Advice wanted

[taytokavo]

Hello 1st time on this site but am feeling a bit more positive reading some of the posts. I am JAK positive Myeloproliferative Disorder and Portal Vein Thrombosis. I was diagnosed with Portal Vein Thrombosis in 2005 and MD in 2008. I was initially put on Interferon but could not tolerate the fevers especially with a 1 year old. Haven't been on any medication for MD since, platelets are 200. I have now got a rash lower part of my legs which burns and sometimes itches and feel I am getting it on my hands also. I am seeing a new consultant on Monday and am wondering if there are any specific medications/questions that I should about. Am worried for Monday but want to be prepared. Thanks

[hunter5582]

Sorry to hear about the struggles with MPN Symptoms. It is rather hard to say what medications you should be considering without knowing more specifically which MPN you have and what your current status is. The good news is that there have been significant advances in treatment options since you were diagnosed in 2008. One of the changes is that they are no longer called Myeloproliferative Disorders as they were reclassified as Myeloproliferative Neoplasms.

I hope you have scheduled to see a MPN Specialist rather than a regular hematologist. Most hematologists do not have the KSAs to provide optimal care for MPNs. If you are uncertain, it is very important to ask about the provider's specific level of expertise in dealing with MPNs. Ask questions like "How many MPN patients have you treated?" "What recent training or conferences have you attended on MPNs?". If this provider is not a MPN Specialist, then make clear your intent to consult directly with one. Here is a list mpnforum.com/list-hem./ .

Note - I have two hematologists. I see a MPN Specialist to consult about my care plan. I have a local hematologist who is a wonderful doc, but not a MPN Specialist. He provides the ongoing care. The MPN Specialist advises us both. This works great.

Your next step is to get a more definitive diagnosis and find out what the status of your MPN actually is. Ask how this will be done. This will lead to a list of treatment options. There will be more than one option. Insist that the provider(s) review ALL of your options and what the risks/benefits are for each option. Ask which of the options is the most likely to provide symptom relief. Be aware that some of the medications used to treat MPNs are much more expensive than others. healthcare systems will push providers to try the cheapest med first, which is not always in the patient's best interests. It is up to you to advocate for yourself for the optimal care plan for yourself.

Case in point is ruxolitinib (Jakavi/Jakafi). RUX is well known for being the most effecting medication for pruritis (rash) that people with MPNs experience. RUX is approved for both Myelofibrosis and Polycythemia Vera. It is also one of the most expensive at about $14,000/month. Some formularies require you to fail on cheaper drugs that are known to be less effective first. This is just a case-in-point as I would have no idea what medication would be indicated at this point. That is something to review in detail with a MPN Specialist. The main point is that you need to be prepared to advocate for yourself. Assertive patients receive higher quality care. Passive patients for not.

Educated patients also can do a better job advocating for themselves. here are a couple of resources than may help.

legeforeningen.no/contentas...

mpnconnect.com/pdf/who-diag...

cancer.gov/types/myeloproli...

These videos are also very informative.

mpninfo.org/conferences/202... .

I hope this helps. Please do let us know how you get on.

[taytokavo in reply to hunter5582]

Thank you so much for your informative reply and all the links. It seems I really have a lot of reading up to do and to also face up to what I have and to find the best course of action for myself. I only have ever seen the haematologist about my JAK II and only found out last week that I had MPN. Where I live in the UK there are no MPN specialists so I will have to see about travelling somewhere to see one. Once again thanks for all the advice, really appreciate it.

[Ebot]

Hi there. What kind of MPN were you diagnosed with? You say your platelets are 200? Is this correct? The itchy rash may be something completely unrelated. You can certainly get itching with MPNs but usually not as a visible ‘rash’. Do post again. Wishing you well.

[taytokavo in reply to Ebot]

Hi Ebot, I will know more on Monday and hopefully know which MPN I have. The replies here have been brillant and I have learned a lot about what questions to ask and about different medications. Thanks for your reply.

[Mazcd Partner MPNVoice]

hello Taytokavo and welcome to our forum. This information on our website might help youmpnvoice.org.uk/living-with...

I would also have a look at the different medications available, so that when you see your consultant on Mon you can discuss the different options available to you and decide on which is most suitable, that is if your consultant decides to re-start you on medication.

mpnvoice.org.uk/about-mpns/...

I hope all goes well for you. Best wishes, Maz

[taytokavo in reply to Mazcd]

Thank you Maz I am so happy to have found this website and support

[asacker]

Hi TaytokavoI think you have a similar background to me. I also had a portal vein thrombosis, discovered in my 20s while I was pregnant with my first child. I was prescribed hydroxycarbamide for many years which help with the itching and brought down my counts. I've never had a rash associated with that though, although I am more prone to heat rash. Do check that the rash isn't some other unconnected problem as mentioned by Ebot.

I was initially diagnosed with PV which was then changed to ET. After many years (I'm now in my late 60s), it has progressed to MF, and I am now on Ruxilitinib. Yes, it did stop the itching that started again with the progression and I have been feeling very well on it. But Ruxilitinib efficacy can wear off after several years, which I think I'm starting to experience now.

So my advice would be not to discard other options first. I have been on hydroxycarbamide for over 40 years and I've tolerated it extremely well. Other people are sometimes wary of it but my experience has been only positive. Interferon didn't work for me - we are all different. But if your platelets are as low as you say then you may not need any medication right now.

You will find many old-timers like me on the forum who have been living with MPNs for almost all their life, at least 50 years. I hope that you are reassured by that. I know that I was most concerned for my children when I was initially diagnosed, but I'm now a grandmother.

So good luck with your appointment.

Amanda

[taytokavo in reply to asacker]

Hi asacker, reading your post has certainly raised my hopes so thank you for that. It is reassuring to hear from someone who has a similar condition as myself, as I have lived with this illness for so long and never had met anyone who I could talk to. I only found out last week from a letter my haematologist sent to my GP that I had MPN so I actually don't even know which one I have. I am meeting a professor haematologist on Monday so hopefully I will know more then and talk about other medications. I even wonder is it important to go on medication for this rash which is more like a purple rash under my skin? I hate taking medication. I have so many questions to ask. Where I live in the UK (Northern Ireland) there are no MPN specialists. I do sometimes feel my haematologist is very blase about me and my condition. Amanda thank you again for your reply as like you I am so concerned for my children and being here for them, so you really have given me hope.

[mhos61 in reply to taytokavo]

Hi taytokavo.

I hope your consultation goes well on Monday. Use the weekend to write down any questions you have. Don’t be frightened to ask for further explanation about anything you don’t understand. The main thing at this point is to find out do they have a conclusive diagnosis, and then take it from there.

Ask which ‘risk group’ you are in - low, intermediate or high. This will determine whether or not medication is indicated. It is quite possible that your previous portal vein thrombosis and Jak2 status may place you in the ‘high risk’ group, suggesting treatment. If that is the case, your haematologist will discuss this with you, but it is always your choice.

There’s a lot of information to take in, I had to do it in bite size chunks when I was first diagnosed with ET.

You can always ask further questions here too.

Good luck

[taytokavo in reply to mhos61]

Hi mhos61,

Thank you for your message. I have started to write down all the questions I need to ask on Monday and this site has certainly given me insight on what to ask, and also what medications that might be available to me. When I couldn't tolerate the interferon the haematologist didn't give me any other options just that. I am so grateful for all the responses to my post and yes Amanda's post certainly did give me hope. Thanks again have a lovely weekend.

[mhos61 in reply to asacker]

Hi Amanda.

Wow! I would just like to say a huge thanks for your reassuring and uplifting post.

You really cannot underestimate the hope your story will bring to so many of us on the forum. Just reading taytokavos response to your post it’s clearly evident how reassured she is. I think it’s wonderful for our young members to read such positive posts.

Also, many years on hydrea without any adverse effects is encouraging to hear. I understand it’s not for everyone, but it certainly has its place, and for the majority of people on hydrea, they do tolerate it well, including myself.

I really hope you can be offered something equally as good as the Ruxolitinib initially offered you in your ongoing MF journey.

Thanks again 👍