Hello MPN community
I was diagnosed with PV in 2020. I am JAK2+ and am currently being treated with Phlebotomies as needed and baby aspirin . I am thinking about going on low dose Busulfan 2mg per day or 500mg Hydroxyurea.I would appreciate any input on side effects . Does it help fatigue or add to it. Thanks.
Like you I have pv with the jak2 mutation. Diagnosed 2018, I'm quiet sensitive to the hydroxy, I was on 7 then 5 now just 3 a week my numbers aren't stable enough to stay on the same dose for long, I'm sure they will up the dose again when I have another blood test. So in my experience we react differently to the same dose with similar symptoms. I do get a horrible tired feeling but if I go out and exercise it goes.
The side effects for Busulphan are not great. I was on it for a while when I was younger although I cannot now remember the dose. My understanding is that it is now usually only given to over 80s because of long term effects. You should discuss it with your haematologist but also do some research yourself using reputable sites like MPNvoice. Your discussion with your haematologist will be much more productive if you have formed an opinion for or against either drug. Best wishes, Jan
Hi Ridenez, I would suggest that you have a look at the information on our website about both of these medications, and then have a discussion with your haematologist or nurse specialist to decide on which is the best option for youmpnvoice.org.uk/about-mpns/...
mpnvoice.org.uk/wp-content/...
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.
Once you have read all the information have a discussion with your haematologist about the best medication for you.
Best wishes, Maz
Maz thank you for the links.I appreciate the information.Good luck and best wishes.
Hi Maz. Do you take any pills to protect your stomach? I am worried taking Hydro which is such a strong drug.
hi Kelly, no I don't, but I don't have any pain or discomfort at all in my stomach. If you experience any pain or discomfort then you will need to discuss this with your haematologist or GP so that they can help you. Kind regards.
Maz
I would suggest broadening your consideration of meds to treat PV should the meds be needed. One key concept is that ALL of the medications used to treat MPN have a risk/benefit profile.
Hydroxyurea - You will hear mixed reviews on HU. The short version is that it is a toxic medication, but some people tolerate it well and/or find the benefits are worth the risks. I am one of those who is HU-intolerant, toxicity even at very low doses. Asthenia (fatigue) is one of the side effects of HU. It can be tricky to figure out, however, as PV can also cause fatigue.
Busulfan is a second-tier medication. Usually only used when other meds did not work out. As others noted, the side effect profile is not favorable.
Pegylated Interferon - There is an emerging consensus that PEG-IFN is a superior form of treatment. (Not everyone agrees just yet) It offers the chance for hematologic and molecular remission. The newest form, Besremi (ropegylated interferon), is much easier to tolerate. Besremi is due for FDA approval soon in the USA. Already approved in Europe. That is not to say that the IFNs are without risks/side effects, but the profile is favorable for many of us with PV. Besremi is one of the meds I am considering.
PTG-300 is a hepcidin mimetic that is under clinical trails in the USA. It has Fast Track designation with the FDA. This med works by altering iron metabolism, controlling erythrocytosis while allowing you to have higher iron levels. The research looks favorable, which is why I am considering participating in the clinical trial.
Like you, I have PV and am on a phlebotomy-only protocol. It is controlling the erythrocytosis, but I am having side effects from the chronic iron deficiency. Alopecia, damage to nails, cold intolerance, and increased thrombocytosis. None of our treatment options is without risk of adverse effects. I am considering a switch to my treatment protocol, but have not made a decision yet. I am consulting with my regular hematologist and the MPN-Specialist is see to make a decision in the near future.
Do please let us know what you learn and decide to do.
This is a bit difficult, you need to consult your haematologist. I have had PV for 11 years. I am no expert but was given Busulfan briefly by my rather old-fashioned haematologist in Spain way back when my other treatment was phlebotomies. Bloody hell, it nearly killed me! I couldn't climb the stairs or walk more than a couple of hundred yards. I read afterwards it is only suitable for the very aged near end-of-life, so I really cannot recommend it. I have been on Hydrea for around 8 years and it works up to a point, it is now not controlling the white blood cells and I have terrible itching. I am hoping I might get changed to Jakafi, but need to wait and see. Haemo appt in France next week.
Like Jan/Beetle I was put on it as initial treatment. However this was in 1985 when I was 32. The only other treatment mentioned at the time was radioactive phosphorous and the consultant said it was for over 60s only. I can't remember exact busulphan dose but both my husband and I remember, as we were both so worried, that it was very high - I had to take 50 pills in one sitting - ghastly! My platelets were 1600 and it took another massive dose to get them down. Oddly enough I don't remember any side effects. I had 2 young children so I may have had more fatigue but was running round so much didn't put it down to drugs/ET. I then coasted for a few years with no drugs and as platelets crept up again I was changed to HU which I stayed on for 15 years till it stopped working. Busulphan was never mentioned again.
At a MPN Voice forum here in the UK a few years ago a consultant said it was very very rarely used now and then only as Jan said for older patients. He asked if anyone had had it and when I raised my hand everyone looked round - in a friendly way - to spot the dinosaur.
It has been mentioned on this forum fairly recently so might be worth a search. I remember the poster was being considered for a very low dose
DJK12 thank you for your candid reply. Your reference to Busulfan as “Dinosaur” kind of says it all.I am leaning toward HU.Best wishes to you.
ridenez
Good luck with whatever path you go down. At least our doctors are generally willing nowadays to discuss pros and cons of different treatments plus there are trustworthy sites like this one to consult. Diana
My wife was diagnosed over 6 years ago with ET, she was started on Hydroxyurea but this was a disaster, she now has been on Busulfan, 2mg, for over four years.
She takes it for a period, on alternative days, until it brings her platelets down and then has a period without. She is 80 this year.
She has had no side effects.
It sounds as if it is prescribed in a considerably more gentle way now. The benefit of the huge strides in mpn knowledge since I was first diagnosed. Glad it is working.
Starting Hydroxyurea 
Results of taking Busulfan
Hydroxyurea, PV and itching...
