I have ET JAK2. Diagnosed 2017. I have had various symptoms since taking this medication all of which my Haemo have dismissed. However since last October I have developed breathing problems. In the beginning only walking upstairs or an incline but recently all the time. It became so bad last Friday that my GP referred me to A&E because he thouclght I may have a clot on my lung. Xray showed I didn't but did show a shadow which the Consultant said was probably inflammation caused by the toxicity of Hydroxycarbamide. He told me to stop it immediately. He would send a text msg to my Haemo. My platelets are 674. I remarked that wasn't this dangerous bearing in mind I am, 81 and have other health issues. No you will be OK. Discharged no other intervention required. I am now left very worried because I am aware of the possible consequences. I have phoned Haemo but have not heard back from them. Your advice help would be appreciated.
Stopping Hydroxycarbamide : I have ET JAK... - MPN Voice
Stopping Hydroxycarbamide
Please ring again . There are different meds if you have a reaction to hydroxy.
It’s their duty to discuss and offer alternatives.
Does your hospital have cancer nurses ? I have found them to be very helpful when I’m concerned about something . Tell them what consultant said
As you said 674 isn’t a dangerous level but could go up now you have stopped meds .
Do hope you can get the support you need
Carolyn x
Yes it was the Harmo Nurse I phoned. I will try again tomorrow. Thanks for your msg.
Let me k ow how you get on
Please check the discussions and lectures by Dr Spivak and Dr Silver on alternate options of treatment that have been posted on this forum .They are Aldo available on YouTube by searching for the name and “ET “ or “PV” . Also check the many discussions I have had on this forum with “ Hunter” . The HU May have really caused severe toxicity for you . Perhaps you could adopt a wait and watch policy fir a month wrt your platelets and other symptoms..... drop your severe worry and stress first of all / they will only cause you harm . Waiting and watching fir a month while you consider other options and discuss with your doctors. There will be a good way to move forward .
There are other options such as Interferon and Jakafi and am sure if you discuss with your hemo - there could be an alternate route of treatment to take ....... keep us posted too ....... regards
I lost my husband last September due to haemotolgy messing messing him up,I would not trust an haemotology unit no further than I could throw them up but all I am
left with is an unbearable life.
I went to a meeting on the 19th February at Blackburn Royal Hospital in Lancashire where it was a complete waste of time, I got no answers and she even went so far as to excuse my husband of telling lies when I told them that he was given hydroxycarbamide with no information whatsoever. She made that assumption because we were never told together anything about those tablets, and now so called medical negligence will not touch it.
All the chairperson was interested in was where I had got the information which Hunter 522 very kindly gave me and which was very informative.
A Yorkshire hospital very quickly found that he had a kidney infection so for Blackburn to
state they could not find it makes it even worse.
Take care and do make sure that you get proper advice from someone not a dratted
Haemotology clinic.
So sorry to hear of your loss. It is certainly very worrying and difficult to know where to go for advice. As I have said above I am going to ask my GP to refer me to Dr Claire Harrison at Guys Hospital. I hope I am doing the right thing. All the best.
HelloHave you seen Claire Harrison? How have you been since you stopped Hydroxycarbamide?
Hi 130396. Yes I saw Claire Harrison in Match who agreed the Drug JAKAFI. It arrived in May and because my platelets had risen from 680 to 1078 she put me on 10 mg twice a day. This I gather is a high starter dose. Most have 5mg twice a day. The high dose has serious side effects for me in particular dizziness which won't subside and is so bad I am unable to drive and worried about going down my stairs. Won't reduce Meds because of High platelets. Feeling pretty rough and depressed.
Oh dear I’m so sorry to read the new meds is making you feel so unwell.Did Claire say it was the HU making you feel so breathless? Is your breathing better now?
Had the HU done anything to your lungs?
How long were you off HU before your platelets went that high?
I’m like you I think. My being breath started mildly. Then I noticed going upstairs made me quite breathless . Also as you said walking up inclines too - not even steep!!!
Best wishes - Rose
Hi Rose. Yes it appears that HU has caused what is called ground glass in my lungs. My breathing is much better but not back to normal. I find it worse if I am Very tired.
Oh dear you poor lady. How did they find out what had happened to your lungs?? Did you have tests?Will these side effects wear off?
Are you staying under Claire Harrison? She’s lovely isn’t she!
Do you have ET?
Yes I have ET JAK2. I had a breathing test plus CT scan and x-ray. I have to see a Respiratory Consultant every 3 months. It appears that all these drugs will have some effect on me.
Hi Heather
I do hope you start to feel better soon. It must be hard for you.
I’m still trying to get to the bottom of my breathing problem. I also started getting stiffness and pain in the muscles of my upper back since starting HU 7 years ago but my haematologist says nothing to do with HU
Best wishes- Rose
Hi Rose. Before starting JAKAFI I had a very painful neck and spine. If you Go on to the MPN website you Will find a video explaining what is called bone pain which is a symptom of ET. I do hope you get to the bottom of your breathing problem I believe it's also a symptom of ET which our Meds should help.
Every box of pills comes with a full leaflet.I had to sign a form that I accepted the treatment.
For the first prescription the hospital pharmacist spent 5 minutes running through all the do's and don'ts.
I can only access the drug from my hospital.
It is difficult to believethat information was not given when the drug is not normally available in general pharmacies (except to order for local convenience)
And anyway, if you have problems on a new drug you question it earlier , not later.
Yes signed the form and was given the form by Pharmacy at the hospital. No in depth info was given by Haematologist. It was when I queried symptoms with Haematologist which were outlined on the leaflet given by Pharmacy I was told they were nothing to do with my medication or PV. This has been the same with so many patients.
Dyspnea is one of the common side effects of HU. It is indeed a sign of HU toxicity. Some people simply cannot tolerate HU, myself included. I had toxic effects even at very low doses.
Your platelet levels are not particularly high, but the combination of age plus other medical conditions with the thrombocytosis are certainly not something to ignore. What the pulmonology consultant was telling you is likely true - in the short term HU toxicity was a greater threat to your health than the thombocytosis. In the longer-term, you certainly need a change in your ET treatment plan.
The good news is that there are other options. The PEGylated interferons, ruxolitinib, anagrelide just to name a few. Not sure if you are seeing a MPN Specialist or just a regular hematologist. In your situation, you do want to be sure to see a MPN expert doc. Not all hematologists have the KSAs to provide optimal treatment for MPNs. Here is a list.
Meanwhile, you certainly do need immediate follow-up. In this sort of situation, a next-day response from the hematologist to a trip to the hospital would be best practice. I expect you may have to be persistent and assertive to get the response you need.
Please let us know how things turn out.
Many thanks for your reply. My haematologist is not an MPN specialist so I will be asking my GP to refer me to Dr Claire Harrison at Guys. I have tried phoning my Haemo but no response in fact I had a phone appointment with her in April but her secretary sent me a message yesterday cancelling it until June. Could you let me know what medication you are taking? I am now feeling very cautious about taking a different one I asked some time ago to have interferon but she refused saying it was not practical for me. No reason why. Luckily I have a very supportive GP.
Hello Heather
The reason I am persisting with this is because Alan was told nothing about Hydroxycarbamide and in the end in June was asked to go into Burnley General Hospital with out telling him to take someone with him where a doctor then told him he had
developed myeloidfibrosis and only had 12 to 18 months to live. That is what hydroxycarbamide does for you when it is not monitored properly.
I, myself have type 1 sugar diabetes and when Alan was started on those tablets then
we should both have been told about side effects etc.
I wasn't told anything about it either. A leaflet was put in with the medication. It may help keep the platelets down but very toxid with so many side effects which unfortunately haematologists won't accept. Keep safe during these difficult times.
I am currently on a phlebotomy-only protocol for PV despite being age 65 where typically cytoreduction would have been initiated. I have a relatively benign MPN so this approach has been OK; however, it has consequences too. The chronic iron deficiency also has adverse effects. I am considering a switch to one or possible two medications. PTG-300 is used (in trials) for PV, but would not apply to ET. The other option is PEGylated Interferon. This is used for both PV and ET. Here in the States Pegasys is currently available. Besremi will soon be available. I would prefer Besremi (ropegylated interferon) as its lower frequency of injection makes it easier to tolerate.
I have no idea why the hematologist would say IFN is "not practical" for you. HU does have a more rapid reduction in platelet levels, but is more toxic than IFN. Both certainly have the own risks/adverse effects, but practical is not the term that would really apply medically. It does apply financially. HU is far less expensive (about 60X) than IFN. That is why it is preferred in the formulary over IFN for age>60 patients. It is more practical for the doc to prescribe it because it is less work to get it approved. It works that way in the States too. HU is typically preferred in patients over 60 since the recognized risk of long-term HU use (leukemic progression when used more than 10 years) is less likely to apply to us older patients since we are more likely to die by then anyway.
If you can see Dr. Harrison, that would be wonderful. Her protocol for ET treatment includes educating the patient about both HU and IFN and letting the patient decide which is preferable. mpnjournal.org/how-i-treat-... .
Even if you cannot see Dr. Harrison, you can certainly change hematologists. The response you are getting from your current provider is not acceptable. I would fire any doc that behaved in the way you describe. I do believe there is a MPN Center at Guys. Perhaps some of the others from the UK can speak to the other docs there.
guysandstthomas.nhs.uk/our-... .
All the best to you.
Many thanks for your comments Hunter they have been very helpful.
Since I replied earlier I’ve come across more info from you and read your links. They are invaluable, thanks so much Hunter! I am very disappointed with my Haemo, after learning I have a chronic, life threatening disease he was stood up to cue me to leave after 4 minutes.I’m in Sydney, btw.
That is an unacceptable standard of care. Suggest you find a better hematologist. I saw your other posts, so I know you saw this list, but will repost for convenience.mpnforum.com/list-hem./ .
Assertive patients receive higher quality care. Passive patients do not. We have the right to high quality care, but also a responsibility to ensure that is what we receive. Inadequate care can only persist when it is tolerated. There is no reason to accept low quality care - ever.
Hi HunterHow do find out if the hemotologist you're under is a specialist in mpns?Many thanks
You can download the full list at mpnforum.com/list-hem/
Hi HeatherDoes this list include all mpn specialists in the uk?
Yes according to MPN voice. I live in Kent and have recently found out that my nearest MNP Specialist is London.
Most hematologists are not MPN-experts. The simple way to find out is to ask. Ethical doctors are always honest about the scope of their expertise and will answer your questions openly and non-defensively. How many MPN patients have you treated? What is your recent training regarding MPN diagnosis and treatment? Do you participate in MPN research? Many MPN expert docs are based out of MPN Centers at major hospitals/research centers. Most other hematologists do not have the KSAs to provide optimal care.
I asked her that question in the beginning but she didn't know. I have since made enquiries from the hospital and it was confirmed that the hospital Trust I am with doesn't have any patients with MPN are referred to Guys. Not sure why I wasn't.
Thanks for replying Hunter and Heather. I have lymphoma as well as Pv so it's a bit complicated but i will ask the questions in regards mpns and lymphoma .Many thanks
That does make it more complicated. I also have another condition, Neurofibromatosis Type 1 that caused a brain tumor. NF1 mutation + JAK2 mutations also increases my risk for leukemic progression. Another rather complicated issue. Sorry to hear about the lymphoma. Not sure which type it is but apparently some types can be linked to the JAK2 mutation.ncbi.nlm.nih.gov/pmc/articl...
Have not looked at the lymphoma + MPN information much. Perhaps you can better educate us based on what you learn. We really are stronger together.
All the best
Hi, Hunter, I was just diagnosed the other day Jak2+ ET, my haemo said little about the HU and I haven’t started it yet as I’m afraid of its toxicity which I’m just learning about (I am sensitive to stuff in general) so, these other drugs you mention above, can you tell me, are they less toxic and as effective as the HU, please?
That is a rather complex question. Comparing the risks/benefits of hydroxyurea to PEGylated Interferon and Ruxolitinib is a bit of comparing apples to oranges. HU is cytostatic medication that interferes with DNA activity in different cells in your body, including hematopoietic stem cells (HSC). HU is a chemotherapy. PEGylated Interferon is a cytokine that works in a different way, apparently more selectively targeting mutated HSCs. PEG-IRN is an immunotherapy. Ruxolitinb is a JAK-inhibitor. As the name indicates, it inhibits the activity of the JAK-STAT pathway that is upregulated by the JAK2 mutation. This disruption of cytokine and growth factor JAK signaling pathways decreases hematopoiesis and the excess production of inflammatory cytokines.
Each one of these medications has a risk/benefit profile. All of them have benefits and potential side effects. We each respond differently to each of these medications. You will hear from some on this forum that they benefit from HU and do not have significant problems with its toxicity. Others, myself included, cannot tolerate HU due to its toxicity. To address your core question,
In order to make a valid decision about what is the appropriate treatment option for yourself, you have to explore each of your options, which for ET would also include an aspirin-only protocol. The treatment protocols define high risks groups for ET that include factors like prior history of thrombosis and age>60 or 65. The treatment protocols may or may not apply to any one of us individually. That is why it is so important to consult with a MPN Specialist (not just a regular hematologist) to create an individual treatment plan.
Your hematologist should have reviewed each of your treatment options with you. Simply offering the one option without a discussion of its impact and reviewing each of your choices is not sound medical practice. Dr. Harrison has a very good explanation of how she handles the treatment of ET in her "How I Treat ET" article. It is worth reading.
mpnjournal.org/how-i-treat-...
Hope that helps.
A world wide list! Thanks.
That list is a Godsend. It is how I found the MPN Specialist I use.
FYI - I believe you are from down-under. This s a link to an Australia-based MPN organization.
Hello ..I’m so sorry to hear what is happening for you ... I take Hydroxycarbomide..,recently have a lot of pain in my lower back ....the heamo I spoke to last week was really abrupt & just said to take another Hydroxycarbomide as my platelets were 517 .... which I thought was ok ...she said no ...I want them below 400 .... I like you don’t know what to do ... but do feel extremely angry with the treatment so many of us have to put up with ...
if I were you I would try talking to someone from the blood wise organisation... I have heard their advice is really helpful...that is what I am going to do ... it makes me wonder how many of these people actually know what they are talking about ... but who else do we have ?
I have a good friend with mental health difficulties ... she never gets a chance to even discuss the meds she takes .. she is always told to keep taking the pills and someone will call her in 3 months ...(just to take more ) I can see that I am in that place now ... just a number ....take your medication &go away .... I’m sorry if my words are unhelpful... I can only tell you my truth & what I’m hearing constantly from our people with these horrible blood cancers ... reading your post has helped me as I am not going to up my medication ... I will take my chances with a lower dose and this time really make an effort to find someone who actually sees me as a human rather than a number or guinea pig ?.. please look for someone to give you good advice ... don’t be fobbed off ... thinking of you & wishing you a good , caring professional to guide you .... love from Lainie 😘
You have certainly echoed how I feel, the breathlessness is dismissed by haematoligist and since I had a lung lobectomy she blames that, despite the fact the breathlessness has gotten worse since starting my hydroxcycarbamide.
I also feel very annoyed. I can't understand how haematologists can say breathlessness has nothing to do with Hydroxycarbamide when the MacMillan leaflet which I was given clearly states it's one of the side effects. Luckily my GP could see how ill I was and stopped it. I am going to ask him to refer me to Dr Claire Harrison at Guys who I understand is an MPN specialist. I hope you get sorted out.
Have you seen her yet?
Distressing to hear you are having the "brush off" by your hospital staff, def not the way it should be! I remember when first being diagnosed and was horrified at having to start Hydroxy and even Macmillan Nurse all but laughed in my face when I asked if there was something else I could take, I was taking 7 a week at that stage,now 21. Luckily for me I am ok on it. Hope you get a good result soon. x
My Haematologist is a lovely lady and very friendly but like you none of my symptoms have anything to do with Hydroxycarbamide. I am pleased to hear you can tolerate it OK. I know a lot of people can but some of us can't.
Same for me, my Haematologist also tells me that my breathlessness has nothing to do with Hydroxy and the itching has nothing to do with the ET Jac2, she reckons it is the Apixaban bloodthinners causing the itching which is a lot of nonsense since I have itched for much longer than what I have been on the bloodthinners
Hi Linda. I think it is very worrying that so many of us have had our side effects of Hydroxycarbamide ignored. When I was told in December by Haemo that breathing was not as a result of Hydroxicarbimide I told her that it was printed on the letter which came with the medication. She said I shouldn't take any notice of that. I told the hospital pharmacist what she had said to which he muttered I must have misunderstood her!!!
FYI. Perhaps some HU data for your hematologist from professional sources.drugs.com/monograph/hydroxy...
online.epocrates.com/drugs/...
ncbi.nlm.nih.gov/books/NBK5....
Regarding itching, from the literature "Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by an increase in blood platelets and dominated by a predisposition to vascular events. Cutaneous manifestations can complicate its course. itching has been the most common symptom reported; however, the percentage has ranged from 3% to 46%, depending on the survey."
pubmed.ncbi.nlm.nih.gov/264....
Perhaps a second opinion with a MPN Specialist is in order.
I am so hoping that I get an appointment with Dr Claire Harrison because although the breathing has been the most problem recently I have many other side effects all of which we are warned about on the leaflet but Haemo says nothing to do with medication or PV. I must add though although I only stopped the Hydroxycarbamide a week ago my breathing has already improved. Man thanks once again
So sorry to hear this. Would you be able to phone haematology again and if no luck, contact your consultant s secretary at the hospital. I always find the staff very helpful and they pass a message straight to the doctor. Really hope you can get some good advice, please let us know how you are.
No reply from Haematology office or Secretary so have left yet another message.
Hello Heather ,This happened to me after 9 yrs of Hydroxy it turned on me!
I am same age as you,Was hospitalised immediately and put on to Jakavi which I have taken now for over 2 yrs...so far all is well.
I hope you soon get sorted out,maybe you have to keep pestering your Drs.
Sending you very best wishes that you soon are given proper care..
Many thanks for your msg. Do you have any side effects with JAKAFI as it's one of the medications Hunter mentioned. Were you ever offered Interferon. My haemo wouldn't give it to me.
Dear Heather first of all well done for trusting your gut on this one and managing to get this through to your health proffesionals. I'm also waiting for a change of meds from Hydroxy and from my experience its always a challenge. Keep calling your team and letting you GP know your concern at not hearing back. Sometimes at these times of important change for me l listen to short breathing meditations they calm me down l practise them through the day and night they help enormously while things are progressing. Stay strong and don't forget to breath.Adiewon
I hope that you find a suitable new treatment quickly, with sympathetic experts to help you. Best of luck!
Hi Heather, I had much less severe reaction to Hydroxyurea but stopped it as I had very good quality of life before that and couldn't see taking something that made me feel so ill. My platelets remained pretty low with a slow increase (425-525) for 6 weeks until my MPN specialist came through with Interferon. So you might not bounce up that quickly and have time to sort things. Do persist with calling them and in the meantime if you have a standing order get your blood checked at least every 2 weeks.The interferon (Pegasys) has been completely tolerable for me which is great.
Oh and I have ET Jak2 mutated and am 66.
Even after a week my breathing has improved but as you say quality of life is so important and like you I can't see the point in taking something which makes me feel so ill.
I am so glad to hear your breathing has improved stopping was the right thing to do. It is very much about living well. Keep us all posted on next steps, Lauren
Did they not run a Covid test ? That would seem most urgent as breathing problems and lung shadows are typical !
Yes I did and was negative as were two 2 chest xrays and blood tests. As Hunter said Dyspnea is one of the side effects of Hydroxycarbamide.
How have you been since you stopped hydroxy? Have your platelets gone up much?
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