sharp pain under my left rib cage so bad I have to push hard with 2 hands till it subsides have you heard of this? I did have a bone marrow aspirate 2yrs ago which favors a diagnosis of pre-fibrotic myelofibrosis due to increased reticulin and megakaryocyte morphology . My health is actually not bad just have lots of aches and pains . Any comment on all of this. Thanks Hunter and I hope you are well

[hunter5582 in reply to Mrbeasley]

The pain you describe under your left rib cage is consistent with splenomegaly. which is a known significant complication of MPNs. While minor in many cases, if it is causing pain then it needs to be followed up. The size of your spleen can be assessed with a simple scan. Ultrasound is the simplest, but sometimes CT or MRI used. This needs follow up ASAP if it has not already been done. It may be something else, but splenomegaly definitely needs to be assessed.

Regarding the lots of aches and pains, this is also a MPN related issue. Deregulation of the JAK-STAT pathway does more than make our bodies make too many blood cells. It also causes out bodies to make too many inflammatory cytokines. This systemic inflammation is thought to cause many of the secondary symptoms we experience. AT the core, MPNs are inflammatory disorders, Controlling inflammation is a key component of treating MPNs and is not thought to be important in preventing progression of the MPN. There are interventions that can help with this, including diet (e.g. Mediterranean) and certain supplements (e.g. NAC and Curcumin).

Given what you describe, including having a cooccurring condition that can impact the MPN (COPD), it is vital that you consult with a MPN Specialist. Most hematologists do not have the KSAs to provide optimal MPN care. You have described some issues that require expert attention, Here are two lists.

mpnforum.com/list-hem./

pvreporter.com/mpn-speciali...

Given that your BMB shows pre-fibrotic myelofibrosis, I would suggest reviewing your treatment options with a MPN Specialist, Current evidence indicates that the interferons have greater efficacy in terms of preventing the progression of MPNs. This is a complex issue that requires MPN expert input to determine what is best in your individual case. Suggest you get a second MPN-expert opinion ASAP.

All the best my friend. Hope you find answers soon. Please do et us know how you get on.