Hello everyone. My name is Tiffany. I've been reading through some of the posts relevant to my interests but haven't had a need to post myself until now.
My mother was diagnosed with polycythemia (follow-up tests show she does have JAK2 mutation) this past August, right around her 74th birthday, after routine lab work. Her body had actually started to go haywire December 2021 with sudden HBP, vertigo, suspected TIA's, etc. that culminated in a stroke in May 2022. Bloodwork had been normal up to and immediately following the stroke aside from a flag for abnormal RDW the month before the stroke.
Her treatment has been phlebotomy along with the 325mg aspirin she was on anyway from the stroke and she was doing well only needing to go in every couple of months to have the draws. However, her platelets had been continuously rising and when they got to just under 1,000,000 the decision was made to start hydroxyurea.
She was put on 500mg 2x a day and started on March 14. Her first labs after that were March 24 but we wouldn't get the results until her appointment on the 31st. March 25 she started experiencing significant back pain to the point that she needed a cane and some days could barely get out of bed. On the 30th we decided to stop the hydroxyurea and see if the pain eased to test if maybe it was a side effect. We had her appointment on the 31 but the hematologist wasn't there so we saw his nurse practitioner who told us the platelets looked great at 536,000. We were shocked at the drop in just 10 days of taking HU. We were going to stay off the HU until we could see the hematologist, monitor the back pain, and see about further testing if it didn't resolve. Side note: we've since seen a chiropractor who dx'd really tight hamstrings causing too much strain on lower back muscles and the stretches he recommended have been easing the pain so likely not a side effect of the HU.
She had a phlebotomy April 3 and labs drawn April 5 with a follow-up appointment today with the hematologist to discuss results which is where we found out her platelets were 126,000 (only 2 weeks after them being perfect and after being off of HU for a week) and hemoglobin dropped from 14 to 10. We had more labs drawn today to make sure it wasn't a lab error and I just got the call that the results were pretty much the same.
The doctor is supposed to come up with a game plan for further testing to see what's going on and we'll find that out next week. I just feel terrible because we were just talking about how phlebotomies every couple of months isn't so bad and oh, how it could be so much worse, and I'm scared that this wonky blood work may be a sign that it just shifted into *so much worse.*
Does anyone have any experience with HU causing such a drastic drop in platelets and/or concurrent with hemoglobin? I've seen where some patients go into cyclical thrombocytosis then thrombocytopenia and it's considered a rare side effect and almost *normal* for them because that's just how they react to it. I don't know what the odds are that this is what we're dealing with in her case seeing how she only took it for 2 weeks.
I'm seeing what other possibilities could be behind it but I'd love to hear personal experiences, good or bad. IMO, the more information one has, the better.
Thank you.
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Hi Tiffany. Such a worry for you and your mother. Not knowing what is happening is the worst thing. I hope someone of greater experience will reply to your post, and I certainly can’t give you medical advice. However I would urge you to get in contact with your Heamatologist’s secretary before you change your mother’s medication and request an appointment as soon a possible. Mum’s back pain could be for multiple reasons, so don’t panic, but do get the best expert advice. HC has given me decades of time, and if it isn’t working for you, there may well be alternatives.
Thank you David. The hematologist agreed we should not be on the HU at this moment due to the drastic drop in platelets and hemoglobin so everyone is on board there. Regarding the back pain we're working on the assumption that the chiropractor was correct about the tight hamstrings and she is doing the stretches he recommended and has had a little relief already. He said in about a week she should see a big difference. We're due to speak with the hematologist again next week so the timing lines up right for us to know if it is muscular and being treated properly or if we need to start looking for other causes.
I'm beginning to think that her dose was just too high for her and it caused excessive myelosuppression. The treatment for that seems to be stopping the HU for 2 weeks (halfway there already) and it should resolve, then try again with a lower dose if necessary.
sorry to hear things are so difficult, if in doubt it’s a good idea to try to see a MPN expert who can be consulted every so often and even work with or write to your local Haem with a plan. I would imagine they will do a BMB to see what’s going on.
The issues with vertigo etc prior to diagnosis is quite common even with apparently normal counts, I had same for a couple of years prior to diagnosis. The CBC doesn’t show all, eg in MPN we have sticky platelets even when platelets count is normal, sticky platelets can cause symptoms , often sorted or partially sorted by taking aspirin.
Thank you ainslie. Her hematologist/oncologist is not on the lists you can find online for MPN specialists but he has a very high rate of treating PV patients. One of the rating sites has his statistic for that as *he treats PV more than 91% of his peers.*
I'm definitely not one to stick around if I feel she's not getting the care she needs so I have no problem uprooting her if need be. We live about an hour and a half's drive from the number one cancer treatment hospital in the US and there are a handful of doctors there from some of the MPN specialists lists so we do have options if it comes to that. We haven't gone that route from the get-go simply because she has been seeing a neurologist and vision specialist in that city and she hates the days we have to make the drive for those.
That is interesting to know that the symptoms can be there without the blood work being abnormal. We just spoke a few days ago about how the PV likely didn't have anything to do with her stroke as it came about afterwards but apparently that isn't the case and it likely still was the cause. Her father (and all of his siblings) died young from massive heart attacks and after her diagnosis we may be onto the reason as to why. Her mother had vascular dementia and her oldest sister died at 70 from some type of bleeding disorder and Alzheimer's. She was very private so nobody really knows what the actual diagnosis was but from what we've been able to gather from various family members combining what little they each knew, her body was producing too much of something, then the treatment for that caused severe anemia and she would have to go in for iron transfusions and there was always some level of internal bleeding or *seepage* they would regularly have to pull off. This went on for years. She also had a history of migraines. Mom would occasionally have what she called *visual migraines* with no pain but visual aura. Again, after Mom's PV diagnosis what my aunt was dealing with now makes sense to us even if we don't know all the details accurately. Definitely seems like blood/vascular disorders are heavily mixed into our family genetics.
one thing to consider is MPN can run in families, my father had it and now my sister has it.
Often MPN goes undiagnosed until someone has a thrombotic event, I mention this as you mention major thrombotic issues in your family. Some Haems recommend that if someone is diagnosed with MPN to get the rest of the family checked for MPN, as you know us with MPN are more at risk of thrombotic risk by a factor of 2 or 3. As we now know the initial stage of MPN may not show in a CBC it may be worth checking Jak2 etc, 95% of PV patients have Jak2, a good Haem should be able to advise.
Visual migraines are common with MPN even with normal blood counts, aspirin usually helps.
Hello and welcome . Sorry to hear about the struggles your mother is experiencing.
PV is a complex disease that can manifest in a variety of ways. At the core, MPNs are inflammatory disorders. The deregulation of the JAK-STAT pathway does more than make too many blood cells. The JAK2 mutation also causes the body to overproduce inflammatory cytokines. This is thought to be responsible for many of the secondary and constitutional symptoms people with PV experience. The increase inflammatory environment is also thought to promote progression of the disease. Controlling inflammation is a key part of managing a MPN. Fortunately, there are things we can do for this. There is evidence to support a Mediterranean diet, which is anti-inflammatory. Some of us here on the forum have had success with supplements such as curcumin and N-Acetylcysteine. Note that should your mother wish to pursue complimentary health interventions, it is important to get expert consultation and let her care team know.
Regarding HU, you will hear a range of responses. It can be a very rapid acting cytoreductive medication for some. It is not effective for others. Some people tolerate it. Others do not. I am one of the latter. HU was not effective and I experienced toxicity even at very low doses. I did opt for phlebotomy-only for about 2 years, but the iron deficiency symptoms became worse than the PV symptoms. I have found the interferons (Pegasys, Besremi) to be far more effective and much easier to tolerate than the other options I have used. Note that we are all different in how we respond to the treatment options.
Here is some information about HU. If you are involved in handling the HU for your mother, please take note of the handling instructions. You would need to wear gloves. Your mother should wash her hands after handling the medication.
MPNs are rare disorders. Most doctors, including hematologists, have little experience with them. Many do not have the KSAs to provide optimal care. The understanding of MPNs and how to treat them is evolving, particularly in recent years. Your mother would need to consult with a MPN Specialist to ensure optimal care. If there is not a MPN Specialist near her, it is possible to consult periodically with a MPN Specialist about the care plan while having a local hematologist handle the care delivery. This is what I do. This arrangement works great. Here are two lists of MPN Specialists.
It helps to build a base of knowledge and learn to speak the same language (medicalese) as the MPN care team. There are some excellent patient education forums that are worth attending or viewing. Here is one example mpninfo.org/conferences/202...
Thank you very much Hunter. Whew, I have a lot to read. We very much would like to incorporate complementary/more natural interventions. It's driving her crazy that she's been so healthy all her life----she never even wanted to take an Advil----then completely overnight she's having to take prescriptions and worry about side effects and interactions, etc.
Complementary health interventions can be very helpful. There are two presentations on this topic in the 2023 conference. Dr Gowin's presentation on Complementary Approaches for MPNs and Dr. Fleischman's presentation on Nutrition and MPNs will be of particular interest.
I have been successful in using several complementary health interventions. I do not take it for granted, however, that everything natural is good. If something is biologically active enough to help you, it can also hurt you and interact with things. That is why I consult with an Integrative-Functional Medicine doctor. Here are two links should your mother wish to pursue that option.
When I was 65, I was diagnosed with ET, with JAK2 mutation, in 2017 and my oncologist prescribed a daily dose of 500 mg Hydroxyurea, along with 81 mg of aspirin.
My PLT count was 500+ at the time and the Hydroxyurea got it down to about 375, with only some tiredness and mouth sensitivity (used Biotene mouthwash to address that) side effects to report. I read that taking 81 mg of aspirin 2x daily was 88% more effective than taking it once a day to reduce the probability of having a thrombotic event and my oncologist didn't have a problem with that, so that's what I did.
I can understand why your mother's oncologist initially put her on Hydroxyurea twice a day if her PLT count was 1000+, because my oncologist was more concerned about my ET than she was about my Lymphoma, but that dosage has proven to be too much for her.
The Nader point for Hydroxyurea is 10 days, so it is not surprising that the large drop in PLT count was observed after that period of time, but the PLT count of 126 was clearly too low. Stopping Hydroxurea until her PLT count gets well into the normal range again is definitely the right thing to do, but it appears that your mother responds well to Hydroxyurea for PLT count reduction, so starting to take it again at a dosage of once per day, or three times a week going forward may be more prudent for her.
Talk to your mother's oncologist about her taking a Folic Acid supplement to address the low hemoglobin issue. From what I've read, that should help.
Thank you Bill. The aspirin timing thing and folic acid are interesting. I feel like all I do anymore is research things. It's come in handy though----when it comes to medical care I've learned to trust....but verify. Some doctors appreciate when patients (or their loved ones) are involved and proactive in their care and some aren't interested in what you think you know. We've had very good luck thusfar with her providers in that regard.
I agree that research is important, so one can help to understand the disease to be your own best advocate for health care going forward.
When I first got diagnosed with ET, I researched it online until there weren't that many articles left to read. My oncologist recommended against doing that, but I did it anyway.
I have to admit that I did spend way too much time focusing on things like 5-year survival rate and worst-case side effects taking Hydroxyurea at first, until I finally realized that it was a waste time doing that and then I started focusing on things that I do have control of, like exercise, nutrition, supplements, and anything else that I could do to help reduce the symptom burden.
... We could demonstrate with high statistical confidence that twice-daily aspirin administration reduced interindividual variability in sTXB2 and lowered the residual sTXB2 level by ≈90%. However, no further improvement in antiplatelet pharmacodynamics was achieved by a thrice-daily regimen, suggesting that a ceiling was reached in matching accelerated renewal of the drug target with a shortened dosing interval. Both experimental regimens similarly reduced in vivo TXA2-dependent platelet activation, as reflected by urinary TXM excretion, consistent with saturability of platelet COX-1 inactivation with twice-daily aspirin administration in ET. The apparent endothelial safety of such a regimen in sparing PGI2 biosynthesis confirms the preliminary findings in a small sample of ET patients,22 apparently at odds with earlier findings in healthy subjects.26 It remains to be established whether the markedly different study design and size and/or the enteric-coated vs plain aspirin formulation used in these studies accounts for the apparent discrepancy.
Based on the present results, we have chosen aspirin, 100 mg twice daily, as the experimental regimen to be compared with the standard once-daily 100-mg regimen for maintenance of superior antiplatelet efficacy, compliance, and tolerability in the long-term phase of the ARES study, in which the same patients with ET are being rerandomized to 1 of the 2 aspirin regimens.
We conclude that (1) the currently recommended aspirin regimen, 75 to 100 once daily, for primary or secondary cardiovascular prophylaxis is largely inadequate in reducing platelet activation in the vast majority of ET patients; (2) the antiplatelet response to low-dose aspirin can be markedly improved by shortening the dosing interval to 12 hours, with no significant improvement seen with additional decreases; and (3) the long-term superiority, compliance, and tolerability of an optimized aspirin regimen remain to be investigated in the ongoing phase of the ARES trial.23
dear Tiffany. I have been on hydroxcombide for three years now it is difficult to say how it is affecting me overall I have No problems and now my platelets have reduced too 220 But I have just received a letter showing my transferrin saturation has increased. Maybe genetic or pills. Don’t know yet. But I would say the hyroxycarbamide is better than a stroke or heart attack Best wishes. Instow 1
Thank you Instow, it's great to hear it's working so well for you. I suspect it will for my mother as well assuming she's just a hyper-responder and tweaking the dosage will resolve the issue she's having now. She had no other side effects. Jury is still out on whether it may have contributed to her back pain but it really seems muscular in origin so I'm doubting it's involved.
Hi and welcome! I am so sorry to hear about your mother’s problems. My reply to you is quick and simple with no “medicalese.” Rather, just a quick summary of my experience with HU. I am about the age of your mother. I was diagnosed with MPD two years ago. No mutations. I was started on HU. It took several dose changes to nail the correct one. My MD carefully explained how tricky this medication is. He also believes that HU has the least side effects of all other (and there aren’t a lot) treatment options. My regimen alternates daily - 500 mg. one day, 1000 the next. I take my meds at bedtime because I felt a bit more tired. I have absolutely no side effects. I take care to use sun screen and stay away from people who have infection and the like. I’ve never been told to wear gloves - don’t be fearful here. Mayo Clinic doesn’t suggest you wear gloves either. You will find all kinds of articles on the internet. Don’t spend a lot of time driving yourself crazy. Just find the best specialist that you can. Then have faith. By the way, I had a consult with Koneti Rao. He is a research specialist in the field of platelets. He concurred with my treatment plan. Good luck to you and your mom. My best - A.
Thank you Alexis. As always, positive experiences are great to read and balance out the sometimes not-so-pleasant ones that we still need to know about in order to make educated, informed decisions. It's still very early in the game for her and HU and as thrilled as I was at the platelet levels at the first draw I guess it's probably too good to be true in most cases that it's a homerun on the first go-round as far as dosage and responses go.
Seems like such a balancing act with all of the cascading effects the management of one blood disorder can bring about with regard to all the other levels. She knows it could be so much worse but it's such an adjustment for her and how she's always lived. We keep telling her she deserves to be able to (forced to) take it easy and let others care for her now as she's always been the one to take care of everyone else. Doesn't seem to make it any easier for her with the loss of independence and having to rely on others so much.
I appreciate all of the thoughtful responses and knowledge I've been getting here.
My MD explained carefully that it takes time to find the right dose. All levels will eventually balance out. Please be careful about what you read online. Find a good MD and have faith.
If it’s any help; I’m 71 years old and was diagnosed with ET last July. I take 500mg HU daily and 1000mg on Sundays. Also 75mg Aspirin daily. I have had absolutely no side effects and my platelet count has continued to drop steadily from c600 to 325. Initially I was having blood tests fortnightly but now once per month and was seeing consultant monthly but now every 3 months. I think I’ve been very fortunate.
Hello,I was on Hydroxyurea for PV for 9 years, starting at age 50 During that time I experienced chronic joint pain and muscle aches, which oncologists dismissed because the Hydroxyurea was controlling my numbers. I went through all types of physical therapies to no avail. Last year Hydroxyurea stopped working for me and now I'm on week 15 of Besremi but with bad side effects. The answer to your question is that Hydroxyurea can indeed cause severe joint and muscle pain.
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