Hi everyone, am newly diagnosed with PV and know at next appt will have discussion around meds, I am 60 no thrombosis events, I am finding it all so overwhelming especially around meds, currently venesection and asprin and antihistamine, is hydroxy always the first med given,I really don't want this I have also previously had BCC , am I right in thinking this does nothing to reduce condition, not only do I not want hydroxy I don't really like the sound of anything else to consider in preference but know will have to, what are peoples experiences can you ask for something other than hydroxy at this stage , I read posts and so many drugs are mentioned and I do not know where in your journey drugs other than hydroxy are introduced, any suggestions, advice would be great on meds to start on,or do I have no choice at this stage, already have aquagenic puritus so do not want that made worse
Medication PV advice please: Hi everyone, am... - MPN Voice
Medication PV advice please
You do have a choice. I was offered hydroxy and said no and asked for pegysus and was given it. Very happy on it. You need to be insistent. Let us know how you get on and good luck
Thankyou for your reply and will let you know how I get on appt is Aug 8th
Interferon could be an option. However, many people do very well on Hydroxycarbamide so it might be worth having a good chat with your haematologist about the most appropriate drug for you. Another thought, if the pruritus is really troubling you, you might be able to have Ruxolitinib - I think it can be prescribed for PV now.
Thanks Otterfield I will look into interferon too as an option but will definitely have a good chat on the day at all options it just so overwhelming at the moment
It is up to you to determine whether to initiate cytoreduction or to continue to treat the PV with aspirin + venesection only. The standard protocol calls for initiation of cytoreduction at age 60. My MPN Specialist told me "65 is the new 35." In short, we do not all age the same. The age-based protocol is guideline, not a mandate. You have to make the risk/benefit calculation based on your individual profile.
Hydroxycarbamide or one of the interferons (Besremi/Pegasys) are the two first-line treatment options for PV when cytoreduction is indicated. It should be up to you to decide which of these options are used if you opt for cytoreduction. Unfortunately, some health care system formularies prioritize hydroxycarbamide due to its much lower coast, particularly for people over the age of 60.
HU = $80.00/month
PEG = $4,400/month
BES = $15,000/month
Given your history of a BCC, you have a very good case to opt for PEG or BES as a first-line treatment option. In addition, the interferons are the only treatment option we have that appear to be disease modifying. The IFNs give the best chance of PV not progressing. If you decide that one of the IFNs is preferable it is your prerogative to opt for this treatment.
All the best to you as you move forward.
Thanks Hunter for your reply and information , wow the costs are significantly different so can see why hydroxy would be the preference to offer but I would really like something that has a chance of stopping progression, I know this is a silly question really but you seem so knowledgeable and obviously interact with lots of people, in your view does it always progress on, I have read including yourself progression from ET to PV over years, is progression inevitable to everyone with the length of time it takes varying person to person, does PV progress over years to MF, also does the AB% of mutation play a major factor in progression, I did not know about this until I saw it mentioned so will be asking about my % at next apptsorry just trying to find some answers to the questions going round and round in my head
The statistical reality is that most people with PV will not progress to MF or AML. Estimates of PV progression to AML range from 3% - 10%. PV usually progresses to Myelofibrosis before it progresses to AML. Risk of progression to MF ranges from 10% - 20%.
There are multiple factors that influence the risk of progression, including non-driver mutations and exposure to certain medications or toxins. It is complex and prediction of individual risk of progression requires a nuanced evaluation of each individual.
Allele burden does tend to play a role in symptom burden and disease progression. People with an allele burden lower than 50% tend to present with a milder MPN. People with ET tend to have the lowest burden, people with MF the highest, and people with PV somewhere in the middle. Emphasis here is on the word tend. There is no straight linear relationship with allele burden.
The JAK2 mutated hemopoietic stem cells have a clonal advantage over wild-type (normal) cells. Over time, allele burden will gradually progress. This can be quite slow or can occur more rapidly when there is a trigger.
The other factor in risk of progression is the presence of non-driver mutations such as TP53, ASXL1, NF1, etc. I do have the NF1 mutation present in addition to the JAK2v617f mutation. Despite this, my MPN had been progressing quite slowly over the course of 30 years. From 2018 - 2019 my allele burden only progressed from 25% to 26%.
It is worth knowing what your allele burden is. It is worth tracking over time as a measure of disease progression or the success of treatment. The hope is to achieve a molecular remission with treatments like Besremi. It remains to be seen what the long-term value of a molecular remission will be. Even in the absence of a molecular remission, preventing progression in the PV is one of the great hopes of the newer treatment options.
It is also worth knowing what the rest of your genetic picture looks like. There are genetic panels specific to MPNs. I believe these will become the standard of care int he future. This is one example files.labcorp.com/labcorp-d...
Hope that helps. All the best.
Thanks Hunter, your very knowledgeable and can give informative information which really does help try to understand this condition, thankyou for your time today and all the information you give on other post too, hope your keeping well
Doing quite well, than you. Just got back from a combined business and pleasure trip to Little Rock, Arkansas. On my way to volunteer at Brainy Camp next week. The Summer camp program run by Children's National Hospital for kids with medical issues is really cool. The kids have a blast and so do the adults who work there. Not long after that I am off for my European adventure.
All the best my friend.
That is a very complete and yet succinct summary- awesome.Thanks
Meds in US so expensive.I am just starting with Pegasys from Kuala Lumpur cost $550 per month only.
Gosh that is so expensive I am in UK so we thankfully have NHS just hoping the costs don't reflect what is offered as treatment first time, thankyou for your reply
Unfortuately, costs do drive what is offered through health system formularies. It depends on the specific healthcare system how luch of a barrier there is when a more expensive medication is desired. Always best to be prepared to advocate for the choice you wish to make.
Thanks for your advice Hunter take care of yourself
Many of these meds are very expensive. Thank goodness for insurance. I only pay $100/month for Besremi. Insurance pays the rest.
Still alot to pay each month but am glad you can get insurance to cover the rest for you ,makes us here in UK realise how lucky we are to have NHS
Hi Hunter
When referring to BCC do you mean Basal Cell Carcinoma ?
If so, has me thinking and wondering as I have had a number of them over the years and worry that cytoreductive therapy would have an impact on this issue.
Any advice is so appreciated.
Again your wealth of knowledge and those on here is fab
Adie
Hi littleluna, yes BCC is short for basal cell circona, that was my worry, I can't give any advice on it I will leave that to Hunter who is very knowledgable just wanted to confirm that was what i mentioned in my original post
Dermal carcinomas are an intrinsic risk of hydroxyurea and Jakafi. If someone has a history of multiple basal cell carcinomas then this is a risk that must be considered. In my view, it would be a contraindication for medications that have this risk when there are other options.
As with all of the decisions we make, we have to weigh the risks and benefits of each choice. There is not one universal right answer for everyone. We have to consider our own unique profile, our treatment goals, risk tolerance, and preferences.
The good news is that some treatment options (e.g. Besremi, Pegasys) do not come with a risk of dermal cancers. These other medications do, of course, come with their own risk/benefit profile. We each have to evaluate what is the best choice based on the facts as they relate to each of us.
Hope that helps answer the question.
Thanks Hunter. That is info I will keep for any future medication interventions.
Exactly- and while I haven't had any yet, my mother had multiple BCC's and died of metastatic melanoma- yet another reason I want to get off Hu and move on to Besremi!
With a family history with BCCs I would make the same decision. Genetics do matter. My daughter is also JAK2 positive. She has ET like I did at her age. I now have PV. Since we also carry the NF1 mutation we are particularly aware of the risk of progression of the risk of other cancers. We do need to consider our genetic profile when deciding on treatment optionsm
The Silver MPN Cancer Treatment Center in New York City has reported the following disease progression statistics based on treating nearly 500 PV patients over a 20-30 year period:
"20 years after diagnosis:
- "85% of PV patients who had been treated with interferon had not progressed to myelofibrosis compared to 59% for the hydroxyurea group and 51% of the phebotomy-only group."
also 20 years after diagnosis:
- "overall survival for patients on interferon was 95% compared to 63% for hydroxyurea and 57% for phlebotomy-only."
Source: crt.org/wp-content/uploads/...
Thank you for those stats that really helps with deciding what meds to consider too just hope it's not a battle with nhs due to the costs involved, hope your keeping well yourself
That's not only a really good population to get statistics from, it's a great analysis of them that I hadn't seen before now-despite my OCD-like searching for same.Thank you!
Reply to PhysAssist
There's a thread discussing this report in some detail. I believe this study is one reason for the revival of INF for MPN. The recent long term Ropeg results help too.
There's also an update from the authors but I recall it's behind a paywall.
healthunlocked.com/mpnvoice...
Hi,
Thanks- I looked at your linked comments [thank you for those], and by following links for the author of the above study found this:
"Genotyping of Transcriptomes links somatic mutations and cell
identity"
ncbi.nlm.nih.gov/pmc/articl...
But as far as I can tell, it's mostly esoteric without direct clinical application thus far.
PA
It seems to be improvements to allele detection. They note this familiar hard-to-ID issue:
<< mutated clone often represents a subset of bone marrow progenitors without distinctive surface markers to distinguish them from non-neoplastic hematopoietic cells>>
The CALR immune therapies we've had threads on rely on identifying CALR cells for attack while Jak2 are cloaked and less amenable. Could be their method could uncloak Jak2 for immune therapy, but this is total guessing and likely not the path they are on:
<<To further demonstrate the ability of circularization GoT to genotype despite significant distance from the transcript end, we targeted JAK2V617F>>
Hi,
I went to the source [The Silver MPN Cancer Treatment Center website] and found this article:
"Optimal therapy for polycythemia vera and essential thrombocythemia: Preferred use of interferon therapy based on phase 2 trials" authored by the folks at the Silver Ctr.
It is a very thorough discussion of the reasons why HU is, but should not be the preferred initial therapy for PV and ET.
Wherein I found this: "It is not unreasonable to suggest that the use of a cytotoxic agent such as HU may predispose to increased clonal evolution, additional cytogenetic abnormalities, and the subsequent development to MDS or acute leukemia..." and also:
"In contrast to HU, rIFNα can be recommended to patients because of its biologic effects on PV stem cells,34–36 megakaryocyte proliferation and morphology, 37 documented regression of marrow cellularity, and reversal of marrow fibrosis.38 In additional, molecular responses, i.e. reduction in JAK2V617F allele burden in PV, are regularly noted39–42 and effects on calreticulin in two patients with ET have been observed.43 These biological properties support the clinical use of rIFNα in PV and ET."
I will be handing a copy of the article [sourced from here:
tandfonline.com/doi/pdf/10.... ]
to my Heme/Onc MD tomorrow.
Just saying...
PA
PS: I can't remember if getting to the article required any jumping through hoops [e.g., pay wall or site membership] and can't figure it out by going in reverse, but if it did- just PM me and I forward a copy to anyone interested.
Here is an even more up to date (2021) article by the same authors: ncbi.nlm.nih.gov/pmc/articl...
Thanks that's great!
...and it also appears to be the article I was trying to recollect discussing interferon-resistant MNP's, their association with non-driver mutations, and combination therapy to overcome the resistance.
Thanks again,
PA
That article includes brief discussion of INF combo therapies. One such combo is with NAC (N-acetylcysteine) implying that NAC could have a similar benefit as adding Rux. But there has been no such trial. I'm taking NAC and in fact just doubled my dose with Dr knowledge.
Another combo is in this post. If Mice were (wo)Men this is the fix to our troubles:healthunlocked.com/mpnvoice...
Unfortunately a Co that was working on an oral ATO is not proceeding to ph 3 trials.
Hi. I was diagnosed 2 years ago in the U.K. at 64 yrs old. Otherwise fit and healthy. I was advised to start medication and given the option of hydroxy or peg. I was very anti hydroxy so reluctantly started peg. I was ok with this for 6 months but then suffered serious side effects ( extreme dizziness and changes in mood). These are known side effects and I had no previous history of depression. Very reluctantly I had to change medication to hydroxy. However, a year down the line and I generally feel very well, some tiredness but nothing that causes me any issues. It also helped the itching! The point I wish to make is please don’t worry, yes these are serious drugs but they can be well tolerated and give you a good quality of life. Keep an open mind and make the decision that feels right for you at this time. Best wishes.
Hi Ellipops, thankyou so much for your reply, I have read hydroxy is tolerated well by lots of people, its the chemo side that scares me plus had BCC before, its hard as its all so new and navigating around it all is overwhelming and guess it may be trialling to see what's good fir me, thankyou again and keep well
You are right that Hydroxycarbamide is well tolerated by most people. Remember that if you start taking it and it doesn't suit you, you can stop.Yes, it is chemotherapy, but just as we often see both ET and PV as cancer with a small c, Hydroxycarbamide can be viewed as chemotherapy with a small c.
It is nothing like the full on chemotherapy that we think of when we hear the word.
Discuss the options with your haematologist (and don't let him/her rush you). Don't be put off anything by retrospective statistics which have no relation to you as an individual.
And remember to ask about Ruxolitinib if the pruritus is a big problem - it can be a life changer!
Thanks Otterfield for that advice and will make sure I fully discuss all options
I don't remember the exact source, but another of my many reasons for not wanting to continue HU despite having achieved CHR in <2 months [along w/ bloodletting] is the reminder that HU is non-selective- it targets and impair the repair and reproduction of healthy cells, just as much as the bad/mutated one.
To Ellipops: Just because you didn't tolerate Peg doesn't mean that you wouldn't tolerate Besremi, and the end results are much more favorable overall. After all, that's why they went on to develop the newer formulation.
Just sayin'
I didn't know that, I have alot to learn about PV , treatment, meds and side effects thankyou
Hi Cityreach, I have been on hydroxide for 10 years and haven't ever had a reaction. I do believe I am quite lucky, have travelled the world, long haul flights etc on it with no problems. Am now 69 nearly seventy still run, swim, gym Hope this helps
Dave
Hi Dave, thankyou for your reply thats really good to hear and am glad you are still getting to enjoy the things you like it's hard because I feel so well and yet I have this chronic condition that needs some serious meds the side effects I have don't relate to a serious condition but its all new to me and will have to get there in the end
I am 66 years and have had PV for 14 years. Like you I am on aspirin and venesections. I'm JAK 2 negative. Other than at my very first appointment with haematologist, 14 years ago, medication has never been mentioned. I've also read at age 60, that the protocol is medication. As my haematologist hasn't mentioned it I'm keeping stum. So I guess it isn't always necessary or my haematologist isn't aware of the protocol. I would like to think she is as she's a Consultant Haematologist.
Hi. I also was diagnosed with PV with Jak negative in 2018 at the age of 52. Have been on venesection every 3 months, aspirin daily and hydroxy 1000 mg per day since. PV is under control as per blood reports and dont have any other issues yet like fatigue etc but only increased depression (which i can still control.)
Just want to note that 1000 mg of hydroxy daily had me feeling depressed. I didn't realize how much until I cut down to 500 mg and my mood went back to normal. I wish I knew sooner it was related to the hydroxy.
Hi can I ask when you cut it down is it because Dr advises it, I have seen a few post post where people may have decreased dosage yo releive a side affect
I moved (from near Yale Hospital to Johns Hopkins) and went to see a different MPN specialist who recommended the change. I thought it was crazy, cutting down to half. My Hematocrit went from 35 to 41, but seems stable now. And it would be worth it to have phlebotomies if I needed them rather than go back to how I was feeling.
Hi, I was diagnosed with PV triple negative 16 years ago aged 51 I have been on Hydroxy for about 4 years and am currently on 500mg daily, Thus far I have tolerated it very well, at my last consultation with my Consultant about 7 weeks ago my HCT was slightly elevated and I had a venesection, my Consultant suggested an increase in my dose but I demured at the time due to the fact that I was seeing an MPN Specialist the following day. Last week my HCT was once again was headed for 45 it was 44.8 so it would seem by the time I see my Consultant again in September I will need another Venesection which will probably mean an increase in my dose.In answer to your own question, from 60 onwards people are more susceptible to stroke and those of us with with an MPN significantly more so, this is why we are often advised to go on meds other than Aspirin. I would certainly recommend that you do go on something and would suggest that you have a chat with your Consultant about this. As to what drug you take will depend on (a) your own preference and or ( b) the availability of the drug in your own Health area.
The best of luck on whatever you decide
Garry
Thanks Gary is the target of HCT regularly just to below 45 or are people given their own target, when having venesection I have been asked what my target is and I have not been given one I just know needs to be 45 or below, maybe all this info is given to me at next appt am very new to it all
Anything over 45 is too high, some Doctors say 50 but most would disagree with this, I had an issue whilst at a clinic when the Doc told I was ok at 46. I insisted that he speak with the Consultant he was very unhappy at this but at my insistence consulted with his boss. I got my venesection!
Thanks Gary I am under Guys and St Thomas and know they say under 45, too
This is one topic on which there is very little disagreement- they did a landmark study a while ago, and the reduction in thrombotic events [clots where they shouldn't be] was dramatically different between the over 45% and under 45% [43% for female patients] groups.ashpublications.org/thehema...
Thanks for the information
Hi, I am PV 72yo. I asked my specialist 2 wks ago about progression and he said his experience was same as averages, about 20% will at sometime progress. I take HU, costs me 5.00 per month, besremi will cost me about 1000.00 per month, so I really don't have a choice. If I had a choice I would try one of the interferons- Pegasys or Besremi. If you can't tolerate one of them you can always go back to HU, as far as I know. Good luck to you on your journey.
Hi Meatloaf, it's shocking the difference in costs, I am lucky enough to be in UK where we have NHS that's not to say you are guaranteed the most expensive and am sure could be budget's to be adhered too and may still only get the cheapest drug, thanks for your reply though, hope your keeping well
I have the same reaction re: relative pricing- but I hope that's not the co-pay I'll have with my insurance.I have my SWMBO calling them to find out- and also what, if any other hoops I have to jump through [besides not tolerating HU- which I'm clearly not].
Re: your previous comments to me- you are of course, welcome!
But perhaps more importantly:
Regarding your comment: "I have alot to learn about PV , treatment, meds and side effects"
As you may have read in the comments between KLCTJC and I, even as medical PA's with decades of previous medical experience, we both feel like we don't know hardly enough about PV and MPN's, and are learning something new every day- a lot of which has come from here.
I say that both to thank EPguy and everyone else who shares information here- including personal experiences w/ their disease and medication, and to ask everyone here to keep posting any [credible] information that you come across, so that we can all keep learning about this thing we're going to be living with [hopefully for a long time- or better yet, not- if the reason is because a cure is developed ].
👍
This site and everyone on it is great and I have found it extremely helpful in the last couple weeks that I joined , the knowledge some people have about MPN is amazing and the readiness to reply to people's questions and worries is so kind too glad I took the plunge to get involved
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