Managing my MPN

[Pappafraser]

Hi all, advice would be appreciated.

Had my ET diagnosis around 2+ years.

Was being treated with Pegasus injections which kept my levels stable at 400.

Had to stop this medication due to bad side effects to lungs 🫁 & breathing etc?

Now on 2000mg 4 tablets a day of Hydroxycarbamide and have been taking for 6 months and my bloods are not dropping at all and are staying at 650?!

My specialist is scratching her head and seems to be very laid back due to her massive work load at my hospital!?

Any suggestions would be greatly appreciated as I feel I’m going nowhere & should I be concerned 🙁

Thanks in advance

Grant

[hunter5582]

That is a puzzler. Hydroxycarbamide is usually an effective agent for cytoreduction. You are on a very high dose. It would appear that you are refractory to hydroxycarbamide. It seems like time to try other options.

Anagrelide would often be the next option for ET when Pegasys and hydroxycarbamide are not viable. Jakafi is sometimes tried as well, though it is off-label. Bomedemstat and Besremi are both in clinical trials for ET. Not sure if that is an option.

There is also the option to just accept a slightly higher level of platelets. The risk of thrombosis is not tied directly to how many platelets you have. Some MPN Specialists consider the delta (degree of change) to be more important. This is something you would want to review in a case-specific fashion with a MPN Specialist to assess what is in your best interests.

It may help to get another opinion from a MPN Specialist. It seems you have a trickier case. You may need additional expert input into your case. Here is a list. mpnforum.com/list-hem./

All the best moving forward.

[Pappafraser in reply to hunter5582]

Thank you so much for your very valuable & interesting reply, I will have a look at all you’ve mentioned.

Thanks again

Grant

[JackLina in reply to hunter5582]

What dose of Pegasys were you on? I am now on 30mcg every fortnight, which is keeping my platelets stable and wthin normal range. I was badly affected when I was first put on Pegasys, 90mcg but after lowering to 45 mcg weekly, I am now able to stay ok at 30mcg fortnightly. I have, all my life, had lung congestion problems but this dose and my Aerobika work well for me.

[hunter5582 in reply to JackLina]

I was on 45mcg/week, which is a standard startling dose for PEG when treating PV. I later switched to Besremi. I have been taking 150mcg/fortnightly. I am going to be bumping up to 175mcg at next dose.

[ainslie]

What were your platelets at prior to meds and why were you put on Pegasys, most experts won’t add cytoreductive drugs for ET unless platelets are over 1 million or other high risk factors or symptoms

[Pappafraser in reply to ainslie]

Sorry I’m confused?

Pegasus was working & maintaining at 400!?

[Wyebird]

Horrid situation to be in. Have you thought of a combination of drugs.

[Pappafraser in reply to Wyebird]

I’m in to them directly soon instead of the std telephone appointment, I will mention it!

Thanks!

Just feel I’m not getting anywhere & my specialist is so busy she doesn’t really explain things properly.

KR

[Arnoldthecat]

Hi I cannot offer any advice but i am in the same position as you, diagnosed with ET a year ago. Started on Hydroxy for 6 months and then Interferon for several more months i am now on a combination of both, platelets have come down a little but still in the 700's. On both occasions platelets came down my quite a lot at first, then stalled then started going back up again. The lowest they got was around 540 for one month.

The new goal is below 600 but i am struggling to get there. My dose of both drugs has been reduced because of the side effects. They are now talking about trying Bomedemstat in the New Year. Hope you find an answer.

[Pappafraser in reply to Arnoldthecat]

Thanks for your info & I wish you well 😍

[Mirror368]

My hematologist has suggested I try Anagrelide. It is used to lower platelets.

I agree with Hunter. You. definitely need to see an MPN specialist.