Hi, I’m a 69 yr old retired nurse. For the past nine years I have been experiencing all the common symptoms of PV and have had three embolitic events, a DVT, Pulmonary embolism and a cerebral venous thrombosis. I was treated for other things and no specific blood tests were ever done. Things got so bad I recently started the whole process of getting investigated and a neurologist referred me to a haematologist. At last, I’m JAK2 + and high HB and Haemacrit . This week I had my first venesection and have started on hydroxyurea. I am so relieved! Strange , I know, to be relieved at a diagnosis but I feel vindicated as I have been touting my bag of symptoms around, mostly bone pain, headaches and dizziness since I turned 60. It’s probably no fault but my own, during that time we were sailing in the Caribbean where medical investigations were limited and most things were put down to age and lifestyle.
So, now I’m on on this cycle of blood tests, venesection and chemo should I expect things to improve rapidly, moderately or learn to live with it.
The information I have found on this site is invaluable and you all seem patient,kind and knowledgeable. Thank you.
Written by
Janbu
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Hello and welcome to the forum. This is a great place to find support and information.
You should find that some of the PV symptoms will resolve with the course of treatment. Some will resolve more quickly than others. There are several different mechanisms of action that underlie PV symptomatology. The overproduction of RBCs causes the blood to be too viscous, which causes some of the symptoms we experience. The JAK2 mutation is thought to cause blood cells to be "too sticky", altering how the blood cells interact with each other and with the vascular endothelium. Thrombosis and microvascular symtopms can occur as a result. Note that dizziness and headaches are microvascular symptoms. Another thing the JAK2 mutation does is cause the body to overproduce inflammatory cytokines. The systemic inflammation is thought to cause many of the secondary symptoms we experience.
Bone pain is in part, inflammation related. "This pain is believed to result from an excess amount of cell turnover in the bone marrow, inflammation of the periosteum (the layer of connective tissue that surrounds bone), and hardening of the bone (osteosclerosis)." mympnteam.com/resources/pai.... There is more about MPN pain here.
You may find that the microvascular symptoms will resolve fairly quickly between the aspirin and HU. Note that some people find they need low-dose aspirin bid to resolve symptoms. HU is reported to have a secondary effect of helping to make the blood a bit more "slippery" which can also help with the microvascular issues.
Venesections will reduce the erythrocytosis. Most people need a series of venesections to achieve the target HCT<42/43% female, HCT<45% male. The intent of the venesections is to make your body iron deficient without anemia, which controls the erythrocytosis. While some tolerate the iron deficiency, there can be adverse effects. In my case, the adverse effects of iron deficiency were worse than the PV symptoms.
HU is chemotherapy as you know, It is a cytotoxin that interferes with DNA activity in hematopoietic stem cells and other cells in your body. It can be effective at reducing hematopoiesis in all three blood cell lines, erythrocytes, leukocytes, and platelets. The normal starting protocol during dose titration is a CBC every two weeks. Usually a CMP as well to monitor for toxicity. Some people tolerate HU and benefit from it. Others are not able to tolerate it or are refractory to HU. I am one of the latter. FOrtunately, I have responded very well to the interferons, Pegasys then Besremi.
Note that we are all different in how we respond to the treatment options. We each need an individualized plan of are directed by a MPN Specialist. If seeing a regular hematologist, it is advisable to get a second opinion from a MPN Specialist.
Starting on aspirin, HU, and vensections is a common PV protocol. You may find it works well. If not, there are other effective options (e.g., Pegasys). The options have expanded considerably in recent years. Besremi and Jakafi are becoming available in many places. Rusfertide and other options should be available in the near future.
Thank you so much. All useful and relevant stuff. I have been taking warfarin for six years and should remain on this. Otherwise I shall see how how it goes.
The warfarin makes sense given a history of three embolic events. It is worth noting that anticoagulants like warfarin have a different mechanism of action from antiplatelet medications like aspirin. While the benefits are similar, they are not the same. Warfarin may not help with microvascular symptoms in the same was as aspirin. As a nurse, you likely already understand this better than most. You are in a good position to have a more informed discussion about how best to manage your symptoms with a MPN Specialist.
Welcome to the Boat! I just want to add that therapies for PV vary greatly from person to person. It is most important to weigh the risk vs benefit for every therapy. I was 69 also when finally diagnosed. I have not had any major setbacks and know how lucky I am. I started with regular venesection because of how high my hematecrit was then onto Hydroxy Urea. I could not tolerate HU and was switched to Jakafi which has worked well for me - going on 4 years now.
I have learned to be my own best advocate which has served me well. Speak up; be the greasy wheel and discuss everything concerning your care. An MPN specialist is so important too. Do not be afraid to change doctors - not every hematologist has the knowledge to treat you appropriately.
There are many treatments in the pipeline that are giving great results too.
My doctor made it clear that his focus would be that I will die with PV not from PV. PV is manageable. You can have a great life and you know your body better than anyone. Don’t accept changes in your symptoms as growing older; speak up and know you are not alone.
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