Hydrea etc

[mark382]

Does anyone know the criteria for going on Hydrea etc. I've had PV for 13 years and am on daily aspirin and venesections as and when required. Venesections used to be about 3 a year and one at a time. The last few years they've become more two at a time with a 2 week gap between. Probably no more than 4 in total over the year. My haematocrit seems to stay low for a long time after the venesections, but then shoots up fairly quickly. Over the last 9 months, and no I'm not pregnant 😐, I've been tired every day. Ferritin level is 3. Haematologist doesn't know why. I did read somewhere that when you reach 60 you are offered Hydrea or other drugs, because you are more prone to clots, due to age. I'm now 65 and nothing mentioned. Could it be because I'm JAK2 negative. Don't particularly want to go on drugs, but would it allow my Ferritin level to increase and get rid of this constant tiredness.

[paradiastole]

Your ferritin is almost certainly low because of the venesections - one of the secondary aims of venesections (the primary one being to remove red blood cells) is to reduce the amount of free iron in your body to limit the number of new red blood cells that can be produced. I am surprised your haematologist doesn't know why your ferritin level is low. I went on hydroxycarbamide to try to allow my ferritin to increase, though that's a long term project like many things to do with these diseases.

[mark382 in reply to paradiastole]

Thanks for reply. I understand the reasons for venesections etc. Sorry, didn't read very well. Haematologist doesn't know why I'm so tired, not why ferritin is low. I'm looking to see if increase in ferritin will reduce tiredness and what is criteria for going on medication. I'd rather stay off medication, but if it gets rid of this constant tiredness, then it's something I'd looked at very seriously. Once again many thanks for getting back to me.

[hunter5582]

Fatigue (asthenia) is one of the symptoms of iron deficiency, even when you are not anemic. Note that anemia and iron deficiency are not the same thing. Fatigue is also the most common constitutional symptom associated with PV. Fatigue is also one of the common side effects of HU, Peg-IFN and other meds. This can make it difficult to figure out the cause.

Given what you describe, the iron deficiency is a plausible cause for the fatigue. Increased fatigue due to iron deficiency is one of the symptoms that caused me to move from phlebotomy-only to Pegasys. I was also experiencing increased thrombocytosis, issues with concentration, cold-intolerance, koilonychia, and alopecia due to the iron deficiency.

In the absence of being in a high-risk group, the reason to move to cytoreductive medications is for better symptom control. Another reason is to prevent/delay disease progression into MF/AML. HU can help with cytoreduction and symptom control for some people, but not everyone can tolerate its toxicity. PEG-IFN can also provide effective symptom control and may be superior in the long-term, particularly in preventing disease progression. PEG-IFN can also have significant adverse effects for some people. As you have already discovered, phlebotomy can also have both benefits and adverse effects. Our treatment needs change over time as we age and the disease evolves. It may be time for a new approach for you.

The good news is that our treatment options are improving. there is a new drug on the horizon called Rusfertide (PTG-300). This is a hepcidin mimetic that allow iron levels to come up while controlling erythrocytosis. It is currently in clinical trials in the USA. it is a very promising option. Meanwhile there are treatment options available if you choose to pursue that course of action. Since you are one of the rare of the rare (JAK2neg PV) it is really important to consult with a MPN Specialist rather than a regulars hematologist. this will be essential in getting the answers you are looking for. here is a list, just in case you have not seen it. mpnforum.com/list-hem./

All the best.

[IrishHiker]

nature.com/articles/s41408-...

[SolasChoir]

My husband on Hydrea for years but only because phlebotomies stopped working (or indicated he needed something more than blood-letting?) He is jak2 positive, also has high platelets and familial high cholesterol. High risk of clots/ embolism. At the time, Hydrea was the best choice of drug. Was only 44 when diagnosed, now 62 and PRV under control. Not sure if protocol the same now for newly-diagnosed? Never heard of the drug you are taking. Hydrea has risk of leukaemia the longer one takes it. Oncologist best advisor. Don't be afraid to ask plenty of questions - it's your body after all. Take care