Progression of EM

Hi Guys,

Several members have asked about the progression of EM symptoms .

Straight off I would first stress EM is an enigma and the little we know suggests symptom progression is extremely variable between persons . In some sufferers, symptom onset may be gradual, with the condition potentially remaining relatively mild and unchanged for years. Some of us may reach what we can describe as a stage of stability in symptomology. However, in others, it may have a sudden acute onset, rapidly spreading, increasing in severity and becoming disabling over a matter of weeks .Reports suggest that, for many sufferers, EM has a chronic course that may gradually increase in severity over time. In some with severe erythromelalgia, involvement may spread (usually bilaterally), such as from the feet up the legs (lower limbs), from the hands up the arms (upper limbs), from the upper to the lower limbs, from the lower to the upper limbs, or to the ears or face. A few of us also develop widespread EM known as sistemic. It affects both externally and internally - even organs .

One piece of longitudinal research by Davis et al 2000 N= 168 follow up 8.7 years reports that ;

  • 14% patients had progression of symptoms from lower to upper and lower extremities, but those who presented only with upper extremity symptoms did not report progression down to the lower limbs.
  • Those patients who reported worsening noted an increase in frequency of attacks.
  • 31% reported general worsening of the symptoms, 27% stayed the same and 31% said their symptoms were reduced; 11% reported complete resolution of the symptoms.

However can EM symptomology be given arbitary percentages so readily? Personally I think not, but it does help put things slightly more into perspective . Like all unknown entities we only focus on the horror stories which perpetuates fear. EM is a wicked , intolerable, miserable syndrome but not all of us get that worst scenario :-) . The general gist from research , articles, blogs and members is that progression does appear more likely in certain sufferers especially primary non familial (sporadic) EM and those with chronicity (frequency/intensity of attacks) . However, we should also mention secondary EM and bear in mind that co existing conditions or underlying disorders can impact on EM progression. For example , often raynauds medication is a calcium channel blocker = vasodilation. EM is excessive vasodilation (put simply) so in some patients even more dilation can exacerbate the co existing EM condition - 'progression of symptoms'. . Primary genetic can also worsen with age (seen as junvenile but can be developed up to 25).

I shall speak of my own onset briefly as full details in profile. My own experience was an acute onset (2 months) ensuing severe disability and diagnosis of chronic sistemic EM which has left me 90% bedridden and little quality of life. Both Alina and I have this type of chronic widespread EM ( external/internal) , which can also affect organs. My EM has certainly progressed throughout the body and intensified in both frequency and voraciousness of attacks. It likes certain places the best but often tricks me and strikes me somewhere new , or hits me doubly hard . However, other co existing conditions have developed - raynauds, fibromyalgia, IBD , autonomic dysfunction, chronic fatigue so its very hard to identify what is true nitty gritty EM progression and what symptoms are aggravated by everything else going on .

EM progression is also highly shewed by ones management tactics. Treatments to try better manage symptoms (polypharmacy approach), minimisation skills (heat, stress, fatigue, exertion reduction - sometime cold also induces EM flare), cooling tips (cold immersion, icing, fans, barefeet, loose cotton clothes) and lifestyle changes (diet, supplements-e.g. omegas, Vit D , magnesium, keeping at your temperature - some prefer cold as can be or a regular warm environment- good idea to find your optimal temperature), all masque progression.

Progression is defined by what criteria? Intensity? Frequency? Widespread/localised? Is it actually spreading to other limbs/parts over time? Side effects such as pernio. ulcerations, lymphadema. Multiplicity of symptoms - being burnt, pricking, itching, swelling, lobster red hot flares, aches, cramps etc. Developing co existing/secondary conditions also bring added complications and confounders into the mix. Is progression the measure of how EM impacts and affects your life. Even the mildest localised erythromelalgia can greatly affect normal functioning and quality of life. I always suggest you get to know YOUR erythromelalgia as it pertains to YOU. Marking its progression by keeping a pain dairy, photos and a log of symptoms. Create your own pain scale and keep a record. EM is debilitating - remember to log those exhausted days when EM seems to attack at its worst. Your psychological and emotional state is just as important as the pathophysiology.

In short , progression of EM is very individualised . It is not a one size fits all.

The community would love to hear your thoughts on EM progression.

How did your EM manifest?

Has your EM progressed?

Some extra reading fodder for everyone. I have a couple of articles on progression which will be uploaded when I can get to my PC - sorry . So pop back :)

NORD: Rare diseases

http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/245/printFullReport

Here are threads from past discussions on progression of symptoms.

http://www.livingwitherythromelalgia.org/forum/topics/link-to-virus-or-stress-and-worsening-of-symptoms

http://www.livingwitherythromelalgia.org/forum/topics/has-anyone-ever-gotten-better-from-em

http://www.livingwitherythromelalgia.org/forum/topics/does-em-get-worse-with-age

http://www.livingwitherythromelalgia.org/forum/topics/em-spreading

http://www.livingwitherythromelalgia.org/forum/topics/worsening-symptoms

http://www.livingwitherythromelalgia.org/forum/topics/how-do-em-symptoms-progress

http://www.livingwitherythromelalgia.org/forum/topics/progression-of-symptoms?id=6418999%3ATopic%3A18653&page=6#comments

http://www.livingwitherythromelalgia.org/forum/topics/em-spreading

http://www.livingwitherythromelalgia.org/forum/topics/erythromelagia-in-face-and-ears

http://www.livingwitherythromelalgia.org/forum/topics/inactivity-makes-it-worse?commentId=6418999%3AComment%3A44848

http://www.livingwitherythromelalgia.org/forum/topics/mayo-clinic?commentId=6418999%3AComment%3A45861

http://www.livingwitherythromelalgia.org/forum/topics/chronic-fatigue-syndrome-do-you-recognise-the-symptoms

Mads I was very interested to read this discussion post of yours because it is a question I've been asking myself all day. When I first saw my neurologist at the end of October he diagnosed an advanced and progressive small fiber neuropathy. The tests I had last week seem to have made him less confident about this initial diagnosis because my EMG and other tests showed everything as normal as did my MRI of brain. The lumbar puncture and blood tests were looking for an immune mediated neuropathy and results are still unknown to me but he seems to think it less likely that my condition is a demylienating one now.

However he acknowledged that EM was a possibility and also that SFN doesn't always show up in nerve conduction studies.

I feel my symptoms are certainly progressing but I'm hopeful that if I can get back onto a disease modifying drug for my autoimmunity then the EM symptoms might also be addressed, or at least minimised. That's what I'm desperately hoping anyhow. I have found some of the tips for ways to reduce EM symptoms on here very useful indeed although I'm still struggling as soon as I stop taking anti-inflammatory drugs. I live in a pretty windy, cold place so I don't really have the issues about over heated environments - although the burning does increase once I've overheated (a bit like a storage heater on overdrive!) - but also if I get too cold.

Meanwhile I was going to ask whether this burning is actually doing physical harm to our bodies in itself as well as mental harm to us through excessive pain and possible harm through the drugs we take to try and reduce the EM pain? I guess this is a very complex question since EM can be primary or secondary and the prognosis will vary accordingly?

NotRed

NotRed.

I share your concern about what physical damage is being done by flares over the long haul. It's been my experience that flares do cause damage if they are severe. I have found that after a really bad flare I will get spontaneous blisters on the top of my toes the next day. And these large blisters are very difficult to heal. Plus, that skin irritation then causes more skin breakdown and additional flaring which just intensifies the problem until it becomes a cycle that is hard to break.

This is especially true now since I added Cymbalta to Lyrica, because the pain from the flares is much less. So, it's much easier to ignore flares than it used to be and overdo things. I think it's really important to stop flares as soon as possible rather than try to tough them out. I suspect that this may be one reason that EM may worsen over time because the elasticity in blood vessels, tissues and skin simply wears out. When I feel a flare coming on now, I try to elevate and use a cooler fan or submerge my feet into TEPID water (in plastic bags!!) and very gradually cool them down until the water is cool but not cold.

I certainly have found that my EM has gotten much worse even though I feel the pain less because of meds. I believe that I am functioning about the same as I did about 18 months ago without meds.

I haven't seen any further study about the course of EM--hopefully someone will know of any research under way.

Cheers,

Dragica

Hi ALL -

Yes this EM is a Mixture of various conditions, diseases, afflictions and whatever else the doctors want to tag onto it because the truth is that unless you were born with it (Primary) anything that Triggers it in the Secondary range there is NO One answer that covers it.... and NO one specific Treatment for it either, most doctors have never heard of it and just DO NOT Understand the disabling effects it has on a person...

All one can do is try to educate the medical staff as to its nastiness and try the range of medications that are available to treat the symptoms and hopefully find something that will reduce the pain, the flare-ups, the swelling and the redness associated with it .... Trial & Error is the only way any of us can hopefully get some relief for the 'EM' ...

There are so many things that cross over with EM symptoms that it can get very confusing and frustrating to find the right path to take regarding medication, but as soon as you do find something - anything, that relieves the pain then stick with it and then try and home in on further reducing the pain - there is NO CURE for 'EM' - just different methods of pain relief - Good Luck to everyone searching for the elusive relief ... It is not easy to find.

Regards to All 'EM' Sufferers -

Greg - frostbite

COOMA NSW

Thank you for this thread!