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
Here are threads from past discussions on progression of symptoms.