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ET: untreated elevated platelets for over 13 years
So I shared a bit on here not too long ago about being misdiagnosed with type 1 von Willebrand‘s disease in my early teens, and then more recently found out that I was misdiagnosed 13 years ago. I’m now 29. Since the VWD diagnosis, my platelet count was ALWAYS elevated, and rising over the years. At the time I found out that I did not have any clotting disorder whatsoever, my platelets were 670k. I was only at that appointment because I had a TON of new symptoms flooding my body that all seemed random and to make no sense, and those were all struck down as fibromyalgia and anxiety… when I shifted the conversation to focus on my platelet count, the oncologist could not give me an answer as to why my platelets were elevated so high for so long, nor could he recommend a medication to at least keep them “within range.” Which, by the way, would have been the first time in at least 12 years. He denied a bone marrow biopsy because I’m allergic to lidocaine and he said that he was uncomfortable with doing any kind of procedure like that with my allergies/hypersensitivities (that all came about basically after the 10 year mark of sustained platelets) and that he “didn’t believe it would be worth it.”
About four years ago, my body started to show signs of other things going on that my oncologist at the time thought were completely unrelated to hematology/oncology and sent me to a rheumatologist. And it went how you’d expect — blamed it on something else and didn’t want to see me again. Which is fine, since I know they have to be coming from the ET.
Now that I’ve been in a constant state of sustained thrombocytosis for well over 12 years now, and not a single piece of blood work ever showing that I’ve been “in range,” I wanna know if this rings true for others with ET (and more specifically if you’re out there — triple negative ET).
Please tell me about your experiences.
How many of you are in your double digit years, and what were some things that you noticed that changed in terms of symptoms and disease progression?
And how many of you had it undiagnosed or missed for years on end and what symptoms did you experience because of your sustained platelet levels? And that going untreated?
Listing all of mine would take up all of the brain power I have for a week, but the most recent and debilitating ones for me:
- erythromelalgia in hands
- shortness of breath that I thought was asthma flaring back up, but this is just constant now
- an increase in the chronic pain
- cognitive impairments that make it seem like I spent an entire day using my brain to solve science, even though I simply woke up for the day
- and extreme fatigue that I have never felt before in my life. I was diagnosed with chronic fatigue syndrome when I was 18 or 19. But over the last two years at the least, I have been getting so fatigued just existing
I see a different oncologist in two weeks and I want to be as prepared for presenting her with an argument that this isn’t normal and not sounding like a hypochondriac. Any advice would be so very much appreciated.
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@janemc have you had many side effects from the HU? That is my fear honestly. Do many lose their hair? Did it help the fatigue? I’ve also heard you are at higher risk for all kinds of other things including skin cancer which is also in my family.
@sdcgcarroll
While HU is indeed a chemo drug, taking the capsules is nothing like getting a chemo infusion. You're not violently ill and your hair doesn't fall out in clumps.
Do you know: HU also treats sickle cell anemia and mouth cancers? It has been in wide use for decades.
As with all drugs, some individuals do have side effects from HU. There may be some hair thinning, for instance.
For me, the worst effect is constipation, which I've addressed by adding fiber to my diet. Another effect is dry skin, so I now keep lotion readily available.
HU does make us more vulnerable to sun damage. Mineral-based sunscreens and floppy hats are my new best friends.
So why am I happy to take this stuff?
Because it frees me from the splitting headaches and utter exhaustion of ET! It protects me from clots and strokes. And it reduces the terrible strain on my bone marrow caused by ET.
It gives me hope of staying alive as long as my family needs me.
With platelet counts in the 600/700s, I was started on just one capsule of HU a week. I'm so grateful to my oncologist for that, because it helped me avoid any dramatic side effects.
I have hard-to-manage MPL-driven ET, so it took almost two full years to get my count under 400.
But relief from exhaustion and headaches started after just six months on HU.
For me, HU has been life-changing.
This is just my story! Keep scrolling through the ET threads to learn more!
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10 Reactions@janemc thank you so much. The fatigue is probably my biggest complaint. Thank you for your encouragement ❤️
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2 Reactions@sdcgcarroll I have been on HU since February 25 my side effects have been fatigue, Thinning of my hair. I get short of breath. My skin & eyes are very dry, I use lotion every day, Also, Systane eye lubricant for my very dry eyes.
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4 ReactionsHi @lexsjx
I’m 63 and just started hydrea (HU) 2 weeks ago. I’ve had elevated platelets for at least 20 years but they were low level elevated, like in the 400+ to lower 600’s. I guess I was on what they call active watch. At that point my response was harumph, my platelets have always been high and shrugged it off - not understanding implications that they might climb at some point and the condition become more serious.
Just about a year ago my platelets started rising more into the 700-800’s. So I saw a hematologist who knew about ET. I’ve now been through the blood tests for gene abnormality, two bone marrow biopsies (the first was not done correctly so was a dud). And my platelets reached 982
Over the past year I have experienced increasing symptoms that I didn’t understand - reduced hearing and loud ringing in my ears, bad headaches, increased fatigue and sometimes tingling in my feet and hands. I’ve had my blood checked twice since starting HU and the first week the platelets dropped 120k. This week the level was steady. But I am optimistic that I will see a reduction in the symptoms as I continue to take the hydrea.
I am considered triple-negative since no gene mutations were evident, even a very rare 4th mutation (JAK2 Xeon, or something like that). Now my Dr suggested a full genomic panel but I am waiting to have my insurance approve the test.
One hematologist I told me that MD’s worry when platelet counts reach 1,000 (really 1M) and to not wait until then to decide next move. Elsewhere I’ve read that at 1,500 is really concerning (red alert) for potential blood clots.
My current Hematologist really reiterated to me that my case is very rare (ET triple negative) so it is not something that all Dr’s understand being a rare thing. And not all Dr’s know what to do; refer you to a hematologist or preferably a MPN specialist.
One primary care Dr continually asked if I’ve ever had a stroke or a heart attack over several visits. Each time I said a shocked and adamant NO yo his question to which he responded “well if you feel any light-light-headedness or pain in my chest be sure to call 911.” His “solution” freaked me out to the point I did call 911 once and also caused me to ask for a referral to a hematologist. Obviously that primary care Dr. was not aware of ET or how to treat it.
At the suggestion of my brother, a hospitalist doctor, I asked for a referral to a hematologist. My brother humbly said that he is not a hematologist and does not have their specialist knowledge, like what triple-negative means.
I share all of this to conclude with a few lessons I’ve learned.
1. Find a Dr who understands ET and its variations
2. Understand it is a rare disorder so not all Dr’s know about it or understand (#1 stated differently)
3. If you aren’t getting your questions answered to your satisfaction find another Dr who can do that
4. Educate yourself on the condition
5. Get questions ready for your visit like:
-What is the next step to get to the bottom of this problem?
-Is a bone marrow biopsy or gene test appropriate?
-Should I start taking an aspirin a day to thin my blood?
-what are the risks that there is a leukemia lurking or mylofibrosis in my bones? (This is the rationale my specialist MD gave me for performing a bone marrow biopsy?)
I hope this helps you.
Best of luck with your appointment!
Meg
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7 Reactions@mmkennedy
HOORAY that your count has dropped 120!! You have had such a long wait for a diagnosis and a good doctor.
Are you taking aspirin? For me, that set off a low-level ringing in my ears, as my thinned blood flows through my head.
As for the primary care doctor warning you about a heart attack . . . he may not have fully understood ET, but with high platelets, the risk of heart attacks (and strokes) goes up. His advice was scary, but on point.
Your suggestions are excellent -- thank you!!
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1 Reaction@janemc
Thank you Jane. It is a relief to be finally taking HU. I don’t know if I really feel result already or imagining them. The power of my mind is so strong. But I am headed in the right direction.
Unfortunately I can’t take aspirin or any NSAIDs because so have anaphylaxis allergy to them. I’ve pressed several Dr’s asking about plavix (what my brother thought they’d do) but evidently the risk or bleeding is too high 3 different Dr’s independently said NO to plavix. (I think a strange development related to von Willebrand disease, VWB, a bleeding disorder) when platelets are high.
I get your comments about the blood clotting concern of my PCP.
Thanks for remembering me and celebrating progress with me!! 😉
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1 Reaction@mmkennedy
Meg, you'd mentioned your allergy to aspirin -- I'm sorry I forgot.
It is WONDERFUL you're responding so quickly to HU!
@mmkennedy
Thanks for sharing your story.
I too am ET triple negative.
I have had this since 2002-2003 so about 24 years ago. I have been on baby aspirin the whole time and Hydrea for the past 11-12 years. I am 71 yo and doing well. I agree with all of your advice. Getting a MPN specialist opinion is key to good care. These specialists can then send recommendations to your local oncologist. My advice is do not let your pcp manage your care (they have even less understanding). The big cancer centers will usually have MPN specialists and they are happy to see you and make recommendations to your local oncologist. Mayo and MD Anderson are two of the big ones in the Midwest but there are others on the coasts. I strongly advise every ET or PV patient to see an MPN specialist at least once.
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7 Reactions@lynnevb
Thank you Lynn! We are part of a very unique and small group of people as I understand it.
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