ET CALR - Heavy Flu like head, neuropathy

[George1976]

Hello all,

64 years old male here in search of reasons for feeling miserable every day. I’m dealing with flu like heavy head within which my heartbeat throbs from my head down my spine. Very little pain just the pressure of my beating heart and sick mild dizziness.

Diagnosed with ET CALR 8 years ago by chance at routine physical. Had been extremely active up until then. Platelets were 1.7m. Put on anagrelide of varied dosages for 2 years and felt awful most of the time with platelets normal to just below 1m. Switched to weekly 45mcg Pegasys in 2016 and although I felt pretty bad the day or two after injection, I felt much better. Platelets in normal range. I could still run laps at high school track and life was pretty normal until July 2020.

In July of 2020 I developed some mild weakness in my feet they said was poly neuropathy along with lumbar spine issues considered by most docs to be too insignificant to cause my weakness. In fall of 2020 I tried to stop Pegasys to see if it’s effects would leave me in remission, and relieve the neuropathy. After a month I went back on irregular Pegasys dosing as platelets rose. The sick head started in October 2020 along with moderate weakness in my neck. Also developed GERD in December 2020. By January 2021 my sick head was quite debilitating especially late afternoon until bedtime. In February 2021 my last dose of Pegasys gave me horrible neuropathic pain in my legs. So with platelets still under 1m I began 500mg daily of Hydrea. This increased to 1 daily and 2 every other day in June 2022. Platelets around 500k now.

Have seen 10 different neurologist and spine docs. And several hematologists, and none of them can explain why I have this weakness but even more importantly why I have felt so incredibly bad for the last two years. I even had a spinal fluid test, results of which one neurologist thought I had an autoimmune disease driving my neuropathy. My other neurologist does not agree but has no clue why I feel so bad. All the while I am feeling absolutely horrible almost all day and night every day.

I am hoping to get yet another opinion on the autoimmune question soon.

Do these symptoms sound familiar to anyone?

Any recommendations on next steps I should take?

Thanks in advance for any thoughts.

[socrates_8]

Hey George...

Are you aware of which CALR Type you are? There are over 50 different Types, however, circa 80% of them either fall into Type 1, (more benign & less likely of thrombosis events), & Type2, which usually indicates, very high Platelet counts, and in my case, I have had a number of TIAs, (minor brain strokes).

Have you ever had a Bone Marrow Biopsy (BMB) done? These can identify if your bone marrow has much in the way of scarring, (aka - Fibrosis). Usually scaled across 4 Levels, from 0-3. Zero most benign, & 3 the worst etc...

The other thing that can also be looked into is whether or not you might have any other 'high-risk-molecular'm mutations?

Next Generation Sequencing, (NGS), might be able to illuminate any other issues, (should they exist at all that is of course...?)

Anyways, best wishes George, & pls keep us informed of what you eventually discover, ok?

Steve

(Sydney)

[George1976 in reply to socrates_8]

Thanks Socrates_8. The biopsy from 2015 says I’ve got a 5-bp insertion/type 2 mutation in Exon 9 of the CALR gene. MF-0 of 3. No evidence of JAK2 or MPL.

I’m hoping the 4 years on interferon at least delayed progression to some extent but it’s weird that my last dose caused so much pain. I’d rather be in that than the Hydrea.

[socrates_8 in reply to George1976]

Hey George... Yes, well it sounds like you are definitely CALR Type2 buddy... Hence, No JAK2 Or MPL, because you are CALR etc...

Your BMB scale of MF - 0 is also a great sign that you have not progressed because there is No Bone marrow scarring present, (Fibrosis), according to what you have revealed here etc...

Just a tad curious to know how High you Platelets usually are etc...?

Sometimes, having very high Platelets can cause Tinnitus, I know that it certainly does in my own case... For I am also Post ET / MF w/ CALR+ Type2...

Best wishes...

Steve

[George1976 in reply to socrates_8]

Socrates_8,

Thanks for the reply.

That biopsy was in 2015. I’ve had a lot of extra blood testing done in these past two years trying to figure out what’s going on and still neither of my hematologists suggest I get another biopsy, but I wonder if I should.

After trying to stop INF platelets have been no higher than 845k. My tinnitus started when they began rising, right around 600k. Before being diagnosed with ET 8 years ago I had no tinnitus and the platelets were 1.7m. So I’m not sure the platelets are the problem now.

[socrates_8 in reply to George1976]

Hey George...

Yes, & those Platelets of yours are still very high, in my view...

Over these past Five to Six years, my Platelets have always ranged up & down between low 700s - 1M marker, & whenever I surpass 1M, a range of symptoms & side effects seem to appear...

My own experience has taught me to attribute a "Red Flag" to those certain symptoms, whenever events seem to be aligning in what others might term a "coincidental" manner... As my tone might indicate, my mind is more a believer of the scientific theory of "Cause & Effect" as a more likely culprit, in those instances...

Blood tests, (effective as they often are), cannot alone reveal many of the more obscure findings, usually uncovered through Next Generation Sequencing (NGS), & BMBs...

2015, is seven years ago, & much can transpire in 7 years George...

My NGS, discovered that I also carry a High Risk Molecular (HRM) mutation known as ASXL1, which generally denotes a more adverse prognosis... However I am still here, w/ my seemingly long, slow burning MPN wick... (?)

MPNs, are after all, chronic illnesses, and most of us will share their company, (unpleasantly or otherwise), for the greater majority of the rest of our lives, in my view...

Only a very small percentage of us usually progress onto (Blast phase) MF, or indeed AML... But hey, that's a good thing right?

So we all just have to best find ways to better manage our wayward MPN shadow company, and learn how to become the most positive, self-advocating force, for our own MPN afflicted lives... do we not?

In my view, our longevity & quality of life, is largely in our own hands...

Stay happy, safe & well George...

Best wishes...

Steve

[Jazzyb50]

Hi, I have PV and have had a similar head problem for a year now that makes me really miserable. It's a constant whooshing/fizzing sound in my head not ears. Seems to be at the left side of my head, not painful. Always there, even during the night and pulsates like a heart beat. Some nausea and dizziness. MRI scan clear. Had some issues with high blood pressure. One doctor said not enough to medicate but another one prescribed tablets to see if the head problem was related and if the tablets would help. They made me feel worse. I hope you get some answers.

Jane

[George1976]

Thanks Jazzyb50.

Sounds similar to what I’ve got going on except the only sound I hear is ringing in my ears.

In my case, one of my theories is that perhaps if I get my platelets down to true normal the head issues will calm down. Have not had true normal platelets in just over 2 years and will need to take even more Hydrea to do that.

[hunter5582]

Sorry to hear that you are having such a rough go of it. It can be very difficult to sort out what is what with MPNs and the related and unrelated conditions we experience.

What you are describing could be any of a number of things. As the one doc suggested, an autoimmune disease is one of the possibilities. Unfortunately, autoimmune disorders are a possible serious adverse reaction to Pegasys. That is not to say that the Pegasys is the issue, but the link between the last dose of Pegasys and the neuropathic pain is suspicious.

I would definitely follow up with an immunologist. That may be what is going on. Hopefully you will get an answer and new treatment plan soon.

Please do let us know what you learn and how you get on.

[George1976 in reply to hunter5582]

Thanks Hunter5582. Will do.

[JaK2ET]

I hope you get help very soon from a doctor who can understand your condition. Best of luck!