Open letter

[Kiruna]

Hi all:

I want to thank all who have posted regarding their experience with MPNs in this forum. I'm very grateful for the information and insight regarding ET, PV, and MF. All of you have educated and helped me a great deal. I write this while experiencing a great deal of stress and sadness; due to my mother's declining state of health, as she recently transitioned from ET to post-ET MF. She's currently in hospital here in Montpellier, France, and is receiving excellent care.

She was first admitted due to a bacterial infection, which has since been treated with antibiotics. She's had at least five transfusions in the last couple weeks, which to me, seems like a lot. Her hematologist is reviewing options going forward (Jakafi, Momelotinib, or others) to determine what Mom can tolerate. It isn't quite clear yet. Because of several prior bleeding events, they're trying to see what might work. Prior to her MF diagnosis, she'd been on Hydroxy and then Anagrelide, until it was determined that she needed a bone marrow biopsy (platelets over 1500 at the time--the upper extreme we were told). The biopsy revealed the genetic mutation (not sure if it was JAK2, CALR) and her hematologist prescribed Jakafi.

Most important to our family is that Mom doesn't suffer. While we hope for more quality time for and with her, we know the reality of MF, particularly when there are the factors of age (Mom is 84) combined with transfusion dependency, etc. . I continue to remain hopeful, as Mom is a fighter. But it's quite difficult, sometimes, to see my once-vibrant and active Mom reduced to a hospital bed, typically exhausted, undergoing test after test. I know each test she takes is testimony to her medical team's dedication to her case--and attention to every detail.

We have friends and extended family supporting us; all of whom have bent over backwards to offer their assistance with rides to/from hospital, dropping by to check on us, etc.. But events have certainly taken their toll on us--particularly on my Dad (also 84) whose health is quite delicate now.

I take great comfort and inspiration reading everyone's stories; and thank all of you for sharing your experiences-- whether you're a patient or a loved one of a patient. Going forward, whatever events unfold for our family in coming days, I plan to keep monitoring this forum, and am forever grateful to all of you for sharing your personal journey(s). Sincerely, Marina

[MCW22]

What your mum is going through is awful, however your description of what is happening is extremely helpful for others who may get to a similar stage. I recently progressed to MF from PV and am unsure of what will happen as the condition progresses. So when others give detailed accounts as you have, it can help us prepare for what may come. I appreciate that it must have been extremely difficult for you to write, but thank you for doing so.My thoughts are with you and your mum and family.

Carol

[Kiruna in reply to MCW22]

Thank you, Carol, for thinking of us. I'm glad that providing Mom's details helps you (and others) going forward. I also wish you and yours the best, and pray for many more years together.

[brightlys]

I'm so sorry about your mom. I don't have anything helpful to add, as I just have ET, but I'm hoping for the best for your mom and your family. Thank you for sharing your experience as a loved one!

[Kiruna in reply to brightlys]

Thank you so much brightlys for your kind words. We take each day as it comes, and pray there are many more with Mom. Hope you are feeling well and receiving all the right care! Thanks again.

[Mostew in reply to Kiruna]

I want to add my warm wishes and thoughts to all the other lovely posts .

When life is difficult community , in all its form is so important. Glad you have family and friends to surround you all and hold you up ..

day and nite someone will be on here , which is one of the many benefits of our wonderful MPN forum

[Kiruna in reply to Mostew]

Thanks Mostew--I agree this forum provides great comfort. We saw Mom earlier today and she was having a pretty good day, albeit drained from various antibiotics and a lingering lung infection. But she was in good spirits. I told her about this forum, and all the well-wishes from others affected by MPNs. And she gave me a big smile. So, that made my day. Wishing you and yours wellness going forward. Marina

[hunter5582]

Sorry to hear about the progression of the MPN. Your mother is very fortunate to have such a supportive family. It makes all the difference when struggling with a serious health issue.

It sounds like her MPn care team is thinking about good options for her treatment. The newer JAK inhibitors may be a good choice. Momelotinib is indicated for MF with anemia. Pacritinib in indicated for MF with thrombocytopenia. We are very fortunate to have more options now than we did just a few years ago.

Wishing you, your mother, and your family all the best. You will all be in thoughts and prayers.

[Kiruna in reply to hunter5582]

Thank you so much. You're so right--family support is so critical. While ours is small, we also have a network of friends here, who are very supportive to us. I'm hopeful that Mom's medical team will pinpoint which medication is most suitable for her current state.

Because of past anemia, I'm guessing Momelotinib might be a good option, but we'll see. She'd been started on Jakafi but was immediately discontinued due to severe bleeding, coinciding with a bacterial infection. I don't believe she experienced the infamous "withdrawal syndrome" but I could be wrong. I agree that we're very fortunate to have these new options.

And thank you for your kind wishes as well. Helps me get through the day a little more easily! All the best to you and yours.

[Lillysue19850705]

So sorry your mother has to go through this. Do her doctors consider to use interferon as part of the treatment? In any event JAK inhibitor seems a standard treatment option. Wish you and your mother all the best.

[Kiruna in reply to Lillysue19850705]

Thank you Lillysue. We'll be at hospital tomorrow and hope to speak with her team about medication options going forward. I assume interferon is available here in France/EU, but we'll confer with her hematologist on what they ultimately recommend. Thanks for your good wishes.

[TimothyHLuff in reply to Kiruna]

There is currently no interferon approved for MF treatment in Europe I believe. However IFN has been there in the MF treatment for decades. Off-label use of Besremi or Pegasys always remains a possibility in this regard. Besremi is approved for PV in Europe and the French authority granted reimbursement for compassionate use of Pegasys as well. Good luck.

[Kiruna in reply to TimothyHLuff]

Thank you ,Timothy, for this information. I'm still not clear on what meds are approved in France and we're trusting Mom's medical team will determine the right course of action.

I understand that MPN specialists in France are limited--and there is one located in Paris--not very practical for our purposes. But we trust the hematologist and hospital team in charge of Mom's care so far. We'll certainly explore all the options available for her specific case. Thanks again.

[PhysAssist in reply to Kiruna]

Hi Marina,

We are so very sorry to hear about your mom's difficulties, but you can be assured that warm thoughts and prayers are heading her/your way- for whatever good they may do.

In addition, may I say that you might consider openly requesting and encouraging her local Heme providers to reach out the the MPN experts in other cities like Paris- something they might do [be doing] anyway, but if you suggest or request it explicitly it might give them more motivation to seek out their expertise, which sometimes we professional forget to think of doing.

It's very easy to get caught up in the care you're busy providing to the point of forgetting to reach out to those who may know more and have access to more advanced treatments and ideas for them.

God bless you and yours, and remember to keep taking deep breaths!

Best,

PA

[Kiruna in reply to PhysAssist]

This is helpful advice. We're supposed to confer with the hematologist this week on next steps. I'll be sure to ask whether they've consulted with MPN experts if they haven't already.

Thanks again for your prayers and kind wishes. We're grateful for everyone's feedback through this amazing forum. Best to you and yours too.

Marina

[Maurabawn]

Sending strength and love to you all during this challenging time. X

[Kiruna in reply to Maurabawn]

Thank you so much. Appreciate your kind words.

[champ30]

Thinking of you and your family X

[Kiruna in reply to champ30]

Thanks so much. I see so much kindness on this forum and am grateful for everyone's well-wishes.

[Janis12]

Your mum is so lucky to have such a great family and network of friends, you all support each other, imagine being on your own with all of this. It sounds like mum is undergoing lots of investigations so you would expect the best treatment option in the long term which can only be a good thing. I have ET and I am 65 years old, I hope I get to be as old as your mum, none of us have a crystal ball. Best wishes to you all.

[Kiruna in reply to Janis12]

Hi Janis--thank you. I hope you are feeling well these days! My Mom was diagnosed in 2018 with ET, but we suspect she had it long before the diagnosis. She was doing quite well until late 2023. We hope to have many more days with her. Wishing you all the best too, and appreciate your support.

[lizzziep]

Sending love and prayers to your mum, you and all family.

[Kiruna in reply to lizzziep]

Thank you for your kindness!

[Bullace]

All good wishes to your Mum and friends who are supporting you right now and hope the hospital is able to find the right treatment. It sounds like you are all in good hands. Hilary

[Kiruna in reply to Bullace]

Thanks Hilary--we're so grateful for everyone around us (including of course, Mom's dedicated hospital team). We appreciate the kindness of everyone on this forum! We're hopeful to have more quality time with Mom. We want her to feel well so she can enjoy time with family and friends. Thanks again, Marina

[Inca]

Hi Kiruna,I am MF & 84 yrs old too .I was P V for 11 yrs progressed to M F 4 yrs ago.I have had transfusions of blood & plaquettaire,several Bone marrow biopsies ……I have excellent treatment at my Heamotology dept some very experienced Drs of M P Ns .My Medicine General is excellent as well checks me monthly & I have pris de sang every 2 weeks from my nurse at my home.The care here in France is excellent.My Very Best to your mum,tell her to keep positive & strong minded.I am often desperately fatigued but I work as a sculptor when I can ,rest a lot of course but my husband & my German Shepherd dogs keep me going.Very Best to you too I know from Hubby & daughter the worry for family is a difficult time.My daughter lives in U K but gets over here when she can.

Pensées Chaleureuse ,Sally

[Kiruna in reply to Inca]

Dear Sally--you're truly an inspiration! My mother is also an artist (painter) and prior to falling seriously ill, actively participated in the artist community here. She worked as an artist for some years. Fully agree about the healthcare here in France. Her caregivers, both in hospital and "chez nous" have been exceptional.

We have to remind Mom to keep positive--and we know she's a fighter. I'm going to relay your story to her (and to our family and friends). A huge thank you for sharing this.

Bisous Marina

[Inca in reply to Kiruna]

Hi Marina,I paint too ,have worked professionally since leaving Art College.Iam finding painting ( just for myself)much more relaxing now as I don’t have to worry about time & getting things finished.Sure if mum starts painting ( splashing madly for herself ) it will help her rotten M F.Believe me it works.Tell her I think of her & know absolutely what she is going thru,& how suddenly the fatigue makes you feel old.Fighting it is necessary, & painting is a big distraction from thru our worries & pains.Very Best to you all & Pensees Chaleureuse.Keep in touch it would help me too that someone my age is suffering MF.I do have a small dose of Jakafi5 morn & 5 evening & also lately a mild anti depressant which does help ☺️Sally

[Kiruna in reply to Inca]

Hi Sally--I agree with your method of encouragement! Mom told me today she's looking forward to getting back to painting, or at least drawing. It's wait and see right now as she has a way to go before they think of releasing her from hospital. We're guessing Jakafi will be the next course. I'll pass your thoughts onto Mom when I see her tomorrow. Glad to know you're keeping up with your art too, as it's clearly effective medicine in so many ways. Will check in with you later. 😉 Marina