I'm 64 , been diagnosed for 3 years now, PV and Jak2, been on venisection and mini aspirin. Have been feeling well most of time, plenty of excercise, walking and tennis . Last bloods have reading of 780 platelets and doc talked about possible hydro, must admit felt pretty scared. We are waiting and seeing but it sort of feels enevitable.
I'm sure others will have been here, are there any alternatives , I just don't like the sound of chemo drugs and the side effects.
Thanks for any help and advice
Pete
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hello Pete, glad to hear that you have been feeling well for most of the time. It is quite scary when your doctor advises that you start taking Hydroxycarbamide, we know how you feel.
Hydroxycarbamide is the most frequently prescribed medication to treat MPNs worldwide and it is classed as a chemotherapy medication because it slows the growth of blood cells, however most MPN patients actually take quite low doses of this drug. You can read more about it on our website and download our information booklet
I myself have ET and have been taking Hydroxycarbamide for almost 14 years and can honestly say it works really well for me, it keeps my platelets very low and reduced the symptoms I had from the ET, like dizziness, fatigue, head pains.
There are though, people who can't tolerate it very well and do take different medication, you can discuss the alternatives with your consultant or nurse specialist, and you can also read about the other medications on our website
hi Pete, not usually no, but your haematologist may alter the dose, many people do have adjustments made to their dose. You will be monitored regularly to check your blood counts and your haematologist will then decide on the correct dose, and you must only stop taking it on the advice from your haematologist. Maz
Hi, I have been on Hydroxycarbamide for 4years and once the dosage was adjusted, 9 a week with no real problem. I asked if I could come off it when stable and told no, it is for life for me. I have a relaxed acceptance of my situation and think how lucky I am that this is managed especially when I visit the Haematologist and see others on full chemotherapy treatment. Good luck, onwards and upwards.
I've been on Hydroxycarbamide for about 8 months now (I have PV, aged 68, diagnosed Feb 2020) and in spite of the list of side effects I only had slight nausea for the first couple of days shortly after taking it and nothing since. I must admit I was initially concerned and disappointed when told I was being put on a 'chemo drug' (because the venesections only sorted out the Haematocrit level) even though I already knew of it. It seems, like a lot of issues with MPNs, that everyone can be affected differently with no fixed pattern. I was started on 500mg daily which was increased to 500mg/1gm alternate days. My blood counts have all reduced (apart from slightly low Haemoglobin) and are all now well within normal ranges if not heading towards low levels. I queried with the consultant only last week whether dosage might be altered in due course and his view was very much they're guided by the counts so I suppose in time it might be reduced if things are 'overdone'.
I would also suggest looking at presentations by people such as Carolyn Katzin of the US (along with others such as Robyn Scherber and Angela Fleischman) on the role of dietary ingredients and their beneficial, or otherwise, impact on Cytokines which are involved in the MPN cell inflammation process. Carolyn has in my opinion an excellent PowerPoint from April 22, 2017 ('Nutrition for MPN Patients') on this which you should be able to find on the pvreporter.com website. Personally I'm all for doing whatever I can to help the condition rather than just only rely on medication.
As Maz rightly says you should discuss any concerns with your medical team and if you do decide to take any supplements (eg turmeric) this should only be done in consultation with, and approval of, your team. I was very firmly told not to take any iron supplements or vitamin supplements containing iron as this feeds the cell growth process!
You will hear from a number of the members of the forum that they have used hydroxycarbamide/hydroxyurea (HU) successfully and benefited from it. You will hear from others that they did not. I am one of those who is HU-intolerant. I experienced toxicity even at extremely low doses. It is important to understand that we each respond differently to each of the medications used to treat PV and the other MPNs. The good news is that there are options to consider.
HU is a cytostatic medication. It interferes with DNA activity in your hematopoietic stems cells (HSCs) slowing them down. This is its benefit. It also interferes with DNA activity in other cells (e.g. endothelial cells). This is part of why it can cause problems. HU is a toxin and there are specific precautions to take when you are using it. It is usually not recommended for people under the age of 60 due to risks associated with long-term use. It is one of the oldest and most well-known medications used to treat MPNs. It is also the cheapest and the easiest to use. Its use is off-label for PV in the USA since it is not FDA approved for this purpose (but it is in common use).
Pegylated interferons (PEG-IFNs) are an immunotherapy. PEG-IFNs more specifically target the abnormal HSCs. Pegasys has been in use for some time for PV, but is off-label. There is a newer form called Besremi (ropegylated interferon) that is easier to tolerate. There is an emerging consensus that PEG-IFN is a superior form of treatment for PV. PEG-IFN offers the opportunity for hematologic and molecular remission. It is the closet thing there is to a cure for PV. There are potential side effects to be aware of with all of the IFNs that need to be considered. Besremi is approved for PV in Europe and is nearing FDA approval in the USA.
Jakafi/Jakavi (ruxolitinib - RUX) is a JAK-inhibitor. It decreases the activity of the JAK-STAT pathway that the JAK2 mutation upregulates. RUX is particularly effective in dealing with specific symptoms of PV such as pruritis. It is the only medication FDA approved to treat PV in the USA. There is some very promising research supporting the combined use of RUX and PEG-IFN for some PV patients. Like the others, RUX does have potential side effects, including being immunosuppressant. RUX is the only medication FDA approved in the USA for PV.
PTG-300 is a hepcidin mimetic. It is currently in clinical trials in the USA. It works by altering iron metabolism in your body. It allows your iron levels to come up without causing erythropoiesis to increase. It is a very promising alternative to phlebotomy (venesection). Therapeutic phlebotomy induces iron deficiency which is what controls the erythrocytosis. Iron deficiency can have side effects. One of the side effects is to increase thrombopoiesis, so it can make the thrombocytosis worse.
Like you, I am treating my PV with phlebotomy-only. My platelets levels historically cycled between 500 – 700 (for almost 30 years). I have been using phlebotomy for about 2 years now. My platelet levels have risen and now cycle 700 – 900. I am also having other symptoms from the iron deficiency (alopecia, damage to nails, cold intolerance). At one point I was over-phlebotomized and actually became anemic (thrombocytopenia). The docs are not particularly worried about my platelet levels since we have ruled out disease progression as a cause and I am not having any symptoms as a result. I am concerned about my body’s response to the iron deficiency. We are monitoring and I am considering my options.
The two things I am considering are PTG-300 and Besremi. Based on the research, I feel like these are the two most favorable options. Besremi should soon be FDA approved. PTG-300 is in clinical trials and I am in contact to see about participating. I am consulting with my care team and will be making a decision in the near future.
Do be aware that in some healthcare systems the formulary will push docs to try HU first due to cost constraints, particularly for those over age 60. If you wish to try something else, it may be an uphill battle to get it approved. What matters is that you be comfortable with the choice you make. It is your body. It is your decision about what is in your best interests. Educated and assertive patients receive higher quality care. Passive patients do not.
Thanks so much for your considered reply. I also have an enlarged spleen, I am worried that here in the uk only hu is offered as first line treatment. How would one know if your doctoris an mpn specialist. Is it normal to ask for second opinions. I heed your reply that one must push for best treatment ,any advise welcome
I always ask my docs how many MPN cases they have treated. Most hematologists have only treated a few. Many other docs have treated none. Here is a list of docs with MPN expertise mpnforum.com/list-hem./ .
It is absolutely normal to ask for second opinions, particularly when your hematologist is not a MPN Specialist. If you live close to a MPN Clinic/Center and can see a MPN specialist on a regular basis, then that is great. If not, you can do what I do. I have a local hematologist who is a wonderful doc, but not a MPN expert (by his own definition). I consult (at least annually) with a MPN Specialist who is about 2 hours way. The MPN Specialist advises me and my treating hematologist regarding my care. The arrangement works great.
I think it depends on the doc most everywhere about what is offered in terms of your treatment. There are formularies in many health care systems, but is is ALWAYS possible to review all of your options no matter what system you are in. Some docs are really great about doing this. Others will only do it when you force the issue. As a side note - I know which kind of doctor I prefer. Regardless, it is up to you to advocate for yourself. Any discussion about your care will always go better when you start from a more informed position and assert yourself regarding your treatment goals, priorities, risk tolerance and preferences.
Others from the UK can speak better to how choice regarding meds works in the UK. From what I have read, you do have choices. It is up to you to assert yourself in the decision making process.
That is very helpful, thank you, perhaps other UK patients can give me their wisdom on treatments available and what has been offered. I have started trying to educate myself, it's a deep and complex topic, with various opinions given.Please if any other UK patients out there can advise would be very welcome
Thank you so much for sharing this great summary of your research on these specific drugs. It was excellent! I read it and re-read it several times. I have had PV since 2002 and only treated it with phlebotomy for the first 13 years. I started Hydroxyurea in 2015 but had side effects (wounds not healing) so was put on Jakafi in 2018 which worked well at first. In 2020 I developed multiple squamous cell carcinomas and had to have several surgeries. My dosage was consequently cut in half but I still continued to develop additional lesions. I am still dealing with one of the lesions on the top of my scalp which will require surgery and grafting of skin which I am dreading. The Jakafi worked extremely well, but the SCC side effects have been hellish to say the least. I see my hematologist next week and plan to discuss another drug option but I understand that those options may also cause SCC side effects.
Thanks again for your great information. I took lots of notes and will have them with me during my discussion next week. Best to you.
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