I was diagnosed late last year with low JAK2 (<2%) PV, with hct .54 and hgb around 170. This was due to routine test for cholesterol. Since then I've had several venesections and my quality of life due to tiredness and feeling washed out has been awful. I have no other constitutional symptoms. In desperation I asked my team (Dudley) about drug options and the only one on the table is hydroxy. I read the leaflet and was terrified.
I am at my wits end. I have just enough energy to work from home and do household stuff but am starting to suffer agoraphobia as I feel woozy after being out and about after a couple of hours. I am reasonable fit but could stand to lose a couple of stone and wonder if that may help but also wonder if anyone has succeeded in fighting for one of the more modern drugs (peg, jakafi etc) and how they went about it? I just feel so lost.
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Santrix69
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There are other drug options, the main one being Peggasy. Unfortunately there is now a world wide shortage of that drug. If I were in your shoes I'd push for Jafaki given that.
I take hydroxy and have done for 17 years now. So far that has worked best for me. Pegs for me didn't work, hence it comes down to trialling the different options to see what suits your situation best. A good haematologist should be able to guide you through the options. The usual tried and true wellness options have helped me i.e. balanced healthy diet, exercise, a good nights sleep. Keeping a good positive mindset also helps i.e. I focus on what is working in my life. That said, be kind to yourself, find your new normal. For me the first year was probably the most difficult. With many venesections to keep hgb levels etc low. Now the venesections are extremely rare which helps a lot with managing fatigue. Wishing you well.
If you read the labels for any of the meds we take, it's plenty scary. HC does have a long list, as does Jakafi and interferon. Interferon has generally fewer while infrequently more severe and sudden ones.
With your low Jak2, it could be you will get a good result with a low dose of HC, while there is no study to know whether this is true.
Is Besremi interferon (IFN) available to you? This or Jakafi are worth considering. But you indicate these are not available to you. Has your Dr explained why? (they are very expensive) Sometimes you need to prove HC is not working or causing bad side effects before you can go to Jakafi or IFN.
Either one of IFN or Jakafi might reduce your low Jak2 to undetectable. But that doesn't guarantee your MPN symptoms will be gone. Has your Dr discussed getting a full mutation test? This looks for other known mutations that can affect the MPN course and could be esp worth knowing with your low Jak2 and high fatigue symptom.
By tiredness, you may be referring to Fatigue. This is the sort that does not respond much to napping or sleeping and is a distinct disease element. I know it very well.
I'm just learning about the other mutations and will be pushing to get a broader picture when I speak to them in October. The fatigue is something that has been repeatedly episodic lasting several months over the past decade. I think I'm going to post separately about it because I've had some very unusual history in my blood tests where my hct has dropped dramatically when fatigue has gone into remission and I've been able to exercise a lot (cycling 15 off-road miles a day). But my fatigue when it is here feels like a jet lagged heavy clumsiness and when I've been up and active for more than an hour or two I have to sit down as I find I do start to feel light headed but then it's hard to separate out anxiety symptoms which have become worse along with the fatigue.
Your HCT dropped a lot without any treatments or obvious reason? That does give your hematologists lots to figure out. Mine also went down a lot right at the time of my PV Dx, before I started HC. My Dr has no comment. With treatments I got droopy if it went under 40.
Having venes long term, esp if they are toward the large size, can lead to iron troubles. Hunter is quite knowledgeable on this area. Hence cytoreduction can be a valid path to have better iron balance and maybe less fatigue.
I now get severe fatigue but it's not MPN related. From this I know that we should not force activity when the body tells us to take it easy.
I understand anxiety well. There is a sort that comes from nowhere, and another that is response to other troubles. I've had both. From your description yours is #2 and indirectly results from high HCT. If so there should be a real solution upon addressing the MPN or related troubles.
Sorry to hear about the PV-related symptoms and limited treatment options. Unfortunately, fatigue is a common problem when managing PV. It can result directly from the PV or from the venesection-induced iron deficiency. It is important to understand that the iron deficiency can cause fatigue and other issues even when the HCT/HGB remains within the target range. Sometimes timing is the only way to tell what is causing the fatigue.
First-line treatment options for PV include hydroxycarbamide (AKA hydroxyurea - HU) and one of the interferons, Pegasys or Besremi. HU and Pegasys are both used off label for PV but are in common use and well recognized for this purpose. Besremi has an indication for PV but is considerably more expensive so more difficult to access. Jakavi (Jakafi) also has an indication for PV, but approval often requires failure on HU first, unfortunately. There is currently a world-wide shortage of Pegasys. Many healthcare systems will require patients to start on HU since it is so much less expensive (HU=$25/month, JAK=$14,300/month, BES=$16,800/month).
We all respond differently to the treatment options for PV. Some benefit from HU and are able to tolerate it. Others are unable to benefit from HU. This is true for all of our treatment options. The only way to know is to try. Note that you can always try a medication and discontinue if it is not suitable.
While you are thinking about cytoreductive medications, there are things you can do to manage the fatigue. Here are some resources.
When I was diagnosed, I was told I should start HU but also told,I could hold off and only take aspirin & have venisection until I was 60 and then, I would have to start treatment. It was all very new to me and I decided to hold off.
At 60, my haematologist took my case to MDT. The team offered me a choice of HU or Peginterferon. I chose Peginterferon and have been taking this for almost a year now. I asked this forum to help me to choose and also chatted to BCUK nursing team. My levels have recently become in the normal range and, I would chose Peg again. I still have fatigue but hoping as my doses start to lower, I'll feel brighter.
I'm wondering if the Peginterferon world-wide shortage has something to do with your offer 🤔
I know we're all different and our levels often determine the timing to start treatment. I probably should have started sooner, having high platelets when I did start treatment. I've not needed a venesection since starting peginterferon.
Please keep us posted. You can always chat or email Blood Cancer UK nurses for support. They helped me. And the people on here, are just the best support.
MPN voice offer face to face forums too. I think the next one is in London.
Thanks for this. I'm now 56. After several venes this year my hct is down to .49 from .55 and my hGb has come down into range. Everything else is looking ok so far apart from low ferretin of 18. Even with an IV bag I struggle with not passing out at the end of a vene as my BP drops so they are always fun. I probably do need to talk to BCUK for some help and support as I feel I need to.
I've had several people tell me how well they are doing on hydroxyl and no bad stories yet although I'm sure there are some.
If I can tolerate the fatigue I'd like to stick with the venes. However, I also want to go on holiday but since I passed out in hospital and feeling this tired and wobbly I have been suffering anxiety and mild agoraphobia. Its affected my physically and mentally.
Bless you, you really seem to be going through it xx
When I was first diagnosed,I found I had more fatigue if my hct went above .45 and I had to have venesection to keep it below this.
I am only being seen by a haematologist currently, but he's amazing and I'm very happy with my care. He is consulting with Guys, which I find reassuring. I'm not sure of your team but you can ask to be seen by a MPN specialist.
Thank you 🙏. My haematology team seem to be quite good and take an interest in what I have to say in consultations and are also very positive about the amount of research I have done into the condition. They have said they work within the framework of the British Haematological Society and that is why they are offering hydroxy only at this point.
I'm going to try and get some mental health support too as I don't want that to complicate things any more than necessary. I'm autistic which probably doesn't help either
I was diagnosed with PV 8 years ago. The diagnosis was by accident -I didn't have any symptoms. I was 65 and recommended to go onto hydroxycarbomide, with 60 being seen as a cut off between being a high risk or low risk patient and therefore treatment being recommended. I have been on hydroxy. since. I have tolerated it well . Over this time my nails have suffered and I suffer from Reynauds syndrome. I do eat a Mediterranean diet and keep active. I wish you all the best and hope your symptoms can soon be managed.
My hematologist/oncologist helped me get Jakafi. One of the side effects is weight gain, so I have to be very careful with my diet. Other than that it’s working very well for me.
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