Everytime l complain about hydroxy side effects to the medics lt feels like lm threatened with Busulfan as the only other treatment option available other than Hydroxycarbamide which has many bad side effects for me. At the moment l am so sun sensitive it's impossible to go outside as it feels as though l will spontaneously combust Have l turned into a vampire haha feels like it.
OK lm over 71 with PV and thrombosithemia But my instincts tell me there may be other alternatives for me if anyone in the hematologhy department Leeds gave a dime.
I don’t know if you have seen my posts as I am also under Leeds. I had my first face to face 2 weeks ago. I felt very let down, some others who are under a Dr Teh are happy with their treatment etc.
I could barely understand him, he spoke very quickly as well and I didn’t get to ask any questions nor the other way round.
I am suffering badly with itching and he told me to buy some tablets to see if they word. I had to ask him to write it me down as didn’t understand him and he spoke to me as though I knew what he was talking about!
The only other sentence I got was hearing him say if they didn’t work he would put me on Interferon but wanted to hold off as long as possible…
Who are you seeing?
Btw, I emailed one of the nurses yesterday to tell her my concerns and not even a reply or call…. 🤷♀️
Sorry to hear this Grendall l was just in the process of reply when it dissappear. Oh hum l will try to find it but yes l agree and recognise your experience its so challenging. .. Adiewon
Your Dr's comment "...wanted to hold off as long as possible" is opposite of what is current thinking for INF; start early to get its potential best long term benefits.
Of course there are other options to treat PV. Hydroxycarbamide is used in some systems as a first choice because it is so much cheaper. It is most certainly not the only choice.
The interferons - IFNs are preferred for younger patients as it is safer for people of childbearing years and does not have the inherent risks associated with long-term use of hydroxycarbamide.
Pegasys has been used for decades as the other first-line treatment for PV. Note that the use of Pegasys for PV is common but off-label.
Besremi - the newest version of interferon. Specifically approved for PV. Very good evidence that Besremi is superior to hydroxycarbamide in preventing progression of the MPN and for long-term treatment outcome. Besremi (and presumably Pegasys) is the only treatment option that is disease altering.
Jakavi - used for people with PV who are refractory or intolerant to hydroxycarbamide. Known to be more effective for certain symptoms like pruritis. It is specifically approved for treating PV.
Busulfan is not commonly used due to its significant adverse effects and risk of leukemic progression. It is still an option for some.
I am hydroxy-intolerant. I experienced adverse effects even at very low doses. It was also ineffective to control the erythrocytosis. Ultimately I opted for the interferons. I started on Pegasys then switched to Besremi when it became available. The IFNs have been far more effective and much easier to tolerate. I am in a complete hematologic response (HCT and PLT are at target) with only minimal side effects. My only regret is that I waited as long as I did to start on the IFNs.
Ultimately this is your choice to make. If you are not satisfied with your treatment plan, then change it. If your current provider is not willing to help you change your treatment plan, change the provider. This is your prerogative. The interferons and Jakavi are well recognized treatment options for PV. In the absence of a contraindication for these meds, that choice should be up to you. Suggest getting another opinion from a MPN Specialist regarding your care plan. Perhaps another doc will be more willing to listen to your very legitimate concerns and help create a more desirable treatment plan.
Bulsafan for all.Hi Hunter just finished printing out your terrific response to my questions, alternatives to Bulsafan.
Thank you things are alot clearer already and l will be ready with some questions at my 2 yearly meeting( covid lockdown) with the powers that be in my neck of the woods.
If your Dr is not aware of interferons, (INF) for MPN that is a good indication to look for another Dr if that is possible.
Your Dr may be aware of them, but only from the past; early INF was not well tolerated and it got a bad reputation among some clinicians 20-30 years ago. Also, in the past, patients over 60-65 were less likely to get INF. The newer ones Hunter noted are more effective, better tolerated, and increasingly used in older patients. Further INF for some can prevent progression and even reverse the disease in certain ways.
I was on HU and now taking Besremi. My 1st hematologist knew about INF but did not believe they were more useful than HU. We now know that is not necessarily true.
You can see my reply here to Otterfield, busulfan could be relevant for one who fails all other 1st line options, but your Dr needs to know about modern INF as the proper starting point.
You should be able to access INF at older ages these days, but not if your Dr won't even talk about it.
For what little it's worth, in my opinion, all of the mentions [from "experts"] about reserving treatment with Interferon for younger/low risk patients are B-S, and as such, they really trip my trigger-
...because what I hear when that is proffered is:
Since I am at the [advanced old] age of 63 y/o, and thus only have a couple of [more or less productive] decades left, why not use a drug that has clearly been shown not to prevent the otherwise inevitable eventual disease progression to either MF or Leukemia? Also, why not just have an extra helping of increased risk for secondary [non-melanoma] skin cancer and/or solid tumor cancers, while we're at it?
After all, we have to save the good, disease-modifying, more expensive medications for the younger patient population.
Sarcasm switch- OFF.
After all, if they [interferons] are safer and effective enough for pregnant women [and presumably their unborn offspring], why haven't they been considered first-line for everyone ever since they became both available and approved?
I am on pegasys and am in the uk. I was offered hydroxycarbamide as first choice but said no and asked for peg and was given it. If you work out which treatment you would like to start you could ask for it and see what they say. Unfortunately you have to be assertive with some doctors. Good luck
Are you positive that it was Bulsufan that your doctor mentioned? It would seem a very odd choice if you haven't yet tried Pegasys or another Interferon.
SO RIGHT OTTERFIELDI was told that because of the diagnosis of ET which after a second opinion (phew) was diagnosed as PV, and my history of a clot thrombosithemia and of course my age, if not hydroxy then Bulfasan would be best for me .
How strange that Pegasys hasn't been mentioned. My understanding is that Bulsufan is usually used as a last resort and generally only for very elderly patients. In your position I would question the doctor closely about alternatives like Pegasys. In terms of side effects, going from Hydroxycarbamide to Bulsufan would be like going from the frying pan into the fire.
I agree, as my reply above, I think Dr might be old fashioned in many ways, possibly still stuck on the early INFs which were troublesome hence not considering what we have today.
There are some small studies I posted before showing busulfan in a better light, but I still presume it's not a modern solution, with INF being well preferred. But it could be interesting for one who all other therapies have failed, as you say "last resort".
"JAK2V617F allele burden is reduced by busulfan therapy: a new observation using an old drug"
<<We treated 6 PV patients with busulfan, all of whom were refractory to multiple drugs including (HU), rIFNα, imatinib, dasatinib, and anagrelide.>>
<<The rapid and dramatic reduction in JAK2V617F allele burden along with complete hematologic response, in both homozygous and heterozygous disease states is noteworthy. That such responses did not occur in all patients treated with busulfan may relate to disease heterogeneity involving other mutations in PV8,9 which may similarly contribute to the variability in disease response seen with rIFNα and HU. Our patients were not screened for other known mutations in PV>>
" My understanding is that Bulsufan is usually used as a last resort and generally only for very elderly patients..."
is missing the implied and widely understood:
"because of the associated [and relatively high] rate of secondary malignancies and malignant transformation to Leukemia..." because only the very elderly are likely to die from something else before those risks mount up.
I agree, and as for me- besides nothing except Aspirin and phlebotomy, Busulfan would be my last Rx choice, with HU running just ahead as second to last.
Yes. Unfortunately another use of Bulsufan is as one of the conditioning drugs used pre SCT. I will be receiving a pretty high dose next week.There are certain things I'm trying not to dwell on at the moment!
Hi Otterfield,I'm sorry to hear that, but it is apparently significantly better than undergoing whole-body irradiation, which was the previous conditioning process pre- SCT.
That seems like a strange suggestion, ie Bisulphan, Dr Teh used to be my local haem, he seemed very bright, but didn’t emit much empathy.It is worth asking why he suggested Bisulphan, as others have suggested Pegasys or Rux seem obvious choices , Peg seems to possibly have long term benefits if you can tolerate it, for some it can increase itch hopefully only temporarily, Rux is best for itch. I hope it’s not this but it may be a cost issue. Both are probably more expensive than Bisulphan especially Rux. Bisulphan is getting slightly better reports than before but it is more risky than Peg or Rux for sure. At only 71 it seems a very odd choice. If you can get a second opinion from ideally a MPN expert I would jump at it.
Hi Ainslie thank you for your response it wasn't Dr Teh who recommended Bulsafan . He is a new MPN consultant here in Leeds moved from Scotland l believe. I think it may be an issue of money and old school ideas who knows. My request for a second opinion with Dr Clare Harrison has left rather a bad smell around me and lm not sure my attempts to look at alternatives to Bulsaphan will be seen in a new light. But still l will talk about it with Dr Teh and again may ask for a second opinion from Clare Harrison.Anxious and afraid of coming of Hydroxy this doesn't help only feeds into big PHARMA power.
Well it not my business but if it was me I wouldnt give up the opportunity to get a good plan in hand from Clair Harrison because you are concerned about the "bad smell" it may cause, it shouldnt if your haems are grown up, she is one of the best and your long term health should be the priority.. I had a situation back in 2011 where I was not tolerating venisections very well so I asked my local haem in Edinburgh if I could have smaller venisections say 150ml instead of 450 and have them more often, local haem said it was not "clinically practical", end of.. I consulted Clair Harrison to see if she had any ideas and she agreed to write to my local haem to suggest the smaller venisections, all of a sudden my local haem made them "clinically practical"., they worked well for 10 years She is very well respected throughout the UK and if your haem is forever changing (as it is in Edinburgh), having a a letter from CH in your file may put away any "bad smell" and more importantly keep you on the optimum track treatment wise. just my 2 cents. Good Luck
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Jak2 V617F.
Raised platelets 
Besremi side effects?
