Recently diagnosed first with ET. put on 500mg hydroxyurea daily with platelets at 617. After bone marrow aspiration and biopsy, diagnosis changed to prefibrotic myelofibrosis. HU had brought platelets down to 270 but I suffered from very bad side effects. Dr didn’t want to change med or dosage. I resisted He finally agreed to 3x a week. (I’m not happy with this doctor, he doesn’t listen to my concerns, doesn’t answer questions, and tells me to go home and not worry so much). Needless to say, I am seeking a new doctor, hopefully one with more experience with my situation. BTW. A month ago switched to 2x a week. On my own. Immediately my side effects almost vanished. Platelets have risen to 470. But won’t go back to HU. There has to be a middle ground somewhere. I’m 83 and feeling fine without the HU. PS I’m generally in very good health!
Prefibrotic myelofibrosis and I hate hydroxyurea... - MPN Voice
Prefibrotic myelofibrosis and I hate hydroxyurea (HU)
Hello Apple238, I feel for you! I'm not a doctor, but you might find my experience helpful. I've had ET for 30 years (diagnosed when I was just 28) and post-ET MF for 15 years. My platelets were over 1 mio. initially, they are now just over 200,000 - they go down as the MF progresses. I have not taken any medication for most of those 30 years - just aspirin for the first 15 years. After the MF had started I was ok for 6 or so years, then the fatigue and bone pain got to a point where I decided I needed help. By that time my spleen was also quite enlarged. I was put on Ruxolitinib in 2014 and took it for 2 years. Now again I'm looking for a new Jak2 inhibitor as Jakavi doesn't work for me anymore. If you're Jak2 positive I think a Jak2 inhibitor is a good idea. Or maybe you decide to take nothing at all. In any case I wish you good luck and all the best! 🌺
hi, I’m not sure where you live and if Pegalated interferon would work for you. (I have ET) but I love it. There is also Ruxolitinib.
It’s worth asking about.
Good luck finding an MPN specialist.
hi Wyebird
I was in hydroxy for seven years and then in Anagrelide for four years but they have just taken me off Anagrelide as my bone morrow has been scarred by it. I’ve been told to stop all medication( except daily aspirin) and go back in six weeks when they will put me on pegalated interferon. I have to say this news has disturbed me. Reading the side effects I’m really worried. Why do you like, how long have you been on it and what has your experience been? Grateful for any feedback.
Tennismad
I love love love love it.
For 6 yrs platelets up meds up haemoglobin down’ til I was on 17 Hu and 9 anagrelide a week haemoglobin 93.
I responded after 1 week 90 units of peg and 17 hu. Platelets halved.
Can’t remember the rest but I posted after each jab.
Just tell yourself you are going to be ill when you take your first jab for about 2 days. Take paracetamol and pretend you have the flu. With each jab the side affects get less.
Now after 7 years of anaemia my heamoglobin is 116. Waiting Gp test results now. I’m now on 90 units every 17 days ( trial) I still put an ice pack on my tummy before injection. I’m a huge baby when it comes to injections but honestly it’s a breeze. It’s tiny.
I’ve been on it about 23 months and still tweaking the dose.
Hope you have the same joy as I do. The only major problem I have is hair loss. I’m considering a hair transplant.
That all sounds very positive! I’m trying to build a positive picture, as I too have had high platelets and low haemoglobin levels so any improvements would be welcome. I feel out of breath a lot and even though I’m very active I still struggle with the anaemia.
What about the hair loss? You skip over that! Are you male or female?
My hair already comes out in fistfuls so I’m a bit concerned about further hair loss!
Thanks for your positive feedback - it’s cheered me up no end!
Tennismad
I’m female and hate the hair loss. Haha I had fine hair before and hu made it a lot finer. I’m trying out ‘REGAINE’
check out you tube the hair loss show on minioxidil
I’m sorry to hear about your hair loss. I do hope you have success with regain. I suppose I must expect some hair loss and other side effects too!😢
Again thanks for taking the time to respond.
Tennismad.
you might not get the hair loss but don’t be afraid of Peg.please Let me know how you get on with it xx
I didn't have hair loss on Pegasys so it's not inevitable. However I did have aching joints, especially my knees, which was genuinely debilitating. I think you just need to try - it's great for some, not for others.
I was diagnosed with ET in 2018. I've been on HU for 5 years, and have bad side effects. Hair loss, ruined my nails to the point it is hard to use my fingers, dry, red, thin, flaky skin, headaches, fatigue. I have had inflamed gums which I finally successfully treated with organic apple cider vinegar, and the gum problem is back, and this time is also affecting my lips. I've about had it with HU. I was supposed to take 500 mg. per day. I personally decided to take it every other day, but this gum and lip problem is so bad, I am going to ask for a reprieve from it, and a venesection/phlebotomy instead. My current Hemo doesn't do those, so have to go back to my prior one. I also syncope after they take my blood, so they have to give me saline at the same time. Dr. suggesting Jakafi. It is outrageously expensive. I would prefer no drugs, as I can't really tolerate many drugs at all. They are all just poison, and enrich the drug companies.
Sorry to hear about the struggles. There are definitely things you can do to make things better. Assertive patients receive higher quality care. Passive patients do not. We must be our own best advocates.
Any doctor that he doesn’t listen to your concerns, doesn’t answer questions, and tells you to go home and not worry so much - should be dismissed from your case. No one deserves to be treated this way. Hopefully the new doc is a MPN Specialist or at least a more appropriate care provider. Suggest you do whatever it takes to consult with a MPN Specialist who will provide proper care. Here are two lists should you need them.
pvreporter.com/mpn-speciali...
While some people can tolerate HU, not everyone can. It is a "highly toxic drug with a low therapeutic index." American Society of Health System Pharmacists. Adverse effects are common and dose dependant. Fortunately, there are other options if HU is not a suitable drug for you. As others have suggested, one of the interferons (Pegasys/Besremi) or a JAK inhibitor like Jakafi (ruxolitinib) may be an appropriate consideration. This would be something to discuss with your new MPN care team. Depending on your insurance, these more expensive options may be viable.
Like you, I am HU-intolerant. I experienced unacceptable adverse effects even at 500mg every other day. I have done much better on the interferons, Pegasys then switched to Besremi. The IFNs have been more effective and much easier for me to tolerate than HU. I have also seen a reduction in my allele burden from 38% to 9% on a relatively low dose. I am higher risk for progression as I also have a non-driver mutation (NF1). Te reduction in allele burden is significant for me.
Wishing you all the best as you move forward.
Hi Hunter- I have finally seen an NF1 specialist and realize that my aches, pains, and issues such as Hypertension and renal stenosis are all from NF1. You mentioned above that having NF1 raises the risk of progression of MPN. I have ET Jak 2 . What do you mean by that? Thanks for your help!
Glad you went to see a NF Specialist. It is essential to be followed as issues can occur throughout the lifespan. Hypertension and renal stenosis are just two of the issues that NF1 can cause. In my case, NF1 caused a very rare adult pilocytic astrocytoma in my occipital lobe that we found are age 63. It is actually very long odds on that even for people with NF1.
The NF1 mutation is a known risk factor for progression into AML for people who have a MPN. It is considered a non-driver mutation for MPNs. One study showed that approximately 16% of people with PV have a NF1 mutation; however, it appears that this is a somatic NF1 mutation, not germline. There is no data on a comparison of the impact of a germline vs somatic NF1 mutation. Moreover, the NF1 gene is a very large gene with several thousand mutation variants. Very little is known about genotype - phenotype presentation. We only have data on 4 of the variants. At this point it is not possible to quantify the degree of risk that having the NF1 mutation presents. It may vary by type of NF1 variant. We simply do not know.
The other factor is is how the NF1 gene alters the action of the RAS/MAPK pathway. The deficiency of neurofibromin causes a variety of issues, including the propensity to form tumors. (neoplasms). The RAS/MAPK pathway is down stream from the JAK/STAT pathway and these two kinase systems interact. Deregulation of either pathway increases risks for other neoplasms. Deregulation of both pathways theoretically could have a compounding effect, but we just do not know. There are too few of us with both the JAK2 and NF1 mutation to generate adequate data.
The ultimate answer to your question is that having both JAK2 and NF1 mutations likely increases our risk for progression and other neoplasms to some degree. We have no idea by how much. It is likely a different answer for each of us. For myself, I do not worry about it. I am aware of it and it is a factor in the decisions I make about how I treat both conditions. I have opted to treat the PV with the interferons, in part to reduce my risk of progression. I have also made lifestyle changes and treatment choices that improve my quality of life and I believe reduce my risk of progression. Controlling inflammation, maintaining cardiovascular health and avoiding toxins are key components of my strategy to manage the MPN/NF1 combination. Ii is my attempt to put the Serenity Prayer in action. Serenity to accept the things I cannot change. Courage to change the things I can. Wisdom to know the difference.
Hi. Hunter is right that you need to have an MPN specialist overseeing your care. There’s no comparison between an expert doc & one who isn’t. Especially one as terrible as yours. I encourage you to make that your top priority. And if there isn’t one near you, try & travel to one & then ask her/him to work with a new & better local hematologist. Good luck. Katie
Hi Apple238,
My husband just turned 86. He was diagnosed with CALR ET several years ago. After about 6 weeks on HU, he had a very bad reaction and stopped it. Continued just on a baby aspirin daily. Considered Peg or Besremi but HE decided to wait and see what happens with just the aspirin. His platelets rose to over 1 million and stablized between 1000 to 1200. HE HAS NO SYMPTOMS. He might take a few more naps than before but that could be related to age. He is on a bunch on supplements that control inflammation and we wonder if that is why he has no symptoms (other than high platelets). He takes Curcumin, NAC, Garlic, Vits C,D andK, Resveratrol, and Tocotrienols. He has a good quality of life and at his age, that is number 1. He has no history of blood clots. He is at a good weight, limits sugar and eats a pretty healthy diet. He gets blood work every other week so if his Platelets jump up, we can reassess things. He is happy with this "WAIT AND SEE" approach.
Like others have said on this site, everyone is different. You have to decide what is best for you. How are you feeling? Do you have any history of blood clots? Other health concerns? These are all things you need to consider to make YOUR decision. And find a new Dr that will listen to you.
Nancy
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