Up and Down!

[Trueblue8]

I was recently diagnosed with ET JAK 2, I'm on 1000mg Hydroxycarbamide and low dose aspirin. I've posted on here about the fatigue, which I know is a common problem. However, I just wondered if anyone is suffering emotionally? I've always been a very glass half full person but this is really testing me. Outwardly I'm fine as hardly anyone knows about my condition (only close friends and family) but I'm very up and down much of the time, which is quite difficult as I have a busy life with a young family.

[Bobthecob]

Hello there sorry to hear you are struggling. I am ET jak2 on 1000mg Hydroxycarbamide, over the years I too have struggled emotionally. I find stress debilitating in terms of affecting my fatigue and stay away from situations I know will be stressful. In the last few months I have increased my exercise and trying to drink more water, and reduced my working week, this has really helped with fatigue and anxiety. Hope sharing this helps, take care

[Trueblue8 in reply to Bobthecob]

Thanks for your reply, it's very much appreciated

[katiewalsh]

Hi. I think it’s completely natural to feel that way. I know I have a few times since my diagnosis. I, and some others on here, have benefited from being on medications &/or talk therapy. It’s especially hard in the beginning when there are a lot of unknowns & adjusting mentally to the diagnosis. I’d encourage you to find a therapist to talk to and, if need be, get on some medication. Your therapist or doctor can help you with this. Good luck & let us know how you do. Katie.

[Trueblue8 in reply to katiewalsh]

Hi Katie, thanks for your reply with some good advice, it's very much appreciated.

[Wyebird]

Yes 3 1/2 years on I still suffer emotionally . The fatigue stops me from working at my normal pace that in turn makes me upset frustrated and anxious because I’m so slow keeping on top of things.

[Trueblue8 in reply to Wyebird]

Thanks for your reply, it's much appreciated.

[Mazcd Partner MPNVoice]

Hi Trueblue8, I am sorry you are feeling like this, it can be difficult living with a MPN and coping with the side effects and daily life. It might help you to have a buddy to talk to, a buddy is someone who has the same MPN as you and takes the same medication, and can give advice and support from the perspective of someone who really does understand how you feel, if you think this might help please email me at [e-mail], or have a chat with your GP who can give you lots of advice. Kind regards, Maz

[Trueblue8 in reply to Mazcd]

Hi Maz, thanks for your reply. I will give the buddy option some serious thought. This is a fantastic site, thanks for all you do, as someone recently diagnosed it has made such a difference being able to chat to other people with MPNs. The haem dept have been good at Torbay Hospital and I'm back there every 4 weeks at the moment. One thing that is slightly strange, although it doesn't make any difference to me, is that the hospital doesn't class it as a blood cancer but everywhere else, including the WHO and this site does! I find this slightly puzzling and confusing.

[Jlah]

Hi. Sorry to hear this. Yes I too am up and down. I’ve just stopped work as realised any level of stress just made me poorly. Now have the task of facing up to high risk ET- which work helped me avoid. But trying to be proactive. Booked a counselling session this week and have just got a new puppy. Take care. Jx

[Trueblue8 in reply to Jlah]

Hi, thanks for your reply it's much appreciated. All the very best

[wormwood]

I was ok for the first 6 weeks, then I got upset and emotional, this us when my friends stepped in and supported me.... I'm ok now, still have a few worries, but I keep them to myself these days... because it's an ongoing thing, I think people can get tired of hearing my fears!!! I am however, glad to be here in the u.k where we get meds and good support from the heam teams were all under....

[Trueblue8 in reply to wormwood]

Hi, thanks for your reply, it's much appreciated. I haven't told many people, which is sometimes difficult but I have young children and don't want them finding out.

[hunter5582]

I was diagnosed with ET over 30 years ago. The ET progressed to PV about 6 years ago. Still alive and kicking! We all face challenges dealing with our MPNs, but there are ways to deal with the issues we face. Please be sure to find people you can talk to about what you are experiencing. You just found out you have a form of blood cancer that cannot be cured. While it can be managed, there will be challenges along the way. That is a big deal to take in and figure out while managing all the other challenges of life with a young family. The good news is that there is some very promising recent research and significant changes in thinking about treatment for MPNs. In addition to finding a good support group, please find the time to educate yourself about your condition. Knowledge is power and feeling/being empowered to manage your treatment will go a long way to returning to being a "glass half full" person. A few topics to look at include: emerging trends in the treatment protocols for someone with ET your age, JAK2 and Inflammatory Cytokines role in your MPN, JAK2 Mutant Allele Burden and prognosis/treatment, Hydroxycarbamide (aka hydroxyurea) risks and benefits - and alternatives to the use of this medication.

If you stay on HU there are some issues with managing its use that are important to know. HU is a cytostatic medication that interferes with DNA activity - leading to the benefit of reducing hematopoiesis. However, that comes with inherent risks. HU is a teratogen - it causes birth defects. You must not have children while on it. Men must wait 1 year post-discontinuing HU, women 6 months to have children. Women of childbearing years not on HU are advised to wear gloves if even handling the bottle. HU passes into semen and vaginal secretions so the use of a condom is recommended for sexually active couples to prevent exposure to the partner. HU is also a mutagen and a carcinogen (particularly skin cancer). Prevention and monitoring for these effects is very important. HU is immunosuppressant, so you must avoid live vaccines and avoiding infections when you can is a wise course of action. For men, HU can cause hypogonadism. This is just a bit of the information about this medication for you to consider. There is more to know regarding risks and benefits of this or any other chemotherapy. you may never encounter any of the negative side effects of HU. We are all different on how we react to this medication.

Let me end this with the thought that you have a long life ahead of you in which you will learn to manage your variant of ET. You will need to learn how your unique presentation of this MPN presents and what the right treatment is for you. You will face challenges like fatigue along the way, but these challenges can be managed and overcome. Take the time to educate yourself about ET/MPNs and their treatment. Build a support group to lean on. None of us can do this all alone - we are stronger together. Forums like this are a great place to start. Find a true MPN expert doc to consult with. MPNs are rare at 1/3000,000. Many docs, even hematologists, lack the experience to provide the level of care you deserve. I found a great consulting doc on this list of patient recommended MPN expert docs mpnforum.com/list-hem/ . Never forget that you have a fundamental right to be in control of your own treatment. Assertive patients receive higher quality care. Passive patients do not. The more educated and empowered you become, the more likely you will be o return to the "glass half full" status in your life.

All the best to you on this journey.

[Trueblue8 in reply to hunter5582]

Thanks so much for your very comprehensive and informative reply. One thing I find quite strange is that my hospital (Torbay) doesn't class MPNs as a blood cancer, whereas everywhere else seems to. It makes little difference to me other than when I tell people - if you tell them you have cancer they understand but if you say you have an MPN they look at you with a vacant expression! As someone who has had the condition for a long time, your thoughts on this would be appreciated. Kind regards Ian

[hunter5582 in reply to Trueblue8]

It may actually make a difference how your base hospital classifies ET in two ways. The WHO reclassified all MPNs as blood cancers in 2008. If Torbay has not followed suit, they are woefully behind the current understanding of MPNs. I have heard commentary on this forum from UK residents regarding issues related to this, particularly when seeking disability status. (I am from the USA so I have only a vague comprehension of how your health care system works).

The second, and more important way the classification can affect you is that you may be based in a hospital system that lacks competence in treating MPNs. Other UK folks on this forum have reported needing to change their base of services in order to get access to a MPN competent provider. We run into this issue in the USA too. Many hematologists (including entire private hematology practices) do not have the KSAs to treat MPNs due to their rarity. As I noted above, I found a great consulting doc on this list of patient recommended MPN expert docs mpnforum.com/list-hem/ . I would encourage you to do whatever is necessary to find a doc who really has expertise in treating MPNs. The approach that is working for me is that I found a local hematologist who is a great doc - he actually spends time with me, listens to me, and answers my questions. I am only his 6th MPN - 2nd PV patient in 20+ years of practice. I found a true MPN expert doc from the list above. He is the Director of the MPN Clinic at Johns Hopkins Hospital (one of the leading hospitals in the entire USA). My local hematologist does not always agree with the MPN expert doc, but he listens to the consultation and respects my right to choose my own course of treatment. That is why I keep him as my doc.

The reason this is all so important is that MPNs are not all the same. Your variant of ET may not present in the same way as another person with the same diagnosis. Most hematologists have to default to a standard treatment protocol when treating an MPN patient as they do not have the KSAs to do otherwise. These protocols have also changed over time. Some hematologists will not use HU to treat ET in patients below the age of 60 with platelet levels below 999K in the absence of symptoms such as thrombosis. They monitor and use aspirin only. However, recent research has also indicated that for some ET/PV patients, their risk of hemorrhage rather than thrombosis is greater when their platelet levels go up. Add to this, any cooccurring conditions you may have that need to be factored into your treatment plan. MPN assessment and treatment can be quite complex. It is really important to have a MPN expert provider who will look at your individual profile and individualize your treatment plan.

I will close with a note about why this is so important from my own most recent journey in dealing with an MPN. I am Age>60, PV - JAK2+ (25% allele burden). No hx of thrombosis in 30+ years and only mild splenomegaly. Have noted easy bruising and mild tendency to bleed too much. I had to go back on HU for the third time in my life after a bout of reactive thrombosis following a surgery about 1.5 years ago. Turns out on this go-round with HU, I am HU-intolerant. Toxic effects even at low doses. When I consulted with the MPN expert doc, he took me off the HU - benefits not worth the risks/adverse effects. After looking at my individual profile, he also took me off the aspirin - I was more at risk for bleeding than clotting. This is NOT a standard protocol for Age>60 PV. The MPN expert rather prophetically stated "You really do not want to get a brain bleed." Two weeks after seeing this MPN-expert doc, I was diagnosed with a hemorrhagic brain tumor which was surrounded with edema. Going off the aspirin may have been one of the best decisions in my life. It may ultimately have saved my life. Now - one craniotomy on June 17 later - I am doing fine. Much wiser and better informed thanks to my consultation with the MPN expert.

I hope you will be able to find a real expert on the treatment of MPNs. You deserve a treatment plan that is based on an assessment of your unique presentation of ET. We are not all the same in how our MPNs present and in how we respond to treatment options. Please do whatever it takes to ensure that your treatment plan reflects your unique set of needs, as well as your goals and priorities regarding your own treatment.

[Cassie11]

I've just been diagnosed with ET. Bit of a shock isn't it. On aspirin and have appt next month to see if I need to go on chemo tab. My rbcs are not that high. I have periods of anxiety and depression anyway but haven't noticed any worseni g yet. You could talk to your GP about this. Hope you feel better soon.

[Trueblue8 in reply to Cassie11]

Hi Cassie, thanks for your reply it's much appreciated

[Cassie11 in reply to Trueblue8]

You're very welcome.