Besremi

[Phantasia]

I see a lot of references to Besremi. Is Besremi also an interferon medication?

In Australia we only have access to hydroxyurea or Pegasys.

[hunter5582]

Besremi (ropeginterferon alpha 2b) is an interferon. it is similar to Pegasys (PEGylated interferon alpha 2a). Besremi differs in that it is a monpegyated forumaltion that is loner actiong. Standard dosing is every-other week instead of the weekly dosing used with Pegasys. It is still not clear whether Besremi will have greater efficacy than Pegasys. It is thought that Besremi will prove to be easier to tolerate than Pegasys due to its monopegyated forumaltion an longer-actiting forumlation.

I would not hesitate to start on Pegasys as an option if you are considering a IFN. That is what I did until Beremi was approved in the USA. i imagine Besremi will ultimately be approved in Australia too. Meanwhile, Pegasys remains an appropriate option.

[Phantasia in reply to hunter5582]

Thank you. I haven't handled Hydroxy very well. It was okay initially but after 7 months I developed peripheral neuropathy so have been taken off it. Pegasys will be my only other treatment option.

[hunter5582 in reply to Phantasia]

I was also unable to tolerate HU. I experienced toxicity even at low doses. I did much better on PEG. It was both more effective and much easier to tolerate. It also offers an advantage in actually being potentially disease modifying in a way that HU does not offer. I would note that there are two other second-line options. Jakafi (ruxolitinib - primarily for PV, but ET too) and anagrelide (for thrombocytosis specifically).

Knowing what I know now, I would opt for Pegasys over HU based on my treatment priorities. Hope you access the treatment of your choice ASAP.

[Kelly2 in reply to hunter5582]

Hi Hunter, its been a while! With covid everywhere I did not care about my MPN much, just taking my aspirin. But a month ago my platelets dropped from 1175 to 950 and my whites went up to 18. So I traveled to see my doctor who immediately sent me to do a new BMB. At the hospital they also did a blood examination and they found that the platelets had risen to 1.500.000 in a month's time!! So they insisted that I restart the dreaded hydroxyurea, under the name of Hydreasyn this time, till we get the results of the BMB in about a months time. The doctor took the sample easily which is a good thing but will have to wait for the rest... They told me that in the case of Besremi, they will have to order it abroad and this will take 1,5 months. Isn't Besremi for myelofibrosis? Or also for polycythemia vera? Do we know how many years its efficacy lasts?

[hunter5582 in reply to Kelly2]

Besemi (ropeginterferon) is approved specifically for PV. It is in clinical trials for ET as well. I have not seen anything about using Besremi for MF. It is a reasonable question as Pegasys has been looked at for use with MF.

The one drug that is approved for both MF and PV is Jakafi (ruxolitinib). Ruxolitinib is considered a first-line treatment for MF and a second-line treatment for PV. Besremi is considered a first-line treatment for PV. Hydroxyurea and Pegasys are also considered first-line PV treatment options even though their use for this purpose is off-label in the USA (no FDA approved for this purpose).

Given what you are reporting, cytoreduction would certainly be recommended by most MPN Specialists. Hydroxyurea is faster-acting than Besremi and should result in a rapid reduction in thrombocytosis, erythrocytosis, and leukocytosis. It is actually the long-term efficacy of Besremi that is superior to hydroxyurea. Sustained hematologic response and progression free survival are both superior with Besremi. Besremi also offer the chance for molecular remission, which is not evident with hydroxyurea.

For what it is worth, I would think using hydroxyurea or anagrelide in the short-term would be a reasonable step providing you tolerate these meds without unacceptable adverse effects. This will give you a more rapid response while you wait for Besremi to be available. You could start on Pegasys if that is available, but it will be a slower initial response. You can always make the transition from hydroxyurea in a few months. There is a protocol for how to make the transition from one to the other.

Hope that helps. Please do let us know how you get on.

[hunter5582 in reply to hunter5582]

Part 2 of the answer - how long its efficacy lasts. Besremi is too new in clinical practice to be 100% sure, but the efficacy does seem to be enduring. The long-term efficacy for Besremi for PV is superior for hematologic response. It is also superior for progression-free survival. Some people even achieve molecular remission. There is a clinical trial underway to see how long the remission lasts once the Besremi is discontinued after molecular remission is achieved. It is also known that once someone has reached complete hematologic remission that the dosing frequency can be reduced. Besremi is disease modifying in a way that other treatment options are not.

If the first dose is going to take 1.5 months to access, then I would be sure to ensure that the supply is steady and on-time after that. I would not wait 1.5 months to get started on something in the meantime. Pegasys or hydroxyurea if targeting multiple blood cell counts. Anagrelide is an option of only focussed on platelets. I would be very cautious with venesection to reduce erythrocytosis at this point due to how high your platelet levels are (risk of reactive thrombocytosis).

All the best to you.

[Kelly2 in reply to hunter5582]

You really are a treasure for us all in here! Nobody even doctors take the time to analyse the medicines and the desease. Tomorrow I'll have the first blood test after starting Hydreasyn, one every week, till the results of the BMB. So far so good, still early for side effects if any this time. I will post the result of the blood exam. Thank you very much and wish you well always!❤️

PS. I have asked them to keep the bone marrow sample and do the genetic test for other possible mutations apart from Jak2+ because i have not done that. What makes me more reassured and not left alone, is that now at the hospital that I finally went (it specialises only in haematological deseases), there is a team with whom I can communicate and an email where I will send my results every week.

[hunter5582 in reply to Kelly2]

Not sure where you live or what genetic tests are available there. This is the MPN Myeloid panel that i did. files.labcorp.com/labcorp-d...

I would encourage you to review all of your options with your care team. Sometimes we have to be quite assertive. Assertive patients receive higher quality care. Passive patients do not.

All the best.

[Kelly2 in reply to hunter5582]

I had impressive results after two weeks of Hydro. From 1500000 it dropped to 500000! Tomorrow is the third week draw and I am curious to see what happens. No side effects except for pruritus which is still very unbearable. I will certainly ask for Besremi when the time comes.

[EPguy in reply to Kelly2]

I've posted this before, but you may like to see that HU can be effective, it controlled by PLT well. Your response seems even faster. I am now on Besremi and it is also holding my bloods well.

PLT MAR-22

[hunter5582 in reply to Kelly2]

HU is one of the fastest acting meds for thrombocytosis. One approach is to start on HU for the rapid response, then switch to one of the interferons. Besremi should be available now if you want to move to it. Pegasys certainly is.

All the best.

[Kelly2 in reply to hunter5582]

Hi. Even lower this week. From 500.000 to 430.000. I am taking Hydreasyn. Lets see what happens this week. I am also expecting the results of the BMB next week.🤞🙏 .

[Phantasia in reply to Kelly2]

That really is impressive. Unfortunately for me HU hasn't been helpful. 7 months and my platelets only dropped by about 80000. All I got out of it was peripheral neuropathy. 10 weeks after my haematologist took me off the meds I am still experiencing PN. Seems to be very slowly improving. I won't go near HU again.

[hunter5582 in reply to Phantasia]

We do not all respond well to HU. I certainly did not, It was just too toxic for me to tolerate and it did not control the erythrocytosis. Some people do tolerate HU and see significant reduction in hematopoiesis. Hopefully, we will find better treatments for all of us.

[EPguy in reply to Kelly2]

Good idea to keep your sample. I recently read of an institution that is doing 600 genes in their gene panel. I had only 54. It's changing almost monthly.

I did well on Hydrea, all blood counts were well controlled, if not symptoms. But I've switched to Besremi for its potential long term benefits as noted by Hunter here.

[Phantasia]

My haemo is waiting for my PN to settle, no sign that's happening yet but hopefully by the time I see her again next month things will be looking better. We discussed anagrelide but not available on the PBS here (cheaper price option.) No way I am going back on HU. These last couple of months have been awful. So Pegasys it will have to be. I am triple negative ET so a JAK inhibitor not needed I would assume. Appreciate your advice.

[hunter5582]

I would not assume that a JAK-inhibitor would not help, bour I expect Pegasys is a more viable option since it is the other first-line choice for treating ET. One of the issues we are hearing references to is the noncanonical mutations of JAK2 and the other MPn driver mutations. There are atypical mutation of JAK2 that are not c617f or Exon 12. These mutations are not picked up on the standard tests.

As someone who is triple-negative, I am hoping your care team has already done on of the more comprehensive genetic tests, looking at the other mutations that bear on MPN progression and disease status. It is important to understand your genomic profile in managing your MPN.

It sounds like you are headed towards using Pegasys. I hope you find it as successful as I have.

[Scottishterrier]

Hi phantasiesI have been on hydrea for many years because of having three blood clots to the liver my consultant gave me two choices interferon or hydrea I had just read a horror story in the paper about someone who was on interferon and didn't like the sound of side effects so I didn't hesitate about my though with it being chemo drug will I lose my hair no was the answer but got explained I could not have children but was a no brainer if going to save my life yes I felt sick at start of taking them but with the liver being damaged I was sent to a gastroenterologist consultant who I am still under he prescribed me domperidone which is an anti sickness drug works a treat and also I do still suffer tiredness but hydrea does keep platelets under control also with COVID you do have to shield when it is at its peak I go for second booster on Tuesday but please go for hydrea does help

Stay safe best wishes

Scottish terrier xxx

[Phantasia in reply to Scottishterrier]

Unfortunately my response to Hydroxyurea wasn't good. Started out okay. No real side effects but 7 months in I developed peripheral neuropathy which has been very unpleasant. The PN has led to insomnia and anxiety. The HU wasn't working very well anyway with a limited reduction in platelets even after a dose increase. 3 months into treatment my platelets actually increased to 671,000. The highest they've been in the 9 years since diagnosis. After a further 4 months at an increased dosage they finally dropped down to 567,000. Once the PN developed my haemo took me off HU. Now waiting for the PN to subside before looking at other options.

[Mufftreacs]

Hi Phantasia,

I too live in Australia in NSW. I became unable to take HU after many years of coping quite well.

I had moved from the U K to live near my family who all lived near Sydney.

I was recommended to Dr Cecily Forsyth who is an expert in the MPN field. She is situated in Gosford.

I knew Anagrelide was not on the PBS list and could not afford to finance it myself. As I had little choice at the time, Dr Forsyth organised my treatment with this drug and I paid only a nominal sum.

Sadly, this did not work for me either. She finally put me onto Peg as there were no options left……… no plan ‘B’!

I have not looked back and my bloods are good. I too have ET but am jak2 and have been diagnosed since 2005.

If you are in NSW, I can thoroughly recommend Dr Forsyth. She will certainly sort out your options.

Best wishes and good luck.

[Phantasia in reply to Mufftreacs]

Thank you. I am in Tasmania so options are limited when it comes to haematologists. So far I don't have any issues with my assigned specialist but I don't think MPNs are her area of expertise.

[Phantasia in reply to Mufftreacs]

Did you receive 3 primary plus booster Covid shots? I have had two AZ plus Moderna booster. My haemo never recommended a third primary for me and I assumed it was because I am not considered "severely" immunocompromised. It seems though that under ATAGI recommendations I should have qualified.

[Mufftreacs in reply to Phantasia]

Yes I did have my three primary shots and am due for my booster this month. My GP and consultant both say these are very important. Just had a flu shot too.

[Phantasia in reply to Mufftreacs]

I had my booster 26 Feb. Flu shot tomorrow. See my haemo again 18 May. I will ask her about a second booster.