When I was first diagnosed with PV (age 59) my dr. had me do one phlebotomy (RBC 5.55, HCT 46.6, WBC 12, PLT 632 & ANC 9). He also discussed that guidelines say once you reach 60 you should go on Hydroxy but said it was just an arbitrary number.
He did not have me do another phlebotomy. 3 months later I started seeing an MPN specialist at one of the leading cancer hospitals. He did not have me go for another phlebotomy right away either. About 8 months later they had me try to get another phlebotomy. By then my blood seemed to be so thick, 2 hospitals and 1 infusion center could not get my blood out. Finally 10 months from the first one, I was able to get a successfully phlebotomy. It took several but eventually I got my HCT down to 42 or below and can keep it there for several months before needing a few phlebotomies in a row, then I am good for several more months. In addition to the phlebotomies I take 1 low dose aspirin twice a day. I am now 61.
My question to all of you is "how did you decide on your treatment"? Did you just accept whatever treatment you dr. recommended? Did you go by any symptoms you were having and how it was affecting your quality of life? Was your only concern potential blood clots?
I am also wondering how many of you are concerned with the potential side effects of some of these medications? Several mention potential skin and other cancers, which I have read some of you reporting on.
If you have already had a very rare/aggressive skin cancer, and a not so rare skin cancer, and some pre-cancers, would you risk going on any of these drugs that list additional cancers as a potential side effect?
Finally, how did you justify going on any medication based on the research? I find the experts are not in agreement about what really has an impact and what does not. I read research that says just because your numbers come down does not mean you are at less risk of blood clots or disease progression. I've read research that says the opposite. How do you decide to take on a new medication (because your dr. is strongly urging you to based on age) that may do you more harm than good?
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Making the decision about which treatment option to use is a very individualized process. There are a number of factors that go into making the decision. At the core of the decision making process is the patient setting the treatment goals and defining their risk tolerance. Each person's unique MPN profile, including actual symptoms and co-occurring risk factors have to be taken into account.
As you are already aware, controlling the erythrocytosis is the key to reducing risk of thrombosis for people with PV. While some use HCT<45% for all people with PV, there are others who differentiate the goal for males and females since females tend to have naturally lower HCT. The different target used for females is 42/43%. My suggestion is to individualize this target based on each individual's response. Some clearly find that the lower number is preferable based on their own experience.
Another factor is the actual symptoms that you experience. Symptoms like splenomegaly, pruritis, headaches, erythromelalgia can all suggest specific treatment approaches. The degree to which these symptoms impact quality of life impact the risks that one would be willing to take to manage the symptoms.
It is correct the age is just a number. Not everyone ages at the same rate. Using age 60 as a risk indicator is based on older research that indicates a higher risk of thrombosis in those older than 60. It is not an individualized risk assessment. It would be up to you to work with your care team to determine if this is a risk factor that should be applied to your case, My MPN Specialist told me "65 is the new 35." I did not initiate cytoreductive medication based on my age. I started for other reasons.
Regarding whether reduction in blood cell numbers reduces risk of thrombosis, it depends on which type of blood cell you are talking about. There is no question that uncontrolled erythrocytosis increases thrombosis risk. There is mixed evidence about leukocytosis, but it it may well be a risk factor if present. There in not good evidence that thrombocytosis increases risk of thrombosis just based on the number of platelets. There is evidence that PLT > 1 million increases risk of hemorrhage. Understanding how the risks work drives how you set your treatment goals.
You are correct that skin cancers are an intrinsic risk with some cytoreductive medications, specifically hydroxyurea and Jakafi. Some debate whether this should apply to Jakafi, thinking it may be the prior exposure to hydroxyurea that is the issue. It would be prudent to assume there is skin cancer risk with both. It would also be prudent to first consider other options for anyone who has a prior history of skin cancers, particularly an aggressive cancer.
The best way to decide about starting a new medication is through the process of shared decision making. It is not a matter of just blindly following a treatment recommendation. You have to decide for yourself what is in your best interests. Your doctor can advise, consult and educate, but only you can decide. The process starts by you making the determination of what your treatment goals will be. You also need to determine your risk tolerance. What risks are you willing to take in order to achieve your treatment goals? Note that not taking any medication is a choice that also has intrinsic risks. Phlebotomy-induced iron deficiency also has risks. You have to decide for yourself which option best meets the risk/benefit profile that is in your best interests.
The best doctors will take the time to walk through each of your choices and not just recommend one without discussion of the others. The NCCN guidelines recognize hydroxyurea, Besremi/Pegasys as preferred first-line options. Jakafi is also recognized as a front-line option but the FDA label indicates failing on HU first (which will hopefully change).
We are all different. We all have different profiles and different preferences. I prefer immune therapies over chemotherapy. I would also consider JAK inhibition and use of a hepcidin mimetic over chemotherapy. I also prefer cytoreduction over phlebotomy-induced iron deficiency. That is my treatment preference based both on treatment philosophy and my experience with hydroxyurea, phlebotomies, and the interferons. However, the right answer for me is not necessarily the right answer for anyone else. We are all different.
Hope that helps. All the best as you move forward.
Thank you for the response hunter5582. Your input has helped me focus on my treatment goals. My #1 concern is blood clots (and what they can cause), which it seems I may be able to keep at bay by keeping HCT <42, and my #2 goal is paying attention to my allele burden and when (if) it gets to be 35% or above, re-evaluate treatment options. I'm currently under 20%.
When I was first diagnosed I had a very long list of symptoms, which negatively impacted my life in a significant way. Although I still have many of those same symptoms, some don't happen as often, and others seem more tolerable. I'm willing to deal with all of them if it means avoiding adding a new drug with potential side effects.
The phlebotomies do cause iron deficiency and some anemia, but my tolerance has gotten better. I have some really bad periods, but lately have been having more good periods than bad, so may have turned a little corner. I think the PT and OT I've been doing are helping tremendously. I've also upped my Vitamin D intake. It really helps with some of the fatigue & joint pain.
This has been one of my main concerns,THE medication issue. I was on hydroxurea for 6 weeks, my platelets are about normal. Two Sundays ago I had some kind of reaction that had me shaking all over. This lasted about two hours. I quit taking it and have not had another episode. Not saying to quit taking what has been prescribed but to use your own judgment. I am 70 years old and still in in construction business. I am outdoors alone and I don't need something that causes skin cancer, just saying. Hope all is well with you today. Greetings from South Alabama
Strumin210 that must have been quite unnerving to all of a sudden have that shaking. Was the dr. able to shed any light on why it happened? Working in construction outdoors certainly makes it difficult to avoid the sun. Although I do not actively sunbath per se, my skin dr. and I have come to an "understanding" that I would be outside in the sun for 10 - 15 minutes a day before 10:00 am. This way I can still boost my Vitamin D (in addition to supplement), because it had been down to 13. I've managed to get it up into the 20s :-).
Thanks for sharing your experience. It helps to understand what others go through on these medications.
You’re right there are a few grey areas but some are black and white. Having HCT over 45 or some say 43 for women will definitely increase your thrombotic risk, hence it is essential to keep it under those numbers by testing regularly eg every 3 to 4 weeks if venisecting only and if it’s at or near those numbers get venisected asap. ALL good haems follow those guidelines. Whites and platelets are more grey area, it is generally accepted that high whites maybe a risk and most experts won’t treat platelets alone unless other health issues or symptoms if they are under 1 million.
Starting meds for the sake of it at 60 is just box ticking, it depends on your other health etc.
Drugs usually have some sides , some more than others , but they can also have benefits.
There is a good section on the MPN voice website explaining the ins and outs of the different treatments. There is some evidence that some of the drugs may slow progression for some people, that’s a big subject in itself.
If skin cancers , Hydroxy can cause them, Ruxolitinib may affect them.Pegasys etc won’t affect them but tolerance can be a issue for some.
I may be wrong but I suspect you MPN docs may not be giving you optimal treatment or guidance. Having good advice is essential. Most of the veterans I know have a local Haem for regular treatment but have a expert they see from time to time to set direction and they often write to the local Haem to advise optimal treatment, I think that is essential.
ainslie, thank you for your detailed response and for the reference to section on the MPN Voice site re: ins and outs of the different treatments. Very helpful.
I do get bloodwork every 3 weeks or so and am automatically scheduled for a phlebotomy right after. If my HCT comes back <42 then the appointment gets canceled. I was able to go 4 months and almost felt normal but then in 3 weeks between labs shot up from 42 to 47 so had to start the TP process again. So far I've had 2 and only down to 44 so hoping tomorrow's will get me below 42 and I'll be able to go another few months.
My MPN specialist (on the list that circulates in this group) is part of a major cancer hospital. He is my primary. I do all my lab work and treatments at the hospital. He is the one pushing Hydroxy. I also have a local Hematologist/Oncologist as well. He is still fine with just the phlebotomies while my numbers stay fairly stable. I see both every 3 months (but have labs ever 3 weeks and treatment as needed). The hospital has a great portal setup that gives me instant access to my dr.'s office whenever the need arises. I'm very fortunate.
I see my cardiologist soon so will once again ask about blood clot risk and get his input regarding my heart and risk of heart attack or stroke. That really is my biggest fear (right after another skin cancer possibility).
It’s a bit unusual for your Hct to go from 42 to 47 in 3 weeks, I wonder if that is real. Do you test at the same time of day at the same lab with the same hydration. Hct can be very different at 9am to 12 or 3pm. When I started venisecting in 2010 I had similar issues with contradictory Hct readings. Sometimes I got tested at the hospital during or after a venisection and sometimes at clinic, I learned taking blood to measure Hct during a venisection was nuts because your testing diluted blood, my Hct was all over the place. I learned to get it done at my GP same time of day, similar hydration, not after flying or drinking alcohol the previous day.
I note you are wary of taking meds, most of us were initially. To drug or not and which drug can be difficult to decide on. Sure they are imperfect, but, MPN’s are serious and potentially dangerous conditions hence can need powerful treatments. I venisected for 12 years but on reflection had my drug Rux been available sooner I would have started much earlier, I feel so much healthier. Some report same on the other drugs. We all hope certain drugs may slow progression, they might for some. After a while many get side effects from venisection, some do fine on them for many years. I wouldn’t be too scared about trying the meds if that’s your decision, remember you can always come off again. Note I am necessarily in favour of meds.
ainslie, I also find it odd that my HCT jumps so quickly seemingly out of the blue. My blood is taken at all different times. Although I am always very well hydrated (daily I drink at least 120 oz of water), I sometimes wonder if I am less hydrated for early morning blood draws due to less water intake overnight and morning coffee. If the labs are super early, I do try to forgo the caffeine but it's not easy
The biggest fear I have with the medications is the skin cancer risk. As I've mentioned, I've had Merkel Cell Carcinoma and was extremely lucky and survived. I got it during a time when they didn't have the drugs they do today and it was often considered a death sentence. When I read articles like the following, I become paralyzed with fear and know I will not be able to get myself to take hydroxy and some of the others.
If you take bloods at different time of day the Hct reading will almost certainly be different. I venisected for about 10 years and had about 200 blood tests, I learned that Hct is higher in the morning and lower as the day goes on, if it has to be a 9am test get up at 7 and drink two tumblers of water, one coffee is not a problem, flying the days before can increase Hct as its dehydrating as is alcohol the days before. I get my bloods done at around 3.30pm at my GP every 3-4 weeks. If you have to do 9am then try to make them all 9am otherwise you may be venisecting at the wrong times hence increasing the risk of symptoms you report. Even noon is better than 9am. The body likes steady eddy so first thing is get the testing right and then most people end up with one venisection every 3 months or so, two or three right after each other is quite likely to cause symptoms. I couldnt even tolerate the 450ml so I had 125ml or so monthly and my Hct was always 43.
Re your biggest fear and meds and skin cancer. Your wise to be careful with that, unless you have other thrombotic issues I wouldnt be pressurised by your doc to start meds until you feel comfortable. Your two years in to PV and your AB is under 20% so from what you have written no rush.
I read the papers you poste d re skin cancers and they look scary. I am not in anyway suggesting Rux but I wonder if those papers show the whole picture. The patients were on Rux and they got skin cancers while on it , but was that the cause , they dont know for sure. Both patients had previous HU use. In the second paper they say Rux might have been the cause, might. I have asked several Rux experts about Rux and skin cancer the first one said its not clear if its the Rux or previous HU. The second one which was at Mount Sinai in Oct 23 said Rux didnt cause skin cancer, he reckoned it was previous sun exposure or previous HU. I think with MF the immune system is not so strong as with PV , that is relevant. Personally I think its a grey area but I suspect previous HU use is a part of it. I had daily UVB for itch for 7 years before starting Rux and I have fair hair so burn easily, I have been on Rux almost 7 years , my skin so far is perfect according to my derm at 6 monthly checks, I have avoided sun exposure since diag at 2010 though. I havnt been on HU.
I think most experts would suggest you try Peg or Bes first IF you want to start meds, I note you mention severe depression, for some but most not, you can always stop the drug if you dont like it, when I stopt Peg mood sorted itself in a week. If you wanted to try Rux then maybe wise to see a few Haem Rux experts and maybe a few expert derms. I think the guys that wrote those papers were mainly derms or oncologists not Haems, hence they dont mention if the patients had PV or MF which is relevant. I would say HU is risky for you.
Your in a good position PV wise , recently diag with low AB so no rush, I know many healthy PV patients pushing 70 and no meds, dont stress and take your time. I hope that helps
All of these people have given great advice. I would recommend if you are not seeing a MPN specialist that is where you really need to start. General oncology gets a handful of us throughout their careers. I would see someone that only does MPNs. They are the best to help guide you. A lot of us have a MPN specialist and general oncologist. Good luck! I think it is just great we now have choices. That is always better than just one path!
Thanks for the response KLCTJC. I am fortunate to have an MPN specialist at a major cancer center (one on the MPN specialist list) who is my primary MPN dr. I feel very fortunate to have the option to see him.
I was diagnosed 15 years ago and still on venesection and aspirin. Blood Test and clinic every 3 months. Keep HCT below 45. If I don't feel well and HCT is less than 45, providing it won't make me anemic they will do a venesection. Usually about 4 a year, although it may be blocks of 2. I'm 68 and no one has mentioned any medication, At the moment I'm keeping stum with regard to medication due to all the potential side effects.
mark382 I suspect we are similar with regard to not wanting to take medication due to the potential side effects. I do understand that venesection is not an option for many people for a variety of reasons and therefore they need medication options.
I am noticing I can go longer between venesections but when it is needed, I usually need to get 2 or 3 in a row to get back down to my target number, then I'm good to go for a few more months. I'm learning to live with it (it's only been a couple years for me).
Ligeba, I have PV, tho sometimes it seems like ET would be a better label, and discovered it just as I turned 65, so big commotion ensued. I had a knee jerk antipathy to chemo and its side effects, so I nixed HU as a treatment. I could not imagine taking it forever, as it appeared would be necessary. My local clinic is not MPN oriented, so I did a few video appointments with a Mayo specialist, and decided on Besremi, as it offered a perhaps mild chance of temporary remission. I hoped I could take it for 5 years, get the magical remission, and make it through my likely last 15 years without suffering from stroke and clot danger. After only a few months, I had to go off Bes due to high liver enzymes and the expense, but went back on a half year later when I found financial help and my liver stabilized with help from supplements, acupuncture or qigong practice, who knows which helped if any? Bes has worked brilliantly to reduce platelets quickly, and my HCT hardly needed inching back into normal. I have plenty of side effects--exhaustion, bloating issues, achy joints, GERD, and worst, insomnia. I am going to present these quality of life issues to my doctor this week, and tho I know they are not necessarily due to the interferon, I hope to reduce my already low dose (started and stayed at 100 mcg) in the near future to see if I could improve my life a bit.
Best of luck deciding. There is an element of luck here for sure.
Ovidess thank you for sharing your story and the pros and cons of your experience with Besremi. Based on the studies I've read so far, it seems this drug does have the capability to lower the allele burden over time (the 5 years your mentioned). Just like any other drug, there's always that potential for quality of life issues (most of which scare the living daylights out of me).
Good luck on your journey. If you wouldn't mind, I would love to hear what your dr. has to say after you visit with him and mention the quality of life issues. Thanks.
IFN, when it is effective on allele burden, (VAF) usually shows its fastest results by year 2. If you've been browsing this forum likely you came across this familiar plot from the trial for Besremi. So if you want best odds of fast VAF reductions IFN is a good option.
On your comment "scare the living daylights out of me" most pts end up with minimal or quite tolerable QoLife issues, with the qualifier, "you can feel you're taking the med." But you're right the rare worst case outcome is dire and distinct for IFN. That is the black box warning. I have suffered this.
If that rare outcome is too much for you, and you still want possibility of VAF reductions, ask your Dr about Jakafi (Rux). It is now known to also be disease modifying and able to reduce VAF. It has a good list of sides too, but none are black box level. Most common bad one for most is modest weight gain. I have experienced some increase hair growth on it, another known side. I have found the experience on it to be entirely boring, and effective, so far. But I still feel IFN is a great option while off limits for me.
I have read the research on Besremi and Ruxolitinib and neither would work for me. Besremi can cause severe depression and other psychiatric issues and Ruxolitinib has the non-melanoma risk (including Merkel Cell Carcinoma which I've already had once). I suppose if my side effects become unbearable or if I get blood clots I will need to re-evaluate. Thanks for all the informative information.
I understand on the concerns and that you're at risk for all. The mental health effects are part of the black box for IFN. I've not found whether these are always reversible. I can say directly the autoimmune hazard is not always so.
There is some discussion I've seen whether prior HU use aggravates the skin cancer hazard with Rux. But it does have at least some such separately.
When it gets approved you may want to check Rusfertide for HCT control with supposedly better iron control than phlbs.
Ligebra, Update from doc and lab appointments yesterday: He says that yes, my problems with tiredness and bone/joint pain are likely due to PV/treatment, but the bloating not so clear. He is not an MPN specialist, but listened carefully to my list of side effects, and since my results have been stable and in a good zone for a few months (remarkable after many decades of high platelets), we agreed that I will lessen my already low Besremi quantity to 50 or 75 mcg/twice a month for two months to see if the results will hold. I kind of doubt I will feel much better from this, but it is worth a try I think to see just how little of this scary drug I can take and still have some positive effects from. I seem to come home with more questions after such a session, so I may mail them into the doctor.
I thank Hunter for the Hyaluronic Acid/MSM suggestion; at least I think that's where I got the idea for that supplement to help me with the terrible crochetiness my joints felt while building benches last week. I'm also taking some powdered tumeric in a compound with mushroom extracts. Tastes awful but I hold my nose and down it whenever I remember it.
Ovidess thanks for taking the time to give an update from your dr.'s appointment. Happy to hear you can give a lower dose a try. I wonder, is anyone able to get completely off the drugs if they reach 0% VAF and their numbers are holding?
What I am noticing while reading this message board is many symptoms I was getting even before being diagnosed are still the same symptoms I continue to get even with treatment (phlebotomy), but are also some of the same symptoms some on the different drugs continue to have. That makes me wonder even more if I am better off sticking with the phlebotomies and low dose aspirin for now while I can still tolerate the side effects I currently have.
I was having terrible leg cramps (and still do but a bit less), and joint pain in various places. It seems to be better since last year, and I don't know if it's the CoQ-10, or the Allopurinol, or the Vitamin D supplements, or a combination along with some PT & OT that has helped along with more movement. I also eat a lot more plant food and do use a lot of herbs, tumeric, nuts, seeds, legumes and other beans. It's so hard to tell what, if anything, is really working and what works for 1 doesn't necessarily work for another.
I'm in the UK so It's Pegasus for us or Hydroxu. I'm 61 so wanted something that might have better longer term outcomes. Having done a lot of reading on here I opted for the chance IFN gives in that respect. 12 weeks in and my HCT is down on minimum dosage weekly but my Neutrophils are low. Haematology have said if that stays the case it's worth doing Biweekly injections. I'm also using the excuse to eat dark chocolate 😁.As Hunter said its a personal thing and don't be afraid to advocate for yourself.
I looked at what would hopefully have the biggest long-term benefit. The prospect of a reduction in allele burden and associated reduced risk of progression was the prime motivation for me b
DougyW I really wish there was some concrete proof that allele burden & reduced risk were absolutely correlated. From the reports I've read there is conflicting & inconclusive data. It's what makes all of this even more confusing. Thanks for the response.
Agree there's no definitive correlation but I've applies engineering principles and the growing weight if evidence would seem tonpount that way. I can't find any long term studies looking at this so it will be unlikely to get a definitive ruling for some time. Each unto their own, there is no generic wrong or right.
I am in the same situation as you are. I am a former paramedic, and know that to weigh all treatments, what is good for one might not be for the next. I was diagnosed about a year ago, but probably had it for five years prior (PV)(ET) my RBC and platelets were high but not crazy high, it was thought it was Testosterone, which increases RBC. I do have the JAK2V617 marker and have been extremely hesitant on BMB because of back issues I am 54 now only symptoms I have other than the bad labs; headaches I have several different types of migraines and I guess I can add PV ones too, puritis mild left hand and arm acts up for a little bit a few times a week, alopepacia thought it was normal aging. Treatment that I went with was phlebotomy and aspirin, doctor immediately had me Hydroxyurea it made me feel terrible, and he is not really listening. I don’t think he’s hearing that the quality of life I have is fine with the phlebotomies and aspirin why upset the apple cart, but the anemia is no joke it takes about a week to recover and does not hit you right away. I would donate blood too before I was diagnosed last year 3 times a year so I guess that saved me some time getting use to that, but it made me wonder why didn’t they test for that? I feel 1-2 weeks of real bad anemia I rather deal with, than the other potential side effects, that might effect other conditions I have. I am soon to get a new dr assigned to my case he stated he was MPN specialist, the one I had, unknown on the new one hopefully he listens and not just checks the boxes. It truly is about quality of life and if you can control it, different ways of treatment for hopefully the same outcome.
FINFAN1970 thanks for the detailed response. I feel the same re: none side effects with phlebotomy vs other meds. Very interesting about testosterone increasing RBC. I have PCOS, which causes my testosterone to be very high. Will have to investigate further.
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