I am new to this forum. I am 39. I have been diagnosed with ET with JAK2 at the age of 27 and have been on aspirin since.
Unfortunately, my platelet count went up to 1100 this year and my hemacritis increased as well. I did blood mass test in the spring and it was confirmed that I have PV.
My hematologist started me with Pegasys (the dose of 45 per week). I didn't have the best of starts: my nurse made an error and gave me double of the dose the very first time. I ended up in an emergency with severe difficulties breathing... I am back to 45 now
I have had 7 injections now. I am a very active person, but with interferon, I feel like a zombie. I find that I don't have much energy and get completely out of breath just walking up the stairs or bending down to pick something up. Walking through a grocery story is now a huge challenge for me.
I have my shots on Friday afternoons, so I can try to rest on the weekend.
I have a very demanding job and work full time. But now I get out of breath and energy just from being on a conference call for 45 minutes.
I worry as I need to work, take care of my family and I have a mortgage to pay.
Has anybody been in the same situation? Do the symptoms get easier as the time goes by?
My hemotologue told me there is no treatment, the goal is to try to reduce the platellets. He can't say how lng I have to stay on Pegasys. I know it is what it is, but I can't help and hope that I can go back to some sort of "normality".
Thank you very much for any advice.
Mary
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It varies , many do get used to it over time, some not. 7 weeks isn’t long in Peg world. Could you consider a lower dose to see if you get used to it and increase slowly. If needed you could also temporarily venisect to control Hct if a lower dose of Peg takes longer to control Hct but of course venisect won’t lower platelets, you could also consider a second opinion from a MPN expert.
My specialist told me that phlebotomy was not an option for me due to my platelet count, unfortunately. He did give me the option to skip my injections every other week until the end of summer to help me get used to them. I am just not sure if I should tough it out and try and push through (as this is a treatment).
phlebs alone would not be suitable with such high platelets but combining them with lower dose Peg may work to help you to get used to the Peg, that is assuming that the Peg is not controlling your Hct, if it is and you can stay on the Peg , of course forget the phlebs.. Your doc maybe right re skipping a dose to try to get used to it but in my humble opinion your body is more likely to get used to it by lower dose say weekly. . When I started on Rux, unusually, I could not tolerate the standard dose of 10 BID, so I reduced it to 2.5mg BID and increased it by 0.64mg per week, it took a while but now I am on 20+18.75 mg and tolerate it easily with no sides. Rux may work for you but if it was me I would give Peg longer for sure, start low and go slow would be my plan but thats just my opinion and I am not a doc of course.
Reduce frequency of injection your body has to slowly get used to drug mine was dropped to monthly for body to cope as it’s about quality of life not just platelets they will drop slowly. Mine took a year on Peg but MPN was more concerned to help me cope with drug which I didn’t on frequent dose. Now 45 monthly 👍 Julia
I am starting to think think that this will be a long term treatment (I didn't know what to expect in term of length of treatment). And if that is the case, I agree. It should be also about the quality of life. Thank you Julia
With your PLT at 1,100 you are at increased risk for hemorrhage. This would be a particular concern if you have noticed an increase in bleeding/bruising or if your prothrombin times are running a bit long. Your hematologist would likely have run a von Willebrand panel to check on that as well.
With your progression from ET to PV, it is worth noting that the most important thing to control is the erythrocytosis. The goal to maintain HCT < 42/43% for a female, HCT < 45% for a male. Using venesection (therapeutic phlebotomy) is considered a first-line choice for PV in anyone age < 60. With your PLT so high, the hematologist may be reluctant to recommend venesection since it can cause reactive thrombocytosis.
We all react differently to PEG and the other meds used to treat MPNs. My energy levels actually improved when I went on PEG to treat the PV. I was able to let my iron levels get higher, which resulted in a significant improvement in my quality of life. Some people find that their body will adjust to the PEG and adverse effects like fatigue will diminish over time.
Treating a MPN is always a matter of balancing the benefits with the risks/adverse effects. Finding the right balance requires an individualized approach to care. You do have a number of options. As ainslie suggests, you could try reducing the dose of PEG, though that will be less than what is considered a therapeutic dose. You could also consider alternatives to PEG. If you live in the USA, you can also consider Besremi and Jakafi for cytoreduction if your insurance will cover at an affordable rate. At age 39, hydroxyurea would not be a good alternative to treat PV.
It is worth noting that nutritional status can have a huge impact on fatigue. When I was using venesections to treat the PV, the chronic iron deficiency caused more symptoms, including fatigue, than the PV did. I did a complete nutritional analysis and was found to also be deficient in Vit B/folate, Vit D, and Magnesium despite a very healthy diet. You may want to consider checking on your nutritional status just to see if there is something that may help.
While there is no cure for PV, there is certainly effective treatment available. It is a matter of finding the right approach for your individual situation.
Checking into an anti-inflammatory diet is a very good idea. Most use a Mediterranean Diet as it is easy to follow and very tasty. I would not expect a huge change in platelet levels due to diet. I would expect to simply feel better as a healthier diet that supports reduced inflammation, which is a good thing.
I agree! Yes the meds can cause fatigue but you also may be dealing with low iron. Please look into your iron levels which are commonly low at time of diagnosis of PV and cause shortness of breath. I am 46 jak2 positive EF and switching from hydroxyurea to Pegasys, I too found myself so sluggish on Pegasys… I was previously very active daily (two hours a day), now long walks intimidate me. But I am happy to say I might be seeing a light here… after three months of Pegasys 45mcg every two weeks I haven’t been as tired or depressed after the last two shots. My energy is returning, I am hopeful and hope the same for you.
A double dose would be 90 then? That's not a huge dose and breathing difficulties seems like a very serious reaction. And if a 45 minute conference call is leaving you out of breath it may be worth asking your haematologist to reconsider the medication. I was on Pegasys for a year. It controlled my blood counts but the side effects continued.
In your position I think I would request a new bone marrow biopsy, as your symptoms are quite extreme. You shouldn't have to live like this. Good luck.
I also experienced fatigue and esp malaise on IFN (Besremi) I did not have this on the prior HU. IFN worked perfect on my blood counts and reduced the mutation.
Fatigue is listed on the Bes label, near half the patients had this:
"Includes asthenia (abnormal physical weakness or lack of energy), malaise, and fatigue"
Hi there. Are you under the care of an MPN specialist? I had a similar case of ET to PV (although in my fifties) but the thought is the disease did not ‘progress’ but just took time to show its true colours. And when it did and my HCT began to rise ( and my platelets were hovering around the 1100 mark) I began with venesections. It was only when my HCT was not being brought under control (it was never wildly elevated) and more to the point, my platelets were hitting 1700, that I went on to Hydroxy. This brought my numbers tumbling, I felt heaps better and I’ve tolerated it very well.
There are a lot of different treatment options for PV with many more coming on stream. MPN Voice website has a very useful recording of a recent panel discussion on PV chaired by Prof Harrison from Guy’s Hospital. Take a look. It gives an excellent overview of the disease and treatment options. And has you’ll see from the discussion, symptoms are just as an important part of assessing drug ‘success’ as blood count numbers.
The fact that the nurse gave you double the initial dose and venesections were immediately ruled out does raise concerns. I’d be more reassured (though not about the nurse!) if I knew the advice was coming from an expert MPN team. Wishing you well.
There are a number of people on this Forum who live in France, and I believe, have good care. Not sure where you / they live but perhaps they will respond to this thread or maybe you could put out a fresh post with a specific question? All the best.
hi Mary I’m so sorry you are breathless. I too had this at the beginning of diagnoses. It was totally unrelated but didn’t realise. I even used to collapse and end end up having an ambulance a few times. I was eventually referred to a lung clinic. Tests revealed I had hyperventilation syndrome.
( you’r breathing technique is wrong 24/7)
It seems I’ve had it for years but now my body ( ET) couldn’t cope.
Proof that MPNs make you very tired.
I eventually had months of physio which improved me drastically even after one session. I’m not saying that’s what you have but I would urge you to go to your Gp. Any breathing improvement will help enormously.
I’m on Peg and love it. Is it reducing your platelets? It didn’t /doesn’t interfere with my breathing but we are all different.
2 yrs down the line and I’m still reducing my dose.
Oh, that is very interesting. Thank you very much Wyebird.
I have been on Peg for only 2 months now.
I followed my hematologue's advice and decided to try 1 injection every 2 weeks and instead. I skipped last week and all the issues with breathing have gone away. I feel like I have come out out of the fog...
I think I will have to see if this will help and if I can reduce my dose.
Bril glad you’ve seen an improvement. I think it took at least 6 months before I stopped having any sort of side affect at all. ( they finished with each jab) .
Look forward to having an up date. Just remember on your next jab take yourself to bed and be kind to yourself.
I used to take mine about 4 pm as it took about 2 hrs to give me side affects.
If I were in your shoes I'd consider lowering the dose to 30 mcg for 8-12 weeks then raising it back to 45 if possible. Pegasys is something many of us hope to stay on for life because of these known life extending benefits: The Silver MPN Center in New York City treated 470 PV patients over a 30 year period and this is how their outcomes differed depending on their treatment:
20 years after diagnosis:
95% of PV patients who had been treated with interferon were still alive15% of them had progressed to post PV myelofibrosis
57% of PV patients who had been treated with phlebotomy-only were still alive49% of them had progressed to post PV myelofibrosis.
63% of PV patients who had been treated with hydroxyurea were still alive41% of them had progressed to post PV myelofibrosis.
I am sorry to hear of your struggles, and wish to share that also as a relatively young PV patient, diagnosed in late 40s, I hover around the 1000 mark with my PLT but have been as high as 1500s. (usually due to a marked stress such as bereavement or house move) However I have been allowed to stay with aspirin only, with occasional vene (about three times a year now as this has stabilised - at first it was about 8 times a year)as my fear was that that the currently available drugs were more 'harmful' esp for long term use - this is a personal opinion, and I stress that! - that it is not an ideal strategy for all. I am just sharing this to say that you have authority over your own body, and to encourage you to take a balanced view on what you feel will serve you best in the long term. I manage my condition also a lot of holistic health and practices, with nutritional home cooked foods, supplements, energy healing, NAET therapy, dancing, meditation and cacao, mostly alcohol free, and lots of quality sleep at night. The specialists are always amazed by my energy levels and wellbeing that I now enjoy and that my levels are in the main stable or even lowering (as in the case of the PLT) Claire Harrison has also shared that the doctors originally felt it was high PLT that caused thrombotic incidences, but that it's now felt that its the WBC that are the more risky for this aspect - unfortunately for me these remain stubbornly high at around the 15 mark, and so for that reason I am considering attempting a low dose interferon and have an appt with Claire in Nov at Guys Hospital to discuss this further.
In summary, I am sharing that you are master of your own body, so do feel that you are able to make choices within the range of treatments available, and primarily for all of us our lifestyle choices are 'KING', or 'QUEEN' in navigating this health journey. i.e. at least take a complementary approach of balancing the meds with good health practice and above all stay positive and minimise stress, where poss - I wish you all the very best and Keep on swimming with the tides
I think you are right. I am starting to realise that there might be other options and I need to be a part of tge solution. Thank you and take care of yourself
It will only be the doseage they are giving you causing the problem. Peg Interferon is a brilliant drug BUT only if given at a dose your body copes at.
That is disgusting you suffered at wrong dose by nurse . You should be taking secondary advice from an MPN expert . I use Professor Harrison at Guys London for phone consultations due to similar past experiences like yours .
On weekly 45 I was horrendous lifeless exhausted. I usually very fit. Haemotology prescribed weekly . However after my phone consultation with MPN expert I changed to 45 monthly which was a game changer.
The interferon works slowly but does work efficiently. I am now back to fitness plenty of energy. You must speak to an MPN.
I do my own injections in a morning into squeezed flesh lower stomach monthly. Travel sail hike swim etc .
Please ask for advice on doseage as our bodies are all different. You will be fine on correct dose . Julia . 👌
Any difficulties with breathing on pegasys should be investigated as soon as possible. There are some rare lung side effects from it and it is important for these to be ruled out. From personal experience unfortunately you have to be assertive about this. Personally I would get that checked out asap. As already mentioned there are alternative drugs.
I went to see my GP after my admission to tge emergency. She was concerned about a possibility of a clot in my lunght. She listened to me breath and measured my oxigen. She told me that my oxigen level was good, which allowed her to rule out any serious issues.
Are there any other tests that should be done, do you think?
I have taken a break from injections last week and all my breathing difficulties have gone away. Hope they won't be back this week
That's good your breathing problems have improved - it is scary- and also your GP checked you out for clots - that and pneumonia are two of very very rare side effects. When you go back on it hopefully you will be fine. Just keep an eye on your temperature and if you haven't got one get an oximeter so you can check your oxygen levels yourself, as if you have telephone appointments I've noticed it is assumed I have one. Actually a decent digital thermometer and oximeter are good to have anyway now so many of us have telephone appointments for all illnesses. Hope all goes smoothly for you.
I’m sure you’ll have already considered this but if you have a mortgage with certain insurance cover then ET does qualify for that insurance. Just a suggestion.
I was 39 when I was diagnosed with PV. Platelets got as high as 1.7 million. That was not the reason they wanted me on cytoreduction though. After almost 2 years my wbcs started to rise which I think had to do with an underlying issue of MS more than anything. So I started Besremi about 5 months ago and it has been great. As Dr V put it, it is cleaner than pegs and therefore may have less side effects. I say less as they technically have the same risks, but it is longer acting so you can take less. It has been amazing. My counts are way better. I still think it needs some tweaking but I finally see a future. I work full time, have a 4 year old, and a family. I work out 3-4 days a week for 30-50 minutes. I try to eat good, fruit and salads during the day, especially during the week, but my husband is a meat and potatoes guy at night! I still enjoy alcohol but I cut back and limit myself but just had labs and everything checked out ok. But I feel this medication has changed my life. On July 7th here in the US it was approved for both low and high risk PV. So will be harder for insurance to deny it, The phlebotomies wear me out which didn’t used to be the case, but I get really short of breath and exhausted. Could be because I lost more weight trying to stay fit! But I drink lots of water, stay hydrated. I hope they find something that works for you, I know the feeling of being scared and of the unknown. But it has changed my life. I kiss my family everyday and if I want to do something, I do it. Best wishes!
Welcome....I reduced my Platelets from 765 to 242 normal in 2 months on Cayenne Pepper, having been on Blood Thinners for over 2 years, they didnt do it for me.
I'm Alternative Medicine, so thought I would give Cayenne Pepper a try.
Hi There. I am sorry you are having the fatigue issues, as they sound rather debilitating. I do hope it's just the titration on the meds and that it improves with adjusted dosage and/or time. You're getting a lot of sound advice, so I will simply tell you about my journey for comparison. I was diagnosed with PV in my mid 30's (I'm now 66). I could have had EV and not known it, but that is irrelevant now. My hematologist did Phlebotomies until I was over 1 million on the platelet count, and then started me on meds instead. I never was on Peg, but I recall being on Agrylin and Hydroxyurea, both for years, and my platelet counts stayed in a low range. Other blood counts were nout normal, but suitable, given my condition. I tested positive for JAK2, and was put on a drug called Jakafi. I transitioned to Mylo Fibrosis about 4 years ago, and I'm on my 2nd drug to treat it. I am also a chronic skin cancer patient, and some of the drugs used to treat MF are known to increase the skin cancer incident rate. Throughout this journey I have always fought fatigue, but it was never as bad as yours sounds, so I, like many others responding, believe it must be the Peg and your reaction to it. I won't pretend to be a Peg expert, but I will say I have had issues with meds treating my PV/MF, and generally they go away once the dosage "sweet spot" for each is found. I did have a bad reaction to one of the drugs many years ago (pretty severe diffused pain in random locations of my body), and had to transition off of it, but otherwise I have managed ok.
I do hope your medical team determines what causes and then reduce the fatigue to a tolerable level. I agree diet will help, and also believe attitude is key as well. You will get through this! Good luck!
I heard that for some people it takes time for our body to adapt to interferon. Also, if you are in the US, there are Besremi (a newer type of interferon) and other medications available. I wish you will feel better soon.
Well said by Hunter as always. The only thing I would add is that treatment with interferon ultimately increased my energy level from when I was treated with phlebotomy only. It takes a while for interferon to take effect and to get the correct dose. I work 50 hours a week and exercise four days a week with PV at age 57. My platelets were 1.5 when I started the drug and are now at .5. I still have to get the occasional phlebotomy but spacing them out more allowed my iron levels to rebound which was a big help for the fatigue. Drink lots of water and get regular exercise of some kind even when you feel too tired. You got this! Best of luck.
I skipped my injection 2 weeks ago and felt great! That was until I took the new dose last week... Since then I have been completely breathless (worse than before). I went to see my GP yestersay. She did an ECG, said it looked OK, althought I had an increased heart rate and my oxygen is down to 95 (compared to the usual of 98). My blood pressure seems to be going up as well.
She is convinced it is a side effect of interferon, but said not much more she can do, as I need to discuss with my specialist. I skipped my dose this week. I will try calling his secretary to see if he has any further advice (I am not due to see him until October).
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