Anyone have Myelofibrosis (CMS-HCC)?

Good morning.

I’m 71. I was diagnosed with myelofibrosis a few years ago. Was asymptomatic at first, then the symptoms appeared. Weight dropped from around 188 to 138. I was a bag of bones. Had difficulty getting up from the sofa .

Went to MD Anderson in Houston to see about having a transplant. They advised that my liver fuctions were too low vs. the risks from the procedure. U of Miami said the same.

Now treated at MCI / Baptist in Miami. They’re great!

Abdominal discomfort led me to the Baptist ER. PetScan revealed suspicion
for Erdheim Chester Disease (ECD, an extremely rare blood cancer). Biopsies (2) confirmed. So, I have myelofibrosis and ECD - very rare combo. I’d prefer surf and turf. LOL.

But, the good news is, there’s a pill for ECD from Genentech called Cotellic. The pill immediately attacked all my symptoms -symptoms we had thought were from the myelofibrosis.

I’m back to life. 170 pounds. Hemoglobin is 13ish. Not the same person as before the first diagnosis, but back to being me - more or less.

MCI gave me Jakafi last year, which totally crashed my levels. I end up in the hospital in need of a transfusion with hemoglobin in the 6s. I stopped the Jakafi and got better immediately. So, Jakafi isn’t for everyone.

Hi @samlupowitz

I'm curious... When you say "...was diagnosed with myelofibrosis a few years ago. Was asymptomatic at first, then the symptoms appeared. " Would you be willing to share how long you stayed asymptomatic in wait-and-observe mode before opting for chemo pills?

(Anyone one else in the wait-and-observe mode out there?)

@gajokos Sure. I’m working from memory, but I would say I had zero symptoms for about a year and half, and then the night sweats and abdominal pain and bone pain in my feet and burning nerve endings in my toes and anemia, and 50 pounds of weight loss came. But, then I was diagnosed with ECD and given the targeted med (not chemo), and all the symptoms went bye-bye. Not sure what symptoms I ever had that would be solely or directly attributable to the myelofibrosis. Probably my slightly enlarged spleen!

@samlupowitz
I saw on your profile that you joined this group 3 years ago, so I guess that's when it all started, right? You mentioned that the jakafi crashed all your levels and you had to discontinue it. So where are you at now with or without treatment for the myelofibrosis?

@gajokos I’m bad with remembering dates and timelines, but yes, I probably joined this forum
fairly soon after being diagnosed with Myelofibrosis. Other than the Jakafi that was given
to me sometime last year -
and which crashed my levels - I have taken no medicine for the Myelofibrosis. I have my blood checked and see the oncologist every 6 to 8 weeks, and I have scans and MRIs every 6 months, but that more to check for the ECD. My weight is steady at 170. Zero night sweats. No pains worth mentioning. I sometimes feel anemic
although my hemoglobin was 13.2 yesterday. And, when I drive, I feel a bit off, which my oncologist thinks is my thyroid. I’m not what I was 10 years ago. I think it’s the diseases, not age. I do occasionally fast for 48 hours. I think we (humans in general) eat too much. So, I don’t do 3 meals a day anymore. I’ve had a total of 4 bone marrow biopsies - not fun - and they say my Myelo hasn’t progressed. And the Cotellic seems to have stopped the ECD, but it causes cystic acne - hate that. Not sure if this is the type of information you were after, but … that’s my story.

I was diagnosed with Myelofibrosis last summer. My platelets were high and I had funny looking red blood cells. Also had JAK mutation. I started 20mg Jakafi and red blood cells and hemoglobin went way down. Platelets went down to normal. Reduced Jakafi to 10 mg and became anemic and depressed and didn't want to live with no energy to do the things I enjoy.
No longer on any meds at present time. Spleen is ok. I get the itchiness about two times a week and have good days and not so good days as far as energy is concerned. See dr in April.
I was very disappointed Jakafi did not work for me.

Good morning, Lori.

No, just the Cotellic. I do take some meds for blood pressure and thyroid, but that’s about it.

I will say this though - and it’s
kind of in response to your comment regarding the human body - I’m no expert - just a guy with common sense, I think, and my common sense way of thinking leads me to believe that extreme fasting - meaning, 48, 72, 100 hour plus fasts - are cancer killers. I further gather from all my experiences that although many doctors are wonderful - and many aren’t - there does seem to be a disconnect in the profession, whereby, as a whole, they don’t seem to be open to the very obvious idea that we are what we eat, we are how
much we eat, and that just maybe we ought to allow
our bodies to do what they’re capable of doing by not inundating our system with food every few hours - which our bodies simply don’t need. It’s just an addiction like all others. We don’t need to be constantly eating.

Hi @samlupowitz. I’m in thought alignment with you. I remember from an early age when my mom would say, “You are what you eat”. That little nugget has stuck with me throughout my entire life. Our bodies are created with the ability to heal. What we put into them, long term, I believe has an impact on every cell. Unfortunately we can’t control every aspect of input into our bodies with pollutants, toxic chemicals, gamma radiation, etc., but it’s up to us to do the ‘best we can with what we know.” Alas, even our greatest attempts at remaining healthy can go awry. I did everything I could in my adult life to avoid getting cancer. Having a blood cancer never even entered my thoughts. Well, life had other plans….
Anyway, back to what you’re saying…There are some very interesting studies discussing the health benefits of fasting. While in recovery from my bone marrow transplant I had a supportive conversation with my dietitian regarding fasting. However at the time, since I was already malnourished it was not even a discussion! But there was mention of the benefits of intermittent fasting and for the most part, I guess that’s how I live. Also, I’m no longer a vegetarian but follow the Mediterranean diet with healthy proteins, whole grains, veggies, with avoiding excess carbs/sugar. Lots of exercises, mental activities, solid sleep and the like.
Finding doctors who align with your thoughts is another issue. Functional Medicine Doctors are less conventional and so if you’re finding it challenging securing a doctor who shares your views, you might want to consider a FMD. https://www.ifm.org/functional-medicine

Wishing you continued good health. Thank you for sharing what has been working for you. How often do you have follow-up labs?

Hi @samlupowitz. I’m in thought alignment with you. I remember from an early age when my mom would say, “You are what you eat”. That little nugget has stuck with me throughout my entire life. Our bodies are created with the ability to heal. What we put into them, long term, I believe has an impact on every cell. Unfortunately we can’t control every aspect of input into our bodies with pollutants, toxic chemicals, gamma radiation, etc., but it’s up to us to do the ‘best we can with what we know.” Alas, even our greatest attempts at remaining healthy can go awry. I did everything I could in my adult life to avoid getting cancer. Having a blood cancer never even entered my thoughts. Well, life had other plans….
Anyway, back to what you’re saying…There are some very interesting studies discussing the health benefits of fasting. While in recovery from my bone marrow transplant I had a supportive conversation with my dietitian regarding fasting. However at the time, since I was already malnourished it was not even a discussion! But there was mention of the benefits of intermittent fasting and for the most part, I guess that’s how I live. Also, I’m no longer a vegetarian but follow the Mediterranean diet with healthy proteins, whole grains, veggies, with avoiding excess carbs/sugar. Lots of exercises, mental activities, solid sleep and the like.
Finding doctors who align with your thoughts is another issue. Functional Medicine Doctors are less conventional and so if you’re finding it challenging securing a doctor who shares your views, you might want to consider a FMD. https://www.ifm.org/functional-medicine

Wishing you continued good health. Thank you for sharing what has been working for you. How often do you have follow-up labs?

@loribmt Just read your message again. I’m sorry; I
seem to have missed what you’ve been going through. It was very early when I responded. Yes, I understand that fasting wasn’t possible for you in the immediate aftermath of a transplant. Sounds like you have a good balance in terms of diet and exercise though. Regarding the Med diet, I heard that by far the reason the diet is good is mainly only from the benefits of high quality organic olive oil.