One month on: My story with EM

It's now been a month since I first found this community and I want to share an update on where my treatment currently stands.

I first introduced myself here:

http://www.livingwitherythromelalgia.org/forum/topics/what-type-of-doctor-do-you-see-for-erythromelagia-my-story

The past month has seen a lot of positive change.

First, I ended up switching doctors. The rheumatologist who diagnosed me with "seronegative arthritis" was no longer serving my needs. I set about finding a new rheumatologist and returned to the hematologist who originally treated my anemia in 2013. Since I essentially diagnosed myself, my appointments with them were basically to pitch my own ideas. Both doctors agreed I was experiencing flares of erythromelalgia. 31 days ended up being the magic number for a correct diagnosis, from the day I first noticed my knees were red (January 29) to the day I settled on erythromelalgia as the likely cause (March 1). Not bad, right?

Second, I wanted to investigate my hematocrit as a possible causation. Because I had been treated for anemia, I had a detailed record of my hemoglobin and hematocrit over the past 2 years. Both had moved higher than at any previous point and were now slightly elevated above normal averages. This was a point of contention with my previous rheumatologist and primary care physician. Neither thought my levels were that high. If you just go by averages, that's true. However, I am my own unique individual. I thought they still might be too high for me in this particular case. The hematologist agreed and ordered phlebotomies.

Third, the phlebotomies seem to have been very successful. My symptoms have not entirely resolved, but they have dramatically reduced. I am no longer experiencing flares like I once was. I sleep normally, no longer needing to keep my legs elevated or cool my knees at night. I am able to stay in bed and sleep in normal, undisrupted sleep cycles. I've been able to return to some physical activity, as well. These are all very welcome improvements!

So where do I go from here? I had my second phlebotomy on Thursday and continue to see improvement. The flares still occur in some capacity, but it's like the fuel has been removed. The power and intensity are gone. The erythema (redness) and warmth are but a fraction of what they once were. The dominant feature now is a basic tingling. Nerve pain? If I had to put a number on it, I'd say my symptoms have reduced 80-90%.

I want to cure this entirely. I'll continue to evaluate over the next week and then decide if further intervention is needed. I've attached some BEFORE and AFTER photos in the comments to show what the flares were like before the phlebotomy and what they are like now after.

Thanks for all the support!

Pictures of what my flares looked like before my first phlebotomy.

98-BEFORE_March13.JPG (1.49 MB) 99-BEFORE_March19.JPG (1.16 MB)

Pictures during and after the second phlebotomy. The first picture is during the procedure. The 2nd picture is what a flare looked like the evening after the procedure. The final picture I took just now. My knees feel flared, but they no longer appear flared. It is a prickly, tingling sensation.

95-April2_During_2nd_Phlebotomy.JPG (1.22 MB) 96-AFTER_April_3.JPG (1.76 MB) 97-AFTER_April_6.JPG (1.91 MB)

Dear CarterDK,

Massive thank you for updating the community. Your experience of erythromelalgia highlights how some cases can be better managed , and even dramatically improved. This is such uplifting news!

Just to briefly clarify for our newest members. Carter developed a case of secondary EM to a hemocrit disorder (PVC) . With secondary EM it is often possible to treat the underlying 'cause' and minimise symptoms - even remission. A high platelet count and/or a high hematocrit (larger then 50%), underlies myeloproliferative blood disorders - several can be ascribed to secondary EM . Carter was given phlebotomy - a common treatment for patients with polycythemia vera. 'Phlebotomy is a procedure that removes blood from the body. Regular phlebotomy treats people who have too much iron in their blood, such as with hemochromoatosis, or who are producing too many red blood cells, such as with polycythemia' . Chemotherapy is also sometimes given to lower platelet count. Other usual adjunct therapies are intermittent ice application, daily aspirin (500 mg po each day), elevation and avoiding over-warming the extremities.

Carter's story highlights how being ones own advocate is imperative in terms of securing diagnosis and any treatments that may facilitate better management of EM. However, diagnosis, treatment and 'remission' within 31 days is atypical. Sadly it takes most of us 5-7 years just to get diagnosed - however hard we fight :( Medications are also several years of trial/error before you can deduce what, if anything, works for you. With myeloproliferatives, the symptoms of erthromelalgia often occur up to 2.5 years before the disorder is diagnosed.

A comprehensive listing of causes of secondary erythromelalgia would include: polycythemia vera, essential thrombocythemia, myelofibrosis and chronic myelogenous leukemia. This condition is often related to rheumatoid arthritis, gout, vasculitis, pernicious anemia, SLE , possibly drug induced (bromocriptine, nicardipine, nifedipine, pergolide and verapamil), surgery and injury.

Re: Secondary EM. Dont forget we have many discussion posts on secondary EM and nunmerous research articles. If you have problems encountering anything please ask moderator team (alina, nel and myself).

Carter , I am thrilled that you are well on the road to recovery .Best news I have heard all week. Yay!!!!! Well done for being such a warrior ! :).

Big hug

x

PS:When you have time maybe you would be amenable to delineating your argument so I could put it as a fact sheet in our files. How did you pitch your own ideas? Did you give them research to read ? etc....

Thanks mads! That was a great recap! I knew I could have been more detailed, but was lazy and just put in a link to my introduction, LOL.

I don't want to oversell my improvement. I am a lot better and hopeful my EM can go away entirely. At the same time, I remain limited in certain areas. Cardiovascular exercise remains out of reach. I still need to cool occasionally during the day. I continue to carry a degree of pessimism, as I don't want to get my hopes too high. That said, I am grateful for the progress I've made. From where I was to where I am now is a big difference. I know that's atypical for most EM patients.

Because my health insurance is an HMO, it likely did speed my diagnosis. For those that are unfamiliar (particularly outside the United States), an HMO is both the insurer and the health care provider. I go to specific centers for my care that look much like a hospital. There are drawbacks to this arrangement, for sure, (and i'll get to those in a moment) but it did help streamline the diagnosis process. Most helpful has been my insurer's online interface. You can get test results and send online messages to any of your providers 24/7. They'll often answer the same day. Consequently, though I only had a handful of physical appointments, I was in near daily contact with a team of doctors. I was constantly bouncing ideas off them. I also received very prompt appointments when I did need them. When an MRI was ordered, I got one the very next day. In this respect, they were very efficent. It helped quickly rule out other potential causes.

The downside is I don’t think any of my providers have actually treated an erythromelalgia patient before. I would prefer to see a doctor with experience treating it. I could try to get an out of network referal, but I haven’t gone to that trouble yet. Since I effectively diagnosed myself, I do have some apprehension. What if something is being overlooked? I have a background in exercise science and understand basic physiolpogy, but i’m not a doctor. I’d feel more secure if I was under the supervision of someone with specific experience treating EM.

I’ll update this post soon with the specific approach I took to convince my doctors of the EM diagnosis.

This is a really fascinating thread - I've had my condition for some years (chronic episodic) and have only very recently come across EM as a possible diagnosis, and will be approaching my GP (am UK based) very soon to suggest this as an area to explore - so I'm really eager to hear how you approached the medics/doctors.

Great to hear success story though, well done!

Oh Carter - you shouldnt flatter me lol!. You were terribly detailed - its so marvellous to hear of your improvement. I just wanted to clarify that your EM is secondary, PVC related, and to mediate what that means in terms of expectations. No diagnostic will tell you whether you are 'cured' or even whether you have EM , but should you feel that you would like to go see someone more EM aware please let Alina, Nel or myself know. We do have some names in our database for your state :).

NMO is new concept to us Brits. It sounds pretty nifty. I think , however, YOU did most of the work by knowing your own body, getting informed and pounding home that diagnosis. I cant stress enough that EM'ers MUST act as their own advocates. Carter , know you will anyway but please do keep us informed x

Thanks mads! I may take you up on that database, for sure. I do feel like i'm still making slow progress, so we'll see. Since midnight last night, I've only used cold packs twice for about 15 minutes each. That is a far cry from how it once was, when I was wearing them upwards of 20 hours a day! The tingling sensation is also less today. I even did 10 minutes on the elliptical last night at the gym. That did cause a minor flare, but I was able to quickly bring it under control.

I do want to make one thing clear though. My doctors don't think I have an actual myeloproliferative disorder. It's very unlikely I have Polycythemia vera or Essential thrombocytosis. My platelets are normal, which would rule out Essential thrombocytosis. They also did genetic testing for the JAK2 mutation present in 95% of Polycythemia vera patients and I was negative for that, as well.

That doesn't mean my EM isn't secondary to a blood issue. The logic for that was discussed in prior comments. It can't be underscored enough though -- the phlebotomies were experimental. One CBC in early March showed my hematocrit elevated at 53%. The CBC i had immediately prior to the first phlebotomy was 51%, exactly the upper limit of normal. My hematologist was willing to order the phlebotomies because the rise of my symptoms corresponded to the increase in my hematocrit. Had my hematocrit not been regularly tracked for 2 years, that wouldn't have been entirely clear. I thought it was my best shot, which is why I pursued it. Though some doctors I saw dismissed it, the results seem to vindicate my theory.

That said, I recognize I am a very unique case, likely not applicable to most EM patients.