PV ?MF weight loss and fatigue

[FamilyPV]

Hi all,

My mum has PV and has done for about 30 years... recently her normal meds have stopped working and she has been changed to Anegralide. She’s currently taking 2 a day (and we’re about 6 weeks into the change over).

However, she has extreme fatigue and keeps losing weight. Obviously they’ll keep upping the anegralide and then biopsy if no change.

I guess my questions are;

How long did it take for Anegralide to work for you?

Did anyone have major weight loss and manage to put weight back on?

Is there a chance the fatigue will get better as they adjust the meds?

Thank you so much in advance for your time (and we are also being realistic about the bad news we may be looking at after 30+ years of very successful management).

[Applesnpears]

Hydroxyurea can become ineffective over time. However I think your mum should be asking for a BMB to help you understand if there are underlying changes in the bone marrow.

It will also provide a baseline for the transition to anagrelide.

[FamilyPV in reply to Applesnpears]

Thanks. Will ask about BMB my understanding is they don’t want to give one if they don’t have to as it’s so invasive. Will ask about baseline though.

[Applesnpears in reply to FamilyPV]

A BMB is not very nice but given your mum has so much fatigue and is losing weight it would be worth the discomfort to understand what is going on.

[Selvamok]

God Bless you

[tracey13]

My husband is post PV MF we were so worried when his diagnosis change but to be honest he's much better on ruxolitanib he's been on this a year now and is doing really well on it.

Maybe her medication isn't right for her there's other options so don't worry too much.

Tracey

[FamilyPV in reply to tracey13]

Thanks for your response. My mum doesn’t have the JAK mutation so not sure that ruxolitanib is an option for her.... Hopefully they can get it under control, she just looks so poorly all of a sudden.

[tracey13]

Hope your mam gets sorted out soon.

Maybe she needs a bone marrow biopsy to see what's going on .

Tracey

[lizzziep]

Hi, I was changed from hydroxy to Anagrelide about 3 years ago, I had a reaction to the hydroxy after being on it without trouble for 3 years - ulcers on my feet. When I first went on Anagrelide I had a few palpitations, nothing too scary, but I was warned about it, that gradually settled down. My platelets dropped quite quickly on Anagrelide and are currently in normal levels. I do still occasionally get palpitations, but not often, and it's usually a couple of hours after taking them. I am on 4 x 0.5mg per day. Two in the morning and two in the evening - after meals. I do get tired, but I'm nearly 67 so don't expect to be like a spring chicken, as for weight I have GAINED - unfortunately, and am having trouble getting it off. I think everyone reacts differently to the various drugs we take.

[FamilyPV in reply to lizzziep]

Thank you for taking time to share. It’s good to hear Anegralide worked for you. I’m hoping the more she can take the better she will feel/look and weight gain would make me feel happier!

[Aneliv9]

She has PV or ET? Cause it is confusing not having the Jak2 mutation.

[FamilyPV in reply to Aneliv9]

It’s not confusing. Not all PV patients have the JAK 2 mutation. About 95% do. She does not. She was diagnosed with PV back in the 90’s so it’s not a new diagnosis and it’s definitely PV.

[Aneliv9 in reply to FamilyPV]

Ok clear. Is then a possibility of progression to MF?

[FamilyPV in reply to Aneliv9]

That’s what we are thinking (which is why I put ?MF) in the title. Was wondering whether anyone else had weight loss as a symptom of a change to MF.

[Hidden]

Yes I suddenly lost a lot of weight for no obvious reason - I had to point it out to the consultant at my next visit and a BMB was done (my 4th) confirming I had progressed to MF.

It wasn't a complete surprise as a previous consultant (who I had seen for 15 years) had said there were signs my bloods were changing but wouldn't be drawn any further so I had suspected it. at that point I did ask my GP if anything was being said on letters to him from hospital as I was worried about not being accurate when applying for travel insurance - he took the time to go back through the correspondence and said nothing was in writing so told me just to declare ET.

My fatigue though wasn't any worse - wasn't great to start with. However my background at that stage was 25 years of ET with CalR mutation (Jak2 neg) and treatment of busulphan, HU and then anagrelide. I had been on anagrelide for nearly 7 years at point of progression. My platelet count was 1600 at diagnosis when I was 31.

I had been on HU, perhaps 17 years, and it stopped working so I was changed to anagrelide - it didn't work very well to start with but a low dose of HU was added for a year or so and my platelets came down eventually. I was lucky and although I had some palpitations I felt a lot better on it - had been on such a high dose of HU eventually had felt quite toxic (otherwise HU had been manageable) and I'd had awful migraines which stopped completely when changed drugs - odd as anagrelide has reputation of bad headaches.

Now 7 years on from progression I am on ruxolitinib and EPO injections . Have had one more BMB 5 years ago. I had a very very rare reaction to Pegasys which led to further large weight loss but my weight has stabilised now though never gone back to original figure. If I recall correctly, at time CalR was identified it wasn't thought ruxolitinib would work for any mutation other than Jak2 but by time I needed it it it had been shown it worked for it as well.

I hope your mother gets some helpful advice from her hospital and you can get to the bottom of the weight loss and increased fatigue- I'm afraid we are all though very different.

[FamilyPV in reply to Hidden]

This is so incredibly useful. Thank you so much for sharing your experience.