Advice please - Raynauds and ET

[Janlol]

Hi everyone, I am really struggling at the moment, diagnosed with ET JAK + in 2016, currently on clopidogrel and Hydroxy, splenectomy and small bowel resection due to splenic infarct and inflammatory mass in bowel. Was admitted to hospital in November due to having a white toe which my GP thought may be caused by a micro blood clot, long story short got passed to the vascular team, ct scans etc carried out and they think no blood clot but not 100% sure, they are thinking it is now Raynaud’s. The issue with the toe has now resolved but I have now got the same problem on 2 toes on the other foot, one purple and one white and very painful. Have spoken to my haematologist who is saying not related to ET and let vascular team deal with it, vascular team are saying haematologist should be involved and I am currently stuck I the middle feeling very fed up with everything. Has anyone else suffered with Raynaud just in their toes (hands etc fine) and is it related with ET?

Thank you

[hunter5582]

This does sound like a microvascular issue related to the ET. You can seacrch the literature and find articles like this specific to Reynaud's. raynaudsnews.com/2017/01/18...

People with ET can als develop erythromelalgia or similar microvascular issues. this is just one article on that. pubmed.ncbi.nlm.nih.gov/888....

So the short version is that this is very likely related to the ET. Some hematologists are not aware of the complications that people with MPNs experience. Suggest consultation with a MPN Specialist on this topic. here is a list of experts you can consult. mpnforum.com/list-hem./

[Janlol in reply to hunter5582]

Thank you Hunter, very useful information. The more I have read the more it sounds like I have erythromelagia and Raynaud’s. I have had a few instances where the soles of my feet have been so painful i haven’t been able to put them on the floor, I just thought I had overdone it in the gym or something but it is getting more frequent. I have upcoming appointments with both the vascular team and haematologist so I think I am going to be a bit more firm and ask for a diagnosis!

[hunter5582 in reply to Janlol]

The problem you may run into is a lack of knowledge about how MPNs can manifest. That is why consulting with a MPN Specialist is so important. Here is another source regarding MPNs and erythromelalgia.

"Primary and secondary forms of erythromelalgia exist. Secondary erythromelalgia has been linked to a wide variety of diseases, the most common of which are certain myeloproliferative disorders: polycythemia vera and essential thrombocythemia"

pubmed.ncbi.nlm.nih.gov/264....

Being firm may well include insisting on consulting with a doctor with the expertise you need. Hope you get answers soon.

[Janlol in reply to hunter5582]

Hi, apologies for the late reply however it has taken this long for me to get an appointment with a rheumatologist! I took matters into my own hands and researched and found a consultant specialist who deals with erythromelalgia. My haematologist (who is an MPN specialist) agreed to refer me to her even though she said unlikely to be connected to MPN. Saw the consultant last week who confirmed most likely secondary erythromelalgia due to ET, provided lots of information and risks etc and said will be emailing my haematologist to discuss medication to try and manage symptoms which are now worse. I just wanted to thank you for your initial advice and say to others be firm and follow up on your instincts when something doesn't feel right!

[hunter5582 in reply to Janlol]

Good news! Glad you followed your instincts. When I was having this issue both of my hematologist immediately said "common PV symptom." I was off aspirin at the time. It stopped immediately when I went back on aspirin.

Wishing you continued success.

[Janlol in reply to hunter5582]

Thank you Hunter, appreciate it. One of the first questions the rheumatologist asked was whether I was taking aspirin which I'm not, Also asked me what is my haematologists plan to reduce my platelets further which I couldn't answer.( Currently 500 / 600 ) . It was the fact that she understood MPN and the effects of this and took ownership to speak with my haematologist, goes a long way when I feel most medical professionals just want you out of the door!

[hunter5582 in reply to Janlol]

Assertive patients receive higher quality care. Passive patients do not. Glad to hear that you are succeeding in advocating for yourself.

Suggest that you may want to have a conversation with your care team about the underlying mechanism for the symptoms we experience with MPNs. With MPNs like ET, there is more to what is going on than the number of platelets. It is also about how the blood cells behave. Reducing the number of platelets would not necessarily reduce the erythromelalgia. This is a microvascular symptom. Aspirin helps the blood cells to be "less sticky" - reducing risk of thrombosis and microvascular symptoms. At 500/600, your hematologist may not feel that the intrinsic risks of additional hydroxy/cytoreduction are worth the benefits in your case. It is worth noting the hydroxy tend to reduce microvascular symptoms.

Since you are also on clopidogrel, dose titration if adding something like aspirin would be really critical. It sounds like you have a pretty detailed conversation to have with your MPN Specialist. Please do et us know how you get on and what you learn.

[Janlol in reply to hunter5582]

I certainly will. My haematologist thinks I'm a bit of a difficult case as I had a splenic infarct when my platelets were in the 400's hence why I am low dose hydroxy with the aim of reducing platelets, this is how i was originally diagnosed with ET Jak2 positive. I have felt like a bit of a hypochondriac and hate mentioning that I have yet another symptom but for some reason this seems to be happening more and more

Thank you again for sharing your knowledge and taking the time to reply.

[hunter5582 in reply to Janlol]

I can certainly sympathize with the plethora of symptoms. In the last four years I needed four surgeries, including heart and brain surgeries. I have a host of inflammatory issues, including GERD, esophagitis, eczema, osteoarthritis, insomnia... I have become quite aware of how wide ranging the impact of MPNs can be. Systemic inflammation is real and problematic for people with MPNs. Add thrombosis, hemorrhage, and microvascular issues and we can have quite a few symptoms to report.

You are most certainly not a hypochondriac. Your care team can only provide adequate care if you report everything you are experiencing. This is just part of being your own best advocate.

All the best.

[Wyebird]

Oh no! Isn’t it awful when you get bounced from doctor to doctor . Hope you get advise soon

[Janlol in reply to Wyebird]

It certainly is frustrating, fingers crossed I will get the advice I need, thank you for your reply.

[Bluetop]

Sorry to hear of your problems, but yes, I have had something similar. I have been experiencing numb fingers very frequently and recently had to visit a 'Minor Injuries' unit with a very red, swollen and extremely sore toe. I suggested at the time that it could be related to my PV but was told it was a chilblane (even though the weather was warm). I have now just had my blood results and indeed my platelets have, for some reason shot up from 250 9/l to nearly 700. I don't think this is a coincidence. I am tweaking my dose of hydroxy. so will see what happens.

Hunter's post very useful, as ever! (Thanks Hunter)

Good luck with managing your two teams, but hopefully Hunter's posts will help.

[Janlol in reply to Bluetop]

Sorry to hear you have had similar issues, I do think it’s so frustrating as the majority of doctors etc just do not know enough about MPN’s and look for the easiest diagnosis which the majority of the time isn’t correct.

I hope you feel better soon.