Brilliant Jane - finally got round to reading this. Thank you for your kindness in sharing your knowledge
blessings
ajh
Jane said:
Ooooh! Sorry to bombard you all, but I just found a potentially useful site - an article called "Recent developments regarding voltage-gated Sodium Channel Blockers for the treatment of Inherited AND Acquired Neuropathic pain" - that's all of us (I haven't read the article yet, its's a long one - but I'm fixing to). It's at:
We NEVER recommend treatments on livingwitherthyromelalgia or give diagnostics. I am surprised that during your 2 week battery of tests you were not given the 30 minute 'lidocaine challenge', which is usual procedure in the UK.. Most specialists tend to 'test' their patient before administering any SCB's. Apart from identifying whether you are a candidate for SCB (ie: you respond positively), the test will prevent the exact thing you complain of ie:unpleasant side effects/wasting time. In these instants ,when you respond negatively, the test is stopped. How very dreadful for you that this procedure was not followed, and you suffered so .I find it quite unethical that you were given lidocaine continuously for 7 days since you were responding so adversely.Moreover, having responded negatively to SCB on previous occasions - seems a no brainer! .
Sadly, as most of us know , continuous severe flaring is intolerable and quite impossible to control - you poor thing! .Only the triggers are managable to varying degrees. As lidocaine/SCB have a half life of 8 hours I hope you are now fully recovered from your terrible ordeal.
SCB treatment is having extremely favourable results BUT in the right candidates when it comes to managing refractory pain.However it should be 'tested' with the challenge and tritated up very slowly over several months until dosage right . Your experience just reiterates EM is not a 'one size fits all' syndrome when it comes to symptoms/treatment. Its really just a continuous case of trial and error for all of us. Stay strong.We are all here to support you.
I'm so sorry you have had this horrible experience. I admire your courage in trying new meds and am grateful that you feed back to us all - this is dodgy territory and the contributions from you have helped me enormously. I don't know your consultant, but just because this happened doesn't mean he cannot still be of use to you. I believe your persistence will pay off in the long run, and what is good is that you have the understanding of the underlying channelopathies that will enable you to put this into a complete picture of your case and make sense of it with your consultant and that will guide you both as to where to go next.
with love
ajh
elizabeth said:
Just got out of hospital after 2 weeks of tests and treatment, including 7 days of continuous lidocaine infusion. It was a disaster. It made things much worse, I ended up with a constant flare of symptoms which couldn't even be controlled with icepacks, which is exactly what happened with other sodium channel blocking drugs I've tried. It also caused very unpleasant cardiological side effects, I had very bad tachycardia and severe muscle tremor the entire time I was on it. I'd be very wary of recommending it to anyone else.
If you read my reply carefully and calmly , I did NOT say that your Doctor or protocols were unethical. As a chronic EM sufferer I am entitled to give my opinion. More to the point as a Dr myself,
"I find it quite unethical that you were given lidocaine continuously for 7 days since you were responding so adversely.",
You say that the experience was a disaster. You say that you had a negative response. You then state that a short infusion would have helped - agreeing with the short test hypothesis.
"To be honest, if I'd had a short infusion I'd probably have thought it helpful, for the first couple of days I did feel better"
Still dont quite understand why you were administered lidocaine when you say you had tried SCB without success.
If I remember correctly you havent actually got EM . Anyway, you are certainly very privileged to be at NHNN and UCLH.
Thank you all for your experiences both good and bad with this method of treatment.
I am working with my doctors to get the lidocaine infusions done here in the US. My Doctor believes it is worth a try and is just getting it approved with insurance.
With any luck it should be soon. I will be sure to let everyone know how it goes!
Take care,
Alina
Thats great news Alina.Fingers crossed SCB proves to be positive for you. SCB need to be tritated up very slowly over the course of several months. Imperative that the right dose attained. Too much can cause increased pain sensitivity ie: flaring.
Hi everyone. Just to clear some of the air up about the studies. There is a specific reason they are looking for inherited EM patients. There are 3 types that need to be studied separately because they all act differently.
Secondary: Studies suggest that EM attacks or episodes typically occur at the same time or immediately following symptoms of the underlying disease. This theoretically means if you can stabilize the underlying disease you could also stabilize the EM with it.
Primary Inherited: Being the same genetic mutation is passed down through genetics the study is probably to see if same strands react the same. If they do it would mean that they could predict EM progression and patterns more easily. This is solely for inherited cases since a stranger with different genetic code would react differently to the same strand of EM.
Primary New Strand: This is the rarest and most difficult to diagnose since there is not history of medical issues and the person appears healthy on all tests. (I have this form.) It is the most unpredictable since there is not underlying disease to trigger it and no pattern to look for. Patients with this form never know what to expect since it can escalate at any given time without triggers.
Just thought I should mention it. They need to conduct three different studying since all three types react differently. But everyone is right they should not be focusing on just one type since the pain and symptoms are the same for all three.
Thank you Melly , but may I ask - what do you mean with *react* different? What kind of reaction are you talking about? and reaction to what ? Medication? We all know that we react different to medications or other stimulus but I don't think that has anything to do with what *type* of EM we have. I can see and understand the difference between genetically caused EM and not genetically caused but were is the proof that another health problem may cause EM? I have never heard of anyone being cured or stabilized of an *underlying* disease and then being cured of EM.
I was told by drs here in UK that if your EM is secondary then treating the primary condition can in fact ease or get rid of the secondary condition (ie EM). He did say however that it all depends what it’s secondary to and often they never find out the primary condition let alone cure it!
thanks laura - that is what some doctors are saying - the dermatologist who diagnosed me said the same thing - treat your pericarditis and your EM will go away. My cardiologist says - get rid of EM and your pericarditis will go away. I just don't quite believe that they know what they are talking about because they have not come up with any PROOF about EM being secondary to another disease. What if EM causes other diseases? But whatever - I wanted to know what Melly meant about *different reactions*. Take care
Hi Laura, I'll just clarify a little bit since I wrote a lot last time.
Secondary EM reactions occur after symptoms to the underlying disease. This means they are predictable to some degree and can potentially be cured/managed with the underlying disease.
Primary EM reactions may be predictable if inherited to due patterns that may appear throughout generations. If not inherited than reactions are completely unpredictable.
Reactions in EM are different in all three because it involves a function called action potential. Action potential involves sodium and potassium channels. EM is a mutation/abnormality in the sodium channels which causes a lot of the symptoms. Sodium channels react abnormally according to the type of EM you have. All EM patients get the same pain and many of the same symptoms with slight variations but the sodium reaction abnormality reacts differently causing episodes at different times. Secondary is right after the underlying disease symptoms, inherited potentially occurs in a pattern through generations, and new strands are spontaneous.
Hi, it was Domina that was asking for it to be clarified, I have fairly good understanding of secondary EM even if my MS brain doesn’t let me explain myself properly…! I am sure there are lots of people who will find your information helpful, thanks. You seem very educated about it, something that we all need to do for ourselves if we don’t have drs who are knowledgable.!
Melly said:
Hi Laura, I’ll just clarify a little bit since I wrote a lot last time.
Secondary EM reactions occur after symptoms to the underlying disease. This means they are predictable to some degree and can potentially be cured/managed with the underlying disease.
Primary EM reactions may be predictable if inherited to due patterns that may appear throughout generations. If not inherited than reactions are completely unpredictable.
Reactions in EM are different in all three because it involves a function called action potential. Action potential involves sodium and potassium channels. EM is a mutation/abnormality in the sodium channels which causes a lot of the symptoms. Sodium channels react abnormally according to the type of EM you have. All EM patients get the same pain and many of the same symptoms with slight variations but the sodium reaction abnormality reacts differently causing episodes at different times. Secondary is right after the underlying disease symptoms, inherited potentially occurs in a pattern through generations, and new strands are spontaneous.
I was actually misdiagnosed as having Raynaud's by one ER and I was given Nifedipine; all heck broke loose and I was unable to sleep for 3 days! They're trying me on Inderal; have you tried that? I ask as I am curious..
Thanks in advance and I hope you treatments bring you relief!
Erythromelalgia is so rare that Pfizer has located just a handful of families in the U.S. to participate in the trial, says Ruth McKernan, chief scientific officer at a Pfizer unit in Cambridge, U.K., that focuses on pain.
Really! They have got to be kidding... Maybe Pfizer needs to look at the amount of people on here that are from the U.S. that have EM.... What a joke!
The sad thing is, my 20 year old son is showing signs of this as well; when he takes a shower and comes out, his ear turns beet red, feels hot to the touch, and he says it's uncomfortable.. He gets something with his feet as well.. This disturbs me as he also has Ehlers-Danlos Syndrome Hypermobility form as well as POTS.. Scares me to pieces to think my son could have this as well.. :(
Sorry you’re having to deal with your pain as well as the worry that your son is showing signs.! I worry everytime my daughter comes home with hot red feet even though I’ve had genetic testing and I don’t have ‘the’ gene so my EM is secondary.!